Most Missed topics Board Review week of June

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Most Missed topics Board Review week of June 14
Question 1 (39 from 2009) most missed – clinical features of glycogen storage disease (this
question deals with management of glycogen storage disease. Would you have recognized it?)
Answer B – corn starch
The hepatomegaly, severe fasting hypoglycemia, lactic acidosis, hyperuricemia, hyperlipidemia, and
ketonuria described for the thin child in the vignette are most consistent with glycogen storage
disease type I (GSD I) (von Gierke disease). GSD I is an autosomal recessive disorder resulting
from deficiency of the enzyme glucose-6-phosphatase, and it is the most serious of all the hepatic

The laboratory findings result from complete blockage of the release of glycogen. Affected children
typically have massive hepatomegaly without splenomegaly on physical examination, and they may
have a wasted appearance. Kidneys are enlarged and may be palpable on examination. Parents may
give a history of irritability and pallor, especially prior to feedings (after fasting). Some of the
children develop seizures.

The mainstays of treatment for GSD I are the avoidance of fasting and frequent administration of
free glucose. The approaches that have been most successful include continuous nocturnal
nasogastric or gastrostomy feedings or administration of uncooked cornstarch every 4 hours during
sleep or other times of fasting. Maintenance of euglycemia reverses clinical and biochemical
abnormalities in most patients.

Coenzyme Q often is administered to individuals who have mitochondrial disorders and is of unclear
benefit, but it plays no role in the management of GSD I. Similarly, carnitine supplementation and
protein and long-chain fat restrictions are of no benefit in GSD I.

The management of disorders of carbohydrate metabolism, regardless of their cause, is aimed at
ensuring the availability of energy for cellular metabolism without compromising necessary fat and
protein stores. This requires frequent delivery of carbohydrates, and gastrostomy tube placement or
venous access may be necessary to ensure success.

American Board of Pediatrics Content Specification:

       Know how to manage carbohydrate metabolic disorders (eg, glycogen storage diseases, type I)

Question 2 (140 from 2010) answer B tube the kiddo!

The sudden deterioration and development of toxicity 5 days after the onset of an apparent
crouplike illness described for the boy in the vignette is most consistent with development of
bacterial tracheitis. The stridor, retractions, and decreased oxygen saturation all are consistent with
this diagnosis. The rapidity of symptom progression and potential for increased purulent secretions
to obstruct the airway mandate early endotracheal intubation to maintain and clear the airway.
Parenteral antibiotic therapy, including coverage for Staphylococcus aureus, is another appropriate
component of treatment. Of note, bacterial tracheitis is a potential complication of measles
infection. A prolonged period of intubation may be necessary until the secretions clear with
antimicrobial therapy.

Although dexamethasone may be indicated for treatment of viral croup, the duration of illness and
acute deterioration described for this boy argue for an alternative diagnosis. A lateral neck
radiograph and pulmonary function testing are not helpful in the diagnosis of bacterial tracheitis and
might be dangerous in a potentially critically ill patient by delaying antibiotic therapy and airway
control. An emergency tracheostomy is not indicated if endotracheal intubation can be accomplished
successfully. However, a tracheostomy may be necessary and possibly lifesaving in the presence of
a fixed obstruction in the upper airway, such as epiglottitis, to bypass the lesion. Epiglottitis
typically presents acutely, with rapid progression over hours from onset of symptoms to toxicity and
impending airway obstruction. Fortunately, the advent of the conjugate Haemophilus influenzae
type b vaccine has made acute epiglottis an extremely rare entity.

