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2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Question: 1

You are counseling the mother of a 3-month-old breastfed infant whose family has been urging her to introduce cereals to her baby’s diet. She asks your advice.

Of the following, the MOST likely outcome of introducing solid foods at this age is to

A. accelerate the development of oral-motor skills

B. help the infant sleep through the night

C. increase the risk of food allergies

D. increase the risk of gastroesophageal reflux

E. increase the risk of gastrointestinal infections

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Critique: 1

Preferred Response: E

The most likely consequence of early (before 6 months of age) feeding of complementary foods such as cereals to breastfed infants is an increased likelihood of gastrointestinal infection. The direct relationship between early complementary feedings and the incidence of diarrheal illness is based on several case-control studies. In one investigation from Belarus, a large group of infants who were exclusively breastfed for more than 6 months was compared with a group receiving a mixed diet of human milk plus solids, with solids introduced between 3 and 6 months of age. Exclusively breastfed infants had a significantly reduced risk of one or more gastrointestinal illnesses. Furthermore, other observations suggest that this effect may be enhanced with greater duration and exclusivity of breastfeeding. However, prior studies have failed to show any clear risk reduction in the prevalence of upper and lower respiratory tract illnesses, asthma, and otitis media among exclusively breastfed infants compared with infants who received a mixed diet of human milk and solids. No available evidence supports the hypothesis that the introduction of solid foods either accelerates the development of oral-motor skills or helps infants to sleep through the night. Data concerning the effect of early introduction of solids on the development of allergies are conflicting. The Belarus study found no reduction in risk for atopic eczema in exclusively breastfed infants; a Finnish investigation showed a reduced eczema risk at 1 year but not at 5 years of age in a similar group. Although the Finnish study demonstrated a small reduction in any atopic condition for exclusively breastfed infants, the results were not statistically significant. Evidence also failed to demonstrate that early solid food introduction was associated with an increased incidence of positive skin prick tests. Results of obesity studies also are inconclusive. In exclusively breastfed infants, solid food introduction prior to 6 months of age generally is associated with reduced human milk intake without accelerated weight gain. However, formula-fed infants may be encouraged to consume the same amount of formula, even after complementary feedings are introduced. This practice may lead to increased calorie consumption and excessive weight gain. Gastroesophageal reflux (GER) is the result of transient relaxations of the lower esophageal sphincter. Studies using intraesophageal pH probe monitoring data have shown that the reflux index (RI) (percent time that esophageal pH is less than 4) is significantly greater in infants (RI mean upper limit of normal: ~12) than in older individuals (mean: ~6). The addition of solids to the diet does not influence the time to resolution of clinical GER during infancy, although the frequency and severity of symptomatic reflux episodes may be reduced, at least in part, by thickening feedings or increasing solid consumption in appropriately aged infants. The appropriate timing for introducing solid foods to the infant diet depends on development of both neuromuscular function and gastrointestinal maturation. The American Academy of Pediatrics supports exclusive breastfeeding for the first 6 postnatal months. However, from a developmental perspective, term infants often are capable of accepting solids (complementary foods) between 4 and 6 months of age. Maturational readiness to tolerate complementary feedings is indicated by loss of the extrusion reflex (usually by 4 months) and by the ability to swallow non-liquid foods. The most obvious risk posed by solid food consumption prior to reaching these developmental milestones is that failure to achieve oropharyngeal coordination for consuming solids may lead to aspiration.

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

References:

Braganza SF, Adam HF. In brief: gastroesophageal reflux. Pediatr Rev. 2005;26:304-305. Available at: http://pedsinreview.aappublications.org/cgi/content/full/26/8/304

Fein SB, Labiner-Wolfe J, Scanlon KS, Grummer-Strawn LM. Selected complementary feeding practices and their association with maternal education. Pediatrics. 2008;122:S91-S97. Available at:

http://pediatrics.aappublications.org/cgi/reprint/122/Supplement_2/S91?maxtoshow=&HITS=10&h

its=10&RESULTFORMAT=&fulltext=Selected+complementary+feeding+practices+and+their+asso

ciation+with+maternal+education&searchid=1&FIRSTINDEX=0&sortspec=relevance&resourcetyp

e=HWCIT

Kleinman RE. Complementary feeding. In: Pediatric Nutrition Handbook. 5th ed. Elk Grove Village, Ill: American Academy of Pediatrics; 2004:103-115

Kramer MS, Chalmers B, Hodnett ED, et al. Promotion of Breastfeeding Intervention Trial (PROBIT): a randomized trial in the Republic of Belarus. JAMA. 2001;285:413-420. Available at:

http://jama.ama-assn.org/cgi/content/full/285/4/413

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Question: 2

You are called to labor and delivery to attend the vaginal delivery of a 37 weeks’ gestation male to a 24-year-old primiparous mother. She reports that her membranes ruptured 36 hours ago. She is afebrile.

Of the following, the maternal condition that is MOST likely to require antibiotic therapy for this neonate is

A. chorioamnionitis

B. diabetes mellitus

C. group B streptococcal colonization

D. preeclampsia

E. urinary tract infection in the first trimester

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Critique: 2

Preferred Response: A

The mother described in the vignette has premature rupture of the membranes, which occurred 36 hours prior to her delivery. Premature rupture of the membranes is defined as rupturing of the amniotic sac not followed by the onset of labor within 8 hours. Preterm rupture of membranes is defined as rupturing of the amniotic sac before 37 completed weeks of gestation. Prolonged rupture of membranes (PROM) before delivery of the fetus increases the risk for early-onset neonatal sepsis (EONS); PROM of 18 hours or more is considered significant. Both term and preterm infants are at risk for EONS associated with PROM. However, the preterm infant delivered after PROM has a greater risk for infection than the term infant due to immature innate immune function (eg, immature phagocytic and neutrophil function) and potential lack of passively acquired transplacental immunity (prior to 32 weeks’ gestation). Two maternal conditions can increase the risk for EONS in the face of PROM: chorioamnionitis and maternal group B streptococcal (GBS) colonization. The former confers a risk for fetal infection that may not be treated adequately with maternal antibiotics. Hence, the newborn may continue to have partially treated bacteremia, pneumonia, or meningitis and require treatment for presumed sepsis. The latter is of import because infants born before 36 weeks’ completed gestation are more susceptible to GBS infection, especially in the face of PROM or chorioamnionitis. A sepsis evaluation and empiric treatment with a penicillin and an aminoglycoside is recommended for the preterm infant following PROM. Maternal GBS colonization status is of most importance in deciding whether to evaluate and treat a preterm but not term infant unless the term infant has symptoms of infection. Maternal diabetes does not affect the treatment of an infant with antibiotics. Preeclampsia does not confer risk to the fetus or newborn for acquiring an infection in the face of PROM, but it may be associated with leukopenia, especially in the very low-birthweight newborn, and may have a negative impact on the newborn’s ability to clear infection. A first-trimester maternal urinary tract infection is of little import because it is remote from delivery. However, in a GBS-positive pregnant woman who experiences a urinary tract infection that is due to GBS, the potential inoculum is greater in the neonate, which affects the evaluation and presumptive treatment of the newborn.

References:

American Academy of Pediatrics. Group B streptococcal infections. In: Pickering LK, Baker CJ, Kimberlin DW, Long SS, eds. Red Book: 2009 Report of the Committee on Infectious Diseases. 28th ed. Elk Grove Village, Ill: American Academy of Pediatrics; 2009:628-634

Centers for Disease Control and Prevention. Guidelines for GBS: Recommendations. 2008. Available at: http://www.cdc.gov/groupbstrep/guidelines/recommendations.htm

Puopolo KM, Madoff LC, Eichenwald EC. Early-onset group B streptococcal disease in the era of maternal screening. Pediatrics. 2005;115:1240-1246. Available at:

http://pediatrics.aappublications.org/cgi/content/full/115/5/1240

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Schrag S, Gorwitz R, Fultz-Butts K, Schuchat A. Prevention of perinatal group B streptococcal disease. Revised guidelines from CDC. MMWR Recomm Rep. 2002;51(RR-11):1-22. Available at: http://www.cdc.gov/mmwr/preview/mmwrhtml/rr5111a1.htm

Thilo EH, Rosenberg AA. The newborn infant. In: Hay WW Jr, Levin MJ, Sondheimer JM, Deterding RR, eds. CURRENT Diagnosis & Treatment: Pediatrics. 19th ed. New York, NY: The McGraw-Hill Companies, Inc; 2009:Chapter 1. Available for subscription at:

http://www.accessmedicine.com/content.aspx?aID=3396500

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Question: 3

A mother brings her 9-year-old boy to your clinic because he has been complaining of being tired in physical education class at school for the past few months. When you ask him about his symptoms, he reports having trouble catching his breath after he runs. Past medical history is negative, and a review of systems reveals only a cough that occurs primarily at night several times a month. He has grown well, and findings on physical examination are normal.

Of the following, the MOST likely reason for his exercise intolerance is

A. cystic fibrosis

B. exercise-induced asthma

C. iron deficiency anemia

D. vocal cord dysfunction

E. Wolff-Parkinson-White syndrome

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Critique: 3

Preferred Response: B

Exercise intolerance is the failure to tolerate physical exercise at a level that would be expected for a person’s age and condition, such as described for the boy in the vignette. For the child, it is important to determine whether exercise intolerance is due to a primary pulmonary or extrapulmonary cause. Pulmonary causes include asthma, cystic fibrosis, and acute and chronic infections of the lung. Among the extrapulmonary causes of exercise intolerance are cardiac disorders such as congestive heart failure, neuromuscular disorders such as muscular dystrophy, anemia, and deconditioning. Exercise intolerance is measured primarily by the maximal oxygen consumption test, and examining the components of maximal oxygen consumption can be useful in understanding the reasons for exercise intolerance associated with various disease states. The Fick equation for maximal oxygen consumption is:

VO2max = SVmax x HRmax x (CaO2 — CvO2)max

where VO2 = oxygen consumption, SV=stroke volume, HR=heart rate, CaO2=oxygen

content of arterial blood, and CvO2=oxygen content of mixed venous blood. A sedentary lifestyle and certain cardiac diseases such as congestive heart failure and cyanotic heart disease can cause a decrease in stroke volume. Diseases such as asthma, cystic fibrosis, anemia, and vocal cord dysfunction lower the oxygen content of arterial blood. States causing muscle weakness, such as muscular dystrophy or general deconditioning, can result in decreased oxygen use by the tissues. Alterations in any of these components can lead to decreased maximal oxygen consumption and exercise intolerance. The shortness of breath after running and a nighttime cough described for the boy in the vignette make exercise-induced bronchoconstriction (EIB), also called exercise-induced asthma, the most likely diagnosis. Children who have EIB generally experience shortness of breath, chest tightness, and cough approximately 10 to 15 minutes after beginning exercise.

Administration of a short-acting beta2 agonist or inhaled cromolyn sodium prior to exercising can help to prevent the symptoms. For patients who have poorly controlled asthma and experience EIB, the most appropriate management is the use of inhaled corticosteroids and possibly other maintenance medications to control overall asthma symptoms. If a child who has presumed EIB

fails to respond to pretreatment with beta2 agonists or inhaled cromolyn sodium, other diagnoses such as vocal cord dysfunction should be considered. Vocal cord dysfunction is the paradoxic adduction of the vocal cords during inspiration, causing airway obstruction during exercise. Inspiratory wheezing and throat tightness are common symptoms, but cough at night is not. Cystic fibrosis is unlikely in any child who is growing well and has no extrapulmonary symptoms. Iron deficiency anemia can cause exercise- induced dyspnea, but the boy’s history is not suggestive of this condition. Wolff-Parkinson-White syndrome causes re-entrant tachycardia; syncope rather than exercise intolerance is the usual clinical manifestation.

References:

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

McColley SA. Extrapulmonary diseases with pulmonary manifestations. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, Pa: Saunders Elsevier; 2007:1846-1847

O’Byrne PM. Exercise-induced bronchoconstriction. UpToDate Online 16.3. 2008. Available for subscription at:

http://www.utdol.com/online/content/topic.do?topicKey=asthma/13974&selectedTitle=1~43&sour

ce=search_result630

Owens S, Gutin B. Exercise intolerance. Pediatr Rev. 2000;21:6-9. Available at:

http://pedsinreview.aappublications.org/cgi/content/full/21/1/6

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Question: 4

You are seeing a 1-year-old patient in your clinic for a health supervision visit. You explain the recommended screening tests for this visit to the medical student who accompanies you.

Of the following, the MOST appropriate recommended screening test at this visit is

A. blood lead concentration by fingerstick

B. blood lead concentration by venipuncture

C. complete blood count with differential count

D. serum ferritin

E. serum iron

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Critique: 4

Preferred Response: A

The diagnosis of lead poisoning or increased lead absorption depends on the measurement of blood lead concentrations. In the 1990s, both the American Academy of Pediatrics and the Centers for Disease Control and Prevention recommended universal blood lead screening of 1- and 2-year-old children, but because of the substantial decrease in the prevalence of elevated blood lead concentrations, the criteria for screening are changing in many communities. Thus, it may be helpful to contact your local health department to determine if children in your area are at risk for environmental lead exposure. Although blood lead concentration can be measured most accurately from a sample obtained by venipuncture, a capillary specimen obtained by fingerstick is the most appropriate screening test for the toddler described in the vignette. The specimen must be obtained carefully to avoid contamination from lead on the skin. Capillary specimen values greater than 10 mcg/dL (0.5 mcmol/L) must be confirmed by a venous sample because of the possibility of skin contamination

causing a false-positive result. Although obtaining a complete blood count with smear and measuring serum ferritin and serum iron may be useful in the diagnosis and management of children who have anemia, including that associated with environmental lead exposure, these tests are not definitive for determining exposure to environmental lead. Finally, hair evaluation for lead poisoning is neither sensitive nor specific due to the lack of correlation with blood lead values and should not be used.

References:

American Academy of Pediatrics Committee on Environmental Health. Lead exposure in children:

prevention, detection, and management. Pediatrics. 2005;116:1036-1046. Available at:

http://pediatrics.aappublications.org/cgi/content/full/116/4/1036

Binns HJ, Campbell C, Brown MJ for the Advisory Committee on Childhood Lead Poisoning Prevention. Interpreting and managing blood lead levels of less than 10 mcg/dL in children and reducing childhood exposure to lead: recommendations of the Centers for Disease Control and Prevention Advisory Committee on Childhood Lead Poisoning Prevention. Pediatrics. 2007;120:e1285-e1298. Available at:

http://pediatrics.aappublications.org/cgi/content/full/120/5/e1285

Laraque D, Trasande L. Lead poisoning: successes and 21st century challenges. Pediatr Rev. 2005;26:435-443. Available at: http://pedsinreview.aappublications.org/cgi/content/full/26/12/435

Rischitelli G, Nygren P, Bougatsos C, Freeman M, Helfand M. Screening for elevated lead levels in childhood and pregnancy: an updated summary of evidence for the US Preventive Services Task Force. Pediatrics. 2006;118:e1867-e1895. Available at:

http://pediatrics.aappublications.org/cgi/content/full/118/6/e1867

Yeoh B, Woolfenden S, Wheeler D, Alperstein G, Lanphear B. Household interventions for

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

prevention of domestic lead exposure in children. Cochrane Database Syst Rev.

2008;2:CD006047

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Question: 5

During the health supervision visit for a 14-year-old boy, you note that his body mass index (BMI) is at the 95th percentile. At last year’s health supervision visit, his BMI was at the 85th percentile.

Of the following, the complication for which this boy is at the HIGHEST risk is

A. atrial fibrillation

B. hypothyroidism

C. narcolepsy

D. social isolation

E. steatorrhea

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Critique: 5

Preferred Response: D

Childhood obesity is one of the most common chronic conditions of childhood. It is believed to be strongly associated with a number of morbidities, including type 2 diabetes mellitus, hypertension, the metabolic syndrome, and psychosocial conditions. For individual children, the immediate psychosocial effects of social isolation, discrimination, and peer problems may accompany childhood obesity. Obese adolescents have been reported to have lower self- esteem as well as increased rates of sadness, social isolation, loneliness, and nervousness. Several studies have demonstrated the relationship between obesity, as measured by body mass index (BMI) and the psychosocial development of children. Adolescent obesity is a strong predictor of adult obesity, and adult obesity has been associated with depression, especially in women. Studies also have suggested an association between depression in adolescence and higher BMI in adulthood. Whether depression leads to obesity or obesity causes depression is unclear. In contrast, there is a strong association between lower self-esteem and higher BMI across the elementary school years. In many children, the presence of an increased BMI and obesity precedes low self-esteem, suggesting a causal relationship. Accordingly, prevention and management strategies for children who are overweight and obese should be undertaken early to minimize the impact on self-esteem and the other important psychosocial aspects of healthy development. The boy described in the vignette, who meets the diagnostic criteria for obesity (BMI >95th percentile), may be at increased risk for obstructive apnea and resulting effects on the right ventricle, but he is not necessarily at a greater risk for atrial arrhythmias such as atrial fibrillation. Similarly, he may have poor sleeping habits, but obesity does not lead to narcolepsy. Although hypothyroidism can be associated with weight gain, no evidence suggests that obesity leads to hypothyroidism. Finally, obesity is not known to be a cause of steatorrhea.

References:

Goodman E, Whitaker RC. A prospective study of the role of depression in the development and persistence of adolescent obesity. Pediatrics. 2002;110:497-504. Available at:

http://pediatrics.aappublications.org/cgi/content/full/110/3/497

Hesketh K, Wake M, Waters E. Body mass index and parent-reported self-esteem in elementary school children: evidence for a causal relationship. Int J Obes Relat Metab Disord. 2004;28:1233-1237. Available at: http://www.nature.com/ijo/journal/v28/n10/full/0802624a.html

Ludwig DS. Childhood obesity–the shape of things to come. N Engl J Med. 2007;357:2325-2327. Available at: http://content.nejm.org/cgi/content/full/357/23/2325

Stunkard AJ, Wadden TA. Psychological aspects of severe obesity. Am J Clin Nutr. 1992;55(2 suppl):524S-532S. Available at: http://www.ajcn.org/cgi/reprint/55/2/524S

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Question: 6

A mother brings in her otherwise healthy 4-year-old son because of problems walking. He has not wanted to walk since this morning and only stands on tip-toe or crawls. He had an upper respiratory tract illness with a fever 1 week ago. On physical examination, the boy appears well, but he complains of leg pains bilaterally. He reports no back pain. His calf muscles are tender to palpation, but joints are not warm, red, or swollen. Patellar and ankle jerk reflexes are present bilaterally, and there is no clonus.

Of the following, the MOST appropriate initial diagnostic procedure is

A. antinuclear antibody measurement

B. lumbar puncture

C. magnetic resonance imaging of the spine

D. nerve conduction studies/electromyography

E. serum creatine kinase measurement

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Critique: 6

Preferred Response: E

A child who has an acute or subacute gait disturbance represents a medical emergency.

Diagnostic evaluation must be thorough, considering causes at all neuroanatomic levels:

brain/cerebrum, cerebellum, brainstem, spinal cord, root, nerve, junction, and muscle. Prompt identification of hydrocephalus, spinal cord lesions, or Guillain-Barré syndrome can prevent lifelong neurologic damage or save a life. The boy described in the vignette has symptoms only in his legs, with both weakness and leg muscle tenderness. Such findings typically localize to muscle and suggest the diagnosis of acute myositis. An elevated serum creatine kinase value (sometimes to more than 1,000 units/L)

confirms the diagnosis. In children, acute myositis most often occurs after respiratory infections such as influenza. The condition is self-limited, rhabdomyolysis is unlikely, and no treatment is needed.

A spinal cord process that necessitates neuroimaging of the spine is very unlikely for this

boy. Findings suggestive of such a diagnosis include flaccid reflexes (initially), loss of bowel and bladder function, and sensory deficits with a sensory level. A lumbar puncture is used to assess for acute inflammatory demyelinating polyneuropathy, also known as Guillain-Barré syndrome. The absence of back pain and the preservation of reflexes make this diagnosis unlikely. Nerve conduction studies/electromyography often are helpful when neuropathy is suspected in a child who has motor and sensory deficits plus loss of reflexes. The boy has no history or examination finding to support a rheumatologic disease that requires antinuclear antibody measurement.

References:

Compeyrot-Lacassagne S, Feldman BM. Inflammatory myopathies in children. Pediatr Clin North Am. 2005;52:493-520. Abstract available at: http://www.ncbi.nlm.nih.gov/pubmed/15820377

Mackay MT, Kornberg AJ, Shield LK, Dennett X. Benign acute childhood myositis: laboratory and clinical features. Neurology. 1999;53:2127-2131. Abstract available at:

http://www.ncbi.nlm.nih.gov/pubmed/10599793

Millichap JG. Benign acute myositis and influenza viral infection. AAP Grand Rounds. 2000;3:32. Available for subscription at: http://aapgrandrounds.aappublications.org/cgi/content/full/3/3/32

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Question: 7

You are called to the neonatal intensive care unit to evaluate a newly admitted 34-week gestational age male infant who has respiratory distress. When you arrive, the baby is receiving oxygen supplementation by hood. You note that the baby’s weight, length, and head circumference are all below the 10th percentile. He has excess hair over his forehead, shoulders, and back (Item Q7). In addition, he is very irritable, despite correction of his oxygen saturation to 95%.

