18-10

A venous hum common innocent murmur heard during childhood. Produced by turbulence of
blood in the jugular venous system; no pathologic significance and may be heard in the neck or
anterior portion of the upper part of the chest. A venous hum consists of a soft humming sound
heard in both systole and diastole; it can be exaggerated or made to disappear by varying the
position of the head, or it can be decreased by lightly compressing the jugular venous system
in the neck. These maneuvers differentiate a venous hum from the murmurs produced by
organic cardiovascular disease, particularly a patent ductus arteriosus.
Atrioventricular Septal Defects (Ostium Primum and Atrioventricular Canal or
Endocardial Cushion Defects)
Partial Anomalous Pulmonary Venous Return
One or several pulmonary veins may return anomalously to the superior or inferior vena cava,
the right atrium, or the coronary sinus and produce a left-to-right shunt of oxygenated blood.
Partial anomalous pulmonary venous return usually involves some or all of the veins from only
one lung, more often the right one. When an associated ASD is present, it is generally of the
sinus venosus type (see Chapter 419.3). When a sinus venosus ASD is detected by
echocardiography, one must search for associated partial anomalous pulmonary venous return.
The history, physical signs, and electrocardiographic and roentgenographic findings are
indistinguishable from those of an isolated ostium secundum ASD. Occasionally, an anomalous
vein draining into the inferior vena cava is visible on chest radiography as a crescentic shadow
of vascular density along the right border of the cardiac silhouette (scimitar syndrome); in
these cases, an ASD is not usually present, but pulmonary sequestration and anomalous
arterial supply to that lobe are common findings. Total anomalous pulmonary venous return is a
cyanotic lesion and is discussed in Chapter 424.7. Echocardiography generally confirms the
diagnosis. MRI and electron beam CT are also useful for defining pulmonary venous drainage.
At cardiac catheterization, the presence of anomalous pulmonary veins may be demonstrated
by selective pulmonary arteriography
The prognosis is excellent, similar to that for ostium secundum ASDs. When a large left-to-right
shunt is present, surgical repair is performed. The associated ASD should be closed in such a
way that pulmonary venous return is directed to the left atrium. A single anomalous pulmonary
vein without an atrial communication may be difficult to redirect to the left atrium; if the shunt
is small, it may be left unoperated.
Scimitar syndrome
Anatomic or functional abnormalities of the lungs, diaphragm, and thoracic cage may result in
displacement of the heart to the right (dextroposition). However, in this case the cardiac apex
is pointed normally to the left. This anatomic position is less often associated with congenital
heart lesions, although hypoplasia of a lung may be accompanied by anomalous pulmonary
venous return from that lung (Hypoplasia of right lung, anomalous pulmonary venous
return to inferior vena cava) (in these cases, an ASD is not usually present, but pulmonary
sequestration and anomalous arterial supply to that lobe are common findings.)
Pulmonary Sequestration
A non functioning area of the lung that receives its blood supply from the systemic circulation
The lung tissue in a sequestration receives its arterial supply from the systemic arteries
(commonly off the aorta) and returns its venous blood to the right side of the heart, usually
through the inferior vena cava. As a result, the sequestration functions as a space-occupying
lesion within the chest that does not function in gas exchange and does not contribute to leftto-right shunt. Most sequestrations do not have a communication with the airway, and thus do
not contribute to dead space.
PDA
Thus, a PDA persisting beyond the 1st few weeks of life in a term infant rarely closes
spontaneously or with pharmacologic intervention, whereas if early pharmacologic or surgical
intervention is not required in a premature infant, spontaneous closure occurs in most
instances.
82.10.d
The incidence of congenital heart disease in the normal population is approximately 0.8%
and this incidence increases to 2-6% for a 2nd pregnancy after the birth of a child with
congenital heart disease or if a parent is affected. This recurrence risk is highly dependent on

