The answer is B. Pityriasis rosea typically occurs in children and young adults. It is characterized by an initial herald patch, followed by the development of a diffuse papulosquamous rash. Pityriasis rosea is easier to identify when the general eruption appears with smaller secondary lesions that follow Langer’s lines (cleavage lines) in a “Christmas treelike pattern.” Many diseases can mimic pityriasis rosea including drug exanthems, but the most worrisome condition to be ruled out is secondary syphilis. Topical or systemic steroids and antihistamines are often used to relieve itching. Asymptomatic lesions do not require treatment.

Additional Reading: Pityriasis rosea. Nelson Textbook of Pediatrics, 19th ed. Philadelphia, PA: Elsevier/Saunders; 2011.

Category: Integumentary

The answer is B. Poststreptococcal glomerulonephritis is the leading cause of acute nephritic syndrome. The condition is most frequently encountered in children between 2 and 6 years of age with a recent history of pharyngitis. It is rare in children younger than 2 and adults older than 40. The incidence of poststreptococcal glomerulonephritis appears to be decreasing. The condition typically develops approximately 10 days after pharyngitis or 2 weeks after a skin infection with a nephritogenic strain of group A hemolytic Streptococcus. It has not been determined whether antibiotic treatment of the primary skin infection affords protection from the development of poststreptococcal glomerulonephritis. The classic presentation of poststreptococcal glomerulonephritis is a nephritic syndrome with oliguric acute renal failure. Most patients have milder disease, and subclinical cases are common. Patients with severe disease experience gross hematuria characterized by red or smoky urine, headache, and generalized symptoms such as anorexia, nausea, vomiting, and malaise. Inflammation of the renal capsule can lead to flank or back pain. Physical examination may show hypervolemia, edema, or hypertension. Acute poststreptococcal glomerulonephritis is usually diagnosed on clinical and serologic grounds without the need for biopsy, especially in children with a typical history. The overall prognosis in classic poststreptococcal acute proliferative glomerulonephritis is good. Most patients recover spontaneously and return to baseline renal function within 3 to 4 weeks with no long-term complications.

Additional Reading: Evaluation of poststreptococcal illness. Am Fam Physician. 2005;71:1949-1954.

Category: Nephrologic system

The answer is B. Motor development during the preschool years results in children running, jumping, and climbing. Children learn to balance on one foot and hop. Vocabulary continues to develop rapidly with the mastery of hundreds of words. Language development proceeds with multiword sentences, the use of pronouns, and the gradual improvement in articulation skills. Children normally master the concept of numbers 1, 2, and 3 by 3.5 years. Four-year-old children should know basic colors and clearly articulate most words.

Additional Reading: Screening for developmental delay. Am Fam Physician. 2011;84(5):544-549.

Category: Psychogenic

The answer is A. Either one or both testes may be absent from the scrotum at birth in about one in five premature or low-birth-weight male infants and in 3% to 6% at full-term infants. Cryptorchism is found in 1% to 2% of male children after 1 year of age but can be confused with retractile testes that is associated with a strong cremasteric reflex, which requires no treatment. Cryptorchism should be corrected before age 12 to 24 months in an attempt to reduce the risk of infertility, which occurs in up to 75% of male children with bilateral cryptorchism and in 50% of male children with unilateral cryptorchism. It is not clear, however, whether such early orchiopexy improves ultimate fertility. Some patients have underlying hypogonadism. Cryptorchism is also associated with testicular carcinoma mainly in the undescended testicle and particularly with intra-abdominal malposition; however, up to 10% of cancers can occur on the unaffected side.

Additional Reading: Undescended testis (cryptorchidism). Nelson Textbook of Pediatrics, 19th ed. Philadelphia, PA: Elsevier/Saunders; 2011.

Category: Reproductive system

The answer is C. Testicular torsion should be suspected in patients who complain of acute scrotal pain and swelling. Testicular viability is in jeopardy with delay in diagnosis, ultimately impacting the patient fertility. Associated conditions that may resemble testicular torsion not warranting surgery are torsion of a testicular appendage, epididymitis/orchitis, trauma, incarcerated hernia, varicocele, and idiopathic scrotal edema. Testicular torsion is most common in men younger than 25 years old, although it can occur in men of any age. A prepubertal or young male adult with acute scrotal pain should be diagnosed as testicular torsion until proven otherwise. Physical exam finding of higher testicular lie and absent cremasteric reflex are supportive evidence for this condition. Scrotal imaging with Doppler color flow ultrasound is necessary when the diagnosis remains unclear. Once the correct diagnosis is established, prompt surgical evaluation should be performed. It is reasonable to perform manual detorsion for immediate noninvasive treatment followed by elective orchiopexy.

Additional Reading: Testicular torsion. Am Fam Physician. 2006;74(10):1739-1743.

Category: Reproductive system

The answer is A. Pediculosis capitis or head lice is an infestation of lice in the scalp hair as it feeds on human blood. Modes of transmission are through fomites and head-to-head contact. For some unknown reason, head lice rarely affects African Americans. Head lice can live off the body up to 1 month. The hallmark of all types of pediculosis is pruritus.

There is known resistance of head lice to pyrethroids. Malathion 0.5% in isopropanol is the treatment of choice for head lice. This medication is applied to dry hair and scalp until wet and washed 12 hours after initial application. A reapplication 7 to 9 days after initial treatment may be necessary. Household members should be treated at the same time. Fine-toothed combs are used after application of a damp towel to the scalp for 30 minutes. Washing or dry-cleaning clothing and bed linens is advised. Brushes and combs used should be disposed of by coating with a pediculicide for 15 minutes followed by rinsing in boiling water. Children are cleared to go back to school after the initial treatment. Spinosad (Natroba) is safe and effective for the treatment of head lice for ages 4 years and above; nit combing following treatment is not necessary.

Additional Reading: Pediculosis. Nelson Textbook of Pediatrics, 19th ed. Philadelphia, PA: Elsevier/Saunders; 2011; and Spinosad (Natroba) for head lice. Am Fam Physician. 2013;87(12): 874-876.

Category: Integumentary

The answer is A. Short stature may affect children as a result of intrinsic growth defects or because of acquired extrinsic factors that impair growth. In general, delayed bone age in a child with short stature is suggestive of an endocrinologic or systemic disorder, constitutional delay, and undernutrition. Normal bone age in a short child is more likely to be caused by familial short stature.

Additional Reading: Analysis of growth patterns. Nelson Textbook of Pediatrics, 19th ed. Philadelphia, PA: Elsevier/Saunders; 2011.

Category: Endocrine system

The answer is C. In children older than 6 years, lymphoma is the most common cause. Intussusception is the most common cause of intestinal obstruction in the first 2 years of life. It is more common in boys than in girls. In most cases (85%), the cause is not apparent. Associated conditions that can result in intussusception include polyps, Meckel’s diverticulum, Henoch-Schonlein purpura, lymphoma, lipoma, parasites, foreign bodies, and viral enteritis with hypertrophy of Peyer patches. Intussusception of the small intestine occurs in patients with celiac disease and cystic fibrosis related to the bulk of stool in the terminal ileum. Henoch-Schonlein purpura may also cause isolated small-bowel intussusception. Intermittent smallbowel intussusception is a rare cause of recurrent abdominal pain.

Additional Reading: Gastrointestinal emergencies. The Harriet Lane Handbook: A Manual for Pediatric House Officers, 19th ed. Philadelphia, PA: Elsevier/Mosby; 2012.

Category: Gastroenterology system

The answer is B. Labial adhesions are common in prepubertal girls. The cause is thought related to low levels of circulating estrogen. Most women with small areas of labial adhesions are asymptomatic. However, interference with urination or accumulation of urine behind the adhesion can lead to discomfort and symptoms. Dysuria and recurrent vulvar and vaginal infections are associated symptoms. In rare situations, urinary retention may occur. Asymptomatic labial fusion usually does not require treatment. Symptomatic adhesions may be treated with a short course of estrogen cream applied twice daily for 7 to 10 days; this may separate the labia. A new alternative treatment is to use estrogen transdermal patches in close proximity to the labia. When medical treatment fails or if severe urinary symptoms exist, surgical separation of the labia is indicated. This can be done as an office procedure using 1% to 2% topical xylocaine gel. Because of inadequate levels of estrogen, recurrences of labial adhesion are common until puberty. Following puberty, the condition usually resolves spontaneously. Improved hygiene and removal of vulvar irritants may help prevent recurrences.

Additional Reading: Labial adhesions. Textbook of Family Medicine, 8th ed. Philadelphia, PA: Saunders/Elsevier; 2011.

Category: Reproductive system

The answer is D. The incidence of iron deficiency in children has decreased substantially due to improved nutrition and the increased availability of iron-fortified infant formulas and cereals. Normalterm infants are born with sufficient iron stores to prevent iron deficiency for the first 4 to 5 months of life. Thereafter, sufficient iron needs to be absorbed to maintain the needs of rapid growth. For this reason, nutritional iron deficiency is most common between 6 and 24 months of life. A deficiency earlier than age 6 months may occur if iron stores at birth are reduced by prematurity, small birth weight, neonatal anemia, or perinatal blood loss or if there is subsequent iron loss due to hemorrhage. Iron-deficient children older than 24 months should be evaluated for blood loss. Iron deficiency, in addition to causing anemia, has adverse effects on multiple organ systems. Symptoms and signs vary with the severity of the deficiency. Mild iron deficiency is usually asymptomatic. In infants with more severe iron deficiency, pallor, fatigue, irritability, and delayed motor development are common. Children whose iron deficiency is due in part to ingestion of unfortified cow’s milk may be fat and flabby, with poor muscle tone. A history of pica is common.

Additional Reading: Anemia. The Harriet Lane Handbook: A Manual for Pediatric House Officers, 19th ed. Philadelphia, PA: Elsevier/Mosby; 2012.

