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Question 1 An infant has the following findings at 5 minutes of life: pulse 130 bpm, cyanotic hands and feet, good muscle tone, and a strong cry and grimace. This infant Apgar score is:
A. B. C. D. E. 6 7 8 9 10
Question 2 Jaundice is most likely to be physiologic in a term infant in which of the following situations:
A. Jaundice at 12 hours of age B. Serum bilirubin level increasing less than 5 mg/dL/24 hours in the first 2-4 days C. Direct serum bilirubin greater than 2 mg/dL D. Jaundice at 13 days of age E. Serum bilirubin level 15 mg/dL at 7 days of age
Question 3 Which of the following is most appropriate for treating hyperbilirubinemia (11.2 mg/dL) in a 3-week-old, breast-fed infant with normal growth and development?
A. B. C. D. E. Phototherapy Exchange transfusion Phenobarbital Replace breast milk by formula for 48 hours None of the above
Question 4 Newborns are obligate nasal breathers for the first few months after birth. Of the following, the most common cause of neonatal nasal obstruction is:
A. B. C. D. E. Choanal atresia Craniofacial malformations Intranasal encephalocele Lacrymal cyst Mucosal inflammation
Question 5
A term newborn appears dusky at rest, but becomes pink with crying. Attempts to pass a catheter through each nostril have failed. Physical examination of the infant reveals other congenital anomalies. Of the following, the most likely clinical syndrome in this infant is:
A. B. C. D. E.
Apert syndrome CHARGE association Crouzon syndrome Pfeiffer syndrome Treacher-Collins syndrome
Question 7 The maternal serum screening test or quadruple test is done between 14 and 22 weeks gestation. It includes all of the following Except
A. Alpha-fetoprotein B. -hCG C. Unconjugated pregninolone D. InhibinA E. Unconjugated esteriol
Question 10
You are called to the delivery of a boy at 42 weeks GA with thick meconium-stained fluid and type II decelerations. The obstetrician rapidly delivers the baby and hands him to you. The boy hypotonic, cyanotic, apneic, and bradycardic. The most appropriate action is to A. B. C. D. E. Stimulate the infant to breath Administer epinephrine Provide positive-pressure bag-and-mask ventilation Intubate and provide positive-pressure ventilation Intubate and apply negative-pressure suction
Question 11
You are called to see one-hour-old term baby in normal newborn nursery with ambiguous genitalia. Your plan includes all the following Except
A. Inform the parents that their babys genitalia are incompletely developed, and you need to do some investigation to identify their babys gender Advise the parents to use unigender name Urgent chromosomal analysis Pelvic ultrasound Serum electrolytes with daily monitoring
B. C. D. E.
Question 12 Incidence of respiratory distress syndrome increases with all of the following Except
A. Prematurity
B. Maternal diabetes
C. Black race D. Male gender E. Cesarean section delivery
Question 13 Normal findings in a newborn baby includes all of the following Except
A. A mongolian blue spot
B. A strawberry nevus
C. Vaginal blood loss D. Lanugo hair E. Erythema toxicum
Question 14
The pharmacologic effects of caffeine in the treatment of apnea of prematurity include stimulation of the medullary respiratory center, increased sensitivity to carbon dioxide, and enhanced diaphragmatic contractility. Of the following, caffeine exerts most of its effects by A. B. C. D. E. Antagonism of prostaglandin activity Blockage of adenosine receptors Enhancement of catecholamine secretion Stimulation of phosphodiesterase Upregulation of gamma-amino-butyric acid receptors
Question 15
When clinical zinc deficiency occurs in term and preterm infants, almost all affected infants are exclusively breastfed and receiving breast milk that has substantially lower-than-average zinc content. Of the following, the most common clinical manifestation of zinc deficiency in infants is A. B. C. D. Alopecia Diarrhea Failure to thrive Irritability
E.