American Board of Pediatrics Content Specification:

       Know the typical clinical course of bacterial tracheitis, including biphasic illness, precipitous worsening,
        requirement for intubation, and relatively prolonged intubation

Question 3 (148 from 2008) answer C bacterial tracheitis

Bacterial tracheitis is a serious infection causing significant upper airway obstruction. It typically
occurs as a secondary staphylococcal infection following viral laryngotracheobronchitis, but it also
may develop as a primary infection without preceding symptoms of croup. Clinical features include a
toxic appearance, with high fever, tachypnea, and brassy cough, as described for the girl in the
vignette. Airway obstruction can be pronounced due to purulent airway secretions, and lower airway
disease such as wheezes or rales also may be present. Neck radiographs often reveal a ragged air
column or subglottic narrowing, and peripheral white blood cell counts frequently are elevated.
Treatment consists of intravenous antibiotics and airway control. Endotracheal intubation frequently
is required until the infection is treated and the severity of secretions improves.

Acute viral laryngotracheitis typically causes a barking cough with occasional inspiratory stridor, but
affected children usually do not have high fevers or appear ill. Angioneurotic edema is a response to
histamine release with an allergic reaction and often is accompanied by urticaria. Upper respiratory
tract mucosae become edematous, so inspiratory stridor may be present, but fever is unlikely.
Children who have epiglottitis appear toxic and have significant airway compromise and inspiratory
stridor due to the swollen epiglottis. Cough is rare. Lateral neck radiograph reveals an enlarged
epiglottis ("thumb sign") (Item C148A) rather than a ragged air column. Further, epiglottitis is
unlikely in a child whose immunizations are up to date. A retropharyngeal abscess can develop with
pharyngitis, and inspiratory stridor and respiratory distress with high fever is typical. The lateral
neck radiograph reveals widening of the prevertebral tissues (Item C148B), and computed
tomography scan of the neck can confirm the presence of the abscess (Item C148C).

Question 4 (28 from 2008) answer A bacterial tracheitis
Although the question looks the same as above, the answer discusses the offending organism in more
detail. Learn your code words for tracheitis vs epiglottitis vs croup!!!!
The boy described in the vignette has bacterial tracheitis, a complication of a primary viral illness
caused by a secondary bacterial infection of the upper airway (excluding the epiglottis).
Staphylococcus aureus, Moraxella catarrhalis, nontypeable Haemophilus influenzae, and oral
anaerobes all have been implicated as common causative agents. Mucosal swelling at the cricoid
cartilage plus thick purulent secretions (Item C28A) are responsible for the respiratory distress. The
affected child typically has a high fever, appears toxic, and has a "brassy" cough. Treatment of
bacterial tracheitis includes broad-spectrum antimicrobial agents that have antistaphylococcal
coverage and may require placement of an artificial airway until the airway swelling and copious
purulent secretions improve.

The patient who has epiglottitis usually cannot lay flat, drools, and has dysphagia. In parts of the
world where the H influenzae type b vaccine is used, bacterial tracheitis occurs more frequently
than epiglottitis. Most patients who have laryngotracheobronchitis improve with the administration
of racemic epinephrine, are not highly febrile or toxic-appearing, and are younger than 3 years of
age. Although acute bronchitis commonly is preceded by a viral upper respiratory tract infection, it
is characterized by frequent dry hacking cough in an otherwise nontoxic-appearing patient.
Similarly, patients who have aspirated foreign bodies usually are not febrile and toxic-appearing.

Question 5 ( 113 from 2009) answer c hereditary pancreatitis
The child described in the vignette has a history of at least three episodes of pancreatitis before age
5 years, and gallstones have been excluded by ultrasonography. Children who have hereditary
pancreatitis (HP) can present with recurrent episodes of pancreatitis and often are well between
episodes. In adulthood, affected individuals are at risk for developing pancreatic insufficiency and
Systemic lupus erythematosus (SLE) may be associated with pancreatitis in the context of severe
vasculitis, but SLE is rare before 5 years of age, and the child has no other signs of the condition.
Alpha-1-antitrypsin deficiency and primary sclerosing cholangitis may cause chronic liver disease,
but they rarely affect the pancreas. Colipase deficiency may result in pancreatic insufficiency and fat
malabsorption, but it usually does not cause pancreatic inflammation.