the type of lesion in the 1st child. When two 1st-degree relatives have congenital heart
disease, the risk for a subsequent child may reach 20-30%. When a 2nd child is found to have
congenital heart disease, it will tend to be of a similar class as the lesion in their 1st-degree
relative (e.g., conotruncal lesions, left-sided obstructive lesions, atrioventricular septation
defects). The degree of severity may be variable, as is the presence of associated defects.
Certain cardiac lesions, for example, left-sided obstructive lesions, may be associated with a
much higher rate of recurrence because of the presence of mild and clinically silent defects in
other family members, such as a bicuspid aortic valve.
85.09.e
Vascular problems other than a diminished radial pulse and occasional long-term
arm length discrepancy are rarely seen in the upper extremity supplied by the subclavian
artery used for the anastomosis.
Physiology of Ebstein anomaly of the tricuspid
valve. Circled numbers represent oxygen saturation
values. Inferior displacement of the tricuspid valve
leaflets into the right ventricle has resulted in a thinwalled, low-pressure "atrialized" segment of right
ventricle. The tricuspid valve is grossly insufficient
(clear arrow). Right atrial blood flow is shunted right
to left across an atrial septal defect or patent foramen
ovale1 into the left atrium. Some blood may cross the
right ventricular outflow tract and enter the
pulmonary artery; however, in severe cases, the right
ventricle may generate insufficient force to open the
pulmonary valve, and "functional pulmonary atresia"
results. In the left atrium, desaturated blood mixes
with saturated pulmonary venous return. Blood enters
the left ventricle and is ejected via the aorta. In this
example, some pulmonary blood flow is derived from the right ventricle, the rest from a patent
ductus arteriosus (PDA). Severe cyanosis will develop in neonates with a severe Ebstein
anomaly when the PDA closes.
1

atrial septal defect - well and truly a defect in atrial septum

patent foramen ovale - due to increased volume or flow in R. atrium functioning foramen ovale
persists(except in probe patency-clinically asymptomatic)
25-10
Acute Poststreptococcal Glomerulonephritis
Pathology
The kidneys appear symmetrically enlarged (As in most forms of acute glomerulonephritis).
light microscopy, all glomeruli appear enlarged and relatively bloodless and show diffuse
mesangial cell proliferation with an increase in mesangial matrix. Polymorphonuclear
leukocytes are common in glomeruli during the early stage of the disease. Crescents and
interstitial inflammation may be seen in severe cases. These changes are not specific for
poststreptococcal glomerulonephritis. Immunofluorescence microscopy reveals lumpy-bumpy
deposits of immunoglobulin and complement on the glomerular basement membrane (GBM)
and in the mesangium. On electron microscopy, electron-dense deposits, or "humps," are
observed on the epithelial side of the GBM.
serum complement (C3)
Clinical Manifestations
Common in 5-12 yr
Uncommon before 3 yr
Antecedent streptococcal pharyngitis 1-2 wk acute nephritic syndrome
Streptococcal pyoderma3-6 wk
Renal involvement - asymptomatic microscopic hematuria with normal renal function to
acute renal failure

hypertension,
oliguria and
edema

nephrotic syndrome may develop in 1020% of cases

+ hypervolaemia
encephalopathy and/or heart failure

directly from the toxic effects of the bacteria on the CNS

Specific symptoms - malaise, lethargy, abdominal or flank pain, and fever - common
Acute subglottic edema and airway compromise.
The acute phase generally resolves within 6-8 wk. Although urinary protein excretion and
hypertension usually normalize by 4-6 wk after onset, persistent microscopic hematuria may
persist for 1-2 yr after the initial presentation.

Causes of Osmotic Diarrhea

.
Malabsorption of water-soluble
nutrients
.

Glucose-galactose malabsorption
Sorbitol
Disaccharidase deficiencies (lactase
Lactulose
and sucrase-isomaltase)
Magnesium hydroxide
Excessive intake of carbonated fluids
Excessive intake of nonabsorbable
solutes
Causes of Secretory Diarrhea

Bacterial
toxins:
enterotoxins
of
.
cholera, Escherichia coli (heat-labile),
Bacterial toxins: Clostridium difficile
Shigella, Salmonella, Campylobacter
enterotoxin
jejuni, Pseudomonas aeruginosa
Neurotransmitters:
acetylcholine,
Hormones:
vasoactive
intestinal
serotonin
peptide, gastrin, secretin
Paracrine agents: bradykinin
Anion surfactants: bile acids, ricinoleic

acid
.
Activation
of
cyclic
adenosine
.
monophosphate
Bacterial toxins: E. coli (heat-stable)
Activation
of
cyclic
guanosine
enterotoxin,
Yersinia
enterocolitica
monophosphate
toxin
Calcium-dependent
Congenital
Acquired

INFANCY
Postgastroenteritis
malabsorption
syndrome
Cow's milk/soy
protein intolerance
Secondary
disaccharidase
deficiencies
Cystic fibrosis

Anatomic
Anal stenosis
Imperforate anus
Anteriorly displaced
anus
Intestinal stricture
(post necrotizing
enterocolitis)

Abnormal
Musculature
Prune belly
syndrome
Gastroschisis

Common Causes of Chronic Diarrhea

CHILDHOOD

Chronic nonspecific
diarrhea
Secondary disaccharidase

deficiencies
Giardiasis

Postgastroenteritis

malabsorption syndrome
Celiac disease

Causes of Constipation
Down syndrome

Intestinal Nerve or
Muscle Abnormalities
Hirschsprung disease
Pseudo-obstruction (visceral
myopathy or neuropathy)
Intestinal neuronal dysplasia