Of the following, this infant’s unusual findings are MOST likely related to prenatal exposure to

A. alcohol

B. cocaine

C. marijuana

D. methamphetamine

E. tobacco

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Critique: 7

Preferred Response: A

The infant described in the vignette has features consistent with fetal alcohol spectrum disorders (FASDs). FASDs are characterized by a range of recognizable outcomes in infants exposed to alcohol prenatally, the most severe of which is fetal alcohol syndrome (FAS). FAS includes the presence of specific facial anomalies, such as short palpebral fissures, thin vermilion border of the upper lip, and smooth philtrum, as well as evidence of pre- or postnatal growth restriction (height or weight <10th percentile) and findings consistent with abnormal brain growth (head circumference <10th percentile) or brain development (structural brain anomalies). Maternal alcohol exposure need not be confirmed to make a diagnosis of FAS, but other syndromes and conditions that have overlapping features should be ruled out. Other categories of FASD include partial FAS with or without confirmed maternal alcohol exposure, alcohol-related birth defects, and alcohol-related neurodevelopmental disorder. Newborns affected by FAS frequently are irritable and tremulous, and although these symptoms suggest neonatal withdrawal, they can continue for months. Infants also can be unusually hirsute (ethnicity always must be considered when judging hirsutism), and this feature typically dissipates over the first 6 postnatal months (Item C7). Although there is no well-characterized neonatal alcohol withdrawal syndrome, the physician should be alert to signs of drug withdrawal when FASD is suspected due to the frequent concomitant use of alcohol and drugs. Despite numerous publications describing deleterious effects of cocaine on the developing embryo and fetus, the impact of prenatal cocaine exposure remains uncertain. It is generally accepted that cocaine use in pregnancy increases the likelihood of placental abruption, and there is an increased incidence of sudden infant death syndrome in exposed infants. There also may be an increased risk for genitourinary and limb anomalies. There is no generally agreed- upon "cocaine syndrome." Because cocaine often is used in combination with other drugs, cigarettes, and alcohol, it can be difficult to discern what fetal abnormalities are cocaine-related. Marijuana use in pregnancy is not known to be associated with an increased risk for birth defects, dysmorphic features, or developmental delay in exposed offspring. Methamphetamines have not been shown to increase the risk for birth defects in exposed infants, although decreased birthweight has been reported in some exposed infants. A neonatal withdrawal syndrome that includes abnormal sleep patterns, tremulousness, poor feeding, and increased tone frequently is described. Concern has been raised for neurodevelopmental problems in later years, but further investigation is needed. The effects of maternal smoking on pregnancy outcome continue to be an active area of study. Cigarette smoking is associated with an increased risk for miscarriage, reduced fetal weight, and abnormal placentation. There may be an increased risk for facial clefting, but cigarette smoking is not otherwise associated with major congenital anomalies.

References:

Cigarette smoking (tobacco). Teris. Available for subscription at:

http://depts.Washington.edu/terisweb/teris

Cigarette smoking. Reprotox®. Available for subscription at: http://www.reprotox.org

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Cocaine. Reprotox®. Available for subscription at: http://www.reprotox.org

Cocaine. Teris. Available for subscription at: http://depts.Washington.edu/terisweb/teris/

Hoyme HE, May PA, Kalberg WO, et al. A practical clinical approach to diagnosis of fetal alcohol spectrum disorders: clarification of the 1996 Institute of Medicine criteria. Pediatrics. 2005;115:39-47. Available at: http://pediatrics.aappublications.org/cgi/content/full/115/1/39

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Question: 8

A 15-year-old girl presents to the emergency department with right upper quadrant pain for 2 days that is severe enough to keep her out of school. Her appetite is decreased and she has nausea but no vomiting or diarrhea. She has mild discomfort with urination but no vaginal discharge. The only medication she is taking is combined oral contraceptive pills. Her last

menstrual period was heavier that usual. Laboratory tests reveal:

White blood cell count, 7.4x103/mcL (7.4x109/L) with 64% segmented neutrophils and

26% lymphocytes

Total bilirubin, 0.4 mg/dL (6.9 mcmol/L)

Alanine aminotransferase, 14 units/L

Aspartate aminotransferase, 16 units/L

Her urine has 7 white blood cells per high-power field. Abdominal ultrasonography reveals a normal liver, spleen, gallbladder, and kidneys.

Of the following, the MOST likely diagnosis is

A. cholecystitis

B. Fitz-Hugh-Curtis syndrome

C. hepatitis A infection

D. infectious mononucleosis

E. pyelonephritis

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Critique: 8

Preferred Response: B

Both the American Medical Association’s Guidelines for Adolescent Preventive Services and Bright Futures recommend that all adolescents should be asked annually about involvement in sexual behaviors that may result in unintended pregnancy and sexually transmitted infections (STIs), including human immunodeficiency virus infection. In addition to annual screening, the possibility of a pregnancy or an STI should be considered at every visit with an adolescent, and the last menstrual period should be documented. The laboratory results for this girl rule out hepatitis, including that caused by mononucleosis, and biliary tract obstruction. Fitz-Hugh-Curtis syndrome or perihepatitis presents as right upper quadrant pain that results from inflammation of the liver capsule from ascending pelvic infection. Although typically associated with salpingitis, it can exist without other signs of pelvic inflammatory disease and may mimic other abdominal emergencies. The absence of fever and the location of pain for this girl make pyelonephritis unlikely. Pyuria raises the possibility of urethritis, which commonly occurs with Neisseria gonorrhoeae and Chlamydia trachomatis infections. C trachomatis can cause inflammation of the genital tract without the classic symptoms and signs of pelvic inflammatory disease. Often, heavier menstrual flow may be the only symptom.

References:

Burstein GR, Murray PJ. Diagnosis and management of sexually transmitted disease pathogens among adolescents. Pediatr Rev. 2003;24:75-82. Available at:

http://pedsinreview.aappublications.org/cgi/content/full/24/3/75

Elster AB, Kuznets NJ, eds. American Medical Association Guidelines for Adolescent Preventive Services (GAPS): Recommendations Monograph. Chicago, Ill: American Medical Association; 1997. Available at: http://www.ama-assn.org/ama/upload/mm/39/gapsmono.pdf

Hagan JF Jr, Shaw JS, Duncan P. Bright Futures: Guidelines for Health Supervision of Infants, Children and Adolescents. 3rd ed. Elk Grove, Ill: American Academy of Pediatrics; 2008

Hammerschlag MR. Chlamydia trachomatis and Chlamydia pneumoniae infections in children and adolescents. Pediatr Rev. 2004;25:43-51. Available at:

http://pedsinreview.aappublications.org/cgi/content/full/25/2/43

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Question: 9

You are evaluating a 4-year-old boy in the emergency department for septic shock. On physical examination, his heart rate is 140 beats/minute, respiratory rate is 30 breaths/minute, and blood pressure is 65/40 mm Hg.

Of the following, the MOST appropriate next step is administration of

A. 5 mL/kg of 25% albumin

B. 5 mL/kg of 3% normal saline

C. 10 mL/kg of 5% albumin

D. 20 mL/kg of 0.45% normal saline

E. 20 mL/kg of 0.9% normal saline

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Critique: 9

Preferred Response: E

The boy described in the vignette is exhibiting signs and symptoms of septic shock, which is defined as the presence of sepsis and cardiovascular organ dysfunction. Septic shock is a medical emergency that requires prompt recognition and treatment. One of the most important factors in lowering the mortality associated with septic shock is early and aggressive fluid resuscitation, defined as isotonic fluid boluses of 20 mL/kg titrated according to clinical assessment of adequacy of cardiac output, such as heart rate, urine output, and level of consciousness. Often, 60 mL/kg is needed for the initial resuscitation of a child who has septic shock. The choice of fluid (crystalloid versus colloid) for use in shock resuscitation has been the subject of much debate, with little evidence to support the superiority of one over the other. In this scenario, 20 mL/kg of normal saline is preferred over 10 mL/kg of 5% albumin solely due to the larger volume administered. Initial fluid boluses should be 20 mL/kg, and often 60 mL/kg or more is required in the first hour of shock resuscitation. The decision to use 5% albumin or normal saline often is dependent on institutional preferences. Both fluids are isotonic and increase intravascular volume, although a greater volume of saline is needed acutely to achieve the same effect. When endothelial integrity is altered, albumin can leak into the interstitium and increase edema formation. In addition, albumin is considerably more expensive than normal saline, can produce hypocalcemia, and has a small risk of allergic reaction. In a large double-blind study comparing the use of 4% albumin with normal saline for fluid resuscitation of nearly 7,000 adult patients in the intensive care unit, investigators were unable to demonstrate statistical differences in mortality, length of intensive care unit and hospital stay, days of mechanical ventilation, or need for renal replacement therapy. A post-hoc analysis demonstrated an association with increased mortality in those patients who had traumatic brain injury and were treated with 4% albumin. Although research is ongoing on the use of smaller amounts of hypertonic solutions for hemorrhagic shock, there is little research to date on its use in septic shock. Therefore, neither the administration of 3% normal saline nor 25% albumin is indicated. A 0.45% normal saline solution is a hypotonic fluid that would not be used for initial resuscitation.

References:

Brierly J, Carcillo JA, Choong J, et al. Clinical practice parameters for hemodynamic support of pediatric and neonatal septic shock: 2007 update from the American College of Critical Care Medicine. Crit Care Med. 2009;37:666-688

Dellinger RP, Levy MM, Carlet JM, et al. Surviving Sepsis Campaign: international guidelines for management of severe sepsis and septic shock: 2008. Crit Care Med. 2008;36:296-327. Abstract available at: http://www.ncbi.nlm.nih.gov/pubmed/18158437

Finfer S, Bellomo R, Boyce N. French J, Myburgh J, Norton R; SAFE Study Investigators. A comparison of albumin and saline for fluid resuscitation in the intensive care unit. N Engl J Med. 2004;350:2247—2256. Available at: http://content.nejm.org/cgi/content/full/350/22/2247

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Gupta M. Saline or albumin in the ICU. AAP Grand Rounds. 2004;12:16-17

McKiernan MA, Lieberman SA. Circulatory shock in children: an overview. Pediatr Rev. 2005;26:451-460. Available at: http://pedsinreview.aappublications.org/cgi/content/full/26/12/451

SAFE Study Investigators; et al. Saline or albumin for fluid resuscitation in patients with traumatic brain injury. N Engl J Med. 2007;357:874-884. Available at:

http://content.nejm.org/cgi/content/full/357/9/874

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Question: 10

You are examining a 6-year-old girl at her annual health supervision visit. She has Sexual Maturity Rating 3 pubic hair, but no axillary hair. Her mother notes that the child has had an adult body odor for about 6 months. You cannot detect breast tissue, and she does not have clitoromegaly. You examine her growth chart for height (Item Q10).

Of the following, the MOST important test to perform at this time is

A. bone age radiography

B. magnetic resonance imaging of the head

C. pelvic ultrasonography

D. serum estradiol measurement

E. serum testosterone measurement

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Critique: 10

Preferred Response: A

Bone age radiographs can offer useful information about the adult height potential of healthy children who have bone ages of more than 7 years. In addition, a bone age that is advanced, in contrast to chronologic age, can offer some understanding of the length of time or the severity of exposure to sex steroids, particularly estrogen, which is responsible for epiphyseal advancement. Similarly, a bone age that is delayed in relation to chronologic age can offer some inference as to the length of time of endocrine deficiency disorders such as hypothyroidism or chronic nutritional disorders such as celiac disease. However, bone age radiographs do not permit accurate height predictions in children who have bone ages less than 7 years and cannot offer appropriate height predictions for children who have abnormal growth patterns because of bone disorders or other growth disorders. Studies have shown that in children who have isolated premature pubic or axillary hair development (premature pubarche or adrenarche), a bone age radiograph that is more than 1 year advanced compared with chronologic age increases the possibility of a serious underlying disorder such as late-onset congenital adrenal hyperplasia. The girl described in the vignette has premature adrenarche or pubarche, the early appearance of the physical signs of adrenal puberty. Her growth chart reveals a small acceleration in height, which is consistent with this diagnosis (Item C10). She has no evidence of either ovarian puberty (thelarche or breast tissue) or exposure to potent androgens (clitoromegaly). An increase in adrenal production of weak androgen precursors (dehydroepiandrosterone [DHEA] and dehydroepiandrosterone sulfate [DHEA-s]) can be identified in most children between 4 and 6 years of age. However, the earliest signs of adrenal puberty (adult body odor, pubic hair) usually occur at the same time or a few months after thelarche. Rare children may have higher DHEA and DHEA-s values before the age of 7 years or may be particularly sensitive to low circulating concentrations of these hormones. Such children, including the girl in the vignette, come to medical attention because of early adult body odor and pubic and/or axillary hair. The most likely worrisome possibility in the differential diagnosis for children who have premature adrenarche is late-onset congenital adrenal hyperplasia. A bone age radiograph reading within 1 year of the child’s actual age effectively rules out the diagnosis. Magnetic resonance imaging of the head might be indicated in a female child who has early true sexual precocity (activation of the hypothalamic gonadotropic axis with thelarche), but there is no indication for such a study in this child. Pelvic ultrasonography is indicated if there is suspicion about an androgen-producing ovarian tumor, but the slow progression and lack of clitoromegaly makes this very unlikely for this girl. Measures of serum estradiol and testosterone are not useful in this situation. The child’s lack of thelarche or clitoromegaly indicates low estradiol and testosterone concentrations, respectively, and standard commercial assays do not provide accurate results at the low concentrations of estrogen and testosterone found in early puberty. Her growth at a higher percentile is typical of girls who will be slightly early maturers and are having the mid-childhood growth spurt, sometimes attributed to adrenarche.

References:

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Ibánez L, Jiménez R, de Zegher F. Early puberty-menarche after precocious pubarche: relation to prenatal growth. Pediatrics. 2006;117:117-121. Available at:

http://pediatrics.aappublications.org/cgi/content/full/117/1/117

Kaplowitz P. Clinical characteristics of 104 children referred for evaluation of precocious puberty. J Clin Endocrinol Metab. 2004;89:3644-3650. Available at:

http://jcem.endojournals.org/cgi/content/full/89/8/3644

Kaplowitz PB. Precocious puberty. eMedicine Specialties, Pediatrics: General Medicine, Endocrinology. 2007. Available at: http://emedicine.com/ped/TOPIC1882.HTM

Muir A. Precocious puberty. Pediatr Rev. 2006;27:373-381. Available at:

http://pedsinreview.aappublications.org/cgi/content/full/27/10/373

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Question: 11

You are examining a 4-year, 11-month-old child during her health supervision visit. Her mother has been home with her full time since birth. The friendly, happy child tells you about her trip to the zoo last week. She asks if you like animals and counts out loud 10 different animals she saw. When given a crayon, she is able to write her name stating the letters as she writes them. Her mother expresses concern about her beginning kindergarten next month because she just meets the age cut-off for kindergarten entry. She inquires about the factors that will help determine if her child is ready to attend school.

Of the following, the MOST appropriate response is that the girl should

A. be able to maintain attention during story time for at least 20 to 25 minutes

B. be able to state her birthday and address

C. be able to tolerate separations from her parent for several hours at a time

D. identify upper and lower case letters

E. undergo psychoeducational testing to assess kindergarten readiness

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Critique: 11

Preferred Response: C

Children are socially ready to attend school when they are able to separate from their parents for several hours at a time. They are expected to pay attention to the teacher, follow the classroom routine, play well with others, and take turns. During classroom group-based activities, the young child should be able to pay attention to the teacher for 10 to 15 minutes, sit quietly, and listen to the teacher without bothering peers or disrupting the activity. Children also should be able to relate personal experiences and tell stories. Children entering kindergarten should know color names, be able to count to 10, retell a story, identify some printed letters, and print their names. Knowing their addresses and birth dates and being able to identify both upper and lower case letters is expected of children entering first grade. Because the child described in the vignette has the prerequisite skills for kindergarten, a psychoeducational evaluation is not indicated at this time.

References:

High PC and the Committee on Early Childhood, Adoption, and Dependent Care and Council on School Health. School readiness. Pediatrics. 2008;121:e1008-e1015. Available at:

http://pediatrics.aappublications.org/cgi/content/full/121/4/e1008

Kaplan-Sanoff M. School readiness. In: Parker S, Zukerman B, Augustyn M, eds. Developmental and Behavioral Pediatrics: A Handbook for Primary Care. 2nd ed. Philadelphia, Pa: Lippincott Williams & Wilkins; 2005:285-288

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Question: 12

A

family comes to your office for consultation regarding a 3-week trip to India they are planning

to

take in 3 months. The children, a 9-year-old boy and a 7-month-old girl, are well, and their

immunizations are up to date.

Of the following, the MOST appropriate prophylaxis to provide in preparation for travel is

A. chloroquine for both children

B. hepatitis A vaccination for both children

C. measles vaccination for the girl

D. polio vaccination for the boy

E. typhoid vaccine for both children

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Critique: 12

Preferred Response: C

Protection against infectious diseases is an important issue in preparing children and adults for international travel. Clinicians can obtain specific knowledge of available vaccines and prophylaxis for certain conditions from the American Academy of Pediatrics 2009 Report of the

Committee on Infectious Diseases (Red Book®) and the travelers’ health site of the Centers for Disease Control and Prevention. Travel to India involves a potentially increased exposure to malaria, hepatitis A, measles, polio, and Salmonella typhi. However, there are other considerations in recommending various preventive measures for travelers. Measles may be encountered more commonly in many parts of the world, including India. Accordingly, measles vaccine is recommended for 6- to 11-month-old children, and the 7-month- old girl in the vignette should be given a dose of measles vaccine. She still will require two doses of measles-containing vaccine after 1 year of age because the immune response may be suboptimal at her young age. If the 9-year-old boy is up to date on immunizations, he requires no additional measles vaccination. Although exposure to malaria is a concern on a prolonged trip to India, resistance to chloroquine is a major concern in this region, as it is in all of South and Southeast Asia, sub- Saharan Africa, and tropical areas of South America. Available agents for resistant malaria prophylaxis in infants and children include atovaquone/proguanil and mefloquine. Doxycycline can be used in children older than 8 years of age. Hepatitis A is a concern, but hepatitis A vaccine is not approved in children younger than 1 year of age. Intramuscular immunoglobulin is recommended for children younger than 1 year of age, as the baby in the vignette, traveling to an endemic area. The boy should receive his first dose of hepatitis A vaccine at least 2 to 4 weeks before departure if he has not been immunized previously, with completion of the two-dose series 6 to 12 months later. Although polio exposure may be a concern, if both children are up to date in their vaccination series, no additional polio vaccine is indicated. Finally, typhoid vaccine might be indicated for a trip to India that lasts longer than 2 weeks, but neither of the two licensed vaccines is indicated in children younger than 2 years of age.

References:

American Academy of Pediatrics. International travel. In: Pickering LK, Baker CJ, Kimberlin DW, Long SS, eds. Red Book: 2009 Report of the Committee on Infectious Diseases. 28th ed. Elk Grove Village, Ill: American Academy of Pediatrics; 2009:98-104

Centers for Disease control and Prevention. Travelers’ Health Web site. Available at:

http://wwwn.cdc.gov/travel/default.aspx

Centers for Disease Control and Prevention (CDC). Update: measles—United States, January–July 2008. MMWR Morbid Mortal Wkly Rep. 2008;57:893-896. Available at:

http://www.cdc.gov/mmwr/preview/mmwrhtml/mm5733a1.htm

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Question: 13

You are evaluating a 2-year-old girl who was adopted from an orphanage in Eastern Europe. She has had a pruritic rash since she was brought to the United States 3 weeks ago. According to the mother, the rash is so pruritic that the girl must wear socks on her hands at night to prevent her from scratching. Physical examination demonstrates multiple 2- to 3-mm erythematous papules and vesicles around her waist, in her inguinal folds, on her neck, and on the palms and soles (Item Q13). No other focal findings are evident on physical examination.

Of the following, the MOST appropriate agent with which to treat this patient is

A. acyclovir orally

B. hydrocortisone topically

C. hydroxyzine orally

D. permethrin topically

E. prednisone orally

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Critique: 13

Preferred Response: D

Scabies is a common disorder caused by infestation with the mite Sarcoptes scabiei. Scabies is contracted by prolonged close personal contact with an infected person, usually in situations such as families with school-age children or individuals living in close quarters, such as the orphanage described for the girl in the vignette. Permethrin, an insecticide that has been available since 1989, is a safe and effective treatment for scabies. Permethrin acts by disrupting the sodium channel current, resulting in delayed repolarization, paralysis, and death of the parasite. It is effective during all stages of the life cycle of the parasite. Because of its excellent safety profile, 5% permethrin cream is the first- line drug for the treatment of scabies, especially among patients who have neurologic disorders and infants and young children. Lindane, an agent that was used in the past, no longer is recommended. The 1% solution of permethrin used to treat head lice has too low of a concentration to treat scabies effectively. Permethrin has a low potential for toxicity but occasionally may cause redness of the skin, burning, and stinging with application and has been associated with rash and diarrhea. It is not recommended for use in infants younger than 2 months of age or for pregnant women. Acyclovir is an antiviral agent used to treat herpesvirus infections and is not effective in the treatment of scabies. Topical hydrocortisone and oral hydroxyzine can be used to alleviate the itching, and oral prednisone may decrease the inflammation of scabies, but none of these agents is used to treat the infection.