Pancreatitis generally is less severe in children than in adults. A child who has acute pancreatitis
typically presents with epigastric pain and vomiting, and epigastric tenderness can be appreciated
on palpation. More severe pancreatitis can cause intravascular volume depletion, third spacing,
oliguria, pulmonary edema, and pleural effusions. The most common laboratory abnormalities in
mild pancreatitis are elevated amylase and lipase values. Lipase is a more specific enzyme for
pancreatitis; hyperamylasemia also can be seen with salivary gland inflammation, ovarian tumors,
renal insufficiency, diabetic ketoacidosis, and ruptured ectopic pregnancy. Laboratory abnormalities
associated with more severe pancreatitis include hyperglycemia, hypocalcemia, high blood urea
nitrogen and creatinine, low hematocrit, and coagulopathy. In severe cases of pancreatitis,
abdominal computed tomography scan can identify the extent and severity of pancreatic injury.

Some children experience recurrent episodes of pancreatitis without an obvious cause (ie, no history
of medications that can cause pancreatitis, gallstones, trauma, or systemic vasculitis). Anatomic
and genetic causes should be considered in such children. Anatomic causes such as choledochocele,
pancreas divisum, or a long common channel connecting the pancreas and bile ducts can be
identified by either magnetic resonance imaging or endoscopic retrograde

The most common genetic causes of recurrent pancreatitis are cystic fibrosis (CF) and HP. Patients
who have CF and present with pancreatitis often have minimal pulmonary disease and no clinical
signs of pancreatic insufficiency or malabsorption. In some cases, a single mutation in the CFTR
gene may cause a heterozygous carrier for CF to present with recurrent pancreatitis. HP is caused
most commonly by a mutation in the pancreatic cationic trypsinogen gene and is inherited in an
autosomal dominant pattern. Although there often is a family history, some patients have no family
history because a de novo gene mutation has developed.

American Board of Pediatrics Content Specification:

        Recognize the signs, symptoms, and laboratory findings of acute pancreatitis in children

Question 6 (181 from 2006) E thyromegaly
The child described in the vignette has chronic lymphocytic thyroiditis (Hashimotos thyroiditis) without
hypothyroidism. Chronic lymphocytic thyroiditis is an autoimmune endocrine disorder characterized by
invasion of the thyroid by lymphoid cells, which often form follicles. The presence of circulating antibodies
directed against components of the thyroid (eg thyroperoxidase) in an individual who has asymptomatic
thyroid enlargement provides laboratory confirmation of the diagnosis.
The most common clinical finding in Hashimoto thyroiditis is a painless, enlarged, and somewhat firm
thyroid gland that has a slightly pebbly consistency because of invasion of the thyroid gland by lymphoid
follicles. Cervical lymphadenopathy may be an incidental finding but should not be due to autoimmune
thyroiditis. The goiter associated with chronic lymphocytic thyroiditis rarely is large enough to cause
dysphagia. Hypoactive reflexes, a sign of severe hypothyroidism, are noted only in those young people
who have developed hypothyroidism as a result of chronic lymphocytic thyroiditis. Thyroid pain is seen in
subacute and acute thyroiditis, but not in chronic lymphocytic autoimmune thyroiditis.
Chronic lymphocytic thyroiditis has a prevalence of about 1 in 600 adolescents. Antithyroid antibodies (
thyroperoxidase, microsomal, or thyroglobulin) circulate in the blood of most of the affected people and
are diagnostic for the disorder. Occasionally, biopsy proven chronic lymphocytic thyroiditis is antibody
negative. Many people who have thyroid enlargement and positive circulating antibodies eventually
become hypothyroid or even hyperthyroid, but some have persistent thyroid enlargement and positive
antibodies without laboratory signs of thyroid dysfunction.