Rash
Question 16
Assisted reproductive technology is associated with significant risks to the fetus and the mother. Of the following, the greatest risk to the fetus is related to
A. Cesarean section B. Gestational diabetes C. Maternal pre-eclampsia D. Multiple gestations
E. Placental abruption
Question 17
A term male newborn has severe intrauterine growth restriction, triangular face with broad forehead and pointed chin, leg length asymmetry, fifth finger clinodactyly, and cryptorchidism. Of the following, the most likely genomic imprinting disorder in this infant is
A. B. C. Albright hereditary osteodystrophy Angelman syndrome Beckwith-Wiedemann syndrome
D.
E.
Prader-Willi syndrome
Silver-Russell syndrome
Question 18
A preterm newborn has multiple fractures of long bones, wormian skull, dark blue sclera, and beaked nose. Osteogenesis imperfecta type II is suspected. Genetic molecular tests are ordered for confirmation of the diagnosis. Of the following, the genetic mutation in this infant is most likely to involve the
A. B. C. D. E. ADAMTS2 gene Collagen 1A1 gene Fibrillin-1 gene Filamin B gene Transforming growth factor receptor 1 gene
Question 19 Of the following, the nasal maldevelopment most likely to be associated with holoprosencephaly is:
A. B. C. D. E. Cepocephaly Choanal atresia Nasal agenesis Piriform aperture stenosis Proboscis lateralis
Question 20
Question 21 The most prevalent clinical manifestation of late-onset group B Streptococcus disease is
A. Arthritis B. Cellulitis C. Meningitis D. Osteomyelitis E. Pneumonia
Question 22
In contrast to classic bronchopulmonary dysplasia (BPD), the new BPD in the more contemporary clinical setting is characterized by different histopathologic findings on examination of the lung tissue. The most striking abnormality in the lungs of infants who have new BPD is
A. B. C. Decrease in alveolar septation Diffuse leukocytic infiltration Epithelial squamous metaplasia
D.
E.
Question 23
Several risk factors during prenatal, perinatal, and postnatal development have been proposed as predictors of cerebral palsy in preterm infants. Of the following, the highest rate of cerebral palsy among preterm infants is associated with
A. B. C. D. Bronchopulmonary dysplasia Necrotizing enterocolitis requiring surgery Parenchymal brain injury Sepsis or meningitis
E.
Question 24
A 1700-g infant is born at 36 weeks gestation complicated by severe oligohydramnios. The Apgar scores are 3 and 5 at 1 and 5 min respectively. The baby required intubation as part of the resuscitation and continued MV to improve the ABGs. At 1 hour of age, the baby showed acute deterioration with cyanosis, bradycardia, and hypotension. The most likely diagnosis of this acute change is A. B. C. D. E. Patent ductus arteriosus Intraventricular hemorrhage Hypoglycemia Pneumothorax Severe respiratory distress syndrome
Question 26
A 14-day-old preterm infant, whose birthweight was 980 g at an estimated gestational age of 27 weeks, is receiving full enteral feedings of fortified human milk. The infant is breathing spontaneously in room air and has no evidence of cardiac, renal, or intracranial abnormalities. Of the following, the most desirable goal of enteral nutrition in this infant is to achieve A. B. C. D. E. Fetal rate of body weight gain Fetal rate of crown-heel length gain Fetal rate of head circumference gain Intrauterine body composition Normal blood urea concentration
Question 27
The interruption in the transfer of nutrients from the mother to the fetus that occurs following birth can be minimized by early administration of parenteral nutrition in VLBW neonates within the first 24 hours after birth. Of the following, the most common metabolic consequence of early parenteral nutrition with amino acids is A. Hyperammonemia
B.
C. D.
Hyperglycemia
Hyperkalemia Increase in blood urea nitrogen values
E.
Metabolic acidosis
Question 28
A 3600-g, breast-fed female, 42 weeks GA, is noted to have persistent hyperbilirubinemia at 2 weeks of age. On examination, the infant has not gained weight since birth and has decreased tone, an umbilical hernia, and an anterior fontanel measuring 4 x 6 cm. The most likely diagnosis is
A.
B. C.
Crigler-Najjar syndrome
Gilbert disease Biliary atresia
D.
E.