Spinal Cord Defects

Tethered cord
Spinal cord trauma
Spina bifida

Drugs
Anticholinergics

Cystic fibrosis

ADOLES
CENCE
Irritable bowel
syndrome
Inflammatory
bowel disease
Giardiasis
Lactose
intolerance

Narcotics
Antidepressants
Chemotherapeu
tic agents
(vincristine)
Pancreatic
enzymes
(fibrosing
colonopathy)
Lead
Vitamin D
intoxication

Metabolic
Disorders
Hypokalemia
Hypercalcemia
Hypothyroidism
Diabetes mellitus

Intestinal
Disorders

Celiac disease
Cow's milk protein
intolerance
Cystic fibrosis (meconium
ileus equivalent)
Inflammatory bowel disease
(stricture)
Tumor

Connec
tive Tissue
Disorders
Systemic lupus
erythematosus
Scleroderma

Psychia
tric Diagnosis
Anorexia
nervosa

99. 28
Effects of Asphyxia-Systemic Effects
CNS-Hypoxic-ischemic
Adrenal-Adrenal hemorrhage
encephalopathy, infarction,
GIT-Perforation, ulceration with
intracranial hemorrhage, seizures,
hemorrhage, necrosis
cerebral edema, hypotonia,
Metabolic-Inappropriate secretion
hypertonia
of antidiuretic hormone,
CVS-Myocardial ischemia, poor
hyponatremia, hypoglycemia,
contractility, cardiac stun, tricuspid
hypocalcemia, myoglobinuria
insufficiency, hypotension
Integument-Subcutaneous fat
RS-Pulmonary hypertension,
necrosis
pulmonary hemorrhage, respiratory
Hematology-Disseminated
distress syndrome
intravascular coagulation
Renal-Acute tubular or cortical
necrosis
100.39
Maternally derived DENV-neutralizing and E protein-reactive IgG titers declined to below
measurable levels in >90% of infants by 6 months of age. In contrast, IgG reactive with
whole DENV virions persisted until 12 months of age in 20% of infants. Serological
surveillance identified 10 infants with asymptomatic DENV infection for an incidence of
1.7 cases per 100 person-years. DENV-neutralizing antibodies remained measurable for
> or = 1 year after infection. These results suggest that whereas DENV infection in
infants is frequently subclinical, there is a window between 4 and 12 months of age
where virion-binding but nonneutralizing IgG could facilitate antibody-dependent
enhancement.
Antibodies of all four dengue virus serotypes were detected by hemagglutination
inhibition (HI) in 97% of 2,000 infants cord sera at the time of delivery. In comparison
with 250 motherinfants paired sera, we found that 53% of the infants serum HI titers
were higher than those of the mothers. The mother/infant IgG subclasses 1, 2, 3, and 4
titers were 53.1/87.0, 8.4/11.7, 0.14/0.11, and 1.1/1.0 mg/dL, respectively. In 18 months
of follow-up of 100 infants studied, we observed that antibody to dengue virus
disappeared in 3% by two months of age, in 19% by four months of age, in 72% by six
months of age, in 99% by nine months of age, and in 100% by 12 months of age, with a
half-life of 41 days. We conclude that the antibodies to dengue virus disappeared in the
first year of life. We suggest that the most appropriate age for vaccination with a liveattenuated dengue vaccine in an endemic area is one year of age.
To establish the role of maternal dengue-specific antibodies in the development of
dengue hemorrhagic fever and dengue shock syndrome caused by dengue 2 virus in
infants, we examined sera from mothers of infants and toddlers with dengue
hemorrhagic fever or dengue shock syndrome and mothers of infants with pyrexia of
unknown origin. The mean titers of hemagglutination inhibition, neutralization, and
infection-enhancing activities against dengue 2 virus were not statistically different
among the three groups. However, among infants who developed dengue hemorrhagic
fever/dengue shock syndrome there was a strong correlation between the mothers'
dengue 2 neutralizing titers and infant age at the time of onset of severe illness, where
no such correlation was found among the other two groups. Furthermore, the actual age
at which dengue hemorrhagic fever/dengue shock syndrome occurred in each infant
correlated with the age at which maximum enhancing activity for dengue 2 infection in
mononuclear phagocytes was predicted. This critical time for the occurrence of dengue

hemorrhagic fever/dengue shock syndrome was observed to be approximately 2 months