Category: Hematologic system

The answer is A. Acute bacterial infection of the sinuses that lasts <30 days and completely resolves is called acute bacterial sinusitis. The maxillary and ethmoidal sinuses most commonly involved when mucociliary function and drainage are impaired by an upper
respiratory infection (URI) or allergic rhinitis. Both the ethmoid and maxillary sinuses are present at birth, forming in the third to fourth gestational month. The sphenoid sinuses pneumatize as an extension of a posterior ethmoid cell by age 5 years, and the frontal sinuses form from an anterior ethmoid cell appearing at about age 7 to 8 years. Frontal sinusitis is unusual before age 10 years.

Additional Reading: Sinusitis. Nelson Textbook of Pediatrics, 19th ed. Philadelphia, PA: Elsevier/Saunders; 2011.

Category: Respiratory system

The answer is B. ADHD is manifest by inappropriate-for-age hyperactivity, impulsivity, and lack of attention. ADHD cannot be easily diagnosed by a specific test or biologic marker, and some are unclear if the disorder is a truly pathologic condition or just one end of the behavioral spectrum. ADHD is more frequently diagnosed in children with behavioral difficulties and academic underachievement. The prevalence of ADHD is between 6.8% and 10.3%, with boys affected threefold more often than girls. Associated psychiatric conditions, including oppositional-defiant disorder, conduct disorder, depressive disorder, and anxiety disorders, are common. The Conners’ ADHD Index and symptom scales from the Diagnostic and Statistical Manual of Mental Disorders, 4th ed. (DSM-IV) are ADHD-specific checklists and have a high sensitivity for identification of children with the disorder. Reviews of the pharmacologic management of ADHD with methylphenidate hydrochloride (Ritalin, Concerta), dextroamphetamine sulfate (Adderall, Dexedrine), and pemoline (Cylert) show these drugs to be generally effective for most children affected. Nonpharmacologic treatments that may have some beneficial effect on behavior and academic performance are behavioral modification and intensive contingency-management therapy. Combining drug therapy with psychosocial therapy shows no clear advantage when compared with drug therapy alone. However, the addition of behavioral therapies to medication may have some benefit, including reduction of anxiety and improvement in social skills. The symptoms of ADHD tend to decrease over the long term, but may continue into adolescence and adulthood. The most common treatment is stimulant medication.

Additional Reading: Current strategies in the diagnosis and treatment of childhood attention-deficit/hyperactivity disorder. Am Fam Physician. 2009;79(8):657-665.

Category: Psychogenic

The answer is E. A diagnosis of “growing pains” must meet three criteria: (1) the leg pain is bilateral; (2) the pain occurs only at night; and (3) the patient has no limp, pain, or symptoms during the day. To inaccurately diagnose a limping child with growing pains can be dangerous, as the physician risks missing the underlying pathology. However, if a child does fit the criteria for growing pains, the parents should be reassured that this is a benign, self-limited process that occurs for unknown reasons.

Additional Reading: Growing pains. Nelson Textbook of Pediatrics, 19th ed. Philadelphia, PA: Elsevier/Saunders; 2011.

Category: Musculoskeletal system

The answer is E. Peritonsillar abscess is most common in persons 20 to 40 years of age. Young children are seldom affected unless they are immunocompromised, but the infection can cause significant airway obstruction in children. The infection affects men and women equally. Chronic tonsillitis or multiple trials of oral antibiotics for acute tonsillitis may predispose persons to the development of a peritonsillar abscess. The presenting symptoms include fever, throat pain, and trismus. Ultrasonography and CT scanning are useful in confirming a diagnosis. Needle aspiration remains the gold standard for diagnosis and treatment of peritonsillar abscess. After performing aspiration, appropriate antibiotic therapy (including penicillin, clindamycin, cephalosporins, or metronidazole) must be initiated. In advanced cases, incision and drainage or immediate tonsillectomy may be required. Streptococcus pyogenes (group A β-hemolytic Streptococcus) is the most common aerobic organism associated with peritonsillar abscess. The most common anaerobic organism is Fusobacterium. For most abscesses, a mixed group of both aerobic and anaerobic organisms cause the infection.

Additional Reading: Peritonsillar abscess. Am Fam Physician. 2008;77(2):199-202.

Category: Respiratory system

The answer is B. JRA, formerly known as Still’s disease, is a diverse group of diseases that is clinically distinct from adult rheumatoid arthritis. Most children with JRA have long remissions without loss of function or significant residual deformity. There are no specific laboratory tests to diagnose JRA. One must exclude other causes for
arthritis, including reactive arthritis from extra-articular infection, septic arthritis, neoplastic disorders, endocrine disorders (e.g., thyroid disease, type 1 diabetes mellitus), degenerative or mechanical disorders, or idiopathic pediatric joint pain. Diagnosis of JRA requires signs of inflammation rather than simply arthralgias persisting for more than 6 weeks, with onset before age 16 years. JRA has three major subtypes: pauciarticular (40% to 50%), polyarticular (25% to 40%), and systemic (10% to 20%). Each type has different clinical presentations and courses, and treatment is determined by differentiating among the various types. Pauciarticular JRA involves four or fewer joints, usually large joints asymmetrically. Early-onset pauciarticular JRA affects mostly girls younger than 4 years and has a 30% risk of chronic iridocyclitis and a 10% risk of ocular damage. Late-onset pauciarticular JRA affects mostly boys older than 8 years; many of whom later develop spondyloarthropathies; 10% develop iridocyclitis. Slit-lamp ophthalmic examinations are recommended. Polyarticular JRA is defined as arthritis in five or more joints; patients are either RF positive or RF negative. RF-positive patients usually are girls aged 8 years or older, have symmetric small-joint arthritis, and have a worse prognosis than do RF-negative patients. Systemic-onset JRA is characterized by high intermittent fevers (>102°F), rash, hepatosplenomegaly, lymphadenopathy, arthralgias, pericarditis, pleuritis, and growth delay. Anemia, leukocytosis, and thrombocytosis are common laboratory findings. Extra-articular symptoms are usually mild and self-limited. Boys and girls are equally affected. NSAIDs are the first-line treatment for JRA. Clinical improvement may not be seen for up to 1 month. Methotrexate is often used with NSAIDs, particularly for systemic and polyarticular JRA. Corticosteroids are used orally for severe, life-threatening, systemic JRA and intra-articularly for pauciarticular JRA. Most children with JRA require a great deal of physical as well as psychological support. Physical and occupational therapy is important because children often stop using painful joints, adding to disability.

Additional Reading: Rakel R. Juvenile rheumatoid arthritis. Textbook of Family Medicine, 8th ed. Philadelphia, PA: Saunders/Elsevier; 2011.

Category: Musculoskeletal system

The answer is A. Early treatment of asthma exacerbations is important to prevent progression to severe disease. First-line treatment should be with a short-acting inhaled β2-agonist such as albuterol; two to four puffs from a metered-dose inhaler can be given every 20 minutes up to three times, or a single treatment can be given by nebulizer. If the response is acceptable as assessed by sustained symptom relief or improvement in PEFR to more than 80% of the child’s best, the short-acting β2-agonist can be continued every 3 to 4 hours for 24 to 48 hours. For patients taking inhaled corticosteroids, the dose may be doubled for 7 to 10 days. If the child does not completely improve from the initial therapy, with PEFR between 50% and 80%, the β2-agonist should be continued, and an oral corticosteroid should be added. If the child experiences marked distress or if PEFR persists below 50%, the patient should repeat the β2-agonist immediately. Further emergent treatment may be necessary.

Additional Reading: Rakel R. Asthma. Textbook of Family Medicine, 8th ed. Philadelphia, PA: Saunders/Elsevier; 2011.

Category: Respiratory system

The answer is C. Practice guidelines from the American Academy of Pediatrics (AAP) recommend renal ultrasonography in all children 2 months to 2 years of age with a documented first UTI. VCUG is no longer routinely recommended. VCUG is warranted if renal and bladder sonogram reveal hydronephrosis, scarring, presence of findings suggestive of high-grade vesicoureteral reflux (VUR) or obstructive uropathy, or clinical scenarious considered to be complex or atypical. After the age of 2 years, some controversy exists. Some authorities recommend postponing workup for the first UTI in girls.

Additional Reading: Urinary tract infection: clinical practice guideline for the diagnosis and management of the initial UTI in febrile infants and children 2 to 24 months. Pediatrics. 2011;128(3):595-610.

Category: Nephrologic system

The answer is E. Motor vehicle crashes continue to be the leading cause of death in children 1 to 14 years of age. Properly used child safety seats significantly reduce child morbidity and mortality. Although many parents know child safety seats are important, >80% of seats are misused. Children should sit in the backseat. The rearfacing position should be used until the child is 1 year of age and weighs 20 lbs (9 kg). Forward-facing child seats face forward and are for children heavier than 20 lbs and older than 1 year. A child outgrows this seat when the ears are above the back of the seat or when the child passes the height or weight limit of the seat (usually 40 lbs). High-back booster seats face forward and have removable harnesses.
They are meant for use with children heavier than 20 to 30 lbs (9 to 13.5 kg), depending on the manufacturer, and older than 1 year. The high back protects the head and neck in a rear-end collision. The harness should be used until the child exceeds the weight limit of the harness system (usually 40 lbs). Once the child is heavier than 40 lbs, the harness is removed, and the seat is used to position the vehicle seatbelt correctly (over the midclavicle and midchest, and tight over the upper thighs). High-back belt-positioning booster seats boost the child up so that the vehicle seatbelt fits correctly. They can only be used with a shoulder-lap belt system. High-back booster seats are for use with children heavier than 40 lbs and can be used until the child fits properly in the vehicle seatbelt system. Because safer restraint systems are available for children weighing more than 40 lbs, the use of low-back booster seats is not recommended. To properly fit a child in a standard car seatbelt, three elements must be present: (1) the child’s legs should bend over the edge of the auto seat with the buttocks against the seat back; (2) the shoulder portion of the belt should be over the midclavicle and center of the chest; and (3) the lap belt should be tight over the upper thighs or the pelvis. A child should have a sitting height of 29 in. (74 cm) to have a proper seatbelt fit. This sitting height roughly correlates to a standing height of 58 in. (147 cm) and a weight of 81 lbs (36.5 kg). Increased education of parents regarding proper use of child safety seats can protect children from potentially fatal crash forces. Parents may also be educated about community resources and the several types of child safety seats.