References:

American Academy of Pediatrics. Scabies. In: Pickering LK, Baker CJ, Kimberlin DW, Long SS, eds. Red Book: 2009 Report of the Committee on Infectious Diseases. 28th ed. Elk Grove Village, Ill: American Academy of Pediatrics; 2009:589-591

Downs A. Comparing antiscabies treatments. Arch Dermatol. 1997;133:526

Schultz MW, Gomez M, Hansen RC, et al. Comparative study of 5% permethrin cream and 1% lindane lotion for the treatment of scabies. Arch Dermatol. 1990;126:167-170. Abstract available at: http://www.ncbi.nlm.nih.gov/pubmed/1689135

Strong M, Johnstone P. Interventions for treating scabies. Cochrane Database Syst Rev. 2007;3:CD000320. Available at:

http://www.mrw.interscience.wiley.com/cochrane/clsysrev/articles/CD000320/frame.html

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Question: 14

An 18-month-old girl who has a 2-day history of vomiting with reduced oral intake presents to the clinic with a 24-hour history of nonbloody diarrhea. She was previously well. Upon further questioning, her mother reports a reduced number of wet diapers prior to the onset of diarrhea. She states that the girl is having four to five loose stools per day. You estimate the girl to be 5% dehydrated.

Of the following, the MOST likely additional examination finding is

A. bounding peripheral pulses

B. capillary refill of 4 seconds

C. hypotension

D. periorbital edema

E. tachycardia

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Critique: 14

Preferred Response: E

Causes of dehydration in the pediatric patient include fluid losses from the gastrointestinal tract (vomiting or diarrhea) and less commonly, fluid losses due to excessive urinary production, as can occur in children who have urinary concentrating defects (due to renal dysplasia or diuretics). Total body water constitutes approximately 60% of the body weight in children. Two thirds of the body fluid is contained within the intracellular compartment and one third within the extracellular compartment. The extracellular fluid compartment is divided further into the interstitial compartment (75%) and the intravascular compartment (25%). Dehydration affects both the intracellular and extracellular compartments, but most physical signs and symptoms result from a reduction of intravascular volume (IVV). Clinically, the IVV can be expressed as the effective circulating blood volume (ECBV), which represents the volume and pressure providing perfusion to the tissues. A patient who has a low IVV has a low ECBV, but a patient who has a high IVV or normal IVV also may have a low ECBV if cardiac dysfunction also is present. Children who have gastroenteritis and mild-to-moderate dehydration, such as the child described in the vignette, can maintain cardiac output and blood pressure by increasing heart rate and effective myocardial contractility. Therefore, tachycardia is the most common clinical finding in this setting. Hypotension is sign of severe dehydration. Other signs in more advanced dehydration (approaching 10%) are decreased skin turgor, bounding pulses, and decreased capillary refill. Periorbital edema is not expected in the clinical setting of mild dehydration.

References:

Armon K, Stephenson T, MacFaul R, Eccleston P, Werneke U. An evidence and consensus based guideline for acute diarrhoea management. Arch Dis Child. 2001;85:132-142. Abstract available at: http://www.ncbi.nlm.nih.gov/pubmed/11466188

Boineau FG, Lewy JE. Estimation of parenteral fluid requirements. Pediatr Clin North Am. 1990;37:257-264. Abstract available at: http://www.ncbi.nlm.nih.gov/pubmed/2184395

Gorelick MH, Shaw KN, Murphy KO. Validity and reliability of clinical signs in the diagnosis of dehydration in children. Pediatrics. 1997;99;e6. Available at:

http://pediatrics.aappublications.org/cgi/content/full/99/5/e6

Hill LL. Body composition, normal electrolyte concentrations, and the maintenance of normal volume, tonicity, and acid-base metabolism. Pediatr Clin North Am. 1990;37:241-256. Abstract available at: http://www.ncbi.nlm.nih.gov/pubmed/2184394

Rose BD, Post TW. Regulation of the effective circulating volume. In: Clinical Physiology of Acid- base and Electrolyte Disorders. 5th ed. New York, NY: McGraw-Hill Medical Publishing Division;

2001:258-284

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Question: 15

A 10-year-old boy presents to the clinic complaining of tongue and mouth itching within a few minutes after eating apples. His mother states that he has not experienced these symptoms with other foods, but they occur every time he eats a fresh apple. He denies systemic symptoms, and the oral symptoms resolve within a few minutes. Other than allergic rhinitis in the spring months, he is healthy.

Of the following, you are MOST likely to advise his mother that

A. allergy skin testing to fresh apples probably will have negative results

B. cooking the apple will not alter its allergenicity

C. her son should avoid eating all fruits

D. her son should avoid milk products

E. her son’s symptoms are related to his allergic rhinitis

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Critique: 15

Preferred Response: E

The boy described in the vignette is exhibiting a common form of food allergy called food pollen syndrome or oral allergy syndrome (OAS). OAS is seen in 30% to 40% of children who have allergic rhinitis. Certain foods contain proteins that are similar to airborne allergens, and patients who are allergic to an aeroallergen are at risk of developing reactions to the cross- reacting food protein (Item C15). In most cases, symptoms are isolated to the oropharynx, where food comes in contact with a mucosal surface, and include lip, tongue, and oral mucosal pruritus; tingling; and occasionally angioedema. Interestingly, because these food proteins are heat-labile, cooking the food (eg, apple pie) negates its antigenic properties. Although symptoms typically are mild, there are reports of severe reactions. In one recent review involving 1,361 patients who had OAS, 8.7% experienced systemic symptoms outside the gastrointestinal tract, 3% experienced symptoms other than oral symptoms, and 1.7% experienced anaphylactic shock. Because OAS is relatively specific to particular cross-reacting food(s), patients do not need to avoid other fruits or vegetables to which they have not experienced reactions. Avoidance of unrelated foods (eg, milk, eggs) is not recommended unless the history suggests a previous reaction. The decision to avoid causative foods can be based on the severity of reaction. Referral to an allergist typically is reserved for situations when skin testing is desired or if the child has experienced systemic symptoms. Skin testing is performed using a commercial extract or the fresh fruit or vegetable. When using fresh food, the sensitivity of skin testing with a history of reproducible reactions is close to 90%, while the negative predictive value is more than 90%. The skin prick device is pressed into the food and then pressed in the skin (so-called "prick-prick" skin test). Other immunoglobulin (Ig) E food reactions include atopic dermatitis, eosinophilic esophagitis, and specific food allergy. In the United States, 85% of specific food allergies are due to egg, milk, wheat, soy, peanuts, tree nuts, fish, and shellfish. Most children who have IgE food allergies react to only one or two causative foods, although children who have tree nut allergy, atopic dermatitis, and eosinophilic esophagitis often have IgE-mediated reactions to multiple foods.

References:

Hyams JS. Food allergy (food hypersensitivity). In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, Pa: Saunders Elsevier;

2007:1585-1586

Ma S, Sicherer S, Nowak-Wegrzyn A. A survey on the management of pollen-food syndrome in allergy practices. J Allergy Clin Immunol. 2003;112:784-788. Abstract available at:

http://www.ncbi.nlm.nih.gov/pubmed/14564362

Sampson HA, Leung DYM. Adverse reactions to foods. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, Pa: Saunders Elsevier; 2007:986-989

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Sicherer SH, Sampson HA. 9. Food allergy. J Allergy Clin Immunol. 2006;117(2 suppl mini- primer):S470-S475. Abstract available at: http://www.ncbi.nlm.nih.gov/pubmed/16455349

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Question: 16

A 5-year-old boy who has epilepsy and severe developmental delay is brought to the emergency department because of increasing somnolence over the past 12 hours. His mother reports that his activity level has decreased over the past 2 days, and this morning he was difficult to arouse. He has not been otherwise ill and has not had any seizures for the past 6 months. His antiepileptic medications include phenobarbital and oxcarbazepine. The doses recently were increased. On physical examination, the boy is difficult to arouse and moans to painful stimulation. His heart rate is 70 beats/min, respiratory rate is 18 breaths/min, and blood pressure is 80/50 mm Hg.

Of the following, the MOST likely additional abnormality on physical examination of this child is

A. dilated pupils

B. hyperactive bowel sounds

C. hyperreflexia

D. hypothermia

E. tremors

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Critique: 16

Preferred Response: D

The patient described in the vignette is exhibiting signs and symptoms consistent with a sedative-hypnotic overdose, including coma, bradycardia, bradypnea, and hypotension. Although most antiepileptic drugs can cause lethargy at high doses, barbiturates (eg, phenobarbital) are particularly sedating. The central nervous system depressant effects of barbiturates are primarily caused by drug action on the inhibitory neurotransmitter gamma aminobutyric acid (GABA). Barbiturates both increase GABA activity and directly stimulate GABA receptors. They also competitively inhibit glutamate, an excitatory neurotransmitter, from binding to receptors. Other physical findings that may be present in the setting of a sedative/hypnotic overdose include hypothermia, hyporeflexia, hypoactive bowel sounds, and decreased muscular activity. Pupillary light reflex is slowed, but pupillary size usually is normal.

References:

Lafferty KA. Toxicity, barbiturate. eMedicine Specialties, Emergency Medicine, Toxicology. 2008. Available at: http://www.emedicine.com/emerg/topic52.htm

Schachter SC. Pharmacology of antiepileptic drugs. UpToDate Online 16.3. 2008. Available at:

http://www.utdol.com/online/content/topic.do?topicKey=epil_eeg/5220&selectedTitle=1~150&sou

rce=search_result630

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Question: 17

During a routine health supervision visit, the mother of a 2½ month-old male infant tells you that the baby has been experiencing bloating and flatulence. His diet consists of 5 to 6 oz of a cow milk-based formula given five times per 24 hours. Because of frequent spitting-up, his mother recently added rice cereal to each bottle. He has two to three seedy stools per day. On physical examination, the baby is alert and vigorous. His length and weight are tracking between the 50th and 75th percentiles. The infant’s mother asks you whether switching to a soy protein-based formula will help her baby’s "gassiness."

Of the following, the MOST likely the cause of this infant’s symptoms is

A. cow milk protein allergy

B. excessive energy intake

C. incomplete starch digestion

D. lactose malabsorption

E. sucrase-isomaltase deficiency

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Critique: 17

Preferred Response: C

The infant described in the vignette has been given formula thickened with rice cereal to ameliorate spitting-up. Following the introduction of cereal, his mother has noted increased "gassiness." The most likely cause of this symptom is incomplete starch digestion. Development of the digestive-absorptive function of the gastrointestinal tract is not complete at birth. The newborn can assimilate considerable amounts of complex carbohydrates through

hydrolysis by salivary gland amylase until pancreatic function and small intestinal intraluminal pancreatic amylase activity mature. Nevertheless, until pancreatic maturity is achieved, and certainly in infants younger than 4 months of age, dietary starches may be hydrolyzed incompletely. As a result, increased amounts of undigested carbohydrate pass into the colon, where bacterial fermentation results in gas production that may cause the symptoms described for the infant in the vignette.

A diagnosis of cow milk protein allergy frequently is considered in the differential diagnosis

of a variety of diverse gastrointestinal complaints. Symptoms that may be associated with cow milk protein intolerance include diarrhea, failure to thrive, hypoproteinemia, hematochezia, anemia, and vomiting as well as other cutaneous and systemic manifestations of atopy. The relationship between infantile colic and cow milk protein allergy remains highly controversial,

particularly when fussiness or irritability is the sole complaint. For a thriving infant who develops vague gastrointestinal symptoms after the type of dietary changes described in the vignette, cow milk protein allergy should be considered only after ruling out other, more likely causes, such as incomplete digestion of complex carbohydrates.

It is unlikely that the infant described in the vignette has excessive energy intake because

his weight gain is not excessive, and thickening of the formula does not appreciably add to energy intake in an infant who is consuming 25 to 30 oz of formula per day. Lactase concentrations reach mature values in the small intestine by the 36th week of gestation in all healthy infants. Congenital or early-onset primary lactose intolerance is an extremely rare condition that is associated with severe diarrhea and inanition. It typically presents with voluminous diarrhea soon after the first feedings of human milk or cow milk-based formula. During infancy and childhood, secondary lactase deficiency may occur as a consequence of intestinal mucosal damage following a prolonged diarrheal illness, as a result of other intestinal disorders (eg, celiac disease), or in association with malnutrition. Sucrase-isomaltase (SI) deficiency is the most common congenital disaccharidase deficiency. Diarrhea is a virtually universal symptom of SI deficiency and may be associated with poor weight gain. Symptoms usually appear in older infants following the introduction of sucrose-containing foods, particularly fruits and juices. Infants who have SI deficiency also do not tolerate soy or protein hydrolysate formulas because both sucrose and glucose polymers are maldigested and malabsorbed.

References:

Craig WR, Hanlon-Dearman A, Sinclair C, Taback S, Moffatt M. Metoclopramide, thickened feedings, and positioning for gastro-oesophageal reflux in children under two years. Cochrane Database Syst Rev. 2004;3:CD003502. Available at:

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

http://www.mrw.interscience.wiley.com/cochrane/clsysrev/articles/CD003502/frame.html

Hall RT, Carroll RE. Infant feeding. Pediatr Rev. 2000; 21:191-200. Available at:

http://pedsinreview.aappublications.org/cgi/content/full/21/6/191

Montes RG. Carbohydrate malabsorption. In: Rudolph CD, Rudolph AM, Hostetter MK, Lister G, Siegel NJ, eds. Rudolph’s Pediatrics. 21st ed. New York, NY: McGraw-Hill; 2003:1423-1427

Thomas DW, McGilligan K, Eisenberg LD, Lieberman HM, Rissman EM. Infantile colic and type of milk feeding. Am J Dis Child. 1987;141:451-453. Abstract available at:

http://www.ncbi.nlm.nih.gov/pubmed/3494394

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Question: 18

You are asked by your pediatric resident on rounds why you remove umbilical artery catheters in very low-birthweight infants in your nursery by postnatal day 7.

Of the following, the BEST reason for such removal is to prevent

A. anemia

B. hyperglycemia

C. hypotension

D. sepsis

E. thrombocytosis

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Critique: 18

Preferred Response: D

Umbilical access to the arterial and venous circulation is a mainstay of early monitoring for the critically ill newborn. The umbilical artery provides access to the central arterial circulation via the inferior iliac artery, common iliac artery, and aorta. An umbilical arterial catheter (UAC) is indicated for only two uses: the frequent sampling of arterial blood for blood gas analysis in a newborn who has respiratory distress or the provision of inline transduced arterial blood pressure monitoring. In either circumstance, the risks of placing and using a UAC must be considered. Among the risks of using a UAC, infection is a significant concern when catheters remain in place for more than 10 to 14 days. The UAC becomes colonized by commensal staphylococci within 24 hours of placement, and such colonization may lead to bloodstream infection due to the immature immune system of very low-birthweight (VLBW) infants. The risk for infection is increased when hyperalimentation fluid is administered through a UAC. Removal of the UAC by the end of the first postnatal week reduces such risk significantly and generally is feasible because most VLBW newborns have improved respiratory status by this time. Additional risks of inserting and using a UAC include vascular spasm; thrombogenesis; either large-vessel occlusion or embolization of microthrombi in the distal arterial circulation; and damage to the vascular endothelium or further injury leading to a mycotic aneurysm. Hypoglycemia may occur if the UAC is inserted in the thoracic aorta (between T6 and T10) and fluids that have a high glucose concentration are administered, inducing a hyperinsulinemic response by the pancreas. Anemia, a common problem in critically ill VLBW newborns, does not result from UAC insertion or use, unless blood loss occurs when the line is accessed, which is rare. Hyperglycemia is not a complication of inserting or using a UAC. Hypotension is an indication to insert a UAC and monitor blood pressure, not a complication. Thrombocytopenia, not thrombocytosis, may occur when a thrombus forms on the end of a UAC or elsewhere in the circulation.

References:

Coleman MM, Spear ML, Finkelstein M, et al. Short-term use of umbilical artery catheters may not be associated with increased risk for thrombosis. Pediatrics. 2004;113:770-774. Available at:

http://pediatrics.aappublications.org/cgi/content/full/113/4/770

Hermansen MC, Harmansen MG. Intravascular catheter complications in the neonatal intensive care unit. Clin Perinatol. 2005;32:141-156. Abstract available at:

http://www.ncbi.nlm.nih.gov/pubmed/15777826

O’Grady NP, Alexander M, Dellinger EP, et al. Guidelines for the prevention of intravascular catheter-related infections. The Hospital Infection Control Practices Advisory Committee, Centers for Disease Control and Prevention. U.S. Pediatrics. 2002;110:e51-e75. Available at:

http://pediatrics.aappublications.org/cgi/content/full/110/5/e51

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Rodriguez RJ, Martin RJ, Fanaroff AA. Respiratory distress syndrome and its management. In:

Martin RJ, Fanaroff AA, Walsh MC, eds. Fanaroff and Martin's Neonatal-Perinatal Medicine:

Diseases of the Fetus and Infant. 8th ed. Philadelphia, Pa: Mosby Elsevier; 2006:1097-1107

Wortham BM, Galtatzes CG, Rais-Bahrami K. Umbilical artery catheterization. In: MacDonald MG, Ramasethu J, eds. Atlas of Procedures in Neonatology. 4th ed. Philadelphia, Pa: Lippincott Williams & Wilkins; 2007:157-176

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Question: 19

You are evaluating a new patient in your office, a 10-year-old boy who has no significant past medical history or illnesses. His weight and height are both at the 75th percentile. On physical examination, you notice a mild pectus excavatum but no cardiopulmonary abnormalities. When you ask him about it, he replies, "I’ve always had it, and it doesn’t bother me."

Of the following, the MOST appropriate next step in the management of this problem is

A. electrocardiography

B. exercise stress testing

C. psychological evaluation

D. reassurance

E. surgery consultation

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Critique: 19

Preferred Response: D

Pectus excavatum is a skeletal abnormality of the chest wall characterized by concavity of the anterior chest. It may occur in isolation or can be associated with other disorders, such as Marfan syndrome and Ehlers-Danlos syndrome, and it is more common in boys. The deformity usually is present in infancy, although symptoms do not present until later in life. Many children are asymptomatic and are not concerned about the cosmetic appearance of the chest, but some children may experience psychological distress about their appearance. Some children experience symptoms such as dyspnea with exertion, chest or rib pain, and decreased exercise tolerance. Pulmonary function abnormalities, when present, include an obstructive pattern and, less commonly, a restrictive pattern. Cardiac dysfunction due to impaired stroke volume with exercise has been described and is more common in older patients. Because the boy described in the vignette does not complain of any symptoms and is not psychologically affected by the cosmetic appearance of his chest, only reassurance and observation are necessary. Lateral chest radiographs and chest computed tomography scan can demonstrate the deformity, but such tests generally are not necessary in asymptomatic patients. Similarly, electrocardiography and exercise stress testing are not indicated unless exercise intolerance or dyspnea upon exertion is present. Psychological evaluation could be considered for patients who are very concerned about the cosmetic appearance, and corrective surgery should be considered for those who have severe psychological distress. Pulmonary function, if impaired, often is not improved with surgical correction.

References:

Aronson DC, Bosgraaf RP, Merz EM, van Steenwijk RP, van Aalderen WM, van Baren R. Lung function after the minimal invasive pectus excavatum repair (Nuss procedure). World J Surg. 2007;31:1518-1522. Available at:

http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pubmed&pubmedid=17534548

Boas SR. Skeletal diseases influencing pulmonary function. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, Pa: Saunders Elsevier; 2007:1841-1844

Koumbourlis AC, Stolar CJ. Lung growth and function in children and adolescents with idiopathic pectus excavatum. Pediatr Pulmonol. 2004;38:339-343. Abstract available at:

http://www.ncbi.nlm.nih.gov/pubmed/15334513

Rowland T, Moriarty K, Banever G. Effect of pectus excavatum deformity on cardiorespiratory fitness in adolescent boys. Arch Pediatr Adolesc Med. 2005;159:1069-1073. Available at:

http://archpedi.ama-assn.org/cgi/content/full/159/11/1069

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Question: 20

A nurse practitioner in your clinic has asked you to review the chart of an 11-year-old boy referred from school for evaluation of acanthosis nigricans.

Of the following, the physical examination parameter that is MOST likely to be useful in predicting comorbidities in this patient is

A. blood pressure at the 75th percentile for height

B. body mass index at the 95th percentile for age

C. sum of triceps and subscapular skinfold thickness greater than 90% for age

D. weight at the 50th percentile and height at the 25th percentile for age

E. weight at the 95th percentile and height at the 75th percentile for age

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Critique: 20

Preferred Response: B

Because acanthosis nigricans is associated with obesity, the young man in the vignette is likely to be overweight and at risk for numerous comorbidities, including hypertension, hyperlipidemia, and the metabolic syndrome. Although several methods have been used to assess obesity, body mass index (BMI),

defined as weight in kilograms divided by height in meters squared (kg/m2), has become the standard measure for children, adolescents, and adults. The correlation of BMI with measures of adiposity is excellent in adults, but slightly less reliable for children, whose BMIs change with age during childhood and adolescence. Of note, boys have less body fat than do girls at the same BMI. Total body fat correlates with sexual maturational level more than age because as sexual maturity progresses, body fat increases. Those who have a higher waist-to-hip ratio at the same BMI have more body fat. In 2000, the Centers for Disease Control and Prevention (CDC) released new growth curves, including BMI curves for boys and girls from ages 2 to 20 years (www.cdc.gov/growthcharts). Obesity has been defined as a BMI greater than the 95th percentile for age on the 2000 CDC growth curves. Children whose BMIs fall between the 85th and 95th percentiles for age are considered overweight. Children whose BMIs are greater than the 99th percentile are defined as severely obese. Weight-for-height is a less accurate measure and is subject to misinterpretation of overweight versus obesity. In the estimation of risk for comorbid conditions, including diabetes or insulin insensitivity, hypertension, hyperlipidemia, or metabolic syndrome, the BMI now represents a standardized measurement. However, high weight-for-height must be used to assess obesity in children younger than 2 years of age for whom BMI norms are not available. It is unclear whether childhood obesity is an independent risk factor for hyperlipidemia and atherosclerotic heart disease. It is known that the higher the BMI, the greater the risk of left ventricular wall thickness in children who have hypertension and that BMI correlates with arterial wall thickness. Skinfold thickness measurements are accurate in estimating total body fat but are not currently the standard for determining risk for comorbidities of obesity. In addition, they may be difficult to obtain in the primary care office setting due to issues with interobserver reliability, training of staff in the technique, and the expense of the calipers ($200). However, skinfold thickness measurements may be useful in very athletic, muscular adolescent males whose BMIs are artificially elevated by high muscle mass. All children older than 3 years of age should have blood pressures measured at each health supervision visit; children who are overweight should have blood pressures monitored more frequently. A blood pressure that is at the 75th percentile is within the normal range and less likely to be associated with comorbidities than BMI at the 95th percentile.