Hypothyroidism
galactosemia
mothers
B. requires treatment with a sling C. is associated with Erbs palsy D. causes an asymmetric Moro reflex E. is not painful
Question 30
Minimal enteral feeding, also called gut priming or trophic feeding, is designed to improve gastrointestinal function and is used frequently in the nutritional management of VLBW neonates. The most accurate statement regarding minimal enteral feeding is that it
A.
B. C. D.
E.
Increases plasma concentrations of gastrointestinal hormones Is best avoided in infants weighing 500 to 600 g Is contraindicated in the presence of assisted ventilation Is contraindicated in the presence of indwelling umbilical catheters Prevents necrotizing enterocolitis
Question 31
The only inborn error of metabolism associated with neonatal hyperammonemia that is X-linked in inheritance is
A. Hyperinsulinism/hyperammonemia syndrome B. Isovaleric acidemia C. Medium-chain acyl-CoA dehydrogenase deficiency D. Ornithine transcarbamylase deficiency E. Pyruvate carboxylase deficiency
Question 32
A 750-g black female experienced respiratory distress after a preterm delivery at 27 wks gestation. Pregnancy was uncomplicated, but labor was abrupt and therefore the mother did not receive steroid or penicillin therapy. The baby required intubation and MV with 100% O2. The possible causes of respiratory distress include (multiple choices)
A.
B. C. D. E.
Pneumothorax
Respiratory distress syndrome Patent ductus arteriosus Group B streptococcal sepsis Transient tachypnea
Question 33 The patient in Question 32 was also treated with endotracheal instillation of exogenous surfactant. This is likely to be beneficial by (multiple choices)
A. Preventing bronchopulmonary dysplasia B. Reducing the incidence of pneumothorax C. Reducing mortality D. More rapid improvement of RDS
E. Preventing oligouria
Question 34
Approximately 10% of infants born with congenital diaphragmatic hernia (CDH) have significant heart defects. The most common congenital heart defect associated with CDH is
A. Aortic arch obstruction B. Tetralogy of Fallot C. Total anomalous pulmonary venous return D. Transposition of the great arteries
Question 35
Contemporary management of gastroschisis is associated with a 10% to 25% incidence of postoperative intestinal and related complications. The most common postoperative complication of surgical repair of gastroschisis is
A.
B. C.
D.
E.
Renal ischemia
Short bowel syndrome
Question 36
Amniotic fluid volume varies substantially at each week of human gestation, as reflected in the wide range of normal values. The largest variation in amniotic fluid volume occurs at the gestational age of
A. B. C. D. E. 26 to 27 weeks 29 to 30 weeks 32 to 33 weeks 35 to 36 weeks 38 to 39 weeks
Question 37
You wish to determine the incidence and natural course of necrotizing enterocolitis in preterm neonates. Of the following, the best study design to address this question is a
A. Case-control study B. Descriptive observational study C. Prospective cohort study D. Randomized clinical trial
E. Systematic meta-analysis
Decreased absorption of amniotic fluid due to gastrointestinal atresia Decreased fetal swallowing from neuromuscular disorder Excessive transudation of fluid from an abdominal wall defect Increased fetal lung fluid secretion associated with gestational diabetes Increased fetal urine output from hydrops associated with anemia
Question 39
A 4-week-old, A-positive, African-American former 40-weeks-gestational age infant was born to an Opositive mother and experienced hyperbilirubinemia requiring 2 days of phototherapy in the NB nursery after birth. The infant appears apathetic and demonstrates pallor, a grade 2/6 ESM, and a HR 175 bpm. The most likely diagnosis is A. B. C. D. E. Anemia of chronic disease G-6-PD deficiency Hereditary spherocytosis Sicle cell anemia with hemolytic crisis ABO incompatibility with continued hemolysis
Question 40 The following predispose a baby to be small for gestational age (multiple choices)
A. Placental insufficiency