Additional Reading: New recommendations on motor vehicle safety for child passengers. Am Fam Physician. 2013;87(7): 472-474.

Category: Patient/population-based care

The answer is C. Vomiting is the first manifestation of rotavirus in the majority of patients, followed within 24 hours by low-grade fever and repeated bouts of watery diarrhea. Diarrhea usually lasts 4 to 8 days but can last longer in young infants or immunocompromised patients. The WBC count is rarely elevated. As patients become dehydrated from unreplaced fecal water loss, they may become hypernatremic. The stool does not contain blood or white cells. Metabolic acidosis results from bicarbonate loss in the stool, ketosis from poor intake, and lactic acidemia from hypotension and hypoperfusion. Replacement of fluid and electrolyte deficits and ongoing losses is critical, especially in small infants. The use of oral rehydration fluid is appropriate in most cases. The use of clear liquids or hypocaloric (dilute formula) diets for more than 48 hours is not advisable in uncomplicated viral gastroenteritis because starvation depresses digestive function and prolongs diarrhea. Intestinal lactase levels are reduced during rotavirus infection. Brief use of a lactose-free diet is associated with a shorter period of diarrhea but is not critical to successful recovery in most healthy infants. Reduced fat intake during recovery may reduce nausea and vomiting. Antidiarrheal medications are not effective and can be dangerous (loperamide, tincture of opium, diphenoxylate with atropine). Bismuth subsalicylate preparations may reduce stool volume but are not necessary for recovery. Specific identification of rotavirus is not required in every case, especially in outbreaks. Rotavirus antigens can be identified in stool. False positives (which may actually be nonpathogenic rotavirus) are seen in neonates. Some immunity is imparted by the first episode of rotavirus infection. Repeat infections occur but are usually less severe. Prevention of rotavirus is mainly by good hygiene and prevention of fecal-oral contamination. In July 1999, the AAP recommended suspending the use of oral rotavirus vaccine in the United States because of its association with intussusception in the first 3 weeks following vaccine administration. Recently, newer vaccines have been developed with an improved safety record.

Additional Reading: Rotavirus. From: http://www.cdc.gov/rotavirus/

Category: Gastroenterology system

The answer is C. Early sexual activity can have a substantial negative impact on adolescents. Currently in the United States, more than 900,000 teenagers become pregnant each year. Those who give birth tend to have more academic deficiencies, poorer socioeconomic outcomes, and repeat pregnancies, and they are more likely to be single parents. In addition, adolescents who engage in early sexual activity expose themselves to STDs. Of all STD cases reported in the United States, more than two-thirds occur in adolescents and young adults. Prevention strategies should be established to reduce early sexual activity in adolescents.

Additional Reading: Sexual activity. Nelson Textbook of Pediatrics, 19th ed. Philadelphia, PA: Elsevier/Saunders; 2011.

Category: Patient/population -based care

The answer is C. The majority of seizures in children <5 years are febrile type seizures, and children with a positive family history have a higher incidence. A febrile seizure is defined as any seizure occurring in a child who is 6 months to 5 years of age accompanied by a current or recent fever (at least 38°C [100.4°F]) and without previous seizure or neurologic events. Febrile seizures are typically categorized as simple or complex. Simple febrile seizures are characteristically generalized, usually last <15 minutes, and occur only once in a 24-hour period. Complex febrile seizures may have focal features, last >15 minutes, and recur within a 24-hour period. Viral infections are often present with febrile seizures, with human herpes virus 6 and 7 and influenza A and B being important causes. There is
also a significant increased risk of febrile seizures within 24 hours of receiving vaccination for diphtheria and tetanus toxoids and wholecell pertussis, and within 8 to 14 days of receiving a measles, mumps, and rubella (MMR) vaccination. The risk of recurrent febrile seizures is increased in patients whose initial febrile seizure occurred at <12 months of age, patients with a lower rectal temperature at first seizure (<40°C [104°F]), patients with shorter duration of fever before their first seizure (<24 hours), patients with a family history of febrile seizures, and patients with complex features with the first febrile seizure. The risk of development of epilepsy is slightly higher among persons having simple febrile seizures but is significantly increased among those who have one or more complex febrile seizures. Initial evaluation of children with febrile seizure includes airway and circulatory support, ideally with noninvasive measures until the postictal state resolves. A thorough medical history that includes past seizures and other neurologic conditions, exposure to medications or toxins, allergies, or trauma may point to a specific seizure cause. Treatment with antipyretics is rarely necessary in the typical seizure case. Patients with seizures that last longer than 5 minutes should receive benzodiazepines to control the seizure. After the seizure ends, the physician should conduct a mental status examination and a physical evaluation. Routine laboratory studies include only a blood glucose test; an electrolyte test may be appropriate if a metabolic abnormality is being considered.

In most cases no further workup is necessary, but lumbar puncture is indicated in patients with suspected meningitis. A lumbar puncture should be considered in children younger than 12 months who have a febrile seizure as other signs of infection may not be evident. For children 12 to 18 months of age, clinical signs and symptoms of meningitis may be subtle, necessitating a lumbar puncture. Lumbar puncture is similarly indicated when a child greater than 18 months with neck stiffness, Kernig, or Brudzinski signs or a clinical picture pointing to an intracranial infection.

Neuroimaging only is appropriate in patients at risk of cerebral abscess, in those who have clinical evidence of increased intracranial pressure, in patients who have evidence of trauma, or in patients who have status epilepticus or have had a complex seizure. Children with simple febrile seizures can be cared for at home after providing parental education and making plans to follow up with the family. Routine prophylaxis using phenobarbital, valproic acid, oral diazepam, or antipyretics is controversial and usually not indicated.

Additional Reading: Febrile seizures. Nelson Textbook of Pediatrics, 19th ed. Philadelphia, PA: Elsevier/Saunders; 2011; and

Evaluation and management of febrile seizures in the out-ofhospital and emergency department settings. Ann Emerg Med. 2003; 41:215-222.

Category: Neurologic

The answer is A. Teenagers are now entering puberty earlier because of various factors, including better nutrition and improved socioeconomic conditions. In the United States, the average age at menarche is 12.16 years in African American girls and 12.88 in White girls. However, menarche may be delayed until age 16 years or may begin as early as age 10. The first objective sign of puberty in girls is the beginning of the height spurt. This is followed by development of breast buds between ages 8 and 11 years. Although breast development usually precedes the growth of pubic hair, in some girls the sequence may be reversed. Among girls, the growth spurt starts at about age 9 years and reaches a peak at age 11.5 years. The spurt usually ends by age 14 years. Girls who mature early reach their peak height velocity sooner and attain their final height earlier. Girls who mature late attain a greater ultimate height because of the longer period of growth before the growth spurt. Final height is related to skeletal age at onset of puberty as well as genetic factors. The height spurt correlates more closely with breast developmental stages than with pubic hair stages.

Additional Reading: Physiology of puberty. Nelson Textbook of Pediatrics, 19th ed. Philadelphia, PA: Elsevier/Saunders; 2011.

Category: Reproductive system

The answer is D. Group A beta-hemolytic streptococcal pharyngitis, scarlet fever, and in rare cases asymptomatic carrier states are associated with poststreptococcal complications. Children are most commonly affected in streptococcal pharyngitis, acute rheumatic fever, pediatric autoimmune neuropsychiatric disorders associated with streptococcal infection, and poststreptococcal glomerulonephritis. The hallmarks of rheumatic fever include arthritis, carditis, cutaneous disease, chorea, and subsequent acquired valvular disease. Pediatric autoimmune neuropsychiatric disorders include a subgroup of illnesses involving the basal ganglia in children with obsessive-compulsive disorders, tic disorders, dystonia, chorea encephalitis, and dystonic choreoathetosis. Poststreptococcal glomerulonephritis occurs most frequently in children between 2 and 6 years of age with a recent history of pharyngitis and a rash during the winter months.

The clinical examination of a patient with possible poststreptococcal complications should include an evaluation for signs of inflammation (i.e., CBC, erythrocyte sedimentation rate, C-reactive protein) and evidence of a preceding streptococcal infection. Antistreptolysin O titers should be obtained to confirm a recent invasive streptococcal infection. Other important antibody markers include antihyaluronidase, antideoxyribonuclease B, and antistreptokinase antibodies.

Additional Reading: Evaluation of poststreptococcal illness. Am Fam Physician. 2005;71:1949-1954.