References:

Barlow SE and the Expert Committee. Expert Committee recommendations regarding the prevention, assessment, and treatment of child and adolescent overweight and obesity:

summary report. Pediatrics. 2007;120 (suppl):S164-S192. Available at:

http://pediatrics.aappublications.org/cgi/content/full/120/Supplement_4/S164

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Feld LG, Corey H. Hypertension in childhood. Pediatr Rev. 2007;28:283-298. Available at:

http://pedsinreview.aappublications.org/cgi/content/full/28/8/283

Freedman DS, Kettel Khan L, Mei A, Dietz WH, Srinivasan SR, Berenson GS. Relation of childhood height to obesity among adults: the Bogalusa Heart Study. Pediatrics. 2002;109:e23. Available at: http://pediatrics.aappublications.org/cgi/content/full/109/2/e23

Katzmarzyk PT, Srinivasan SR, Chen W, Malina RM, Bouchard C, Berenson GS. Body mass index, waist circumference, and clustering of cardiovascular disease risk factors in a biracial sample of children and adolescents. Pediatrics. 2004;114:e198-e205. Available at:

http://pediatrics.aappublications.org/cgi/content/full/114/2/e198

Schneider MB, Brill SR. Obesity in children and adolescents. Pediatr Rev. 2005;26:155-162. Available at: http://pedsinreview.aappublications.org/cgi/content/full/26/5/155

Sorof J, Daniels S. Obesity hypertension in children: a problem of epidemic proportion. Hypertension. 2002;40:441-447. Available at:

http://hyper.ahajournals.org/cgi/content/full/40/4/441

Summerbell CD, Waters E, Edmunds LD, Kelly S, Brown T, Campbell KJ. Interventions for preventing obesity in children. Cochrane Database Syst Rev. 2005;3:CD001871

Whitlock EP, O’Connor EA, Williams SB, Beil TL, Lutz KKW. Effectiveness of Weight Management Programs in Children and Adolescents. Evidence Report/Technology Assessment No. 170. AHRQ Publication No. 08-E014. Rockville, Md: Agency for Healthcare Research and Quality, U.S. Department of Health and Human Services; 2008. Available at:

http://www.ncbi.nlm.nih.gov/books/bv.fcgi?rid=hstat1b.chapter.139937

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Question: 21

You are demonstrating digital clubbing in a teenage patient at the physical diagnosis course you teach at your local medical school. One of the students asks what condition would predispose an adolescent to this finding.

Of the following, the MOST likely predisposing condition is

A. hypoplastic left heart syndrome after completion of a Fontan procedure

B. pulmonary atresia associated with unrepaired ventricular septal defect

C. tetralogy of Fallot that was repaired in infancy

D. transposition of the great arteries that was repaired at 1 week of age

E. unrepaired atrial septal defect

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Critique: 21

Preferred Response: B

Hypertrophic pulmonary osteoarthropathy, better known as clubbing of the digits (Item C21A), can be seen in a variety of entities, the most common of which is cyanotic heart disease, such as pulmonary atresia with an unrepaired ventricular septal defect and collateral pulmonary blood flow. Other conditions in which it has been described are chronic lung disease, biliary cirrhosis, and infective endocarditis. Finally, clubbing of the digits can be a normal variant, occurring as a familial trait. Clubbing initially becomes apparent when the angle between the proximal nail and the soft tissue of the digit is obliterated or filled in, which can be demonstrated by having the patient place the distal phalangeal joints together in a "mirrorlike" fashion. For the individual who has no clubbing, the maneuver creates a diamond-shaped space (Item C21B). In contrast, the patient who has clubbing demonstrates complete occlusion of this space because all aspects of the nail bed and distal soft tissue directly oppose one another. This finding is referred to as a positive Schamroth sign. Hypoplastic left heart syndrome is an obstruction of systemic blood flow that typically presents in the first several days after birth as the ductus arteriosus and, thus, the route of systemic blood flow constricts. Palliation of this complex disorder involves difficult surgical anastomoses, including the creation of systemic blood flow from the right ventricle through the native pulmonary valve and artery that has been brought to the aorta. The pulmonary blood flow is delivered through an aortic-pulmonary artery shunt (eg, Blalock-Taussig shunt) or directly from the right ventricle to the pulmonary artery (Sano shunt). The second stage of the palliation usually occurs about midway through the first postnatal year and consists of a superior vena cava-to-pulmonary artery passive shunt such as the Glenn operation. Both the first and second stages of the palliation result in oxygen saturations in the 70% and 80% range, but upon completion of the third stage, which brings the inferior vena cava to the pulmonary arteries, saturations reach the 90% range. Clubbing is not expected in affected patients. Similarly, patients who have tetralogy of Fallot may present with or develop cyanosis, but upon complete surgical repair, their oxygen saturations are normal. The same is true of the child who has repaired transposition of the great arteries. The atrial septal defect typically is a left-to-right shunt associated with normal oxygen saturation.

References:

Haddad GG, Green TP. Diagnostic approach to respiratory disorders. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 18th Ed. Philadelphia, Pa:

Saunders Elsevier; 2007:1731-1732

Karnath B. Digital clubbing: sign of underlying disease. Hospital Physician. 2003;39(9):25-27. Available at: http://www.turner-white.com/pdf/hp_sep03_club.pdf

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Question: 22

A 14-year-old boy presents to your office because the side of his face is drooping. His mother

states that he complained yesterday of decreased food taste. Today, while at school, he could

not use the microscope in science class because he couldn't close his left eye, and his teacher noted that his smile was crooked. Physical examination reveals no abnormalities and no vesicles

in his ears. Mental status on neurologic examination is normal, pupil responses are normal,

extraocular movements are full, and there is no nystagmus or reported double vision. He is unable to close his left eye or raise his left eyebrow, has decreased left-side nasolabial folds, and cannot close his mouth to puff out his cheeks (Item Q22). His palate and tongue movements are normal. Motor examination reveals normal proximal and distal strength in both arms and normal regular and tandem gait.

Of the following, the MOST appropriate initial diagnostic procedure is

A. blood test for antistreptococcal antibodies

B. brain magnetic resonance imaging

C. edrophonium (Tensilon®) test

D. no further testing

E. noncontrast head computed tomography scan

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Critique: 22

Preferred Response: D

Acute focal or generalized weakness is a medical emergency requiring a systematic history and neurologic examination to localize the problem. For acute focal weakness, the problem can localize to the brain, brainstem, spinal cord, anterior horn cell, root, nerve, junction, or muscle. Often, the physical examination can localize the problem to one of these levels. In the case of acute unilateral facial weakness, as described for the boy in the vignette, the typical differential diagnosis is acute facial nerve palsy (ie, Bell palsy) or a more rostral disease process of the brainstem or brain (cerebrum, motor cortex) such as a stroke. The key diagnostic point for facial weakness is whether the weakness involves the entire side of the face or the face below the forehead. A 7th nerve palsy affects all the innervated muscles, weakening or paralyzing the entire hemi-face from forehead to chin. A lesion above the facial nerve nucleus typically weakens the face below the forehead. The boy described in the vignette has full left-sided facial weakness, including the muscles in his forehead. Such findings localize to the facial nerve, and in this clinical setting, neuroimaging is not revealing. Accordingly, no further testing is required. When the examination localizes a problem involving facial weakness to the brain or brainstem, brain magnetic resonance imaging (MRI) or, if MRI is not available quickly, noncontrast head computed tomography should be obtained. Facial weakness due to an acute brain process, such as a left middle cerebral artery stroke, usually presents with involvement of both the contralateral right face and the right hand. The brainstem, specifically the pons, is the source of the facial nerve, and brainstem diseases can produce full hemi-facial weakness. However, due to the close proximity of other brainstem nuclei, a brainstem lesion affecting the left face also should affect other functions, including the left 6th nerve, which abducts the left eye. Often, sensory and motor findings on the opposite side of the body, the so-called "crossed signs," indicate brainstem disease.

The edrophonium/Tensilon® test involves administration of this acetylcholinesterase inhibitor to increase acetylcholine at the neuromuscular junction and reverse weakness. The test is used for diagnosis of myasthenia gravis. Myasthenia gravis typically produces bilateral fatiguing weakness, particularly ptosis, as well as weakness in other cranial nerves or generalized weakness. Facial nerve palsy can be caused by a variety of infectious agents, but no specific diagnostic testing is indicated in most cases. However, in regions where Lyme disease is endemic or exposure is possible, testing for Lyme disease may be indicated. Assessment of antistreptococcal antibodies is not helpful because streptococci do not cause facial nerve palsy. The American Academy of Neurology practice parameter states that oral steroids probably are beneficial and acyclovir possibly is beneficial for treatment of facial nerve palsy. Pediatric studies and reviews have concluded that evidence is insufficient to recommend steroids for children. However, many clinicians recommend administering a short course of oral prednisone for Bell palsy.

References:

Ashtekar CS, Joishy M, Joshi R. Best evidence topic report. Do we need to give steroids in

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

children with Bell's palsy? Emerg Med J. 2005;22:505-507. Abstract available at:

http://www.ncbi.nlm.nih.gov/pubmed/15983089

Grogan PM, Gronseth GS. Practice parameter: steroids, acyclovir, and surgery for Bell's palsy (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology. 2001;56:830-836. Available at:

http://www.neurology.org/cgi/content/full/56/7/830

Sarnat HB. Bell palsy. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, Pa: Saunders Elsevier; 2007:2566-2568

Singhi P, Jain V. Bell's palsy in children. Semin Pediatr Neurol. 2003;10:289-297. Abstract available at: http://www.ncbi.nlm.nih.gov/pubmed/14992461

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Question: 23

A 4-year-old girl in your practice has moderate persistent asthma. The child’s mother tells you that she and her husband would like a big family, and she asks if there is anything they can do to reduce the risk of asthma to their future children.

Of the following, increased asthma risk in the offspring is MOST associated with prenatal exposure to maternal

A. alcohol binging

B. barbiturate use

C. benzene exposure

D. cigarette smoking

E. cocaine sniffing

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Critique: 23

Preferred Response: D

A growing body of epidemiologic data show various negative outcomes for individuals exposed prenatally to maternal cigarette smoking. The bulk of the information comes from birth registries or retrospective studies, which show associations between maternal cigarette smoking and miscarriage, fetal growth restriction, preterm birth, and low birthweight. A statistically significant increase in cleft lip +/- cleft palate was shown in at least one study that had large sample sizes, but maternal cigarette smoking is not otherwise reliably associated with congenital anomalies. Strong evidence demonstrates an increased risk for sudden infant death syndrome among exposed children, possibly due to altered autonomic function. Multiple studies examining the association between in utero cigarette smoke exposure (without postnatal environmental tobacco smoke exposure) and childhood respiratory disorders demonstrate a significantly increased prevalence of physician-diagnosed asthma and wheezing. Alcohol binging places the exposed embryo/fetus at greater risk for fetal alcohol spectrum disorders than daily moderate alcohol intake. However, the incidence of asthma is not increased in these individuals. Prenatal barbiturate exposure is associated with birth defects in 10% to 20% of exposed individuals. Anomalies include midface hypoplasia, cleft lip +/- cleft palate, and heart defects as well as pre- and postnatal growth deficiency. Asthma risk is not increased. Prenatal exposure to benzene occurs most commonly in a setting where industrial solvents are used. Benzene has not been found to increase the risk of congenital anomalies in exposed embryos/fetuses, and it is not associated with an increased asthma risk. Fetal and childhood abnormalities associated with maternal cocaine use during pregnancy are difficult to study because cocaine often is used concomitantly with tobacco, other street drugs, and alcohol. An increased incidence of placental abruption and sudden infant death syndrome has been associated with prenatal cocaine exposure, and genitourinary anomalies and limb amputations have been reported. Cocaine alone, however, is not associated with increased asthma risk in prenatally exposed individuals.

References:

Benzene. Reprotox®. Available for subscription at: http://www.reprotox.org

Benzene. Teris. Available for subscription at: http://depts.Washington.edu/terisweb/teris/

Cigarette smoking (tobacco). Teris. Available for subscription at:

http://depts.Washington.edu/terisweb/teris/

Cigarette smoking. Reprotox®. Available for subscription at: http://www.reprotox.org

Cocaine. Reprotox®. Available for subscription at: http://www.reprotox.org

Cocaine. Teris. Available for subscription at: http://depts.Washington.edu/terisweb/teris/

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Higgins S. Smoking in pregnancy. Curr Opin Obstet Gynecol. 2002;14:145-151. Abstract available at: http://www.ncbi.nlm.nih.gov/pubmed/11914691

Hoyme HE, May PA, Kalberg WO, et al. A practical clinical approach to diagnosis of fetal alcohol spectrum disorders: clarification of the 1996 Institute of Medicine criteria. Pediatrics. 2005;115:39-47. Available at: http://pediatrics.aappublications.org/cgi/content/full/115/1/39

Phenobarbital. Reprotox®. Available for subscription at: http://www.reprotox.org

Phenobarbital. Teris. Available for subscription at: http://depts.Washington.edu/terisweb/teris/

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Question: 24

You are examining an asymptomatic adolescent male for his annual health supervision visit. Genital examination reveals genitalia at Sexual Maturity Rating 5 and a nontender mass in his left scrotum that extends from the inguinal canal to the upper pole of the testis (Item Q24). The mass decreases in size when he lies down.

Of the following, the MOST likely diagnosis is

A. direct inguinal hernia

B. hydrocele

C. spermatocele

D. testicular tumor

E. varicocele

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Critique: 24

Preferred Response: E

Varicosity of the pampiniform plexus of veins in the scrotum usually develops slowly and may be asymptomatic or cause a heavy sensation in the scrotum. Varicoceles are more common in males ages 15 to 25 years and are seen most often on the left side of the scrotum. This is attributed to increased pressure in the left spermatic vein as it drains directly into the left renal vein. In contrast, the right spermatic vein drains into the inferior vena cava in the pelvis. On physical examination, varicoceles vary in size, can extend from the testis to the inguinal canal, and feel like a "bag of worms" (Item C24). As described for the boy in the vignette, they increase in size with standing or a valsalva maneuver and reduce spontaneously when the patient lies down. If a varicocele develops suddenly, does not reduce in the supine position, or is on the right side, a cause for obstruction of the spermatic vein should be sought. Inguinal hernias usually present as painless, intermittent groin masses that appear with straining and usually reduce spontaneously. They may increase in size and become obstructed, resulting in a painful scrotal mass. A hydrocele represents fluid within the tunica vaginalis that surrounds the testis rather than being distinct from it. A hydrocele transilluminates, which distinguishes it from a solid mass. Spermatoceles are cystic lesions within the spermatic cord that are above and distinct from the testis and transilluminate. Testicular tumors are painless solid masses within the testicle that do not transilluminate.

References:

Adelman WP, Joffe A. Consultation with the specialist: testicular masses/cancer. Pediatr Rev. 2005;26:341-344. Available at: http://pedsinreview.aappublications.org/cgi/content/full/26/9/341

Diamond DA. Adolescent varicocele. Curr Opin Urol. 2007;17:263-267. Abstract available at:

http://www.ncbi.nlm.nih.gov/pubmed/17558270

Kass EJ. Adolescent varicocele. Pediatr Clin North Am. 2001;48:1559-1569. Abstract available at: http://www.ncbi.nlm.nih.gov/pubmed/11732130

Kumanov P, Robeva RN, Tomova A. Adolescent varicocele: who is at risk? Pediatrics. 2008;121:e53-e57. Available at: http://pediatrics.aappublications.org/cgi/content/full/121/1/e53

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Question: 25

You are called to evaluate a 16-year-old girl who was the unrestrained driver in a motor vehicle crash. She reportedly hit the steering wheel but maintained consciousness and now complains of chest pain. On physical examination, she has a heart rate of 110 beats/min, blood pressure of 120/80 mm Hg, and a respiratory rate of 30 breaths/min. Her oxygen saturation by pulse oximetry is 85% while receiving 8 L/min oxygen via a nonrebreathing face mask. As you observe her breathing pattern, you notice that her right chest moves inward with each inspiration. Her chest radiograph demonstrates several significant right-sided findings, including multiple fractures of the 7th, 8th, and 9th ribs; a small apical pneumothorax; and a diffuse opacification consistent with a pulmonary contusion.

Of the following, the MOST appropriate next step is

A. administration of 10 mL/kg of 0.9% normal saline

B. administration of furosemide

C. endotracheal intubation

D. observation

E. surgical fixation of the rib fractures

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Critique: 25

Preferred Response: C

The pulmonary contusion, multiple rib fractures, and pneumothorax described for the girl in the vignette are consistent with significant thoracic trauma. On physical examination, she has a paradoxic respiratory pattern, as evidenced by inward movement of her right chest during inspiration. This is a result of her multiple rib fractures producing an isolated, unstable area that does not move in the appropriate direction during respiratory efforts, the so-called "flail chest." The diagnosis of flail chest is primarily clinical. It is less common in young children due to elasticity of the thoracic skeleton, which results in traumatic forces being transmitted more readily to internal organs. Respiratory distress, as described for this patient, must be addressed urgently. Initial treatment of flail chest generally consists of intubation, mechanical ventilation, and adequate pain control. The associated hypoxemia is due to the underlying pulmonary contusion and is unlikely to improve with a 10-mL/kg fluid bolus, administration of a diuretic, or simple observation. Surgical fixation of the flail segment 24 to 36 hours after injury is a relatively new technique that has shown initial promise in improving pulmonary function and cosmetic appearance, but it is not part of initial management. Thoracic injuries account for 5% to 8% of pediatric trauma cases, with mortality rates of 7% to 15%, second only to the mortality of head injuries. Blunt trauma to the chest can produce a variety of injuries, including cardiac tamponade, aortic arch dissection, rib fractures, pulmonary contusion, hemo- and pneumothorax, and diaphragmatic rupture. Patients in whom thoracic injuries are suspected due to the mechanism of injury (eg, motor vehicle or bicycle crashes, falls) or signs and symptoms (eg, respiratory distress, failure to respond to supplemental oxygen, visible wounds to the chest, decreased breath sounds, hyperresonance on chest percussion, distended neck veins) should undergo radiography of the chest and cervical spine. Focused assessment sonography for trauma (FAST) may be useful to rule out thoracic hemorrhage and cardiac tamponade. Computed tomography scan of the chest should be performed after initial stabilization.

References:

Dayan PS, Klein BL. Acute care of the multiple trauma victim. In Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, Pa: Saunders Elsevier; 2007:431-436

Herrera P, Langer JC. Thoracic trauma in children. In: Mikrogianakis A, Valani R, Cheng A, eds. The Hospital for Sick Children Manual of Pediatric Trauma. Philadelphia, Pa: Lippincott, Williams & Wilkins. 2008:131-144

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Question: 26

An anxious mother calls your office to tell you that her 5-year-old daughter has a "high blood sugar." The family had been visiting the child’s grandparents, and her maternal grandmother, who has type 2 diabetes mellitus, used her glucose meter to measure her granddaughter’s blood glucose about 90 minutes after the child had a piece of cake and a glass of apple juice. The meter reading was 253 mg/dL (14.0 mmol/L). The girl has been entirely asymptomatic, without nocturia, polyuria, or weight loss. Her mother has been trying to control her daughter’s weight lately, but the girl has a large appetite, and it was hard to control her eating at her grandparents’ house. There is no history of type 1 diabetes mellitus in the family.