after the time calculated for maternal dengue 2 neutralizing antibodies to degrade
below a protective level. In addition, sera of mothers of infants with dengue
hemorrhagic fever/dengue shock syndrome enhanced dengue 2 virus infection to a
slightly greater degree than did sera from mothers of infants with pyrexia of unknown
origin and toddlers with dengue hemorrhagic fever/dengue shock syndrome. These data
are consistent with the hypothesis that maternal dengue antibodies play a dual role by
first protecting and later increasing the risk of development of dengue hemorrhagic
fever/dengue shock syndrome in infants who become infected by dengue 2 virus.
94.13
Initial Breaths and Assisted Ventilation:
First give five inflation breathes (2-3 sec duration, 30cm H20 inflation pressure). If the
chest wall
moves, move on to ventilation breathes (1-2 sec duration), aiming for 30 breaths/min
until the child is
vigorous. If chest wall does not move with your 1st five breathes:
Recheck position

o Chin support

o Jaw thrust

o Double handed Jaw thrust

Consider using a slighter longer inspiratory time or higher pressure
Consider visualisation of the airway and suction to remove any obstruction.

98.14
Fetal
Severe anemia
(hemolysis, fetalmaternal transfusion,
parvovirus B19-induced
anemia, hypoplastic
anemia)
Supraventricular
tachycardia
Ventricular
tachycardia
Complete heart
block

Premature Neonate
Fluid overload
Patent ductus
arteriosus
Ventricular septal
defect
Cor pulmonale
(bronchopulmonary
dysplasia)

Etiology of Heart Failure

Hypertension
Full-Term Neonate
Asphyxial cardiomyopathy
Arteriovenous malformation (vein of
Galen, hepatic)
Left-sided obstructive lesions (coarctation
of aorta, hypoplastic left heart syndrome)
Large mixing cardiac defects (single
ventricle, truncus arteriosus)
Viral myocarditis

Infant-Toddler
Left-to-right cardiac shunts (ventricular
septal defect)
Hemangioma (arteriovenous malformation)
Anomalous left coronary artery
Metabolic cardiomyopathy
Acute hypertension (hemolytic-uremic
syndrome)
Supraventricular tachycardia
Kawasaki disease
Viral myocarditis

ChildAdolescent
Rheumatic fever
Acute
hypertension
(glomerulonephritis)
Viral myocarditis
Thyrotoxicosis
Hemochromatosi
s-hemosiderosis
Cancer therapy
(radiation,
doxorubicin)
Sickle cell
anemia
Endocarditis
Cor pulmonale
(cystic fibrosis)
Cardiomyopathy
(hypertrophic,
dilated)

Congenital complete AV block in children is most often caused by autoimmune injury of

the fetal conduction system by maternally derived IgG antibodies (anti-SSA/Ro, antiSSB/La) in a mother with overt or, more often, asymptomatic SLE. Rheumatoid arthritis,
dermatomyositis, or Sjgren syndrome is rarely the primary autoimmune process.
Autoimmune disease accounts for 60-70% of all cases of congenital complete heart
block and about 80% of cases in which the heart is structurally normal. Complete heart
block is also seen in patients with complex congenital heart disease, abnormal
embryonic development of the conduction system, myocardial tumors, myocarditis,
myocardial abscess secondary to endocarditis, LQTS, postsurgical repair of congenital
heart disease involving the ventricular septum, and Kearns-Sayre syndrome. The
incidence of congenital complete heart block is 1 in 20,000-25,000 live births . In some
infants of mothers with SLE, complete heart block is not present at birth but develops
within the 1st 3-6 mo after birth. Steroids may be helpful in preventing the development
of complete heart block. The arrhythmia is occasionally suspected in the fetus and may
produce hydrops fetalis.
98.11

A
g
e

0
3
m
o
3
6
m
o
6
9
m
o
9
1
2
m
o
1
3
y

Approximate
Weight Gain

D
ail
y
(g
)

3
0

2
0

1
5

1
2

M
o
nt
hl
y

Le

2
lb

1
1/4 lb

1
lb

1
3 oz

8
oz

3.5

2.0

1.5

1.2

1.0

Growth in
(cm/mo)

Recommend
ed Daily
Allowance
(kcal/kg/day)

Head
Circumfer
ence

2.00

115

1.00

110

0.50

100

0.50

100

0.25

100

r
4
6

6
oz

1 cm/yr

90-100

y
r

There are three circumstances in which the diagnosis of acute rheumatic fever can be
made without strict adherence to the Jones criteria. Chorea may occur as the only
manifestation of acute rheumatic fever. Similarly, indolent carditis may be the only
manifestation in patients who first come to medical attention months after the onset of
acute rheumatic fever. Finally, although most patients with recurrences of acute
rheumatic fever fulfill the Jones criteria, some may not.