Category: Nonspecific system

The answer is B. Rheumatic fever is complication of acute group A streptococcal pharyngitis that presents as an acute systemic febrile illness. Associated findings include a migratory arthritis involving the large joints, signs and symptoms of carditis and valvulitis, the erythema marginatum rash, subcutaneous nodules, and choreoathetotic movements of Sydenham’s chorea. Damage to the cardiac valves may be chronic and progressive, resulting in significant cardiac dysfunction. Although the Modified Jones Criteria help with the clinical diagnosis, no specific symptoms, clinical signs, or laboratory tests are pathognomonic for rheumatic fever. Additionally, not all patients with rheumatic fever fulfill the Modified Jones Criteria. The criteria consist of major manifestations that include carditis, erythema marginatum, polyarthritis, subcutaneous nodules, and Sydenham’s chorea. Minor manifestations include clinical (e.g., arthralgia, fever) and laboratory (e.g., elevated C-reactive protein and erythrocyte sedimentation rate, prolonged PR interval on electrocardiograph) findings. A diagnosis of rheumatic fever is supported by evidence of preceding group A streptococcal infection (i.e., positive throat culture or rapid streptoccocal antigen test, elevated or rising antistreptolysin titer), and the presence of two major manifestations or of one major and two minor manifestations. Arthritis is the most frequent and least specific manifestation of rheumatic fever. It usually affects the large joints and may be the first sign of illness. The lower extremities generally are affected first, followed by the upper extremities. Joint involvement occurs early in the illness and is more common and severe in younger patients. The arthritis may be painful, but it is transient; the inflammation lasts about 2 to 3 days in each joint and 2 to 3 weeks in total. Radiographic evaluation can show slight joint effusions but usually the results are normal. The arthritis is self-limited, resolves without complications, and is treated with salicylates and NSAIDs. Carditis associated with rheumatic fever presents as pericarditis, myocarditis, and, most commonly, endocarditis. Pericarditis can present with chest discomfort, pleuritic chest pain, pericardial friction rubs, and distant heart sounds. Myocarditis is rare in isolation and can present with signs and symptoms of heart failure. Endocarditis may be asymptomatic or present with a new heart murmur. Cardiac murmurs do not always indicate valvular involvement, and they may be transient. If valvular disease occurs, it is most likely in the mitral, aortic, tricuspid, or pulmonary valve, in that order. Electrocardiograph and echocardiogam abnormalities may be present in about one-third of patients with carditis. Rheumatic heart disease is an important long-term consequence of rheumatic fever and is the major cause of acquired valvular disease internationally. Rheumatic heart disease typically occurs 10 to 20 years after the original rheumatic fever episode. Significant mitral stenosis can occur and require surgery. Sydenham’s chorea is characterized by involuntary movements, muscular weakness, and emotional disturbances. It is usually more marked on one side of the body than the other and may be completely unilateral. Atypical behavior such as crying and restlessness are seen and, in rare cases, features of a psychosis may be noted. There is no sensory loss or involvement of the pyramidal tracts. Sydenham’s chorea is typically self-limited and occurs in <5% of those affected. The condition typically lasts 2 to 3 months. Antistreptococcal prophylaxis should be maintained continuously after an attack of acute rheumatic fever or chorea to prevent recurrences.

Additional Reading: Evaluation of poststreptococcal illness. Am Fam Physician. 2005;71:1949-1954.

Category: Cardiovascular system

The answer is B. Sigmoid volvulus is a rare problem seen in children and adolescents. Volvulus occurs when a floppy sigmoid loop rotates around its base, producing arterial and venous obstruction of the affected segment, followed by rapid distention of the closed loop. Because the consequences can be life-threatening, sigmoid volvulus should be included in the differential diagnosis of acute and recurrent episodes of abdominal pain or bowel obstruction in children, especially if colonic dilation is seen on radiographs. Boys are more commonly affected than girls. Symptoms can be either acute or recurrent. The most common symptoms are abdominal pain that is relieved by passage of stool or flatus, abdominal distention, and vomiting. Radiographic evaluation often reveals colonic dilation. Barium enema often confirms or suggests the diagnosis and should be performed under fluoroscopic control; a “twisted-taper” or “bird’sbeak” appearance of the affected colon is characteristic. The most common associated conditions include Hirschsprung’s disease and imperforate anus. Although sigmoid volvulus can resolve spontaneously, nonoperative management begins with fluid resuscitation and antibiotics, followed by barium enema detorsion of the sigmoid. Other nonoperative modalities include proctosigmoidoscopy and decompression by rectal tube. Operative management most commonly consists of sigmoidectomy.

Additional Reading: Vomiting. Nelson Textbook of Pediatrics, 19th ed. Philadelphia, PA: Elsevier/Saunders; 2011.

Category: Gastroenterology system

The answer is B. The 2004 acute otitis media (AOM) guideline published by the AAP and the American Academy of Family Physicians suggested an “observation option” for selected children and proposed that the initial antibiotic therapy be prioritized according to diagnostic certainty. The recommendation was to prescribe amoxicillin (assuming no penicillin allergy) when a decision to treat with an antibiotic had been made if the child had not been administered amoxiccilin in the previous month or there was no concurrent purulent conjunctivitis.

Antibiotic resistance is growing among the organisms that commonly cause AOM. Associated risk factors for resistant pathogens include recent antibiotic treatment, children in day care facilities, infections occurring in the winter, and AOM in children <2 years of age. Amoxicillin remains the antibiotic of first choice, although a higher dosage (80 mg/kg/day) is recommended to ensure adequate eradication of resistant Streptococcus pneumoniae. Oral cefuroxime (Ceftin) or amoxicillin-clavulanate (Augmentin) and intramuscular ceftriaxone (Rocephin) are suggested second-line choices for treatment failure. Compliance with antibiotic regimens is enhanced by selecting medications that require less frequent dosing (such as one or two times a day) and by prescribing shorter (5 days or less) treatment courses. Selective use of tympanocentesis if the patient does not respond to empiric therapy can help confirm the diagnosis and guide effective therapy.

Additional Reading: The diagnosis and management of acute otitis. Pediatrics. 2013;131(3):e964-e999.

Category: Special sensory

The answer is A. Influenza vaccine (Fluzone, Fluvirin) should optimally be given in October and November, but can be given throughout the influenza season. Unvaccinated children younger than 9 years should be given two doses at least 1 month apart. Children aged 6 to 35 months are given 0.25 mL IM, whereas children aged 3 years and older are given 0.5 mL IM. (Fluvirin is indicated for use in only children aged 4 years and older.) Because vaccine viruses are first grown in eggs, the vaccine is contraindicated in persons with a history of allergy to eggs or egg products. It is also contraindicated in persons known to be sensitive to thimerosal. The Food and Drug Administration has approved a live attenuated influenza vaccine that is administered nasally (FluMist). It is to be used in healthy children aged 5 to 17 years and adults aged 18 to 49 years. Its safety in asthmatic individuals has not been established, and it is not currently recommended for use in patients with high-risk conditions, such as chronic cardiovascular, pulmonary, renal, or metabolic disorders, and in pregnant women. It is contraindicated in persons with a history of allergic reactions to any vaccine component, including eggs and children receiving chronic aspirin therapy or who are immunosuppressed.

Additional Reading: Seasonal influenza vaccination resources for health professionals. From: http://www.cdc.gov/flu/professionals/vaccination/

Category: Patient/population-based care

The answer is C. Controversy has risen about the safety of some vaccines because of rare but serious adverse effects that have been attributed to them. Pain, swelling, and redness at the injection site are common local reactions to vaccines. Fever and irritability may occur after some immunizations. Currently, no substantial evidence links MMR vaccine to autism or hepatitis B vaccine to multiple sclerosis. Thimerosal is being eliminated from routine childhood vaccines because of concerns that multiple immunizations with vaccines containing this preservative could exceed recommended mercury exposures. Children with a history of egg allergy may be given MMR vaccine, even though it is derived from chick embryo fibroblast tissue culture. However, influenza vaccine should not be given to a person with a history of egg allergy. Traces of antibiotics such as neomycin, which is present in varicella (chickenpox), trivalent inactivated poliovirus (IPV), and MMR vaccines, have been considered possible causes of adverse reactions. A history of anaphylactic reaction to neomycin is a contraindication to future immunization, whereas a local reaction is not.

Additional Reading: Vaccine adverse events: separating myth from reality. Am Fam Physician. 2002;66:2113-2120.

Category: Patient/population-based care

The answer is E. Fifty million cases of pertussis are seen each year, leading to about 400,000 deaths. In recent years, high-income countries see large increases in adolescent pertussis rates; the incidence of pertussis in U.S. teens has risen 19-fold since 1996. Whole-cell vaccines have been available for 70 years. Concerns about adverse effects (e.g., convulsions, encephalopathy, hypotonic episodes, fever, vomiting) paved the way for the development of acellular recombinant vaccines in the 1970s and 1980s.

The Centers for Disease Control and Prevention (CDC) recommend DTaP vaccine at 2, 4, 6, and 15 to 18 months of age, with a booster of the tetanus toxoid, reduced diphtheria toxoid, and acellular pertussis (Tdap) vaccine between 11 and 18 years of age, and a one-time Tdap booster as an adult.

Cochrane review included 52 safety studies and 6 effectiveness trials. In general, acellular and whole-cell vaccines had a low incidence of adverse effects; however, in patient receiving acellular vaccines, there was better compliance to the full series and fewer patients had adverse effects such as febrile convulsions and hypotonic-hyporesponsive episodes. Although not statistically significant compared with whole-cell vaccines, adverse effects from
acellular vaccines increased as the series progressed, including fever (60 to 162 per 1,000 persons), local redness (96 to 162 per 1,000 persons), and swelling (117 to 275 per 1,000 persons). There was no difference seen between vaccines when compared as a cause of death from infection, with such events being rare.

Additional Reading: Cochrane briefs acellular vaccines for preventing pertussis in children. Am Fam Physician. 2011;84(5):504.

Category: Patient/population-based care

The answer is C. Hyperbilirubinemia is very common in term newborns. Current recommendations include the following: phototherapy should be instituted when the total serum bilirubin level is ≥15 mg/dL (257 µmol/L) in infants 25 to 48 hours old, 18 mg/dL (308 µmol/L) in infants 49 to 72 hours old, and 20 mg/dL (342 µmol/L) in infants older than 72 hours. It is unlikely that term newborns with hyperbilirubinemia have serious underlying pathology. Physiologic jaundice peaks on the third or fourth day and declines over the first week following birth. Infants who are breast-fed are more likely to develop physiologic jaundice because of relative caloric deprivation in the first few days of life. If jaundice occurs in breast-fed infants, feedings should be increased to more than 10 times per day. In some cases formula supplementation may be necessary. Pathologic jaundice occurs if it presents within the first 24 hours after birth, the total serum bilirubin level rises by >5 mg/dL (86 µmol/L) per day or is >17 mg/dL (290 µmol/L), or an infant has signs and symptoms suggestive of serious illness. The management consists of excluding pathologic causes of hyperbilirubinemia and initiating treatment to prevent harmful neurotoxicity.