B. Twin pregnancy C. Congenital infection D. Maternal diabetes E. Alcohol consumption
Question 41 The small for gestational age newborn baby is at increased risk of (multiple choices)
A. Hypoglycemia
B. Polycythemia C. Hypothermia D. Hypothyroidism E. Cardiac abnormalities
Question 42 A term infant is born with Apgar score 5 and 7 at 1 and 5 min respectively. The infant has a HR 170 bpm and demonstrates pallor with hepatosplenomegaly. A Kleihauer-Betke test was positive. The most likely diagnosis is
A. B. C. D. E. Erythroblastosis fetalis Hereditary spherocytosis Chronic feto-maternal transfusion ABO incompatibility Blackfan-Diamond syndrome
Question 44 The amino acids derived from swallowed amniotic fluid are important for fetal gastrointestinal development. The amino acid considered conditionally essential for normal growth and maturation of the fetal gastrointestinal tract is
A. Alanine B. Citrulline C. Glutamine D. Leucine E. Valine
Question 47
Bilirubin conjugated in the liver and secreted into bile can be deconjugated in the gut, then reabsorbed into the bloodstream, resulting in enterohepatic bilirubin circulation. The enzyme most responsible for contributing to enterohepatic bilirubin circulation is
A. B. C. D. E. Beta-glucuronidase Bilirubin hydrogenase Bilirubin oxidase Biliverdin reductase Uridine diphosphoglucuronate glucuronosyltransferase
Question 48
The most widely used and accurate method for estimating amniotic fluid volume is by
A. Determination of dye dilution by timed amniocentesis B. Palpation of the fetus by the Leopold maneuver C. Palpation of the uterus for fundal height D. Ultrasonographic measurement of the amniotic fluid index E. Ultrasonographic measurement of the largest vertical pocket
Question 49 A NB female has a ventricular septal defect, cleft lip and palate, imperforate anus. All of the following tests would be appropriate Except
A. B. C. D. E. Karyotype analysis TORCH titer Renal US Cranial US Skeletal survey
Question 50
A 2700-g, 36-wks-GA white male is born after 22 hrs of premature rupture of the amniotic membranes. The Apgar scores are 3 and 5. He immediately experienced respiratory distress and cyanosis requiring ET intubation and MV with 100% O2. Vital signs are T: 35.7C, HR 195 bpm, mean BP 22 mm Hg. WBC 1500/ L, platelets 59,000/ L. The next most appropriate treatment for this baby is to administer
A. B. C. D. E.
E. TORCH infection
Question 52
Question 53 A baby presents in heart failure at 5 days of age. The baby is not centrally cyanosed. The differential diagnosis includes (multiple choices)
A. Atrial septal defect B. Fallots tetralogy C. Hypoplastic left heart D. Coarctation of the aorta
E. Tricuspid atresia
Question 54 A well baby has difficulty sucking. Causes for this could be (multiple choices)
A. Micrognathia
B. Dystrophia myotonica
C. Cleft palate D. Prader-Willi syndrome E. Bulbar palsy
Question 55 The causes for failure to pass meconium in the first 24 hours of life include (multiple choices)
A. Cystic fibrosis
B. Hyperthyroidism C. Hirschsprungs disease D. Hiatus hernia E. Galactosemia
Question 56 Polycythemia in the neonatal period is associated with the following Except
A. Trisomy 21
Question 57 A preterm baby of 30 weeks gestation is intubated and ventilated. On the 2nd day of life he suddenly deteriorates. The differential diagnosis includes (multiple choices)
A. An intraventricular hemorrhage B. A blocked endotracheal tube C. A pulmonary hemorrhage D. Pneumonia
E. Self-extubation
Question 58 The blueberry muffin appearance in infants with TORCH infections most likely represents
A. Dermal erythropoiesis B. Palpable purpura C. Metastatic hepatic tissue D. Viral lesions
Question 60
When a newborn fails to achieve or sustain the normal decrease in pulmonary vascular resistance at birth, the result is hypoxemic respiratory failure or persistent pulmonary hypertension of the newborn (PPHN). Of the following, the mediator most responsible for normal pulmonary vascular transition at birth is
A. B. C. D. Asymmetric dimethylarginine. Endothelin-1 Leukotriene Nitric oxide
E.