Question 7 (26 from 2008) C- recheck TSH in 6 months
Hashimoto thyroiditis or chronic lymphocytic thyroiditis is a common autoimmune disorder of the
thyroid, affecting more than 1 in 600 children. It is more common in girls. The diagnostic criterion is
the presence of antithyroid antibodies directed against the thyroid peroxidase (TPO) enzyme or
against thyroglobulin. Pathologic evaluation of the thyroid would reveal the presence of lymphocytic
infiltrates and lymphoid follicles within the thyroid gland. The spectrum of the disorder ranges from
asymptomatic thyroid enlargement associated with lymphoid infiltration and, in most cases, positive
serum antibodies to frank hypothyroidism with an enlarged or atrophic gland or, occasionally,
transient hyperthyroidism. A child who is euthyroid but has positive antithyroid antibodies, such as
the girl described in the vignette, should undergo thyroid function studies, including measurement
of thyroid-stimulating hormone (TSH) and free thyroxine (fT4), at 6-month intervals or if symptoms
of hypo- or hyperthyroidism are recognized. Most affected children eventually develop
hypothyroidism, but this process may take many years and may not occur until adulthood.

TSH is available as a biosynthetic preparation and can be used as preparative therapy before
radioactive iodine ablation or for evaluation for metastasis in individuals who have thyroid cancer,
but it is not used to treat thyroiditis. Triiodothyronine (T3), the active form of thyroid hormone, has
a relatively short half-life, and is produced as needed from T4 by most peripheral tissues. Therefore,
treatment with T3 rarely is indicated. There is some evidence that treatment with T4 may reduce
the size of the thyroid gland in a child who has chronic lymphocytic thyroiditis, even if the TSH value
is normal, but this is still controversial. A thyroid scan using radioactive iodine may show a
characteristic pattern of patchy uptake related to infiltration by lymphoid follicles in the child who
has Hashimoto thyroiditis, but the study is not indicated if the patient is euthyroid and the gland is
symmetric. Radioactive iodine scans should be reserved for the evaluation of thyrotoxicosis and, in
rare circumstances, thyroid nodules. Thyroid ultrasonography would confirm the enlargement of the
thyroid gland. There is a slightly higher risk of thyroid malignancy in patients who have thyroiditis,
but if the gland is smooth and symmetric, there is no indication for this study.

Question 8 (154 from 2009) answer b hashimotos
Approximately 10% of people who have type 1 diabetes develop autoantibodies against the thyroid
or chronic lymphocytic thyroiditis, sometimes referred to as Hashimoto thyroiditis. This is more
common in children in whom the onset of type 1 diabetes occurs before the age of 10 years, such
as the girl described in the vignette. A smaller percentage, perhaps 50% or more of individuals who
have chronic lymphocytic thyroiditis, eventually develop hypothyroidism, and a very small number
may develop other autoimmune thyroid disorders, including Graves disease.

Iodine deficiency is not very common in the United States because iodine is freely available from
sources such as iodized salt, seafood, iodophors used to clean stainless steel milk storage
containers, and sodium alginate thickeners derived from seaweed that are found in many fast foods.
Multinodular goiter would be unusual in an iodine-sufficient child of this age without pre-existing
thyroid problems. On physical examination, a multinodular goiter has a bumpy or irregular firm
surface. Although the thyroid becomes somewhat more palpable during puberty, perhaps because of
increased need for thyroid hormone during rapid growth, it is soft and not firm.

Thyroid autoimmunity may be associated with other autoimmune disorders. Approximately 3% to
4% of individuals who have chronic lymphocytic thyroiditis develop celiac disease. Vitiligo also can
be found. Less frequently, adrenal insufficiency, pernicious anemia, ovarian failure, type 1 diabetes,
or other autoimmune disorders can develop in an individual who initially has chronic lymphocytic
thyroiditis. Some have classified these disorders as polyglandular autoimmune syndromes type 1, 2,
or 3. Type 1 is caused by a known gene defect in AIRE, an immune regulator. Individuals who have
this rare severe autosomal recessive disorder can exhibit candidiasis, hypoparathyroidism, Addison
disease, and pernicious anemia in addition to type 1 diabetes, vitiligo, and other autoimmunities. A
specific underlying gene defect has not been identified in those who have type 2 and type 3
autoimmune endocrinopathies, but these conditions have linkages to human lymphocyte antigen
types related to the autoimmune response. Type 2 autoimmune endocrinopathy includes
autoimmune thyroid disease or diabetes with adrenal insufficiency. The type 3 disorder usually
occurs in adults and is comprised of variable combinations of autoimmune endocrine disorders, but
not adrenal insufficiency.