Additional Reading: A practical approach to neonatal jaundice. Am Fam Physician. 2008;77(9):1255-1262.

Category: Hematologic system

The answer is B. Baby-bottle tooth decay can occur after a child repeatedly falls asleep with a bottle in his or her mouth. It is more commonly seen in lower socioeconomic groups and can lead to major dental problems with the development of caries. Prevention should be aimed at educating the parents about this problem so that they can avoid bottle-feeding at bedtime.

Additional Reading: A practical guide to infant oral health. Am Fam Physician. 2004;70(11):2113-2120.

Category: Special sensory

The answer is E. Current recommendations for the diphtheria, pertussis, and tetanus immunization of young children state that DTaP is usually given at 2, 4, 6, and 12 to 15 months, with an additional dose at 4 to 6 years. The acellular pertussis form is preferred for all doses to help reduce the occurrence of side effects. Tetanus toxoid, reduced diphtheria toxoid, and acellular pertussis vaccine (Tdap adolescent preparation) is recommended at age 11 to 12 years for those who have completed the recommended childhood DPT/DTaP vaccination series and have not received a tetanus and diphtheria toxoid (Td) booster dose. Adolescents aged 13 to 18 years who missed the 11-to-12-year Td/Tdap booster dose should also receive a single dose of Tdap if they completed the recommended childhood DPT/DTaP vaccination series. Subsequent boosters are recommended every 10 years. Contraindications to the DPT vaccine include the following:

Items 5 through 8 are relative contraindications and should be evaluated individually. The DTaP immunization should be given intramuscularly. A combined vaccine with DPT and Hib (Tetramune), which can be given at 2, 4, 6, and 12 to 15 months, is available.

Additional Reading: DTaP ACIP vaccine recommendations. From: http://www.cdc.gov/vaccines/hcp/acip-recs/vacc-specific/dtap.html

Category: Patient/population-based care

The answer is A. Sabin’s vaccine (oral poliovirus vaccine [OPV]) for poliomyelitis prevention is an oral, live, attenuated, trivalent vaccine that was given at 2, 4, and 18 months, and 5 years. Because of cases of the risk associated with the live vaccine, it is no longer used. In its place, the injectable (Salk) vaccine, referred to as IPV vaccine, should be administered. The IPV vaccine has now been recommended for routine immunization in all infants because of the risk of developing polio from the live attenuated Sabin’s vaccine. The schedule is the same for IPV vaccine.

Additional Reading: Polio vaccine. From: http://www.cdc.gov/vaccines/vpd-vac/polio/default.htm

Category: Patient/population-based care

The answer is C. Commonly, formula preparations provide 20 calories/ounce. Formulas exist as cow’s milk-based, soy-based, and casein-based preparations. Cow’s milk-based formula is the preferred, non-breast milk preparation for otherwise healthy term infants who do not breast-feed or for whom breast-feeding has been terminated prior to 1 year of age. Cow’s milk-based formula closely resembles human breast milk and is composed of 20% whey and 80% casein with 50% more protein/dL than breast milk, as well as iron, linoleic acid, carnitine, taurine, and nucleotides. Approximately 32 ounces meets 100% of the recommended daily allowance (RDA) for calories, vitamins, and minerals. These formula preparations are diluted to a standard 20 calories/ounce and are typically wheydominant protein preparations with vegetable oils and lactose. There are also multiple lactose-free preparations. Most standard formula preparations do not meet the recommended daily allowance for fluoride, and exclusively formula-fed infants may require 0.25 mg/day of supplemental fluoride.

Additional Reading: Nutrition and growth. The Harriet Lane Handbook: A Manual for Pediatric House Officers, 19th ed. Philadelphia, PA: Elsevier/Mosby; 2012.

Category: Nonspecific system

The answer is E. Otitis media usually results as a complication of an URI (viral). It is particularly common in children between 6 months and 3 years of age. The most common etiologic agents include Streptococcus pneumoniae, Haemophilus influenzae, and Moraxella (Branhamella) catarrhalis. In newborns, Escherichia coli and Staphylococcus aureus are major causes. Viral causes include respiratory syncitial virus (RSV), parainfluenza virus, influenza virus, enteroviruses, and adenoviruses. Risk factors include attending day care at or before 2 months of age, in day care >30 hours/week, bottle-feeding, exposure to cigarette smoke, pacifier use, and Polynesian, Native American, or Alaskan/Canadian Eskimo descent. Low birth weight, young gestational age, and a family history of allergies or asthma are not significantly associated with an increased risk of AOM.

Symptoms include earache, nausea, vomiting, diarrhea, hearing loss, and otorrhea. Fever may be present, but it may be absent in as many as 33% of those affected. Signs include bulging of the tympanic membrane with loss of the light reflex and normal landmarks as well as tympanic membrane immobility. Perforation and vestibular dysfunction may also occur. Diagnosis is based on clinical findings and requires the presence of fluid under pressure in the middle ear plus one sign of acute local or systemic illness. Eardrum motion is best assessed by looking at the pars flaccida in the superior part of the drum. A red drum with normal mobility is common in crying children and is not diagnostic of acute bacterial infection. The drug of choice for treatment is amoxicillin in patients who are not at increased risk of being infected with a drug-resistant organism. Complications include mastoiditis, labyrinthitis, conductive and sensory neural hearing loss, and meningitis.

Additional Reading: The diagnosis and management of acute otitis. Pediatrics. 2013;131(3): e964-e999.

Category: Special sensory

The answer is A. A significant number of preschool-age children in the United States have BLLs >10 µg/dL (0.50 µmol/L), and these levels have been associated with a decline in IQ. The CDC advocates the use of a screening questionnaire to identify lead exposure or toxicity in all children. Efforts to remove lead from gasoline and paint have led to a reduction of BLLs in children. Secondary prevention through lead paint removal is effective in homes that have a high lead burden. Children with lead levels of 45 to 69 µg/dL (2.15 to 3.35 µmol/L) should receive chelation therapy using succimer (DMSA) or edetate calcium disodium (CaNa2EDTA). Use of both CaNa2EDTA and dimercaprol (BAL in oil) is indicated in children with BLLs higher than 70 µg/dL (3.40 µmol/L).

Additional Reading: Lead poisoning in children. Am Fam Physician. 2010;81(6):751-757.

Category: Patient/population-based care

The answer is E. Unicameral bone cysts (simple bone cysts) usually affect the metaphysis in long bones of pediatric patients (predominantly the femur, humerus). Most are asymptomatic and come to the attention of the patient, parents, and physician when a fracture
occurs in the area of the bone cyst. Most small cysts heal without difficulty; a larger cyst may require surgery that involves removal of the cyst and bone grafting. Most patients recover without permanent disability. An aneurysmal bone cyst is a cyst that occasionally grows larger; these cysts usually occur before 20 years of age. Areas of involvement include expansion beyond the metaphyseal cartilage of the long bones. Patients may report pain and swelling in the region of the cyst. Radiographs may show well-circumscribed areas of rarefaction with periosteal elevation. Treatment usually involves surgery to remove the cyst. Occasionally, radiation therapy is used for vertebral lesions that threaten the spinal cord if surgery is contraindicated; however, postradiation sarcomas can occur. The prognosis for unicameral and aneurysmal bone cysts after treatment is excellent.

Additional Reading: Benign tumors and tumor-like processes of bone. Nelson Textbook of Pediatrics, 19th ed. Philadelphia, PA: Elsevier/Saunders; 2011.

Category: Musculoskeletal system

The answer is E. Amblyopia is subnormal visual acuity in one or both eyes despite correction of a refractive error. It results when the child suppresses the vision in one eye to avoid diplopia. Organic disease may be present but is insufficient to explain the level of vision. Amblyopia is frequently asymptomatic and detected only by screening programs. It is more resistant to treatment at an older age; thus children should be treated early. Other causes (neurologic, psychologic) must be considered if the history and ophthalmologic examination are not suggestive of amblyopia.

Treatment includes correction of refraction error or removal of cataract, as well as forced use of the amblyopic eye by patching the better eye. Some children cannot tolerate the patch, in which case the sound eye is blurred with glasses or drops (penalization therapy) to stimulate proper visual development of the more severely affected eye.

Additional Reading: Amblyopia. Nelson Textbook of Pediatrics, 19th ed. Philadelphia, PA: Elsevier/Saunders; 2011.

Category: Special sensory

The answer is C. An abnormal pupillary light reflex (called leukocoria if the pupil appears white) may indicate a disorder anywhere within the eye. Associated disorders include corneal opacity, blood (hyphema) or other material in the anterior chamber, cataract, vitreous opacity, or retinal disease. The most serious diagnosis is retinoblastoma, a malignancy that is thought to arise from retinal germ cells. Because it may be hereditary, a family history of retinoblastoma or of enucleation is of special concern. Although retinoblastoma is almost uniformly fatal without treatment, the cure rate is better than 90% when the condition is promptly recognized and treated, and many children can be effectively treated without enucleation.

Additional Reading: Abnormalities of pupil and iris. Nelson Textbook of Pediatrics, 19th ed. Philadelphia, PA: Elsevier/Saunders; 2011.