Thromboxane
Question 61
The triggering mechanism that has become the standard of care in neonatal synchronized ventilation involves
A. Airflow trigger
B. Esophageal probe
C. Pressure trigger D. Surface capsule E. Thoracic impedance
Question 62
The clinical and ultrasonographic feature most consistent with the compensated phase of fetal hypoxemia is
A. Abnormal biophysical profile
B. Absent fetal weight gain C. Diminished cardiac ventricular compliance
Question 63
Harlequin color changes is a sign of
A. Congenital ichthyosis B. TORCH infection C. Erythroderma D. Normal physiology
Question 65 A 2-day-old is noted to have conjunctival and retinal hemorrhage. The most likely etiology is
A. Child abuse B. Maternal alloimmune thrombocytopenia C. Maternal idiopathic thrombocytopenic purpura
Question 66
Inhaled nitric oxide (iNO) has many of the characteristics of an ideal selective pulmonary vasodilator and, therefore, is used widely in the treatment of PPHN. Of the following, the lung disease most resistant to iNO is
A. B. C. Bacterial pneumonia Congenital diaphragmatic hernia Idiopathic PPHN
D.
E.
Question 67 The most common cause of nutritional intrauterine growth restriction is maternal
A. Chronic disease
B. Drug addiction
C. Hypertension D. Infection E. Malnutrition
Question 68
Fetal undernutrition can have long-term effects on the occurrence of chronic adult diseases, a phenomenon described as fetal programming. Of the following, in addition to coronary heart disease and hypertension, the most common chronic adult disease attributed to fetal programming is
A. B. C. D. E. Chronic obstructive pulmonary disease Diabetes mellitus Malignancy Osteoporosis Retinal degeneration
Question 69 A baby with Apgar scores of 1 and 2 at 1 and 5 minutes, respectively, appears hyperalert and has hyperactive deep tendon reflexes and mydriasis. The most likely diagnosis is
A. Stage I hypoxic-ischemic encephalopathy B. Stage II hypoxic-ischemic encephalopathy C. Stage III hypoxic-ischemic encephalopathy D. Kernicterus
E. Intraventricular hemorrhage
Question 70
The most typical inspiratory-to-expiratory time used with high-frequency oscillatory ventilation is
A. 1:2
B. 1:3 C. 1:4 D. 1:5 E. 1:6
Question 72
The clinical manifestation most predictive of intestinal necrosis in necrotizing enterocolitis is:
A. Abdominal tenderness
B. Bloody stools
C. Erythema of the abdominal wall D. Gastric residuals E. Hemodynamic instability
Question 73
A 2-week old preterm neonate (30 weeks) is experiencing recurrent episodes of apnea. The infant is well-oxygenated in room air between episodes and has no clinical or radiographic evidence of lung disease. Mechanical ventilation using the strategy of volume guarantee is started. The most appropriate positive end-expiratory pressure in this infant is A. B. C. D. E. 0 cm H2O 3 cm H2O 5 cm H2O 8 cm H2O 10 cm H2O
Question 74
Many identifiable factors contribute to human preterm birth. Of the following, the largest single category of causes associated with human preterm birth is
A. Idiopathic preterm labor B. Multiple gestation pregnancy C. Placental abruption D. Prenatal infection
E. Substance abuse
Question 75 The most likely syndrome in an infant who has thrombocytopenia and thumb anomalies is
A. Down syndrome
B. Fanconi syndrome C. Kasabach-Merritt syndrome
Question 76
Transient neonatal diabetes mellitus is a rare disorder of genomic imprinting. Its clinical presentation includes intrauterine growth restriction, failure to thrive, hyperglycemia, and dehydration. Of the following, the imprinting defect in transient neonatal diabetes mellitus is most likely to involve A. B. C. Chromosome 6 Chromosome 7 Chromosome 11
D.
E.