American Board of Pediatrics Content Specification:

       Know that Hashimoto thyroiditis may be associated with other autoimmune disorders
Question 9 ( 16 from 2006) answer D hypokalemia
The weakness described for the child in the vignette most likely is a result of ypokalemia due to prolonged
vomiting and diarrhea. The child’s intake of fluids that are high in free water and low in sodium and
potassium have contributed to the electrolyte derangement.
The boy has been drinking fluids and has a normal heart rate, which is inconsistent with severe
dehydration. Mild dehydration is unlikely to be the explanation for his marked degree of weakness. His low
sodium fluid intake has the potential to lead to hyponatremia, not hypernatremia. Hypoglycemia could
cause this child’s symptoms, but his intake of sugar containing fluid make this diagnosis less likely. Finally
a well nourished child who experiences a short illness, such as 3 days of vomiting and diarrhea, is not at
significant risk for starvation.
Diarrhea leads to direct loss of potassium through the GI tract. The loss of potassium during vomiting is
not due to high K content in the vomitus; most of the potassium is lost through the kidneys as an indirect
result of ECF volume depletion, which can lead to secondary hyperaldosteronism. Secondary
hyperaldosteronism causes increased sodium retention and increased potassium loss in the distal tubule of
the kidney. In addition, gastric loss of HCl in vomitus leads to alkalosis. Alkalosis can result in increased
bicarbonate delivery to the distal tubule, which also increases urinary potassium loss because the proximal
tubule preferentially reabsorbs sodium with bicarbonate to restore ECF. Both of these mechanisms can be
circumvented by providing normal saline to expand ECF early in the course of vomiting.
An early management priority is to establish the degree hypokalemia. This can be assessed, in part, by
measuring serum potassium, although serum potassium itself does not accurately reflect total body
potassium stores. The combination of serum potassium and serum pH permits an estimate of the total
body potassium deficiet. ECG also should be considered as part of the assessment, although the absence
of ECG changes does not rule out significant potassium losses.
The boy in the vignette, who has symptomatic hypokalemia, should be admitted to the hospital for further
management. If severe symptoms of hypokalemia are present, IV replacement of potassium should be
considered. Initial replacement should be in the form of individual boluses of K (0.2-0.3 mEq/kg) delivered
over 1 hour. If life-threatening arrhythmias or respiratory paralysis is present, potassium infusion rates of
up to 1 mEq/kg per hour may be used with continuous ECG monitoring. Potassium chloride should be used
in the presences of alkalosis, and potassium bicarb should be used in the presence of acidosis.

Question 10 ( 38 from 2009) answer D tuberous sclerosis
Low tone and seizures are relatively common neurologic problems. Low tone is a nonspecific finding
that may be due to disease in the central or peripheral nervous system, but the occurrence of a
seizure suggests a central cerebral cause. Neuroimaging with brain magnetic resonance imaging
(MRI) generally is recommended for any infant who has a seizure because congenital brain
malformations are more common at this age. The combination of such cerebral symptoms and
pigmentary abnormalities of the skin are an indication to obtain brain MRI to assess for a possible
neurocutaneous disorder. The seizures, hypotonia, hypopigmented macules, and MRI findings
described for the child in the vignette are most suggestive of tuberous sclerosis complex (TSC)
(Item C38A).

Incontinentia pigmenti is characterized by spasticity rather than hypotonicity, and skin findings
include swirled hyperpigmentation following the line Blaschko (Item C38B). Skin findings associated
with neurofibromatosis type 1 include café au lait macules (Item C38C), axillary freckling, and iris
Lisch nodules (Item C38D). Although Sturge-Weber syndrome can be associated with seizures in the
first postnatal year, the primary skin finding is a port wine stain (Item C38E). Neither seizures nor
skin abnormalities are seen with von Hippel-Lindau syndrome.