Category: Special sensory

The answer is E. Kernicterus occurs when the serum unconjugated (indirect) bilirubin becomes dangerously elevated (usually >25 mg/dL) in newborns. Symptoms include poor feeding, flaccidity, apnea, opisthotonos, and seizures; in severe cases, death may occur. Children who do survive may suffer hearing loss, seizures, and mental retardation. Risk factors include prematurity, blood incompatibilities, infection, and acidosis. Physiologic jaundice is the most common form of jaundice and occurs in up to 50% of newborns. The condition is benign and usually resolves in 1 week. Most bilirubin levels peak in 3 to 5 days. The workup of a child with hyperbilirubinemia should include the following:

Treatment for hyperbilirubinemia of newborns includes the following:

Additional Reading: Kernicterus. Nelson Textbook of Pediatrics, 19th ed. Philadelphia, PA: Elsevier/Saunders; 2011.

Category: Neurologic

The answer is D. The composition of breast milk in mothers of preterm infants is different from that of term infants. This difference persists for approximately 4 weeks before the composition approaches that of term infant breast milk. The difference in preterm milk composition reflects the increased nutrient demands of preterm infants. Preterm breast milk contains higher concentrations of total and bound nitrogen, immunoglobulins, sodium, iron, chloride, and medium-chain fatty acids. However, it may not contain sufficient amounts of phosphorus, calcium, copper, and zinc. Preterm infants are more likely to require fortification with human milk fortifiers (HMF) to correct these deficiencies.

Additional Reading: Enteral nutrition components. The Harriet Lane Handbook. A Manual for Pediatric House Officers. Johns Hopkins Hospital; 2012.

Category: Nonspecific system

The answer is C. Slipped capital femoral epiphyses are usually seen in overweight boys between 11 and 14 years of age. The condition occurs when the femoral head slips posteriorly and inferiorly, exposing the anterior and superior aspects of the metaphysis of the femoral neck. When the condition occurs before puberty, an underlying endocrine disorder (hypothyroidism, growth hormone deficiency) should be suspected. Symptoms, including pain and a limp, are usually gradual in onset and usually involve the hips or are referred to the thigh or knee. The condition is bilateral in 20% of cases. Radiographs should be performed, including frog-leg views. Findings include abnormalities with the femoral head, including acetabular dysplasia. Treatment involves orthopaedic referral with surgical pinning. Complications include avascular necrosis of the hip and erosion of cartilage.

Additional Reading: Slipped capital femoral epiphysis. Nelson Textbook of Pediatrics, 19th ed. Philadelphia, PA: Elsevier/Saunders; 2011.

Category: Musculoskeletal

The answer is B. Tinea capitis is a fungal infection of the scalp that usually affects infants and young children. It is contagious and may become epidemic. It is caused by fungi including Trichophyton, Microsporum, and Epidermophyton. Lesions of the scalp usually cause scaly, gray patches that are pruritic. Multiple areas of hair loss may occur with hair shafts broken just above the scalp. Microscopic examination of scrapings after treatment with 10% potassium hydroxide reveals fungal hyphae. Hair examined with black light fluorescences show a greenish-yellow color in cases of microsporosis. Treatment for most tinea infections involves the use of topical antifungals but is not sufficient for tinea capitis, which requires oral administration of antifungals, such as griseofulvin. Severely inflamed lesions benefit from systemic or intralesional steroids. Until tinea capitis is cured, an imidazole or ciclopirox cream should be applied to the scalp to prevent spread, especially to other children, and selenium sulfide 2.5% shampoo should be used daily. Children can attend school during treatment, and the risk of transmission is low.

Additional Reading: Tinea capitis. Nelson Textbook of Pediatrics, 19th ed. Philadelphia, PA: Elsevier/Saunders; 2011.

Category: Integumentary

The answer is E. The incidence of Hodgkin’s disease increases throughout childhood and peaks in the late teens. The most common presenting complaint is a painless mass in the neck. Other presentations include a persistent cough secondary to a mediastinal mass or, less commonly, splenomegaly or enlarged axillary or inguinal lymph nodes. About one-third of children with Hodgkin’s disease present with constitutional symptoms. These symptoms may include intermittent fever, night sweats, and weight loss. These are referred to as “B” symptoms. The “A” designation refers to the absence of these symptoms. Other symptoms include anorexia, fatigue, and pruritus. Any persistent painless mass (especially a neck mass) that does not respond to antibiotics should be evaluated. This investigation should include a lymph node biopsy. Because of sampling errors and difficulties in obtaining an accurate diagnosis, excisional biopsy (rather than needle biopsy) of enlarged lymph nodes should be performed. A persistent cough, especially in the presence of any “B” symptoms, should be investigated. As part of this evaluation, a chest radiograph should be obtained. It is also important to investigate “B” symptoms associated with any lymphadenopathy or splenomegaly. Laboratory tests can often be helpful in confirming the diagnosis. Although nonspecific, elevations in the erythrocyte sedimentation rate, lactate dehydrogenase level, and ferritin level are suspicious findings in children with other signs or symptoms of Hodgkin’s disease. Infrequently, the CBC reveals abnormalities, including anemia and eosinophilia.

Additional Reading: Role of the primary care physician in Hodgkin lymphoma. Am Fam Physician. 2008;78(5):615-622.

Category: Hematologic system

The answer is B. Acne is one of the most common presenting complaints in a family physician’s office. Adolescent patients between 12 and 25 years of age are the most commonly affected. The condition results when keratinization blocks follicular canals. Increased sebum production occurs, and bacterial proliferation causes inflammation. Increased androgen production is often related to the development of acne. The plugged pilosebaceous unit is referred to as a “whitehead” if the lesion is a closed comedone, or a “blackhead” if the comedone is open. The most common infecting bacterium is Propionibacterium acnes, which proliferates and releases chemotactic factors that attract leukocytes. The diagnosis of acne is made by observing characteristic lesions on the face, back, shoulders, and chest. Treatment involves washing with mild soaps on a regular basis; application of benzoyl peroxide; topical tretinoin (Retin-A); or topical antibiotics such as erythromycin, clindamycin, tetracycline, or meclocycline. Oral antibiotics may be necessary for more severe cases. Severe nodulocystic acne that fails to respond to the previously mentioned measures may be treated with oral isotretinoin (Accutane). Close monitoring of liver function tests, triglyceride levels, and CBCs are required. In addition, the drug has many other side effects (i.e., xerosis, epistaxis, myalgias, and arthralgias) and is highly teratogenic. Patients should be aware that acne is not a disease of hygiene. They should not try to scrub the lesions away, and they should not use alcohol-based astringents that can dry and irritate their skin. Patients should be instructed to wash their face twice a day with a mild soap and water. Patients should also be informed that acne has no relationship to diet (e.g., chocolate, pizza, soda). Many think acne is caused by stress, but no studies support this association. It may be that the acne itself causes stress, not vice versa. Cosmetics have long been blamed for the development of acne lesions. Although the causal relationship between cosmetics and acne may be overstated, patients should be directed to use oil-free, noncomedogenic cosmetics. Oil from hair products and suntan lotions can also exacerbate acne. Female patients should be informed that acne usually worsens during the week before menses. Mechanical trauma can make acne worse. Therefore, patients should be encouraged to avoid picking at lesions because doing so may cause more inflammation.

Additional Reading: Diagnosis and treatment of acne. Am Fam Physician. 2012;86(8):734-740.

Category: Integumentary

The answer is B. Absence seizures (formerly called petit mal seizures) are characterized by brief, 10- to 30-second staring episodes, followed by a resumption of normal activity. Attacks may occur up to 100 times daily and can be precipitated by hyperventilation. The seizures usually affect children, and there is a genetic predisposition. Affected children usually have normal intelligence, and most cases resolve before 20 years of age. EEG findings show a characteristic 3-per-second bilateral spike-and-wave pattern. Treatment usually involves the use of valproic acid and/or ethosuximide and clonazepam.

Additional Reading: Seizures in childhood. Nelson Textbook of Pediatrics, 19th ed. Philadelphia, PA: Elsevier/Saunders; 2011.

Category: Neurology

The answer is C. Idiopathic aseptic necrosis of the femoral head is also known as Legg-Calvé-Perthes disease. The disease is usually unilateral and is most common in boys 2 to 12 years of age. Symptoms include hip, groin, or thigh pain and difficulty ambulating, which is usually gradual in onset and progressive. Physical examination may show an abnormal gait (painless limp) and atrophy of the thigh muscles. Lateral radiographs (frog view) are required and show areas of lucency and fragmentation of the femoral head, which may progress to sclerosis and destruction. Radiographs are often normal early in the disease process; however, bone scans may show decreased uptake in the area of the femoral head. Treatment involves expectant observation both clinically and radiographically, abduction casts to contain the femoral head within the acetabulum, and surgery in select cases. It should be remembered that children who present with knee pain may have hip pathology.

Additional Reading: The hip. Nelson Textbook of Pediatrics, 19th ed. Philadelphia, PA: Elsevier/Saunders; 2011.

Category: Musculoskeletal

The answer is D. Infectious mononucleosis is relatively common in patients 10 to 30 years of age who present with sore throat and fatigue, palatal petechiae, posterior cervical or auricular adenopathy, marked adenopathy, or inguinal adenopathy. An atypical lymphocytosis of at least 20% or atypical lymphocytosis of at least 10% plus lymphocytosis of at least 50% strongly supports the diagnosis, as does a positive heterophile antibody test. False-negative results of heterophile antibody tests are relatively common early in the course of infection. Symptomatic treatment, the mainstay of care, includes adequate hydration, analgesics, antipyretics, and adequate rest. Bed rest should not be strictly enforced, and the patient’s energy level should guide activity. Corticosteroids, acyclovir, and antihistamines are not recommended for routine treatment of infectious mononucleosis, although corticosteroids may benefit patients with respiratory compromise or severe pharyngeal edema. Patients with infectious mononucleosis should be withdrawn from contact or collision sports for at least 4 weeks after the onset of symptoms. Fatigue, myalgias, and need for sleep may persist for several months after the acute infection has resolved.

