Chapter 13: Pediatrics

A 6-year-old male presents to your office with his mom. He has been soiling his underwear frequently and it is causing stress at home and school. His parents are frustrated and think he is old enough to know better. At school, he is being teased and being called "stinky pants" and he reports feeling embarrassed. Mom asks you if there is something medically wrong with him.

Question 13.1.1 What percentage of chronic encopresis is functional (meaning no underlying medical or organic cause)?

A) 10%.

B) 25%.

C) 50%.

D) 75%.

E) 90%.

Answer 13.1.1 The correct answer is "E." Ninety percent of chronic encopresis is functional. Functional encopresis is the repeated involuntary passage of stool into underwear in a child who is 4 years of age or older and at a developmental level appropriate for toilet training in the absence of an underlying medical cause. The most common reason is retentive constipation with overflow incontinence. Frequently, the child has an associated psychological problem. Boys are affected more often than girls. Children with this disorder withhold feces voluntarily thereby avoiding defecating. As stool stays in the colon, more water is absorbed creating harder stool that is more difficult to pass. This leads to a self-perpetuating cycle. The excess stool stretches the rectum with eventual loss of sensation to defecate. Liquid stool may leak around the impacted mass, which parents often mistake as diarrhea. In some, a triggering event such as passage of a painful stool leading to fear of stooling, forced toilet training before the child is ready, or dislike of using public toilets (starting school) may have occurred. A common theme is parental misunderstanding of the problem thinking it is due to an undiagnosed medical problem, attention-seeking behavior, or child laziness.

Question 13.1.2 Which of the following conditions is associated with encopresis?

A) Urinary tract infections.

B) Enuresis.

C) Social isolation.

D) ADHD.

E) All of the above.

Answer 13.1.2 The correct answer is "E." Encopresis is an independent risk factor for urinary tract infections. Feces in the underwear allows for close contact with the urethra and introduction of bacteria. Chronic contracting of pelvic floor muscles to withhold stool may lead to dysfunctional voiding and urinary stasis. Enuresis occurs in up to 40% of patients with chronic constipation. A mass of stool in the rectum can compress the bladder leading to uncontrolled voiding. The chronic odor of feces makes socialization rough and teasing frequent (recall that this kid has started to be called "stinky pants"). ADHD frequently is associated with functional constipation and encopresis due to being too distracted to use the bathroom.

Question 13.1.3 Which of the following are medical (organic) causes of constipation?

A) Hypothyroidism.

B) Hypercalcemia.

C) Cystic fibrosis.

D) Lead poisoning.

E) All of the above.

Answer 13.1.3 The correct answer is "E." All of the above can cause constipation and thus fecal incontinence. Organic causes account for only 5% to 10% of encopresis cases and can be anatomical (anterior displaced anus), neurological (Hirschsprung disease), metabolic (Celiac disease), or iatrogenic (medications such as narcotics or anticholinergics). The first step in evaluating a child with encopresis is to rule out an underlying medical condition starting with a good history and physical examination. Do not forget to ask about timing of meconium passage in the past medical history as delayed meconium passage may suggest cystic fibrosis or Hirschsprung disease. Rectal examination is essential. Look at the back for signs of an occult spina bifida such as sacral dimple or hair tuft. Lack of anal wink, cremasteric reflex, or deep tendon reflexes is suggestive of a neurologic cause. Often a painful fissure may initiate a vicious cycle of pain with stooling leading to the withholding of stool leading to even more painful defecation, etc.

Question 13.1.4 All of the following are useful in the treatment of functional encopresis EXCEPT:

A) Polyethylene glycol (MiraLAX^®).

B) Oral sodium phosphate solution (OsmoPrep).

C) Mineral oil.

D) Lactulose.

E) Milk of magnesia.