Of the following, the MOST likely explanation for the child’s glucose reading is

A. a normal blood glucose value 90 minutes after a high-carbohydrate meal

B. maturity onset diabetes of the young

C. postprandial glucose intolerance

D. residual glucose on the fingers

E. type 2 diabetes

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Critique: 26

Preferred Response: D

Assessment of whole blood glucose values using glucose meters is a useful technique for management of diabetes, but methodologic errors occur. One of the most common is contamination of the fingers with glucose-containing food, which is the most likely explanation for the reading described for the child in the vignette. Foods such as juices and some commercial baked goods may contain appreciable amounts of glucose. Causes of falsely low glucose readings include cold temperature (outdoor winter use of a meter) and outdated test strips. Causes of falsely low or high glucose readings include failure to calibrate the strips, if this is required by the meter, by setting the appropriate code. It is also possible to manipulate blood glucose readings willfully by using diluted blood or testing using a standard control solution rather than blood. A healthy little girl should not have a blood glucose reading of 253 mg/dL (14.0 mmol/L) 90 minutes after eating. Random glucose concentrations greater than 200 mg/dL (11.1 mmol/L) on two separate occasions are diagnostic of diabetes. A child’s reading should not exceed the renal threshold for glucose (180 mg/dL [10.0 mmol/L]) after oral intake for more than a few minutes. Maturity onset diabetes of the young (MODY) is a rare autosomal dominant disorder resulting from mutations in at least six different genes, most of which are transcription factors that control the release of insulin from the beta cell. This group of disorders comprises fewer than 5% of all diagnoses of diabetes in childhood. Postprandial glucose intolerance could be a marker of diabetes or impaired glucose tolerance, but the prevalence of diabetes in 6-year-old children is less than 1 per 1,000. Type 2 diabetes, which results from insulin resistance and a diminished capacity for insulin release, typically is seen in adults. This disorder is increasingly recognized in obese children and adolescents, but it would be unusual in a normal weight 6-year-old child.

References:

American Diabetes Association. Diagnosis and classification of diabetes mellitus. Diabetes Care. 2008;31(suppl 1):S55-S60. Available at:

http://care.diabetesjournals.org/cgi/content/full/31/Supplement_1/S55

Craig ME, Hattersley A, Donahue K; International Society for Pediatric and Adolescent Diabetes. ISPAD clinical practice consensus guidelines 2006-2007: definition, epidemiology and classification. Pediatr Diabetes. 2006;7:343-351

Rewers M, Pihoker C, Donaghue K, Hanas R, Swift P, Klingensmith GJ; International Society for Pediatric and Adolescent Diabetes (ISBAD). Assessment and monitoring of glycemic control in children and adolescents with diabetes. Pediatr Diabetes. 2007;8:408-418

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Question: 27

A 13-year-old girl presents with the sudden loss of sight following the violent death of her mother. On physical examination, her pupils are round and equal and constrict briskly to light. When instructed to do so, she is unable to touch the examiner’s hand held in front of her. There are no other neurologic findings on examination. Results of head magnetic resonance imaging are normal, and a dilated ophthalmologic evaluation reveals no abnormalities.

Of the following, the MOST likely diagnosis is

A. body dysmorphic disorder

B. conversion disorder

C. hypochondriasis

D. malingering

E. somatic delusions

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Critique: 27

Preferred Response: B

The girl described in the vignette is displaying a conversion disorder, which simulates disease (mostly acute) and is monosymptomatic. Conversion disorder occurs more frequently in females and is most common in adolescents and young adults. Symptoms are not compatible with physiologic mechanisms or anatomy. Hypochondriasis is concern about a disease or preoccupation with illness. It often occurs in persons 20 to 30 years of age, equally in males and females, and in individuals who have had previous physical disease. Malingering is diagnosed when an individual presents with false or exaggerated physical or psychological symptoms. The individual has a motive either to avoid a situation/punishment/responsibility, to obtain compensation, or to retaliate. The presence of a defined goal distinguishes this from other factitious disorders. The person who has a strong belief or fear that he or she is unattractive or even repulsive despite having a normal or near-normal appearance is experiencing body dysmorphic disorder. Neither compliments nor reassurance alleviate the person’s fear. Somatic delusions encompass the belief that something is physically wrong with the individual. The delusion may involve a medical condition or illness or a perceived deformity, such as a belief that the person’s heart is melting. This condition differs from hypochondriasis, which involves excessive worries about health that stem from nonreality-based interpretations of specific physical signs as being not normal.

References:

Sadock BJ, Sadock VA. Additional conditions that may be a focus of clinical attention. In: Kaplan and Sadock’s Synopsis of Psychiatry: Behavioral Sciences/Clinical Psychiatry. 9th ed. Philadelphia, Pa: Lippincott Williams & Wilkins; 2003:894-900

Sadock BJ, Sadock VA. Somatoform disorders. In: Kaplan and Sadock’s Synopsis of Psychiatry:

Behavioral Sciences/Clinical Psychiatry. 9th ed. Philadelphia, Pa: Lippincott Williams & Wilkins;

2003:643-660

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Question: 28

A 12-year-old boy who has acute lymphoblastic leukemia (ALL) is undergoing reinduction chemotherapy and has an indwelling Broviac catheter. He has received multiple courses of antibiotics for episodes of fever and neutropenia. He recently completed a 6-week course of vancomycin for persistent coagulase-negative staphylococcal bacteremia. He is admitted to the

hospital with a temperature of 39.5°C and a white blood cell count of 0.2x103/mcL (0.2x109/L) (0% neutrophils). Blood culture grows gram-positive cocci that are resistant to vancomycin.

Of the following, the MOST likely pathogen on the blood culture is

A. group B Streptococcus

B. Klebsiella pneumoniae

C. Listeria monocytogenes

D. methicillin-resistant Staphylococcus aureus

E. vancomycin-resistant Enterococcus

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Critique: 28

Preferred Response: E

The boy described in the vignette is immunocompromised, has an indwelling catheter, and has undergone multiple antibiotic courses, including prolonged exposure to vancomycin. Accordingly, he has several risk factors for colonization and infection with vancomycin- resistant enterococci (VREC). Enterococci are normal inhabitants of the gastrointestinal tract, with E faecalis and E faecium accounting for most human infections. They are low-grade pathogens but have been associated with bacteremia in neonates. In older children, they may be isolated from intra- abdominal infections (generally in association with polymicrobial infection), device-associated infections, and urinary tract infections. In adults, they are also a major agent in infective endocarditis. Traditionally, enterococci have been susceptible to ampicillin and vancomycin. Gentamicin may offer synergistic benefit. Enterococci are resistant to all cephalosporins. Over the past 20 years, enterococcal strains resistant to vancomycin have emerged, with spread occurring primarily in the hospital setting. Previous antibiotic treatment, especially with vancomycin and cephalosporins, is the most common risk factor for nosocomial acquisition of VREC. Most of the vancomycin-resistant strains are E faecium. VREC strains also are resistant to ampicillin. Treatment of VREC infections may be difficult. Linezolid shows in vitro activity against the strains and is approved for such treatment in adults. Pediatric safety and efficacy of linezolid has been demonstrated. The Society for Healthcare Epidemiology of America and the Hospital Infection Control Practices Advisory Committee of the Centers for Disease Control and Prevention have published guidelines to prevent transmission of multidrug-resistant organisms, including VREC. Prevention of nosocomial transmission of VREC includes contact precautions (gowns and gloves), hand hygiene, and, if necessary, cohorting of VREC-colonized and -infected patients. Methicillin-resistant Staphylococcus aureus (MRSA) infections of central catheters can occur, and this boy’s recent antibiotic courses plus hospitalizations do increase the risk for acquiring MRSA, but vancomycin-resistant strains are extremely rare to date. Group B streptococcal infections are a concern for immunocompromised hosts, but the organism remains sensitive to vancomycin and is seen less commonly than MRSA or VREC in this setting. Although Klebsiella and Listeria infections occur in immunocompromised hosts and are resistant to vancomycin, they are not gram-positive cocci.

References:

American Academy of Pediatrics. Non-group A or B streptococcal and enterococcal infections. In: Pickering LK, Baker CJ, Kimberlin DW, Long SS, eds. Red Book: 2009 Report of the

Committee on Infectious Diseases. 28th ed. Elk Grove Village, Ill: American Academy of Pediatrics: 2009:634-636

Weinstein JW, Anderson DJ. Epidemiology and prevention and control of vancomycin-resistant enterococci. UpToDate Online 16.3. 2007. Available for subscription at:

http://www.utdol.com/online/content/topic.do?topicKey=hosp_inf/2484&selectedTitle=1~150&so

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

urce=search_result

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Question: 29

You are speaking to a group of medical students about antifungal agents, their potential uses, and the potential adverse effects associated with their use.

Of the following, the MOST common adverse effect of ketoconazole is

A. arrhythmias

B. headache

C. nausea

D. rash

E. seizures

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Critique: 29

Preferred Response: C

Ketoconazole is an imidazole antifungal agent that works by inhibiting the biosynthesis of ergosterol and other membrane lipids that compose the fungal cell membrane. Such inhibition results in fungal cellular membranes that lack the sterol components, causing increased permeability and progressive instability. Ketoconazole is effective in the treatment of chronic mucocutaneous candidiasis, coccidioidomycosis, histoplasmosis, blastomycosis, and paracoccidioidomycosis in nonimmunosuppressed hosts. It is not effective in the treatment of aspergillosis, cryptococcosis, or mucormycosis. Because other antifungals are effective against these pathogens and have fewer adverse effects, ketoconazole no longer is considered the drug of choice. The most frequent adverse effects of ketoconazole are anorexia, nausea, and vomiting, which occur in about 15% to 29% of patients. Allergic rash, headache, and pruritus are seen in 4% to 10% of patients. Mild elevations in liver transaminases occur in 2% to 5% of patients during the course of therapy. However, the most serious adverse effect associated with ketoconazole therapy is fulminant hepatitis. This occurs rarely, in 1 in 15,000 exposed individuals. Arrhythmias and seizures have not been associated with the use of this drug.

References:

Cross JT Jr, Hickerson SL, Yamauchi T. Antifungal drugs. Pediatr Rev. 1995;16:123-129. Abstract available at: http://pedsinreview.aappublications.org/cgi/content/abstract/16/4/123

Dismukes WE, Stamm AM, Graybill JR, et al. Treatment of systemic mycoses with ketoconazole:

emphasis on toxicity and clinical response in 52 patients. National Institute of Allergy and Infectious Diseases collaborative antifungal study. Ann Intern Med. 1983;98:13-20. Abstract available at: http://www.ncbi.nlm.nih.gov/pubmed/6293361

Heel RC, Brogden RN, Carmine A, Morley PA, Speight TM, Avery GS. Ketoconazole: a review of its therapeutic efficacy in superficial and systemic fungal infections. Drugs. 1982;23:1-36

National Institute of Allergy and Infectious Diseases Mycoses Study Group. Treatment of blastomycosis and histoplasmosis with ketoconazole. Results of a prospective, randomized clinical trial. Ann Intern Med. 1985;103:861-872. Abstract available at:

http://www.ncbi.nlm.nih.gov/pubmed/2865921

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Question: 30

A 14-year-old girl presents with a 2-month history of joint pain that is responding poorly to over- the-counter anti-inflammatory medications. She reports some sores in her mouth and mild swelling around her eyes and ankles. On physical examination, her temperature is 37.0°C, heart rate is 76 beats/min, respiratory rate is 14 breaths/min, and blood pressure is 130/86 mm Hg. She has oral ulcers, mild periorbital and pretibial edema, and mild swelling of her wrists and knee

joints. Laboratory findings include:

Sodium, 136 mEq/L (136 mmol/L)

Potassium, 4.8 mEq/L (4.8 mmol/L)

Chloride, 100 mEq/L (100 mmol/L)

Bicarbonate, 22 mEq/L (22 mmol/L)

Blood urea nitrogen, 24.0 mg/dL (8.6 mmol/L)

Creatinine, 1.3 mg/dL (114.9 mcmol/L)

Albumin, 2.5 g/dL (25.0 g/L)

Hemoglobin, 10.1 g/dL (101.0 g/L)

White blood cell count, 3.0x103/mcL (3.0x109/L)

Platelet count, 190x103/mcL (190x109/L)

Urinalysis: 3+ blood, 3+ protein, with 20 to 50 red blood cells/high-power field

Antinuclear antibody titer: 1:1,280

Anti-double-stranded DNA titer: 1:640

Of the following, the next BEST step in management is to

A. admit the patient for intravenous cyclophosphamide treatment

B. initiate treatment with ibuprofen

C. order a 24-hour urine for protein collection

D. refer the patient for a renal biopsy

E. refer the patient for bone marrow aspiration

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Critique: 30

Preferred Response: D

The adolescent girl in the vignette meets the diagnostic criteria for systemic lupus erythematosus (SLE). Her renal involvement necessitates an aggressive approach to diagnosis and treatment, but the severity of renal involvement must be determined before aggressive treatment is initiated. Renal disease in patients who have SLE usually manifests as an immune complex-mediated glomerulonephritis (GN), often associated with hypocomplementemia and positive serologic testing for antinuclear antibody (ANA) and anti-double-stranded (ds) DNA. A recent observational study from Toronto demonstrated that 37% of children have nephritis at diagnosis, 46% within 1 year of diagnosis, and 55% in long-term follow-up. The clinical manifestations of lupus nephritis are those typically seen with GN and may include one or more of the following:

hematuria, proteinuria, azotemia, hypertension, and edema. Lupus nephritis is categorized further by histologic criteria into the World Health Organization classification system: class I (normal), class II (mesangial proliferative GN), class III (focal proliferative GN), class IV (diffuse proliferative GN), and class V (membranous GN). Because of the need to classify the form of nephritis prior to the institution of corticosteroids, the standard of care is to obtain a renal biopsy prior to treatment. Some forms of lupus nephritis, including diffuse proliferative nephritis, are treated with cyclophosphamide as an adjunctive agent, but this medication should not be used for renal indications without a kidney biopsy. Results of the renal biopsy can provide both prognostic and treatment information. Once the renal disease is classified histologically and initial treatment is instituted, the patient can be monitored by periodic assessment of urinary protein excretion as well as measurement of serologic markers such as complement components and anti-ds DNA titers. Patients who exhibit worsening proteinuria, decreasing concentrations of complement components, or rising anti-ds DNA titers require assessment for a disease flare, which may necessitate increasing immunosuppressive therapy (including corticosteroids). Because nonsteroidal anti-inflammatory drugs such as ibuprofen have potential nephrotoxicity, they usually are not administered to children who have lupus nephritis. The 24- hour urine collection typically is not used for quantitating urine protein excretion, which can be assessed accurately with a spot urine protein and creatinine measurement. There is no indication for a bone marrow aspiration in this patient.

References:

Adams A, MacDermott EJ, Lehman TJ. Pharmacotherapy of lupus nephritis in children: a recommended treatment approach. Drugs. 2006;66:1191-1207. Abstract available at:

http://www.ncbi.nlm.nih.gov/pubmed/16827597

Hiraki LT, Benseler SM, Tyrrell PN, Hebert D, Harvey E, Silverman ED. Clinical and laboratory characteristics and long-term outcome of pediatric systemic lupus erythematosus: a longitudinal study. J Pediatr. 2008;152:550-556. Abstract available at:

http://www.ncbi.nlm.nih.gov/pubmed/18346514

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Question: 31

The mother of a 14-year-old girl who has asthma is concerned that her daughter’s recent severe exacerbation is due to mold exposure. Their home sustained flood damage last year. The mother provides you with a list of diagnostic tests she found on the Internet, which she believes will help confirm her daughter’s mold allergy.

Of the following, the MOST appropriate testing to evaluate the girl for possible mold allergy is

A. allergy skin testing

B. applied kinesiology

C. cytotoxic testing

D. provocation-neutralization

E. pulse test

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Critique: 31

Preferred Response: A

Clinicians should be aware that many unconventional and unproven testing methods are still promoted for the diagnosis and management of allergic conditions. For example, applied kinesiology theorizes that an allergen or irritant substance provokes weakness when the person ingests, holds, or even stands in close proximity to the offending agent. To date, no conclusive evidence supports this theory or test. The cytotoxic test is an in vitro test involving microscopic evaluation of blood after placing a drop of the offending agent (eg, food, mold) on a slide containing the patient’s blood. Changes in the appearance, size, shape, and integrity of leukocytes are interpreted as a "positive" response. A thorough United States Food and Drug Administration review has concluded that the efficacy of the cytotoxic test is unproven. The pulse test evaluates whether an increase or decrease in pulse rate follows food ingestion, injection, or sublingual application and is interpreted as an "allergy" if a change occurs. Finally, provocation-neutralization is a nonstandardized test for food and inhalant allergies. A series of dilutions of an allergen are injected in the upper arm until the patient reports subjective sensations. A progressive series of lower concentrations subsequently are administered until the sensation abates. The lower dose is used for injection treatment to build immunologic tolerance for the offending trigger. Both the testing and treatment method are unproven and should be considered similar to placebo therapy. Finally, Candida immunotherapy or avoidance of yeast ingestion for patients diagnosed with yeast or Candida hypersensitivity is unproven. The parent in the vignette is concerned for mold allergy. Molds generally are believed to have three effects: direct infection through ingestion or inhalation, release of toxins or irritants as mold byproducts, and an immune response (eg, allergic bronchopulmonary aspergillosis, allergic fungal sinusitis, allergic rhinitis, asthma). Significant water damage, leaking water sources, or visible mold growth represent real health concerns. Commercial testing for mold can provide some information, but quantifiable amounts are found in 80% of homes in the United States. Thus, the presence of mold does not necessarily indicate causation. For example, Alternaria alternata is linked to fatal asthma but is primarily an outdoor aeroallergen. Allergy skin prick testing or blood testing can aid in detecting immunoglobulin E-mediated responses to molds and other allergens and is reasonable as part of determining the cause of this adolescent’s asthma exacerbations.

As a result of reviewing this information, do you intend to make a change in practice to provide better patient care?

Yes

No

References:

Bush RK, Portnoy JM, Saxon A, Terr AI, Wood RA. The medical effects of mold exposure. J Allergy Clin Immunol. 2006;117:326-333. Abstract available at:

http://www.ncbi.nlm.nih.gov/pubmed/16514772

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Bush RK, Portnoy JM. The role and abatement of fungal allergens in allergic diseases. J Allergy Clin Immunol. 2001;107(3 suppl):S430-S440. Abstract available at:

http://www.ncbi.nlm.nih.gov/pubmed/11242604

Matsui EC, Eggleson PA. Immunotherapy for allergic disease. In: Leung DYM, Sampson HA, Geha RS, Szefler SJ, eds. Pediatric Allergy Principles and Practice. St. Louis, Mo: Mosby Elsevier; 2003:277-285

Nelson HS. Immunotherapy for inhalant allergens. In: Adkinson NF Jr, Yunginger JW, Busse WW, Bochner BS, Holgate ST, Simons FER, eds. Middleton’s Allergy Principles and Practice. 6th ed. Philadelphia, Pa: Mosby Elsevier; 2003:1455-1473

O’Connor GT. Allergen avoidance in asthma: what do we do now? J Allergy Clin Immunol. 2005;116:26-30. Abstract available at: http://www.ncbi.nlm.nih.gov/pubmed/15990768

Terr AI. Unconventional theories and unproved methods in allergy. In: Adkinson NF Jr, Yunginger JW, Busse WW, Bochner BS, Holgate ST, Simons FER, eds. Middleton’s Allergy Principles and Practice. 6th ed. Philadelphia, Pa: Mosby Elsevier; 2003:1711-1728

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Question: 32

The parents of a 12-year-old boy bring him to the emergency department after finding him unresponsive in bed when they tried to wake him for school. They report that he has had no recent illnesses and was in his usual state of health when he went to bed last night. Of note, he has enuresis, treated with imipramine. On physical examination, he is responsive only to pain, his heart rate is 120 beats/min, respiratory rate is 6 breaths/min, and blood pressure is 60/40 mm Hg. His pupils are 6 mm, equal, and sluggishly reactive. All other findings are within normal parameters. He is endotracheally intubated, ventilated with 100% oxygen, placed on a cardiac monitor, and given a 20-mL/kg bolus of normal saline. Electrocardiography demonstrates sinus tachycardia, PR interval of 130 msec, and QRS duration of 140 msec.

Of the following, the next MOST appropriate step is to

A. administer adenosine

B. administer amiodarone

C. administer sodium bicarbonate

D. begin external pacing

E. perform synchronized cardioversion at 0.5 J/kg

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Critique: 32

Preferred Response: C

The coma, respiratory depression, tachycardia, hypotension, and dilated pupils described for the patient in the vignette are consistent with an acute tricyclic antidepressant (TCA) overdose. Among the additional signs that might be observed are seizures, dysrhythmias, and other anticholinergic features such as dry mouth, hyperthermia, urinary retention, flushed skin, and agitation. Tricyclic antidepressants cause these clinical features by inhibiting a variety of neurotransmitter receptors, including muscarinic acetylcholine, alpha-1-adrenergic, gamma aminobutyric acid (GABA), and histamine receptors as well as cardiac fast sodium channels. The most serious toxicities from TCA overdose affect the heart and central nervous system (CNS). Because of the TCA effect on fast sodium channels, conduction velocity is decreased. In addition, repolarization duration and absolute refractory periods are prolonged. These effects, coupled with alpha-1-adrenergic antagonism, are responsible for the hypotension and conduction delays that are seen commonly. CNS excitation and seizures or depression may be related to effects on GABA or histamine receptors. Initial management of a TCA overdose begins with ensuring a patent airway and restoring adequate oxygenation, ventilation, and perfusion. Intubation and mechanical ventilation often are necessary, as is fluid resuscitation with boluses of normal saline. Seizures are treated with benzodiazepines. Alpha-adrenergic pressors (eg, norepinephrine) may be required to treat refractory hypotension. Decontamination should be performed with activated charcoal. Although acetaminophen and aspirin concentrations should be measured, especially in intentional ingestions to evaluate for possible coingestants, measurement of TCA concentrations is not clinically useful. The single most useful diagnostic and prognostic test in the setting of a TCA overdose is electrocardiography. In a study from 1985, toxicologists found that a QRS duration of greater than 100 msec predicted seizures in 34% and dysrhythmias in 14% of patients who had TCA overdoses. The QRS widening is related to fast sodium channel blockade caused by direct TCA effects and exacerbated by acidemia. These effects can be overcome by the administration of sodium bicarbonate boluses. Sodium bicarbonate should be administered until the QRS duration is less than 100 msec. The exact mechanism for this effect is unknown. Adenosine and synchronized cardioversion are treatments for supraventricular tachycardia. Amiodarone is a drug of choice for ventricular arrhythmias. External pacing is appropriate treatment for refractory, symptomatic bradycardia.