Additional Reading: Epstein-Barr virus. Nelson Textbook of Pediatrics, 19th ed. Philadelphia, PA: Elsevier/Saunders; 2011.

Category: Nonspecific

The answer is C. Following birth, there is a modest decrease in the vitamin K (phytonadione)-dependent factors II, VII, IX, and X, which gradually return to normal in 7 to 10 days. The cause of this decrease is inadequate free vitamin K available from the mother and the newborn’s inability to synthesize vitamin K because of a lack of intestinal flora. Therefore, 1 mg of vitamin K is administered intramuscularly at birth to prevent hemorrhagic disease of the newborn in term infants. Larger doses predispose to the development of hyperbilirubinemia and kernicterus. Breast milk is a poor source of vitamin K. As a result, hemorrhagic complications occur more frequently in breast-fed infants. Mothers taking medications that interfere with vitamin K function (i.e., phenobarbital and phenytoin) may have infants at increased risk for early-onset bleeding.

Additional Reading: Hemorrhage in the newborn infant. Nelson Textbook of Pediatrics, 19th ed. Philadelphia, PA: Elsevier/Saunders; 2011.

Category: Hematologic

The answer is B. Down’s syndrome is a condition characterized by an extra chromosome 21. The incidence is reported to be 1 in 700 to 800 births, but it varies depending on maternal age. Older mothers (especially those older than 35) are at increased risk. The disease may result from trisomy 21, translocation, or mosaicism.

Signs and symptoms include a flattened, hypoplastic midface with a depressed nasal bridge, hypotonicity, delayed physical and mental development with decreased IQ, microcephaly with a flattened occiput, slanted eyes with epicanthal folds, Brushfield spots (gray to white spots around the periphery of the iris), single palmar crease, short fingers, and abnormal feet with a wide gap between the first and second toe. Other associated conditions include congenital heart disease (e.g., ventricular septal defects) and GI anomalies (e.g., tracheoesophageal fistula, pyloric stenosis, duodenal atresia, and imperforate anus). The life expectancy of a child affected with Down’s syndrome is reduced by the presence of heart disease and an increased risk of acute leukemia. Some affected women are fertile; however, they have a 50% chance that their fetus will also have Down’s syndrome. Many of these affected fetuses abort spontaneously. All men with Down’s syndrome are infertile.

Additional Reading: Down syndrome and other abnormalities of chromosome number. Nelson Textbook of Pediatrics, 19th ed. Philadelphia, PA: Elsevier/Saunders; 2011.

Category: Nonspecific

The answer is B. Transient synovitis of the hip is the most common cause of hip pain and limping in U.S. children. The condition usually follows an upper respiratory illness and resolves spontaneously within a few days. Children 3 to 10 years of age, particularly boys, are the most commonly affected. Physical examination shows a limp and limited motion of the hip, especially with internal rotation. The hip is usually kept flexed, abducted, and externally rotated. Radiographs are usually negative but may show soft tissue swelling associated with the hip joint. A CBC and the erythrocyte sedimentation rate are usually normal. Treatment involves rest and anti-inflammatory drugs. Symptoms usually resolve in 7 to 10 days. Traction of the hip in slight flexion may also be used. Follow-up radiographs (at 1 and 3 months from time of presentation or perhaps sooner if the child’s limp persists) are recommended because of the risk for development of avascular necrosis of the femoral head. If septic arthritis is suspected, aspiration of the hip may be necessary.

Additional Reading: The hip. Nelson Textbook of Pediatrics, 19th ed. Philadelphia, PA: Elsevier/Saunders. 2011.

Category: Musculoskeletal system

The answer is B. Controversy surrounds the necessity of circumcision. UTIs are 10 to 15 times more common in uncircumcised infants. Many recommend circumcision in infants who are predisposed to UTI, such as those with congenital hydronephrosis and vesicoureteral reflux. Other indications for circumcision include recurrent balanitis (inflammation of the glans), posthitis (inflammation of the foreskin), or paraphimosis (retraction of the prepuce behind the glans that may interfere with blood flow). Routine circumcision is often more a social issue than a medical indication. Because phimosis (tightness of the foreskin so that it cannot be retracted over the glans penis) cannot usually be detected before puberty, it is not an indication for circumcision. Contraindications for circumcision include prematurity, genital anomalies (including hypospadias or ambiguous genitalia), and bleeding disorders. Circumcision should be performed at least 12 to 24 hours after birth and within 6 weeks of birth, preferably before discharge from the hospital. If delay occurs beyond 6 weeks, circumcision should be postponed until after 1 year of age with general anesthesia. Most recommend using a dorsal penile nerve block with 1% lidocaine without epinephrine for local anesthesia. EMLA cream can also be applied.

Additional Reading: Anomalies of the penis and urethra. Nelson Textbook of Pediatrics, 19th ed. Philadelphia, PA: Elsevier/Saunders. 2011.

Category: Reproductive system

The answer is A. Infectious mononucleosis is caused by the Epstein-Barr virus. It usually affects individuals between 10 and 35 years of age. Symptoms include fever, sore throat, anorexia, generalized fatigue, lymphadenopathy (especially affecting the posterior cervical chain), splenomegaly, and a maculopapular rash. Hepatitis with hepatomegaly is often seen with occasional jaundice. Laboratory findings include leukocytosis with many atypical lymphocytes (i.e., larger with vacuolated cytoplasm) and a positive monospot test (with heterophil agglutination) that becomes positive before the fourth week after the onset of the illness. The results of these tests are usually negative in infants and children younger than 4 years. False-positive rapid plasma reagin and Venereal Disease Research Laboratory tests may occur, as well as abnormal liver function tests. Treatment involves supportive therapy with saline gargles, anti-inflammatory drugs, and antipyretic medication. In severely ill patients with severe pharyngitis, corticosteroids can be used to help decrease inflammation. Complications include the development of Guillain-Barré syndrome, myocarditis, and encephalitis. Spleen rupture may also occur with trauma; therefore, contact sports should be avoided until the splenomegaly has resolved.

Additional Reading: Epstein-Barr virus. Nelson Textbook of Pediatrics, 19th ed. Philadelphia, PA: Elsevier/Saunders; 2011.

Category: Nonspecific system

The answer is C. Topical retinoids, benzoyl peroxide, sulfacetamide, and azelaic acid are recommended in patients with mild or moderate comedones. Topical erythromycin or clindamycin can be used in addition in patients with mild to moderate inflammatory acne or mixed acne. A 6-month course of oral erythromycin, doxycycline, tetracycline, or minocycline can be prescribed in patients with moderate to severe inflammatory acne. A low-androgen-containing oral contraceptive pill is also effective in women with moderate to severe acne. Isotretinoin is reserved for use in the treatment of the most severe or refractory cases of inflammatory cystic type acne. Because of its poor side-effect profile and teratogenicity, isotretinoin must be prescribed by a physician who is registered with the System to Manage Accutane-Related Teratogenicity (SMART) program. Serious side effects of isotretinoin include hepatitis, hypertriglyceridemia, intracranial hypertension, arthralgia, myalgias, night blindness, and hyperostosis. Serum liver function tests and triglyceride levels must be monitored monthly in patients receiving isotretinoin. Isotretinoin is teratogenic and can result in severe fetal abnormalities involving several systems. As a result, two forms of contraception must be used during isotretinoin therapy and for 1 month after treatment has been discontinued. To ensure that female patients are not pregnant when treatment is initiated, two negative urine pregnancy tests are required before isotretinoin is prescribed. Pregnancy status is rechecked at monthly visits. The link between isotretinoin and depression is controversial.

Additional Reading: Diagnosis and treatment of acne. Am Fam Physician. 2012;86(8):734-740.

Category: Integumentary

The answer is C. Erythema infectiosum is referred to as fifth disease because it represents the fifth major viral childhood illness (which also includes measles, mumps, rubella, and rubeola). The disease is caused by parvovirus B19 and is characterized by mild constitutional symptoms, such as low-grade fever, malaise, and joint pain (particularly in adult women). Also, there is a classic indurated, erythematous maculopapular facial rash that may progress to the trunk and extremities (but spares the palms and soles). The rash is often pronounced on extensor surfaces. The rash is often referred to as a “slapped-cheek” appearance and can be exacerbated with exposure to sunlight, heat, emotional stress, or fever. The illness usually lasts 5 to 10 days, and only symptomatic treatment is necessary. Occasionally, complications include arthropathies, myocarditis, and a transient aplastic crisis. Fifth disease may occasionally cause fetal death secondary to fetal hydrops; therefore, pregnant women should avoid contact with affected patients. Children are not infectious once the rash develops because the rash and arthropathy (when present) are immune-mediated, postinfectious reactions. Therefore, isolation from school and/or day care is not necessary.

Additional Reading: Parvovirus B19. Nelson Textbook of Pediatrics, 19th ed. Philadelphia, PA: Elsevier/Saunders; 2011.

Category: Nonspecific system

The answer is B. There are five stages described for iron intoxication:

In addition to the previously mentioned symptoms, vomiting, hyperglycemia, leukocytosis, and an abdominal radiograph that shows the iron particles are usually related to a serum iron level >300 mg/dL. Severe cases may cause seizures, coma, pulmonary edema, and vascular collapse. Treatment involves inducing vomiting, as well as gastric lavage, followed by use of the chelating agent deferoxamine. Charcoal does not bind iron. In severe cases, hemodialysis and exchange transfusion may be necessary.

Additional Reading: Poisoning. Nelson Textbook of Pediatrics, 19th ed. Philadelphia, PA: Elsevier/Saunders; 2011.

Category: Nonspecific system

The answer is B. Diaper dermatitis secondary to Candida albicans is an intensely red and scorched-appearing rash that involves the perineal area. The rash may be well demarcated and possesses vesicles that weep, pustules, papules, and the characteristic satellite lesions. Treatment consists of antifungal ointment (i.e., clotrimazole or nystatin) and, possibly, short-term use of hydrocortisone cream for severe dermatitis. Soothing cream (zinc oxide) should be applied with each diaper change. Also, keeping the area dry can help deter the development of yeast dermatitis.