Initial management of this patient centers on the chief complaint, which is the seizure. In most
cases, no treatment is recommended in a child after a first unprovoked seizure. However, the
recurrence risk is much greater in TSC.

Subsequently, diagnostic assessment should be directed toward confirming whether this child has
TSC. In many cases, this is a clinical diagnosis based on the characteristic findings of skin
examination and the cerebral complications. However, many other organs may be involved at
presentation or during the child's lifetime, including the eyes, kidneys, lungs, and heart. In the
future, this child is at risk for developmental learning difficulties, behavior problems that can include
features of autistic spectrum disorders, and malignancies. Given the autosomal dominant
inheritance, proper management involves assessment of the parents and genetic counseling.
Commercial genetic testing is available and is helpful in cases where clinical features, particularly
early in the disease, do not confirm the diagnosis. Genetic testing may have false-negative results
due to mosaicism, ie, some organs may be affected due to TSC1 or TSC2 mutations that are not
present in blood. Given the complexity of this diagnosis, many experts recommend that children
who have TSC be cared for in multidisciplinary specialty clinics.

American Board of Pediatrics Content Specification:

       Recognize the clinical manifestations of tuberous sclerosis, and manage appropriately

Question 11 (40 from 2010) E PCOS
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.

American Board of Pediatrics Content Specification:

       Know the characteristics of adolescents with polycystic ovary syndrome

Question 12 Answer D PPHN
The growth restriction, 42-week gestation, oligohydramnios, and depressed condition requiring
vigorous resuscitation at birth reported for the newborn in the vignette strongly indicate fetal
compromise due to chronic hypoxemia. Although meconium expression does not always equate with
fetal stress and may be a normal finding in many term pregnancies, the risk for meconium
aspiration must be acknowledged when fetal stress is accompanied by abnormal fetal heart rate
findings (bradycardia) and perinatal depression requiring resuscitation. Viewing and suctioning the
trachea is an essential step in this newborn's resuscitation.

Tracheal suctioning may reveal particulate meconium, meconium-stained mucous secretions, or no
meconium. The degree of (any) meconium aspiration cannot be determined by delivery room
suctioning alone. Clinical, biochemical, and radiographic evaluation must follow for the newborn who
has perinatal depression because of the risk for parenchymal lung injury due to aspiration of blood,
amniotic fluid, or meconium (meconium aspiration syndrome); meconium obstruction of the small
and large airways; air-leak syndromes (pneumothorax, pneumomediastinum); and pulmonary
vascular reactivity. The latter is due to hypoxemia and reactive pulmonary vascular constriction,
leading to a condition of pulmonary hypertension.

The difference in pre- and postductal arterial oxygen saturations indicates that the newborn in the
vignette has a right-to-left shunt and persistent pulmonary hypertension of the newborn (PPHN).
The radiograph in the newborn who has PPHN associated with meconium aspiration syndrome
reveals generally hyperinflated lung fields, patchy infiltrates, and varying areas of atelectasis and
hyperaeration (Item C130A).

Congenital diaphragmatic hernia is a defect in the embryologic closure of the diaphragm in which
abdominal contents occupy the thorax and compress lung development (Item C130B). Congenital
pneumonia typically is seen in the presence of prolonged rupture of fetal membranes or
chorioamnionitis with subsequent neonatal respiratory distress and a lobar or diffuse consolidation.
Pneumonia due to group B streptococci may cause respiratory failure in the term newborn and
presents a radiographic picture indistinguishable from respiratory distress syndrome due to
surfactant deficiency in the preterm infant, in which there are low lung volumes, diffuse ground-
glass densities, and air bronchograms (Item C130C). The radiographic appearance of cyanotic
congenital heart disease may show cardiomegaly, pulmonary vascular engorgement, or relative
pulmonary oligemia.

American Board of Pediatrics Content Specification:

       Recognize the clinical presentation of a neonate with persistent pulmonary hypertension following
        meconium aspiration

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