References:

Boehnert MT, Lovejoy FH Jr. Value of QRS duration versus the serum drug level in predicting seizures and ventricular arrhythmias after an acute overdose of tricyclic antidepressants. N Engl J Med. 1985;313:474-479. Abstract available at:

http://www.ncbi.nlm.nih.gov/pubmed/4022081

Hutchinson MD, Traub SJ. Tricyclic antidepressant poisoning. UpToDate Online 16.3. 2008. Available at:

http://www.utdol.com/online/content/topic.do?topicKey=ad_tox/10025&selectedTitle=1~150&sou

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

rce=search_result630

Jacob J. Toxicity, antidepressant. In: eMedicine Specialties, Emergency medicine, Toxicology. 2008. Available at: http://www.emedicine.com/emerg/topic37.htm

Woolf AD, Erdman AR, Nelson LS, et al. Tricyclic antidepressant poisoning: an evidence-based consensus guideline for out-of-hospital management. Clin Toxicol (Phila). 2007;45:203-233. Brief summary available at:

http://guidelines.gov/summary/summary.aspx?doc_id=9906&nbr=005302&string=tricyclic+AND+

antidepressant+AND+overdose

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Question: 33

You are evaluating a 2-year-old daughter of strict vegan parents. Her birthweight at term was 3.5 kg. Since weaning at 12 months of age, the child’s diet has included a homemade, macrobiotic-based formula. In your office today, the girl’s weight is 11.2 kg.

Of the following, the child’s diet MOST likely is deficient in

A. essential amino acids

B. linoleic acid

C. vitamin A

D. vitamin B12

E. vitamin C

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Critique: 33

Preferred Response: D

Strict vegan diets include foods that come solely from plant sources. Such diets generally contain adequate amounts of vitamins A and C as well as essential fatty acids (including linoleic

acid). However, a strict vegan diet instituted after weaning contains very little vitamin B12, a nutrient primarily found in meats, eggs, and dairy products, unless supplements are provided.

Breastfed infants of vegan mothers may develop vitamin B12 deficiency, but only if maternal stores are low. Although the vitamin composition of human milk is directly related to dietary intake of vitamins A, C, D, and the B group, studies comparing the vitamin content of human milk from vegan compared with nonvegan mothers have not demonstrated any significant micronutrient differences. Commercial soy-based formulas are alternatives for vegan mothers who do not breastfeed. In most cases, therefore, the greatest potential nutritional risks for both breastfed and soy formula-fed infants of vegan parents occur after weaning. This is particularly the case when a homemade weaning formula is given. Conversely, commercially available soy milks are supplemented with vitamins. Studies in both the United States and the United Kingdom have shown that vegan children exhibit small but significant differences in growth variables (height and weight percentiles) compared with children eating mixed diets. This observation most likely is the consequence of group differences in total energy consumption, although other studies have demonstrated that the calcium and zinc content of the vegan diet also may be low, indicating the requirement for supplementation. Conversely, the essential amino acid and total protein intake of vegan children has been shown to be adequate to support normal growth. Despite the lower mean height and weight of vegan children compared with children eating mixed diets, a large British study found no evidence of growth failure (weight or height less than the 5th percentile) in vegan children, and no between-group differences were noted in terms of muscle strength and overall health. A routine health assessment of any child should include a careful dietary history. Information about specific cultural or family customs permits identification of patients at nutritional risk and aids the clinician in determining whether caregivers require education regarding appropriate nutrition for growing children. For example, in industrialized countries, vitamin D deficiency rickets is an emerging nutritional problem. Such deficiency is particularly prevalent for dark- skinned infants living in temperate or northern climates, those whose cultural/religious customs may include extensive covering of body surfaces, and in infants and children who receive little direct sunlight exposure (ie, less than 30 minutes to the face and hands three times per week).

References:

Graham EA. Economic, racial and cultural influences on the growth and maturation of children. Pediatr Rev. 2005;26:290-294. Available at:

http://pedsinreview.aappublications.org/cgi/content/full/26/8/290

Hebbelinck M, Clarys P, De Malsche A. Growth, development, and physical fitness of Flemish vegetarian children, adolescents, and young adults. Am J Clin Nutr. 1999;70(3 suppl):579S- 585S. Available at: http://www.ajcn.org/cgi/content/full/70/3/579S

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Joiner TA, Foster C, Shope T. The many faces of vitamin D deficiency rickets. Pediatr Rev. 2000;21:296-302. Available at: http://pedsinreview.aappublications.org/cgi/content/full/21/9/296

Kramer MS, Guo T, Platt RW, et al. Breastfeeding and infant growth: biology or bias? Pediatrics. 2002;110:343-347. Available at: http://pediatrics.aappublications.org/cgi/content/full/110/2/343

Moilanen BC. In brief: vegan diets in infants, children, and adolescents. Pediatr Rev. 2004;25:174- 176. Available at: http://pedsinreview.aappublications.org/cgi/content/full/25/5/174

Sanders TA. Vegetarian diets and children. Pediatr Clin North Am. 1995;42:955-965. Abstract available at: http://www.ncbi.nlm.nih.gov/pubmed/7610022

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Question: 34

You are called to the newborn nursery to examine a 4-hour-old term infant delivered to a mother who had polyhydramnios. The infant’s Apgar scores were 7 and 8 at 1 and 5 minutes, respectively. The nurse reports that the infant requires frequent oropharyngeal suctioning and displays cyanosis when suctioned. She placed a pulse oximeter on the infant and reports frequent episodes of desaturation from 94% to 70% on room air during the cyanotic episodes. Physical examination reveals an appropriately grown infant who has substantial oral secretions, scattered rales on auscultation, normal S1 and S2 heart sounds, no heart murmur, normal bowel sounds, and no abdominal distention.

Of the following, the MOST important next step is to

A. insert a feeding tube

B. insert an umbilical arterial catheter

C. measure bedside blood glucose concentration

D. order emergent echocardiography

E. order renal ultrasonography

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Critique: 34

Preferred Response: A

The newborn described in the vignette displays the classic clinical signs of a tracheoesophageal fistula (TEF) with accompanying esophageal atresia. Attempting to insert an orogastric (OG) feeding tube is the most important next step in evaluating a newborn in whom this diagnosis is suspected. The inability to insert the OG tube confirms esophageal atresia, which can be verified by seeing the tube coiled in the proximal esophageal pouch on chest radiography (Item C34). In the presence of esophageal atresia, TEF is confirmed by radiographic examination of the abdomen revealing air in the lower gastrointestinal tract, which could only reach that locale via a fistula between the upper airway and the esophagus distal to any atretic portion. The intermittent cyanosis exhibited by the newborn may be due to pooling of oral secretions in the hypopharynx, airway obstruction, aspiration of oral secretions through the larynx into the trachea (because the infant cannot swallow these secretions), pneumonitis, or hypoxia resulting from the reflux of gastric contents via the TEF into the tracheobronchial tree. The maternal history of polyhydramnios is a clue to likely swallowing dysfunction or gastrointestinal tract obstruction. The physical examination and judicious use of diagnostic imaging tools such as plain films and ultrasonography to assess for the presence of vertebral anomalies, anorectal stenosis or atresia, structural heart disease, renal anomalies, and limb anomalies is important in determining if the esophageal atresia or TEF are isolated defects or part of the VACTERL association, which occurs in one third of infants who have esophageal atresia. TEF and esophageal atresia is a surgical emergency that requires early evaluation for surgical ligation of the TEF to protect the airway. Until surgery is performed, vigilant oropharyngeal suctioning is required, and the newborn’s head should be kept elevated. Some newborns may require tracheal intubation and assisted ventilation. Early insertion of a gastrostomy tube for gastrointestinal decompression and subsequent feeding until such time as the esophagus can be used also is common. Surgical anastomosis of the proximal and distal esophagus may be accomplished as a later procedure. Measuring bedside glucose concentration is important in newborns who have respiratory distress but should follow evaluation of airway obstruction in the newborn in the vignette. Echocardiography can help in the evaluation of the newborn for structural heart disease, but the documented normal room air saturation of 94% indicates no fixed cardiac shunt or cyanotic lesion, making this test less imperative at this time. Renal ultrasonography is indicated if VACTERL association is suspected but requires an initial diagnosis of TEF. An umbilical arterial catheter may or may not be indicated, depending on the degree of respiratory distress.

References:

Goyal A, Jones MO, Couriel JM, Losty PD. Oesophageal atresia and tracheo-oesophageal fistula. Arch Dis Child Fetal Neonatal Ed. 2006;91:F381-F384. Abstract available at:

http://www.ncbi.nlm.nih.gov/pubmed/16923940

Keckler SJ, St Peter SD, Valusek PA, et al. VACTERL anomalies in patients with esophageal atresia: an updated delineation of the spectrum and review of the literature. Pediatr Surg Int.

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

2007;23:309-313. Abstract available at: http://www.ncbi.nlm.nih.gov/pubmed/17377826

Kulayalat NA, Narchi H. Index of suspicion: case 5. Pediatr. Rev. 2000;21:20-28. Available at:

http://pedsinreview.aappublications.org/cgi/content/full/21/1/20

Magnuson D, Parry RL, Chwals WJ. Selected thoracic gastrointestinal anomalies. In: Martin RJ, Fanaroff AA, Walsh MC, eds. Fanaroff and Martin's Neonatal-Perinatal Medicine: Diseases of the Fetus and Infant. 8th ed. Philadelphia, Pa: Mosby Elsevier; 2006:1373-1380

Nakayama DK. Esophageal atresia and tracheoesophageal fistula. In: Nakayama DK, Bose CL, Chescheir NC, Valley R, eds. Critical Care of the Surgical Newborn. Armonk, NY: Futura Publishing Company, Inc; 1997:227-249

Thilo EH, Rosenberg AA, The newborn infant. In: Hay WW Jr, Levin MJ, Sondheimer JM, Deterding RR, eds. CURRENT Diagnosis & Treatment: Pediatrics. 19th ed. New York, NY: The McGraw-Hill Companies; 2009:Chapter 1. Available for subscription at:

http://www.accessmedicine.com/content.aspx?aID=3396500

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Question: 35

You are working in the newborn nursery when the nurse asks you to evaluate a girl who has just been admitted. According to her records, her mother had good prenatal care and results of prenatal laboratory evaluations were normal. The infant is vigorous and pink. Findings on physical examination are normal except for a reddish-purple patch over her right forehead, eyelid and cheek (Item Q35).

Of the following, the condition that is MOST likely to be associated with this skin lesion is

A. Kasabach-Merritt syndrome

B. neurofibromatosis type 1

C. Osler-Weber-Rendu disease

D. Sturge-Weber syndrome

E. tuberous sclerosis complex

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2010 PREP SA on CD-ROM

Critique: 35

Preferred Response: D

Vascular lesions in the newborn may be characterized as benign vascular neoplasms such as hemangiomas or as vascular malformations such as salmon patches (also called a nevus simplex) or port wine stains (PWSs). PWSs occur commonly and usually are not associated with underlying disorders, but approximately 5% to 10% of infants who have PWSs in the distribution of the first branch of the trigeminal nerve (V1), as described for the infant in the vignette, have Sturge-Weber syndrome (SWS). SWS is associated with seizures and meningeal and cerebral cortex abnormalities ipsilateral to the PWS, and the presence of a PWS in the V1 region is included in the diagnostic criteria for the syndrome. Eye involvement, most commonly glaucoma, also is a feature of SWS occurring in 60% of patients. Large atypical-appearing hemangiomas (actually hemangioendotheliomas or tufted angiomas) and thrombocytopenia are seen with Kasabach-Merritt syndrome (Item C35A). The primary cutaneous manifestations of neurofibromatosis type 1 are café au lait macules, which may be present at birth, and neurofibromas (Item C35B), which typically appear during childhood or early adolescence. Osler-Weber-Rendu disease (hereditary hemorrhagic telangiectasia) is characterized by recurrent epistaxis and cutaneous telangiectases that develop later in life. Tuberous sclerosis complex is a neurocutaneous syndrome; the most common skin lesions are hypopigmented macules (ash leaf macules), angiofibromas (adenoma sebaceum) (Item C35C), and shagreen patches. Other skin lesions include periungual fibromas and facial plaques. PWSs can be treated effectively with pulsed dye laser. Laser treatment may result in complete resolution or significant lightening of the lesion, and the degree of effectiveness is

related to the location of the lesion (Item C35D). Results of studies are conflicting, but lesions in

the periorbital area and central forehead generally respond better than do lesions in the midface

or on the limbs. Other factors associated with improved results include lesion size less than 20

cm and age at onset of therapy. Risks of treatment are small and include atrophy, hypertrophy,

and hyperpigmentation of the treated area. PWSs recur in many patients after treatment.

References:

Jasim ZF, Handley JM. Treatment of pulsed dye laser-resistant port wine stain birthmarks. J Am Acad Dermatol. 2007;57:677-682. Abstract available at:

http://www.ncbi.nlm.nih.gov/pubmed/17658196

Pielop JA. Vascular lesions in the newborn. UpToDate Online 16.3. 2008. Available for subscription at:

http://www.utdol.com/online/content/topic.do?topicKey=ped_derm/4439&selectedTitle=9~150&s

ource=search_result630

Stier MF, Glick SA, Hirsch RJ. Laser treatment of pediatric vascular lesions: port wine stains and hemangiomas. J Am Acad Dermatol. 2008;58:261-285. Abstract available at:

http://www.ncbi.nlm.nih.gov/pubmed/18068263

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Question: 36

You are the physician at a summer camp for special needs children. An adolescent who is a junior counselor comes to the infirmary after being stung by a bee. Physical examination reveals swelling, pain, erythema, and tenderness in a well-circumscribed area on the right upper arm. On close examination, you see a small foreign body in a punctum.

Of the following, the MOST appropriate means of removing the stinger is to

A. apply duct tape and rapidly pull it off

B. pass a needle subcutaneously alongside the stinger

C. probe for the stinger with tweezers

D. scrape the stinger from the skin gently with a tongue blade

E. wait for the stinger to extrude by itself

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2010 PREP SA on CD-ROM

Critique: 36

Preferred Response: D

The honeybee is unique among hymenoptera species in that it may leave a stinger embedded in skin; wasps, hornets, and yellow jackets do not. The stinger should be removed to avoid continued envenomation from the accompanying venom sac. Simple scraping away of the stinger can avoid rupture of the venom sac. In a medical setting (such as a camp infirmary), a tongue blade may be available, but in other settings, the blunt handle of a spoon or other such instrument may be used. The area should be cleansed with soap and water after removal of the stinger. Because allergic reactions are more likely to occur when the stinger is not removed promptly, tweezers may be used if nothing is available to scrape out the stinger. However, use of tweezers in a pincer motion may release more venom. Unnecessary probing using blunt or sharp instruments should be avoided if the stinger cannot be extracted readily. In recent years, a variety of methods, including home remedies using

common household product adhesives such as duct tape and Superglue®, have become popular for removal of transdermal foreign bodies and superficial lesions such as cactus spines and common warts. There is no evidence to suggest their efficacy in removing bee stingers. Waiting for the stinger to extrude increases the risk for allergic reaction.

References:

Booker GM, Adam HM. In brief: insect stings. Pediatr Rev. 2005;26:388-389. Available at:

http://pedsinreview.aappublications.org/cgi/content/full/26/10/388

Mendez E, Sicklick MJ. In brief: hymenoptera reactions. Pediatr Rev. 1995;16:355-356. Abstract available at: http://pedsinreview.aappublications.org/cgi/content/abstract/16/9/355

Vankawala HH, Park R. Bee and hymenoptera stings. eMedicine Specialties, Emergency Medicine, Environmental. 2008. Available at: http://emedicine.medscape.com/article/768764- overview

Visscher PK, Vetter RS, Camazine S. Removing bee stings. Lancet. 1996;348:301—302. Abstract available at:

http://www.ncbi.nlm.nih.gov/pubmed/8709689?ordinalpos=6&itool=EntrezSystem2.PEntrez.Pubme

d.Pubmed_ResultsPanel.Pubmed_DefaultReportPanel.Pubmed_RVDocSum

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Question: 37

You are treating a 4-month-old infant who was born with tetralogy of Fallot. Her mother brings her to the clinic because she has had diarrhea and fever since the previous evening. On physical examination, the infant is irritable and has cyanosis and a heart rate of 180 beats/min.

Of the following, the finding that is MOST consistent with a tetralogy spell is

A. clubbing of the digits

B. hepatomegaly

C. inability to hear a murmur

D. oxygen saturation of 75% in room air

E. S3 gallop rhythm

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Critique: 37

Preferred Response: C

Tetralogy of Fallot (TOF) is the most common form of cyanotic congenital heart disease, with an incidence of approximately 0.2 in 1,000 live births and accounting for 9% of all congenital heart disease. The four components of TOF are right ventricular outflow/pulmonary stenosis, ventricular septal defect (VSD), overriding aorta, and right ventricular hypertrophy (Item C37). The primary lesion is underdevelopment of the pulmonary infundibulum, which has led some to refer to this disease as "monology of Fallot" because all aspects of the tetrad result from this lesion. The result of underdevelopment of the pulmonary infundibulum is deviation of the infundibular septum anteriorly and superiorly, bringing it into the right ventricular outflow tract. This leads to obstructed right ventricular outflow and the commonly seen underdevelopment of the pulmonary valve and pulmonary arteries caused by diminished blood flow through these structures. The underdeveloped pulmonary infundibulum also creates a VSD, which is almost universally large and of the malalignment type. The defect resulting from anterior malalignment of the infundibulum allows the aorta to "override" the ventricular septum. Finally, right ventricular hypertrophy results from exposure to systemic pressures (large VSD and pulmonary stenosis). Most patients who have TOF do not present with cyanosis in the newborn period, but rather come to medical attention because of a harsh systolic murmur. The murmur results from infundibular stenosis and pulmonary stenosis, not from the VSD. The second heart sound is single. Because the degree of pulmonary blood flow obstruction can vary among patients, the degree of systemic oxygen desaturation ranges from mild to severe. Children who have mild obstruction may appear "pink," and those who have severe pulmonary stenosis have significantly reduced pulmonary blood flow and an increase in right-to-left shunting across the VSD into the aorta, leading to more pronounced cyanosis. Furthermore, as pulmonary blood flow decreases with tight pulmonary stenosis, pulmonary venous return to the left atrium decreases, resulting in less highly saturated blood leaving the left ventricle and entering the aorta. Conversely, mild pulmonary stenosis is associated with more pulmonary blood flow, less right-to- left intracardiac shunting, and less systemic desaturation. In the mildest cases, there is left-to- right shunting across the VSD and near-normal or normal systemic saturation. A decreased or absent murmur signifies diminished pulmonary blood flow, as occurs in the cyanotic spell or tetralogy spell. Such spells are marked by distress, crying, inconsolability, hyperpnea, and increasing cyanosis, as described for the infant in the vignette. They frequently occur in the morning or at times of dehydration (eg, fever, gastroenteritis). If not treated quickly, cyanotic spells can lead to serious morbidity and even death. Treatment of cyanotic spells centers on increasing pulmonary blood flow, which is accomplished by several means. The first step is to alter the ratio of relative resistance of pulmonary and systemic beds. Increasing the systemic vascular resistance relative to the pulmonary vascular resistance decreases the right-to-left shunt at the VSD and can be accomplished by placing the patient in a knee-to-chest position or by squatting in older children. Pharmacologic augmentation of the systemic vascular resistance can be achieved with intravenous phenylephrine. Therapy also includes the use of sedation with morphine, which suppresses the sensation of suffocation and can relieve the patient’s fear. The use of high-flow oxygen, which dilates pulmonary vasculature, constricts systemic vasculature and increases

Po2 of pulmonary venous return, and generous intravascular fluid administration to increase

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

preload are important therapies for the patient experiencing a tetralogy spell. Clubbing of the digits can be seen in cyanotic heart disease as well as a variety of other entities, but it is not typical in patients younger than 1 year of age and its presence is not associated with a tetralogy spell. Hepatomegaly is uncommon in the infant who has TOF; its presence suggests right heart failure. Diminished oxygen saturation is a component of a tetralogy spell, although the physical findings and condition of the patient, not the oxygen saturation, define the spell. Finally, an S3 gallop rhythm can be heard in the patient who has myocardial failure but is not expected in a patient who has TOF, particularly with the pronounced tachycardia described for the patient in the vignette.