Additional Reading: Candida. Nelson Textbook of Pediatrics, 19th ed. Philadelphia, PA: Elsevier/Saunders; 2011.

Category: Nonspecific system

The answer is B. Viral croup is the main cause of airway obstruction in children 6 months to 6 years of age. For children with mild croup, symptomatic care and mist therapy may be all that is necessary. Epinephrine has been used in the past to treat more severe cases of croup, but recent studies have found that glucocorticoid use is associated with shorter hospital stays, improvement in croup scores, and less use of epinephrine. Studies have shown that treatment with oral dexamethasone is as effective as intramuscular dexamethasone or nebulized budesonide. While more studies are needed to establish guidelines, oral dexamethasone can be used to treat mild to moderate croup with close follow-up and instructions for further care, if needed.

Additional Reading: Croup: an overview. Am Fam Physician. 2011;83(9):1067-1073.

Category: Respiratory system

The answer is D. Ciprofloxacin is generally not indicated for patients below age 18 years because of the risk of damage to cartilage development. Although amoxicillin is considered the drug of choice, the other options can be used as second-line agents.

Additional Reading: The diagnosis and management of acute otitis. Pediatrics. 2013;131(3):e964-e999.

Category: Special sensory

The answer is B. Premature infants are predisposed to certain problems, including poor sucking and diminished gag and cough reflexes, which can lead to an increased risk of aspiration and difficulty feeding. Other problems include pulmonary immaturity, decreased ability to maintain body temperature, impaired renal excretion, limited iron stores with a predisposition to develop anemia, metabolic disturbances, and decreased ability to fight infection. Immunizations should take place at the same designated times as for term infants with no adjustments made for premature age. One exception to this recommendation is that hepatitis B vaccination should be delayed for 1 month if mothers are negative for hepatitis B surface antigen.

Additional Reading: General immunization practices. Nelson Textbook of Pediatrics, 19th ed. Philadelphia, PA: Elsevier/Saunders; 2011.

Category: Patient/population-based care

The answer is A. The children of teenage mothers have been shown to have cognitive delays on IQ and vocabulary tests. They may also show problems of emotion, including rebelliousness, aggressiveness, uncontrollable anger, and impulsiveness. They are at greater risk for low birth weight and they have an increased risk of experiencing an accident within the home and of being hospitalized before the age of 5 years. There appears to be no link with major affective disorders.

Additional Reading: Adolescent pregnancies. Nelson Textbook of Pediatrics, 19th ed. Philadelphia, PA: Elsevier/Saunders; 2011.

Category: Patient/population-based care

The answer is A. Kawasaki’s disease was previously referred to as mucocutaneous lymph node syndrome. The cause of Kawasaki’s disease is unclear, and unfortunately a specific diagnostic test for its detection does not exist. The majority of affected patients are younger than age 5 years, and boys are more frequently affected. Diagnosis is based on the following criteria and include fever for more than 5 days and at least four of the following features: (1) bilateral, painless, nonexudative conjunctivitis, (2) lip cracking and fissuring, strawberry tongue, inflammation of the oral mucosa, (3) cervical lymphadenopathy (≥1.5 cm in diameter and usually unilateral), (4) exanthema, and (5) redness and swelling of the hands and feet with subsequent desquamation. Adverse cardiovascular effects are the most serious component of Kawasaki’s disease. Cardiovascular complications include myocarditis, pericarditis, valvular heart disease (usually mitral or aortic regurgitation), and coronary arteritis. Coronary artery lesions range from mild transient dilation of a coronary artery to large aneurysm formation. Those at greatest risk of aneurysm formation are boys, children under the age of 6 months, and those not treated with intravenous immunoglobulin (IVIG). The gold standard for diagnosing coronary artery aneurysms is angiography; however, two-dimensional echocardiography is highly sensitive and is the current standard screening test in children with Kawasaki’s disease. Fortunately, most coronary artery aneurysms resolve within 5 years of diagnosis. Giant aneurysms (>8 mm) are much less likely to resolve, and about half become stenotic. Of additional concern, acute thrombosis of an aneurysm can occur, resulting in a myocardial infarction, and can be fatal. The treatment of Kawasaki’s disease consists of therapy with intravenous immunoglobulin and high-dose aspirin. This therapy is effective in decreasing the incidence of coronary artery dilation and aneurysm formation. Currently, corticosteroids are not felt to be effective in Kawasaki’s disease.

During the acute and subacute phases of the illness, patients should be monitored closely by serial electrocardiography, chest radiograph, and echocardiography. Selective coronary angiography is recommended in patients with evidence of myocardial ischemia.

Additional Reading: Kawasaki disease. Nelson Textbook of Pediatrics, 19th ed. Philadelphia, PA: Elsevier/Saunders; 2011.

Category: Cardiovascular system

The answer is A. The etiology of stuttering is controversial. Majority of stuttering cases are classified as developmental problems, although stuttering can also be classified as a neurologic or, less commonly, psychogenic problem. Stutterers also have difficulty coordinating airflow, articulation, and resonance. In addition, small asynchronies have also been found in the fluent speech of stutterers. Stuttering is a common disorder that usually resolves by adulthood. Boys are more frequently affected, and there appears to be an increased genetic risk. Generally, there is cause for concern if a patient’s speech has five or more breaks per 100 words. Almost 80% of children who stutter recover fluency by the age of 16 years. Mild stuttering is self-limited, but more severe stuttering requires speech therapy, which is the mainstay of treatment. Delayed auditory feedback and computer-assisted training are currently used to help slow down speech and control other speech mechanisms. Pharmacologic therapy is seldom used, although haloperidol has been somewhat effective. Differentiating between normal developmental dysfluency and stuttering is important. In general, developmental dysfluency involves the repetition of whole words and phrases, whereas stuttering involves the repetition of word parts and the prolongation of sounds. In addition, stutterers frequently speak at a faster tempo, display silent pauses, have inappropriate articulating postures, become more dysfluent in response to stress, and are more easily frustrated.

Additional Reading: Stuttering: an overview. Am Fam Physician. 2008;77(9):1271-1276.

Category: Special sensory

The answer is A. Rabies in humans is rare in the United States, but the CDC estimates that as many as 39,000 persons receive postexposure prophylaxis annually. The risk of infection must be carefully evaluated by the clinician in the management of potential human rabies exposures. Bats, skunks, raccoons, foxes, and most other carnivores should receive postexposure prophylaxis. Bites of squirrels, hamsters, guinea pigs, gerbils, chipmunks, rats, mice, other small rodents, rabbits, and hares almost never require antirabies postexposure prophylaxis. The CDC considers administration of postexposure prophylaxis to be a medical urgency, not a medical emergency, although Advisory Committee on Immunization Practices (ACIP) emphasizes that decisions about using prophylaxis should not be put off.

Additional Reading: Human rabies prevention—United States, 2008 Recommendations of the Advisory Committee on Immunization Practices (ACIP). MMWR. 2008;57(RR-3):1-28.

Category: Nonspecific system

The answer is C. Coarctation of the aorta is one of the more common congenital heart defects (CHDs). Boys are more commonly affected than girls. The condition occurs when there is discrete narrowing of the thoracic aorta near the ligamentum arteriosus, leading to proximal hypertension and left ventricular overload. Other findings include a ventricular septal defect, patent ductus arteriosus, and bicuspid aortic valve. In most cases, those affected are asymptomatic during infancy. However, congestive heart failure can occur and may require immediate surgical intervention. Signs associated with coarctation of the aorta include diminished or absent femoral pulses, blood pressure higher in the arms than in the legs in infants older than 1 year, 2/6 to 3/6 systolic ejection murmur heard over the apex and upper left sternal border, rib notching on chest radiograph (which is a result of enlargement of the intercostal arteries), and left ventricular hypertrophy. Diagnosis is based on physical findings and echocardiography or with CT or MRI angiography. Treatment depends on the severity of coarctation and the heart’s ability to maintain perfusion. In severe cases, prostaglandin E1 may be used to keep a patent ductus arteriosus dilated until surgery or balloon angioplasty can be performed. In more stable patients, β-blockers and afterload-reducing agents can be used to postpone definitive treatment until the child is 3 to 5 years of age, when the treatment can be performed electively. Those patients who do well initially without evidence of congestive heart failure and requiring no surgery usually do quite well regarding further complications in childhood and adolescence. Those affected are at risk for hypertension and cardiac dysfunction as well as subacute infective endocarditis.

Additional Reading: Coarctation of the aorta. Nelson Textbook of Pediatrics, 19th ed. Philadelphia, PA: Elsevier/Saunders; 2011.

Category: Cardiovascular system

The answer is B. Cystic fibrosis is the most common fatal genetic disease in the United States. It is transmitted as an autosomal-recessive trait. The incidence in the United States is reported to be approximately 1:3,500 in whites and 1:17,000 in African Americans. Those who are heterozygous are unaffected. The disorder involves exocrine glands and affects predominantly the GI and respiratory systems. Complications include meconium ileus present at birth, chronic cough and wheezing with copious mucous production, pancreatic insufficiency with possible development of insulin-dependent diabetes (up to 8%), retarded growth, infertility, and chronic obstructive pulmonary disease. The diagnosis is made by the pilocarpine iontophoresis sweat test. Levels of sodium and chloride >60 mEq/L are usually diagnostic. Survival beyond 30 years of age is occurring more frequently. Death usually results from pulmonary complications such as infections with S. aureus, Pseudomonas aeruginosa, and H. influenzae.

Additional Reading: Cystic fibrosis. Nelson Textbook of Pediatrics, 19th ed. Philadelphia, PA: Elsevier/Saunders; 2011.

Category: Nonspecific system