Chromosome 15
Chromosome 21
Question 77 The plasma constituents most commonly implicated in the pathogenesis of transfusion-related acute lung injury are
A. ABO alloantibodies
B. Anti-IgA antibodies
C. Anti-neutrophil and anti-HLA antibodies D. Anti-T agglutinins E. Inflammatory interleukins
Question 78 The first time the human fetus demonstrates swallowing ability is by the gestational age of
A. 11 weeks B. 13 weeks C. 15 weeks D. 17 weeks E. 19 weeks (Sucking at 18-20 weeks)
Question 79 The function that plays a major role in regulation of the amniotic fluid volume is
A. Fetal lung fluid secretion.
B. Fetal swallowing
C. Fetal urine formation D. Intramembranous absorption E. Transmembranous flux
Question 80 Direct-reacting hyperbilirubinemia on the 10th day of life suggests all of the following Except
A. Cystic fibrosis
B. Galactosemia
C. Neonatal hepatitis D. Byler syndrome E. Gilbert disease
Question 81
The ventilator variable most influential in avoiding atelectasis in mechanically ventilated neonates is
A. Inspiratory time
Question 82
Nearly all preterm neonates are most likely to have passed meconium by the postnatal age of
A. 48 hours B. 96 hours C. 144 hours D. 192 hours E. 240 hours
Question 83
The most accurate statement regarding gastrointestinal development and enteral feeding in preterm infants is that
A. B. C. D. E. Diluted milk empties more slowly from the stomach than does undiluted milk Feeding intolerance is largely due to immaturity of mucosal function rather than motor function Gastric emptying is influenced by the temperature of the milk Large feeding volumes induce maturation of motor patterns of the gut more effectively than small feeding volumes Motor function develops earlier in the stomach/small intestine than in the rectum/anal canal
Question 85
The most critical determinant of ventilator-induced lung injury in preterm neonates is excessive
A. Airway pressure
B. Gas flow C. Oxygen concentration D. Tidal volume E. Time constant
Question 86 The postmenstrual age at which preterm infants who have stable cardiopulmonary status and no neurologic abnormalities are likely to be introduced to oral feeding is closest to
A. 28 weeks B. 30 weeks C. 32 weeks D. 34 weeks E. 36 weeks
Question 87 All of the following are problems of an infant of a recently diagnosed diabetic mother Except
A. Hypoglycemia
B. Hypocalcemia C. Intrauterine growth restriction D. Hypomagnesemia E. hyperbilirubinemia
Question 88
The most critical factor in the development of respiratory distress syndrome in the preterm neonate is
A. Immature composition and biophysical function of surfactant Leaking epithelium/endothelium barrier from lung injury Low lung gas volume with susceptibility to overdistention Low surfactant lipid pool size Saccular versus alveolar stage of lung development
B.
C.
D. E.
Question 89 A newborn baby has profound thrombocytopenia. The causes include all of the following Except
A. Cytomegalovirus infection
Question 91 A severely hydropic infant is about to be delivered. Complications to be prepared for include all of the following Except
A. Pulmonary hypoplasia
B. Abdominal ascites
C. Polycythemia D. Heart failure E. Laryngeal edema
Question 92 You are called to see a tow-day-old jittery baby. The causes to consider are (multiple choices)
A. Maternal diabetes B. Fetal alcohol syndrome C. Maternal hyperparathyroidism D. Maternal thyrotoxicosis E. Congenital adrenal hyperplasia
Question 93 The distribution of surfactant in the lungs is most efficient when surfactant is administered
A. After a period of mechanical ventilation B. As an aerosolized preparation C. At a slow rate of infusion D. At birth in the presence of fetal lung fluid E. Using a smaller volume of the drug
Question 94 Infants at risk of hyperinsulinemic hypoglycemia include all of the following Except
A. Infants with nesidioblastosis B. Infants of diabetic mothers C. Infants with galactosemia D. Infants with leucine sensitivity with hyperammonemia E. Infants with Beckwith-Wiedemann syndrome
Question 95
Question 97 The most common limb defect associated with amniotic bands is
A. Amputation of digits
B. Congenital arthrogryposis C. Preaxial polydactyly D. Proximal syndactyly E. Single bones in extremities
Question 98 The aromatic amino acid most likely to be detected by tandem mass spectrometry is
A. Citrulline
B. Leucine C. Methionine D. Phenylalanine E. Valine
incompatibility
E. Turner syndrome
Question 100
The blood constituents most implicated in the pathogenesis of transfusion-associated graft versus host disease are
A. B. C. D. E. Anti-neutrophil antibodies Anti-T agglutinins Donor-derived lymphocytes Erythrocytic RhD antigens Inflammatory interleukins