References:

Doyle TP, Kavanaugh-McHugh A, Graham TP. Tetralogy of Fallot and pulmonary atresia with ventricular septal defect. In: Moller JH, Hoffman JIE, eds. Pediatric Cardiovascular Medicine. Philadelphia, Pa: Churchill Livingstone; 2000:391-408

Neches WH, Park SC, Ettedgui JA. Tetralogy of Fallot and tetralogy of Fallot with pulmonary atresia. In: Garson A Jr, Bricker JT, Fisher DJ, Neish SR, eds. The Science and Practice of Pediatric Cardiology. 2nd ed. Baltimore, Md: Williams & Wilkins; 1998:1383-1411

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Question: 38

During the health supervision visit for a healthy 4-month-old boy, you note that his head circumference is 46 cm (>98th percentile) and his length and weight are at the 50th percentile. He has mild frontal bossing and widely split cranial sutures. The fontanelle is flat. Arm and leg movements, tone, and reflexes are normal. In reviewing prior growth parameters, you note that his head circumference was at the 75th percentile at birth and the 90th percentile at 2 months.

Of the following, the MOST helpful next diagnostic procedure is

A. electroencephalography

B. head ultrasonography

C. lumbar puncture with manometry

D. plain radiography of the skull

E. three-dimensional head computed tomography scan

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Critique: 38

Preferred Response: B

The boy described in the vignette is healthy, with no abnormalities of neurodevelopment, and his neurologic examination reveals no changes in mental status or evidence of focal brain abnormalities or increased intracranial pressure. However, assuming measurements are accurate, he has steadily crossed head circumference percentiles since birth. Neuroimaging is important to determine the reason for his crossing of percentiles. The differential diagnosis includes hydrocephalus, arachnoid cyst, parenchymal brain lesions, subdural hematomas, and neurodegenerative diseases. Although the most helpful high-resolution image in this case is brain magnetic resonance imaging, sedation usually is required for such imaging in a child of this age. In contrast, head ultrasonography through the fontanelle can be performed quickly and with less risk and cost because sedation is not needed. The results can be used in planning referral to neurosurgery or neurology. Some conditions, such as an arachnoid cyst, may not require urgent intervention, and observation suffices, with magnetic resonance imaging scheduled for a later date. Because the child does not have seizures, electroencephalography is not indicated. Lumbar puncture to rule out elevated intracranial pressure is not needed because the sutures are open. Therefore, in a chronic process, pressure will not rise to a level that is dangerous or requires measurement. Studies of bone are not helpful. Radiography of the skull will not provide information that affects management. Three-dimensional computed tomography scan is helpful for assessing craniosynostosis if the child’s head shape is abnormal and fused ridges at the cranial sutures are palpable.

References:

Haslam RHA. Neurologic evaluation. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, Pa: Saunders Elsevier; 2007:2433-2442

Koenigsberg RA, Bianco BA, Faro SH, et al. Neuroimaging. In: Goetz C, ed. Textbook of Clinical Neurology. 3rd ed. Philadelphia, Pa: Saunders Elsevier; 2007:427-466

Piatt JH Jr. Recognizing neurosurgical conditions in the pediatrician's office. Pediatr Clin North Am. 2004;51:237-270. Abstract available at: http://www.ncbi.nlm.nih.gov/pubmed/15062671

Stoll BJ. The newborn. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. Philadelphia, Pa: Saunders Elsevier; 2007: 675-682

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Question: 39

You are called to examine a newborn girl who has multiple congenital anomalies. On physical examination, you notice several "punched-out" scalp ulcers (Item Q39A), bilateral cleft lip (Item Q39B) and palate, postaxial polydactyly (extra digit on the ring finger side) of the hands (Item Q39C), and a small omphalocele.

Of the following, this infant’s karyotype MOST likely is

A. 45,X

B. 45,X/47,XXX

C. 47,XX+13

D. 47,XX+18

E. 47,XX+21

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Critique: 39

Preferred Response: C

The newborn described in the vignette has a 47,XX+13 karyotype consistent with trisomy 13. Her unusual features of "punched-out" scalp lesions (also known as "aplasia cutis"), bilateral cleft lip and palate, and polydactyly are present in at least 50% of newborns who have this diagnosis. Other common anomalies in affected individuals include holoprosencephaly (incomplete septation of the frontal lobes), microcephaly, and cardiac defects (80%). Trisomy 13 has a poor prognosis, with approximately 50% of affected individuals dying by 2 weeks of age and 90% dying by 1 year. Approximately 50% of individuals who have Turner syndrome have a 45,X karyotype; the remainder have a mosaic karyotype that includes a cell line consistent with Turner syndrome, such as 45,X/47,XXX, or a karyotype with 46 chromosomes wherein one of the X chromosomes is aberrant (eg, ring X, isochromosome Xq). Affected newborns may exhibit dysmorphisms or may appear completely normal. Unusual features include webbed neck with low posterior hairline, broad chest with widely spaced nipples, narrow and hyperconvex nails, and cardiac defects, most commonly bicuspid aortic valve. Individuals who have trisomy 18 usually have a 47,XX(or XY)+18 chromosome complement. Characteristic features include intrauterine growth restriction, prominent occiput, small facial features, clenched hands with overlapping of the second finger over the third and the fifth finger over the fourth, and hypertonia. Trisomy 18 has a poor prognosis; approximately 50% of affected individuals die by 2 weeks of age and 90% die by 1 year. 47,XX(or XY)+21 is the most common karyotype seen in individuals who have Down syndrome. Affected newborns typically exhibit midface hypoplasia with epicanthal folds, upslanting palpebral fissures, small ears with overfolded pinnae, redundant nuchal skin, and hypotonia. Many affected individuals have fifth finger clinodactyly (in-curving) (Item C39), and almost 50% have a single transverse palmar crease. Approximately 45% of affected individuals have congenital heart defects.

References:

Carey JC. Trisomy 18 and trisomy 13 syndromes. In: Cassidy SB, Allanson JE, eds. Management of Genetic Syndromes. 2nd ed. Hoboken, NJ: Wiley-Liss; 2005:555-568

Jones KL. Down syndrome. In: Smith’s Recognizable Patterns of Human Malformation. 6th ed. Philadelphia, Pa: Elsevier Saunders; 2006:7-12

Jones KL. Trisomy 13. In: Smith’s Recognizable Patterns of Human Malformation. 6th ed. Philadelphia, Pa: Elsevier Saunders; 2006:18-21

Jones KL. Trisomy 18. In: Smith’s Recognizable Patterns of Human Malformation. 6th ed. Philadelphia, Pa: Elsevier Saunders; 2006:13-17

Sybert VP. Turner syndrome. In: Cassidy SB, Allanson JE, eds. Management of Genetic Syndromes. 2nd ed. Hoboken, NJ: Wiley-Liss; 2005:589-605

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Question: 40

A 15-year-old girl is concerned about irregular menses and acne. Menarche was at age 11 years and 9 months, and she remembers developing pubic hair around age 7 years. On physical

examination, her vital signs are normal and her body mass index is 32.3 kg/m2. She has facial comedonal and papular acne as well as mild darkening of the skin of her neck (Item Q40) and axilla. You also note hypopigmented, narrow stretch marks on her abdomen and hair in a linear distribution from her umbilicus to the pubic symphysis and on the upper inner surface of her thighs. She is at Sexual Maturity Rating 5, and her clitoral diameter is 2 mm.

Of the following, the MOST likely diagnosis is

A. Cushing syndrome

B. hypothyroidism

C. metabolic syndrome

D. physiologic anovulation

E. polycystic ovarian syndrome

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Critique: 40

Preferred Response: E

The presence of acanthosis nigricans combined with obesity (body mass index >30 kg/m2), acne, and some increase in body hair described for the girl in the vignette as well as irregular menses 3 years after menarche suggests the need for further evaluation for polycystic ovarian syndrome (PCOS). The diagnosis of PCOS, using the 2003 Rotterdam criteria, requires, in

addition to exclusion of related conditions, the presence of two of the following three criteria: 1) oligo- or anovulation, 2) clinical or biochemical signs of hyperandrogenism, and 3) polycystic ovaries. Oligo- or anovulation presents as irregular menses, and hyperandrogenism may present as acne, increased body hair, and rarely, clitorimegaly (a transverse clitoral diameter greater than 3 mm). The severity of hirsutism may be assessed using the Ferriman-Gallwey Scoring system. A score ranging from 0 (no hair) to 4 (frankly virile [extensive hair growth]) is assessed for each of nine body areas most sensitive to androgens. These sites include the upper lip, chin, chest, abdomen, suprapubic region, arms, thighs, upper back, and lower back. A score of 8 or more is considered significant and suggestive of increased androgen concentrations. The severity of acne and hirsutism, however, may not correlate well with the concentrations of androgens because the response of the androgen-dependent follicle to androgen excess varies considerably between and within persons. Therefore, total and free testosterone measurement may be supportive of this diagnosis.

A number of risk factors for PCOS have been outlined at various stages of development.

One of these factors is premature adrenarche, which is the appearance of pubic hair before age 8 years without other evidence of puberty. Whether peripubertal obesity predisposes to PCOS remains to be determined. Those who have risk factors for insulin resistance such as acanthosis nigricans or a family history of type 2 diabetes and cardiovascular disease may be at increased risk for PCOS. Acanthosis nigricans is a velvety hyperpigmentation and thickening of

the skin on the nape of the neck, axilla, and other body folds. It is a nonspecific sign of insulin resistance.

A number of disorders may be considered in the differential diagnosis of PCOS but are not

associated with signs of androgen excess. Patients who have hypothyroidism may be overweight and have menstrual disturbances, but they typically have other symptoms, including hair loss, constipation, and dry skin. A common symptom of Cushing syndrome is sudden weight gain. In addition, affected patients have signs or symptoms of cortisol excess such as muscle weakness; facial rounding and plethora; easy bruising; and multiple wide, purplish striae on the abdomen, not the narrow hypopigmented type exhibited by the patient in the vignette. In addition to central obesity and high blood pressure, patients who have metabolic syndrome have elevated fasting glucose and triglyceride values and decreased high-density lipoprotein cholesterol values. Metabolic syndrome is common in those who have PCOS, and such patients should be screened regularly for metabolic syndrome. Physiological anovulation becomes less likely as an explanation for irregular menses 3 years after menarche.

References:

Hassan A, Gordon CM. Polycystic ovary syndrome update in adolescence. Curr Opin Pediatr. 2007;19:389-397. Abstract available at:

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

http://www.ncbi.nlm.nih.gov/pubmed/17630601?ordinalpos=10&itool=EntrezSystem2.PEntrez.Pu

bmed.Pubmed_ResultsPanel.Pubmed_DefaultReportPanel.Pubmed_RVDocSum

Rosenfeld RL. Clinical practice. Hirsutism. N Engl J Med. 2005;353:2578-2588. Extract available at: http://content.nejm.org/cgi/content/extract/353/24/2578

Rosenfield RL. Clinical review: identifying children at risk for polycystic ovary syndrome. J Clin Endocrinol Metab. 2007;92:787-796. Available at:

http://jcem.endojournals.org/cgi/content/full/92/3/787

Schneider MB, Brill SR. Obesity in children and adolescents. Pediatr Rev. 2005;26:155-162. Available at: http://pedsinreview.aappublications.org/cgi/content/full/26/5/155

Wornham WL. Complementary and alternative medicine for gynecology patients. In: Emans SJH, Laufer MR, Goldstein DP, eds. Pediatric and Adolescent Gynecology. 5th ed. Philadelphia, Pa:

Lippincott Williams & Wilkins, 2005:976-987

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Question: 41

You are called to the pediatric ward to evaluate a 10-month-old infant awaiting liver transplant who began vomiting blood after coughing. She has biliary atresia and a failed Kasai procedure. On physical examination, the thin infant has jaundice and a protuberant abdomen with visible veins (Item Q41). Her heart rate is 150 beats/minute, respiratory rate is 30 breaths/minute, and blood pressure is 65/40 mm Hg. A moderate amount of bright red blood is on her bed sheets near the head of her bed.

Of the following, the MOST likely cause of her bleeding is

A. anterior epistaxis

B. esophageal varices

C. gastric stress ulcer

D. infectious enterocolitis

E. Meckel diverticulum

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Critique: 41

Preferred Response: B

Gastrointestinal (GI) bleeding is a common pediatric problem, but life-threatening GI hemorrhage is relatively rare. The location of acute bleeding (above or below the ligament of Treitz) can be determined initially by inserting a nasogastric tube. If blood is recovered, the source is the upper GI tract. Common causes of hematemesis (vomiting of bright red blood) include swallowed blood (eg, from epistaxis, tonsillectomy), esophagitis, Mallory-Weiss tears, reactive gastritis (eg, "stress ulcer" due to alcohol, nonsteroidal anti-inflammatory drugs, critical illness, radiation), peptic ulcer, and esophageal varices (due to portal hypertension). The child described in the vignette has significant liver disease caused by underlying biliary atresia with resultant portal hypertension, as evidenced by the dilated collateral veins seen on her abdominal wall. Portal hypertension, defined as portal pressures greater than 10 mm Hg, is caused by obstruction to portal blood flow due to intra- or extrahepatic causes. Collateral vessels often form to shunt blood to the systemic circulation, but portal flow remains high, as do portal pressures. Collateral vessels are seen commonly in the esophagus; they may dilate and rupture as the result of chronically elevated pressures. In addition, sudden rises in pressure, such as seen with coughing, can produce acute rupture. Although epistaxis and gastric stress ulcers can cause hematemesis, esophageal varices are a much more likely cause of this child’s bleeding because of her underlying clinical condition. Infectious enterocolitis and Meckel diverticulum are both causes of lower GI bleeding and typically present as either hematochezia (passage of bright red blood per rectum) or melena (passage of black, tarry stools).

References:

Boyle JT. Gastrointestinal bleeding in infants and children. Pediatr Rev. 2008;29:39-52. Available at: http://pedsinreview.aappublications.org/cgi/content/full/29/2/39

Mehta R, Shanley TP. Gastrointestinal bleeding. In: Wheeler DS, Wong HR, Shanley TP, eds. Pediatric Critical Care Medicine: Basic Science and Clniical Evidence. New York, NY:

Springer-Verlag London Limited; 2007:1013-1021

Suchy FJ. Portal hypertension and varices. In: Kliegman RM, Behrman RE, Jenson HB, Stanton

BF, eds. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, Pa: Saunders Elsevier; 2007:1709-

1711

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Question: 42

At her initial health supervision visit with you, a 13-year-old girl confides that she has just moved to the United States to join her father because her family could not afford treatment to make her grow taller in Guatemala. Her father tells you that the doctors diagnosed a chromosome problem when she was 8 years old, handing you a letter from a doctor in Guatemala stating that she has Turner syndrome. On physical examination, the girl is 130 cm in height, and her weight is appropriate for height. She has Sexual Maturity Rating 3 pubic hair but no breast tissue. She has a high-arched palate and a triangular-shaped face with a low posterior hairline. You tell the girl and her father that treatment with growth hormone may be possible, but certain tests must be undertaken to determine if the growth hormone will be effective.

Of the following, the MOST helpful test in determining the usefulness of growth hormone is

A. bone age radiography

B. pelvic ultrasonography

C. serum estradiol measurement

D. serum follicle-stimulating hormone measurement

E. serum growth hormone measurement

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Critique: 42

Preferred Response: A

The girl described in the vignette has features of Turner syndrome, including significant short stature. Growth hormone has been shown to increase linear growth rate and adult height in girls who have Turner syndrome as well as in many other conditions associated with short stature. The average increase in adult height afforded by treatment is about 1/3 of an inch or a little less than 1 cm for each year of treatment. Therefore, the most important determinant of the long-term growth response to growth hormone is physiologic maturation, as reflected in the bone age radiograph. For example, a 13-year-old girl who has a bone age of 11 years would have several more years of growth left compared with a 13-year-old girl who has a bone age of 13 years. A girl who has Turner syndrome generally has streak ovaries and an immature uterus, which is evident on pelvic ultrasonography, but development of the ovaries and uterus is important for linear growth only if there is estrogen production, which advances epiphyseal maturation. Measurement of serum estradiol is not useful for the girl described in the vignette because she shows no evidence of estrogen effect, and most girls who have Turner syndrome do not release much estrogen from their poorly functioning ovarian remnants. In addition, it is difficult to measure low circulating concentrations of estradiol accurately. Measurement of serum follicle-stimulating hormone (FSH) should confirm that this girl has ovarian failure because values usually are elevated in girls who have Turner syndrome. FSH is the pituitary hormone that directly stimulates follicular function, and past the usual age at puberty, values rise if there is ovarian failure, just as they do at menopause. Rarely do girls who have Turner syndrome have functioning ovaries and normal FSH concentrations. Elevated FSH values are common in girls who have Turner syndrome after the age of 11 years. Because girls who have Turner syndrome are not deficient in growth hormone, assessment of the capacity of growth hormone release is not necessary. Further, a single growth hormone value rarely is useful in diagnosing the cause of short stature in children because growth hormone is released in response to various stimuli, and unstimulated concentrations are low or not measurable in the blood.

References:

Bannink EM, Raat H, Mulder PG, de Muinck Keizer-Schrama SM. Quality of life after growth hormone therapy and induced puberty in women with Turner syndrome. J Pediatr. 2006;148:95- 101. Abstract available at:

http://www.ncbi.nlm.nih.gov/pubmed/16423606?ordinalpos=74&itool=EntrezSystem2.PEntrez.Pu

bmed.Pubmed_ResultsPanel.Pubmed_DefaultReportPanel.Pubmed_RVDocSum

Baxter L, Bryant J, Cave CB, Milne R. Recombinant growth hormone for children and adolescents with Turner syndrome. Cochrane Database Syst Rev. 2007;1:CD003887. Available at: http://www.mrw.interscience.wiley.com/cochrane/clsysrev/articles/CD003887/frame.html

Loscalzo ML. Turner syndrome. Pediatr Rev. 2008;29:219-227. Available at:

http://pedsinreview.aappublications.org/cgi/content/full/29/7/219

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Sybert VP, McCauley E. Turner's syndrome. N Engl J Med. 2004;351:1227-1238. Available at:

http://content.nejm.org/cgi/content/full/351/12/1227

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Question: 43

A 10-year-old girl is having difficulty completing her schoolwork in class without making errors. She is capable of doing the work with extended time but not within time limits. She is trying very hard to keep up with her classmates, but she works very slowly. Her parents report that she is able to do her homework but requires a lot of time. Her parents bring you a copy of her school testing results, including both the 10 subtest scores and the 4 index scores of the Wechsler Intelligence Scale for Children-Fourth Edition.

Of the following, the score that is MOST likely to be affected for this girl is the

A. full-scale intelligence quotient

B. perceptual reasoning factor

C. processing speed factor

D. verbal comprehension factor

E. working memory factor

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Critique: 43

Preferred Response: C

The Wechsler Intelligence Scale for Children (WISC-IV) is used to assess a child’s mental ability in comparison with the abilities of other children of the same age via a numerical score referred to as the full-scale intelligence quotient (IQ). The scores on a cognitive test are used to predict how a person will function academically. The WISC-IV consists of 15 subtests: 10 core subtests and 5 additional optional subtests. The subtests are grouped into four composite scales known as factor scores. The individual factor scores provide more detailed information regarding the child’s mental ability than does the full-scale IQ score. The verbal comprehension factor assesses skills such as verbal knowledge and how a person uses verbal skills in novel situations. The perceptual reasoning factor evaluates the ability to reason and organize material that is seen without the use of words. The working memory factor is based on the ability to remember information and either to manipulate it or use it to perform calculations. The processing speed factor assesses the speed of processing information. The girl described in the vignette is unable to complete tasks within time limits, indicating that she has difficulty with the speed of processing simple visual information without making a mistake. Therefore, the score that is most likely to be affected for her is the processing speed factor. Factor scores on the WISC-IV should be fairly similar. A substantial difference between scores suggests a greater likelihood of a learning or cognitive disability.

References:

Braaten EB, Norman D. Intelligence (IQ) testing. Pediatr Rev. 2006;27:403-408. Available at:

http://pedsinreview.aappublications.org/cgi/content/full/27/11/403

Wechsler D. Manual for the Wechsler Intelligence Scale for Children-Revised. New York, NY:

Psychological Corporation; 1974

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Question: 44

A 7 year-old-girl presents to your office with a 1-day history of a temperature of 38.9°C. Notable findings from her past medical history include static encephalopathy, seizure disorder, and recurrent urinary tract infections. She is receiving intermittent straight catheterization and trimethoprim-sulfamethoxazole prophylaxis. Her medications also include phenytoin, albuterol via nebulizer, ipratropium, and ranitidine. Urinalysis reveals more than 100 white blood cells per high- power field and is positive for leukocyte esterase and nitrites.

Of the following, the BEST option for oral empiric therapy pending culture results is

A. amoxicillin

B. azithromycin

C. ciprofloxacin

D. nitrofurantoin

E. trimethoprim-sulfamethoxazole

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Critique: 44

Preferred Response: C

The history of recurrent urinary tract infections (UTIs) and development of a UTI while receiving trimethoprim-sulfamethoxazole (TMP-SMX) described for the girl in the vignette raise concern for a resistant pathogen, making ciprofloxacin, a fluoroquinolone, the best option for therapy, pending culture results and sensitivity testing. Azithromycin is not indicated for treatment of UTIs. Amoxicillin and nitrofurantoin may have roles in treatment of a simple UTI but are not adequate in the possible presence of a resistant pathogen. The development of this UTI while the patient is receiving TMP-SMX prophylaxis suggests that the infecting organism is

resistant to this agent. Fluoroquinolones are derivatives of the urinary tract agent nalidixic acid that have a very broad antimicrobial spectrum, excellent oral absorption, and relatively few adverse effects. Studies in juvenile animals demonstrated arthropathy, which initially limited their investigation and use in children. Subsequent trials and analyses of uncontrolled use of fluoroquinolones in pediatrics have documented no increased incidence of arthropathy. However, fluoroquinolone use to date generally has been associated with rapid development of resistant organisms. The most recent recommendations of the Committee on Infectious Diseases of the American Academy of Pediatrics suggest that fluoroquinolone use in pediatrics be restricted to situations in which the pathogen is multidrug-resistant and there is no safe and effective alternative or when parenteral therapy is not feasible and there is no effective alternative oral agent. Such situations might include UTIs caused by multidrug-resistant gram-negative rods, including Pseudomonas aeruginosa; gastrointestinal and respiratory tract infections caused by resistant gram-negative organisms; and chronic or acute osteomyelitis caused by P aeruginosa. In addition, a fluoroquinolone may be indicated for treatment or prevention of anthrax and for treatment of mycobacterial infection with sensitive strains. Clinicians also must be aware of potential drug interactions that may occur when fluoroquinolones are used. Antacids containing aluminum, magnesium, or calcium as well as warfarin may decrease the absorption of fluoroquinolones. Fluoroquinolones may increase caffeine concentrations or the anticoagulant effects of warfarin. Nonsteroidal anti-inflammatory drugs may potentiate the central nervous system effects of fluoroquinolones and should be used cautiously in patients receiving fluoroquinolone therapy. Fluoroquinolones also can inhibit potassium channels in cardiac tissue and increase the risk for arrhythmias associated with a prolonged QT interval. Finally, they may cause hypo- or hyperglycemia when administered to patients receiving an antidiabetes agent or insulin.

As a result of reviewing this information, do you intend to make a change in practice to provide better patient care?

Yes

No

References:

Committee on Infectious Diseases. The use of systemic fluoroquinolones. Pediatrics. 2006;118:1287-1292. Available at:

http://pediatrics.aappublications.org/cgi/content/full/118/3/1287

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Hooper DC. Fluoroquinolones. UpToDate Online 16.3. 2008. Available for subscription at:

http://www.utdol.com/online/content/topic.do?topicKey=antibiot/8621&selectedTitle=1~150&sour

ce=search_result

Oliphant CM, Green GM. Quinolones: a comprehensive review. Am Fam Physician. 2002;65:455- 464. Available at: http://www.aafp.org/afp/20020201/455.html

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Question: 45

You are evaluating a 5-year-old boy who has acquired immunodeficiency syndrome and whose

recent CD4 percentage is 18%. He presents today with a 5-day history of a temperature to 38.9°C, increased work of breathing, and cough. His mother states that he is normally very active but over the past 2 days has been having a problem catching his breath just walking to

the bathroom. Physical examination shows a tired-appearing boy in mild respiratory distress. He

has a temperature of 38.4°C, respiratory rate of 28 breaths/min, some mild nasal flaring,

moderate intercostal retractions, and scattered crackles at the lung bases bilaterally. Oxygen

saturation by pulse oximetry on room air is 88%, and an arterial blood gas reveals a Pao2 of 60

mm Hg. A chest radiograph demonstrates bilateral perihilar infiltrates (Item Q45).

Of the following, the MOST appropriate antimicrobial agent to start is

A. azithromycin

B. cefotaxime

C. clindamycin

D. trimethoprim-sulfamethoxazole

E. vancomycin

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Critique: 45

Preferred Response: D

The child described in the vignette presents with classic signs and symptoms of Pneumocystis jiroveci (formerly Pneumocystis carinii) pneumonia. This pathogen is an important cause of pulmonary infections in immunocompromised patients. Characteristic signs and symptoms include dyspnea at rest, tachypnea, nonproductive cough, fever, and hypoxia with an increased oxygen requirement. The intensity of the signs and symptoms can vary, and onset may be acute and fulminant. Chest radiographs frequently demonstrate diffuse bilateral interstitial or alveolar disease (Item C45). Pneumocystis pneumonia is diagnosed definitively by demonstrating the presence of the organism in lung tissue or respiratory secretions. The mortality rate in immunocompromised patients ranges from 5% to 40% if treated and approaches 100% if untreated. Treatment should not be delayed until a definitive diagnosis is established in an immunocompromised patient who has a clinically compatible illness. Trimethoprim-sulfamethoxazole is the drug of choice for therapy of a Pneumocystis infection. It also is used as prophylaxis for children whose CD4 percentage is less than 15%. Azithromycin, cefotaxime, clindamycin, and vancomycin have no activity against P jiroveci. Patients who are unable to tolerate trimethoprim-sulfamethoxazole or who have severe disease and do not respond to trimethoprim-sulfamethoxazole after 5 to 7 days may be treated with intravenous pentamidine as an alternate agent. In pediatrics, the sulfonamide agents alone have several uses. They may be used in the treatment of acute urinary tract infections, inflammatory bowel diseases, burns, umbilical cord care, vaginitis, nongonococcal urethritis due to Chlamydia, toxoplasmosis, and bacterial conjunctivitis. Trimethoprim-sulfamethoxazole remains the drug of choice for susceptible isolates in the treatment of urinary tract infections; prevention and treatment of P jiroveci infections in immunocompromised patients; and gastroenteritis due to Salmonella, Shigella, and Isospora belli. It also can be used in the treatment of upper respiratory tract infections (eg, otitis media, sinusitis, pneumonitis) due to sensitive organisms, although it is not the first-line antimicrobial agent for these infections. Trimethoprim-sulfamethoxazole also is effective in the treatment of infections caused by Stenotrophomonas maltophilia, Burkholderia cepacia, Brucella, and methicillin-resistant Staphylococcus aureus.

References:

American Academy of Pediatrics. Pneumocystis jiroveci infections. In: Pickering LK, Baker CJ, Kimberlin DW, Long SS, eds. Red Book: 2009 Report of the Committee on Infectious Diseases. 28th ed. Elk Grove Village, Ill: American Academy of Pediatrics; 2009:536-540

Burchett SK, Pizzo PA. HIV infection in infants, children, and adolescents. Pediatr Rev. 2003;24:186-194. Available at: http://pedsinreview.aappublications.org/cgi/content/full/24/6/186

Smith CL, Powell KR. Review of the sulfonamides and trimethoprim. Pediatr Rev. 2000;21:368- 371. Available at: http://pedsinreview.aappublications.org/cgi/content/full/21/11/368

Walzer PD, Smulian AG. Pneumocystis species. In: Mandell GL, Bennett JE, Dolan R, eds.

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Mandell, Douglas, and Bennett’s Principles and Practice of Infectious Diseases. 6th ed. Philadelphia, Pa: Elsevier Churchill Livingstone; 2005:3080-3094

Zinner SH, Mayer KH. Sulfonamides and trimethoprim. In: Mandell GL, Bennett JE, Dolan R, eds. Mandell, Douglas, and Bennett’s Principles and Practice of Infectious Diseases. 6th ed. Philadelphia, Pa: Elsevier Churchill Livingstone; 2005:440-450

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Question: 46

A

14-year-old boy presents for a sports physical examination prior to participating in football. He

is

required to undergo a urinalysis as part of the evaluation. His temperature is 37.1°C, heart rate

is

74 beats/min, respiratory rate is 14 breaths/min, and blood pressure is 114/68 mm Hg. Findings

on his physical examination are unremarkable. His urinalysis demonstrates a urine specific gravity of 1.025, pH of 6.5, 3+ protein, and no blood. Microscopy findings are negative.

Of the following, the MOST appropriate next step is to

A. collect a first-morning urine specimen to measure protein and creatinine concentrations

B. measure serum albumin concentration and obtain renal function studies

C. obtain a 24-hour urine collection to measure protein and creatinine concentrations

D. quantitate the proteinuria with a random urine collection to measure protein and creatinine

concentrations

E. repeat the urinalysis in 1 year because of the lack of coexisting hematuria

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Critique: 46

Preferred Response: A

The adolescent described in the vignette has asymptomatic, isolated proteinuria without any signs or symptoms of a systemic disease. Furthermore, he has a normal blood pressure and lacks any associated hematuria. These pertinent negative features suggest that he is unlikely to have severe renal disease. Proteinuria typically is detected on urinalysis by the urine dipstick, which screens for albuminuria. The reaction of urinary albumin tetrabromphenol blue impregnated within the dipstick test results in a color change to green. The result can be false-negative if the urine is dilute (urine specific gravity <1.015) or false-positive if the urine is alkaline or highly concentrated (urine specific gravity >1.030). Finally, the dipstick test does not detect other low-molecular

weight proteins such as beta2-microglobulin. The most likely diagnosis for an adolescent who has asymptomatic, isolated proteinuria is postural or orthostatic proteinuria, which occurs in 2% to 5% of children of this age and is believed to have an excellent prognosis. The amount of urinary protein excreted in the supine position is normal, but that excreted in the upright position may be as much as tenfold higher than normal. The mechanism is unclear but appears to be a subtle glomerular defect that is exacerbated by a tendency for protein filtration in the upright position. The upright position is believed to cause venous pooling in the legs, followed by renal vein congestion and increased efferent arteriolar resistance and the subsequent tendency to protein filtration. If orthostatic proteinuria is confirmed, an annual urinalysis is recommended to monitor for progression of proteinuria. If proteinuria has resolved at the next annual visit, further follow-up is not required. A random urine sample in the clinical setting can be used to diagnose orthostatic proteinuria by documenting elevated urinary protein excretion. Serologic testing, renal function tests, serum albumin concentrations, and quantitative measurement of urine protein excretion usually are not performed until orthostatic proteinuria has been excluded. Because of the efficacy of easily obtained random urine samples, 24-hour urine collections are not recommended for the evaluation of proteinuria in the pediatric patient. Finally, patients who have nonorthostatic proteinuria should be followed relatively closely, with a repeat urine check in 1 to 2 months to monitor for the development of hematuria or worsening of proteinuria, which signals progression of the underlying disorder that may warrant a renal biopsy.

References:

Bergstein JM. A practical approach to proteinuria. Pediatr Nephrol. 1999;13:697-700. Abstract available at: http://www.ncbi.nlm.nih.gov/pubmed/10502130

Devarajan P. Mechanisms of orthostatic proteinuria: lessons from a transplant donor. J Am Soc Nephrol. 1993;4:36-39. Available at: http://jasn.asnjournals.org/cgi/reprint/4/1/36

Hogg RJ, Portman RJ, Milliner D, Lemley KV, Eddy A, Ingelfinger J. Evaluation and management of proteinuria and nephrotic syndrome in children: recommendations from a pediatric nephrology panel established at the National Kidney Foundation Conference on Proteinuria, Albuminuria, Risk, Assessment, Detection, and Elimination (PARADE). Pediatrics. 2000;105:1242-1249.

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2010 PREP SA on CD-ROM

Available at: http://pediatrics.aappublications.org/cgi/content/full/105/6/1242

Vehaskari VM, Rapola J. Isolated proteinuria: analysis of a school-age population. J Pediatr. 1982;101:661-668. Abstract available at: http://www.ncbi.nlm.nih.gov/pubmed/7131137

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Question: 47

An 18-year-old girl presents with a 12-month history of severe nasal congestion and anosmia. She was diagnosed with allergic rhinitis at age 13 and has been receiving allergen immunotherapy for the past 3 years. Despite allergy shots, allergy medication (oral antihistamine and nasal corticosteroid), and two 21-day courses of antibiotics, her symptoms have persisted. She describes her rhinorrhea as thick and "peanut buttery." On physical examination, her height and weight are at the 75th percentile for age and she has bilateral nasal polyps. The remainder of the examination results are normal. Computed tomography scan of her sinuses shows complete unilateral opacification of the right maxillary sinus (Item Q47).

Of the following, the MOST likely diagnosis is

A. allergic fungal sinusitis

B. allergic rhinitis

C. chronic bacterial sinusitis

D. cystic fibrosis

E. primary ciliary dyskinesia

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Critique: 47

Preferred Response: A

Sinusitis is an infection of the nasal passages and sinuses. In the past, sinusitis was classified as acute (less than 4 weeks in duration), subacute (4 to 12 weeks), and chronic (more than 12 weeks). Recent nomenclature has altered this classification to include chronic sinusitis with and without nasal polyposis. The finding of nasal polyps for the girl described in the vignette should raise the suspicion for cystic fibrosis. However, there are other, more common causes of nasal polyposis (Item C47), and the teenager lacks any other clinical features consistent with cystic fibrosis (eg, poor weight gain, gastrointestinal symptoms, clubbing, pansinusitis, recurrent pneumonias). The finding of unilateral sinus disease, "peanut buttery" mucin, nasal polyps, and a history of atopy makes allergic fungal sinusitis (AFS) the most likely diagnosis for this girl. This condition accounts for 10% to 15% of chronic rhinosinusitis in adolescents and young adults. The exact mechanism is unknown, but exposure to a particular fungus (usually Bipolaris or Curvularia) results in local inflammation, eosinophilic tissue infiltration, and mucus production. The five primary characteristics of AFS are:

Production of eosinophilic-containing, noninvasive fungal hyphae

Nasal polyposis

Characteristic radiographic findings (unilateral sinus disease)

Immunocompetence

Immunoglobulin (Ig) E to fungi, as determined by skin testing or serum-specific IgE

The management of AFS involves oral corticosteroids after surgical debridement, but recurrences are frequent. Uncomplicated allergic rhinitis rarely results in polyps or thick mucin. Further, allergic rhinitis should respond promptly to usual therapies, such as oral antihistamines, nasal steroids, and allergen immunotherapy. Chronic bacterial sinusitis can present identically to allergic fungal sinusitis. Although polyps may be present in bacterial sinusitis, improvement is expected with a prolonged course of antibiotics. Ciliary dyskinesia represents a defect in the dynein arm or radial spokes of cilia. Patients typically present with recurrent otitis media and pneumonia. Situs inversus is seen in approximately 50% of patients and is termed Kartagener syndrome. Patients experiencing recurrent sinusitis or chronic sinusitis should be evaluated for possible underlying risk factors.

References:

Mazur LJ, Kim J, American Academy of Pediatrics Committee on Environmental Health. Spectrum of noninfectious health effects from molds. Pediatrics. 2006;118:e1909-e1926.

http://pediatrics.aappublications.org/cgi/content/full/118/6/e1909

Meltzer EO, Jamilos DL, Hadley JA, et al; American Academy of Allergy, Asthma and Immunology (AAAAI); American Academy of Otolayngic Allergy (AAOA); American Academy of Otolaryngology–Head and Neck Surgery (AAO-HNS); American College of Allergy, Asthma and

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Immunology (ACAAI); American Rhinologic Society (ARS). Rhinosinusitis: establishing definitions for clinical research and patient care. J Allergy Clin Immunol. 2004;114:155-212

Pappas DE, Hendley JO. Sinusitis. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, Pa: Saunders Elsevier; 2007:1749-1752

Taylor A, Adam HM. In brief: sinusitis. Pediatr Rev. 2006;27:395-397. Available at:

http://pedsinreview.aappublications.org/cgi/content/full/27/10/395

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Question: 48

The mother of one of your patients calls frantically because she just found her 2-year-old daughter with an open bottle of prenatal vitamins and several of the tablets in her mouth. The child is acting normally. The mother reports that the label says there is 30 mg of elemental iron per tablet and five tablets are missing from the bottle she picked up at the pharmacy this morning. Her daughter weighs 25 lb.

Of the following, the MOST appropriate advice to give the mother is to

A. bring the child to office in the morning for assessment of serum iron concentration

B. give the child activated charcoal

C. give the child syrup of ipecac

D. observe the child at home for symptoms

E. take the child to the nearest emergency department

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Critique: 48

Preferred Response: D

Iron overdoses continue to be common among children younger than 6 years of age. Most of these ingestions are unintentional and do not result in significant toxicity. However, iron ingestion can cause fatalities, especially in the setting of an intentional ingestion or exposure to an adult preparation. Management of iron ingestion begins with determining, if possible, how much elemental iron was ingested and if the patient is symptomatic. Any child who is symptomatic within 6 hours after ingesting iron, regardless of the estimated dose, or if the dose is unknown, should be brought to medical attention. In the asymptomatic child, ingestions of less than 40 mg/kg of elemental iron are not significant, and the patient can be observed at home. The mother in the vignette can be reassured that because her child ingested 13.2 mg/kg of elemental iron (<40 mg/kg), she is unlikely to develop toxicity and can be observed at home. If she remains asymptomatic for more than 6 hours, no further evaluation or treatment is necessary. If she develops symptoms, she should be taken to an emergency department for evaluation and treatment. For the patient who has a significant ingestion, laboratory evaluation should include measurement of a serum iron concentration within 4 hours of ingestion, serum electrolyte and aminotransferase determinations, a complete blood count, and coagulation tests. Laboratory indicators of a potentially significant ingestion include serum iron concentration greater than 350

mcg/dL (62.7 mcmol/L), white blood cell count greater than 15.0x103/mcL (15.0x109/L), and serum glucose values greater than 150 mg/dL (8.3 mmol/L). In a symptomatic patient, abdominal radiography should be used to look for the presence of retained tablets in the gastrointestinal tract. If present, gastrointestinal decontamination using whole-bowel irrigation is indicated. Patients who have severe symptoms, anion gap acidosis, serum iron concentrations of greater than 500 mcg/dL (89.5 mcmol/L), or a significant number of pills visible on abdominal radiography should be treated with deferoxamine to chelate free circulating iron. Neither activated charcoal, which adsorbs iron poorly, nor syrup of ipecac is indicated for decontamination. Iron is both a corrosive and a cellular toxin. The clinical phases of significant iron toxicity are attributable directly to these two mechanisms. Phase 1 (gastrointestinal phase) occurs between 30 minutes to 6 hours after ingestion and includes vomiting, diarrhea, abdominal pain, and hematemesis or melena. Such signs and symptoms are caused by the agent’s corrosive effects on the gastrointestinal mucosa. Phase 2 (latent phase) occurs 6 to 12 hours after ingestion but can last as long as 24 hours. Patients often are asymptomatic during this time while free iron is taken up into the reticuloendothelial organs. Phase 3 (shock, metabolic acidosis, hepatic failure phase) may be seen as early as 6 to 12 hours after ingestion and is the result of mitochondrial dysfunction and cell death. Phase 4 (bowel obstruction phase) occurs 2 to 8 weeks after the acute ingestion and results from gastrointestinal tract scarring following iron-induced corrosive damage.

References:

Liebelt EL, Kronfol R. Acute iron poisoning. UpToDate Online 16.3. 2008. Available at:

http://www.utdol.com/online/content/topic.do?topicKey=ped_tox/4912&selectedTitle=1~150&sou

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

rce=search_result630

Manoguerra AS, Erdman AR, Booze LL, et al. Iron ingestion: an evidence-based consensus guideline for out-of-hospital management. Clin Toxicol (Phila). 2005;43:553-570. Abstract available at: http://www.ncbi.nlm.nih.gov/pubmed/16255338

Spanierman C. Toxicity, iron. eMedicine Specialties, Emergency Medicine, Toxicology. 2007. Available at: http://www.emedicine.com/emerg/topic285.htm

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Question: 49

The mother of a 5-month-old boy has come to your office seeking nutritional advice. She exclusively breastfed the infant for the first 4 months, then weaned the baby to a standard, cow milk protein-based infant formula. One week after weaning, she noted that the baby "strained with stool." Because of her concerns regarding the development of constipation, the mother switched him to a formula containing 2 mg/L iron.

Of the following, the MOST important dietary recommendation for this infant is to

A. add pureed vegetables to the diet

B. change to a cow milk protein-based formula containing 12 mg/L iron

C. change to a soy protein-based formula

D. continue the present regimen and supplement with 4 oz/day diluted apple juice

E. substitute oatmeal for rice cereal in the diet

2010 PREP SA on CD-ROM

2010 PREP SA on CD-ROM

Critique: 49

Preferred Response: B

For the infant described in the vignette, the most appropriate dietary recommendation is to return the infant to a formula fortified with 12 mg/L iron. Parents frequently use low-iron formulas in the false belief that iron-supplemented formulas induce constipation. However, the change to a low iron-containing formula not only fails to alter the stooling pattern but can place the infant at risk for iron deficiency. Although the optimal iron content of infant formula has been debated, all available data indicate that iron supplementation is essential for formula-fed infants to prevent a deficiency state. For nursing infants, the lactoferrin content of human milk greatly enhances iron bioavailability and absorption, despite the milk’s low iron content. Therefore, during the first few months, no added iron is required. After 6 months, however, breastfed infants are at risk for iron deficiency, unless dietary supplements are provided. For infants weaned to the bottle, this may be accomplished by using a standard iron-fortified formula. For infants who continue to nurse after 6 months, iron-fortified stage 1 baby foods and cereals meet the requirement. Supplementing human milk with formula or changing formulas are common responses to a wide range of nonspecific complaints. In otherwise healthy and thriving infants, available evidence neither supports this practice nor recommends the consumption of solids for other than nutritionally indicated purposes. In situations such as that described in the vignette, caregivers often try a soy formula, usually on the advice of friends or family. In fact, if constipation is the perceived issue, soy formulas actually may exacerbate the problem. Proprietary soy preparations contain either sucrose or glucose polymers as the constituent carbohydrate. Such di- or polysaccharides may be absorbed more efficiently compared with lactose, particularly in young infants, resulting in a firmer stool. From a nutritional perspective, all commercial soy formulas are iron-fortified. Such fortification is required for soy-based formulas because soy protein products contain phytates, which bind iron and other minerals intraluminally. The consumption of poorly absorbed carbohydra