Answer 13.1.4 The correct answer is "B." In 2008, the FDA placed a black box warning on oral sodium phosphate solutions due to risk of acute kidney injury from deposition of calcium–phosphate crystals. As a result, nonphosphate-containing medications are used. Treatment begins with disimpaction that can be performed manually, with enemas, or with an oral medication such as polyethylene glycol (MiraLAX) or mineral oil. Many treatment guidelines prefer disimpaction using oral polyethylene gylcol solutions over manual disimpaction. It seems like a strange concept but it works just as well as manual disimpaction and is more humane (Pediatrics. 2009;124(6):e1108–15).

Treatment should be individualized. After disimpaction, patients require maintenance therapy (combination of medical, behavioral, dietary, and counseling). Medications such as milk of magnesia (magnesium hydroxide), mineral oil, or polyethylene glycol (MiraLAX) should be used daily and incrementally increased until the patient is having regular, soft bowel movements. Increased fiber and fluid intake should be encouraged to help keep stools soft. Behaviorally, the child should sit on the toilet with active pushing after each meal to make use of the gastrocolic reflex. Even if the child does not stool, he is learning to use the toilet and training his bowels. Use of a footstool may improve Valsalva maneuvers. A sticker chart can give visual encouragement. Families need to understand that there is no quick fix and relapse is common (about 50%). It takes months for the rectum to return to normal size and sensation. Patients who fail to improve after 6 months of compliant treatment should be referred to a pediatric gastroenterologist.

Objectives: Did you learn to…

• Describe encopresis, including its presentation, diagnosis, and causes?

• Differentiate between organic and nonorganic causes of encopresis?

• Medically manage encopresis?

Causes of neonatal polycythemia (central venous hematocrit of 65% or greater) in the immediate postpartum period include all of the following EXCEPT:

A) Delayed umbilical cord clamping.

B) Twin–twin transfusion.

C) Congenital adrenal hyperplasia.

D) Diabetic mother.

E) Sepsis.

The correct answer is "E." Neonatal polycythemia can be caused by all of the above except for sepsis. Other causes include chronic intrauterine hypoxia (e.g., mother is a heavy smoker), intrauterine growth restriction, maternal hypertension, congenital hypothyroidism, and chromosomal abnormalities (e.g., trisomy 21, 18, and 13).

Patients with neonatal polycythemia are at risk for hyperviscosity syndrome including respiratory distress, hypoxia, cyanosis, hyperbilirubinemia, and hypoglycemia. Heart failure, stroke, renal vein thrombosis, and necrotizing enterocolitis may also occur.

The best treatment for these patients is:

A) Phlebotomy.

B) Exchange transfusion with normal RBCs.

C) Exchange transfusion using D5W.

D) Exchange transfusion using normal saline.

E) Aspirin.

The correct answer is "D." Partial exchange transfusion using normal saline is the treatment of choice. "A" is incorrect since phlebotomy alone will reduce overall circulating volume and may actually exacerbate the problem. "B" is incorrect. If you chose this, back home for you! What is the point of taking out cells and then putting more in, we ask you? "C" is incorrect because large amounts of D5W can cause fluid shifts, electrolyte abnormalities, and hemolysis. Of note, patients with a hematocrit of 65% to 70% can be observed if they are asymptomatic. There is no one-to-one predictable relationship between hematocrit and blood viscosity. Also of note, there is no evidence that long-term outcomes are improved in asymptomatic patients with partial exchange transfusion versus observation.

Amy, a 4-day-old female, comes to clinic because "she is yellow." She was born at term to a 25-year-old woman (well of course…it wouldn't be a male). Maternal lab results are as follows: blood type A+, syphilis negative, rubella immune, group B streptococcus (GBS) negative. Amy has been breastfeeding every 3 to 5 hours. She has had one stool and three urine diapers a day. Her weight is 15% less than her birth weight. On your examination, you notice jaundice from the head to the thighs. Her total bilirubin level is 25.2 mg/dL. The conjugated (direct) fraction is 0.4 mg/dL. The unconjugated (indirect) bilirubin is 21.2 mg/dL.

Question 13.2.1 Which of the following statements is correct?

A) She has breastfeeding failure jaundice.

B) She has physiologic jaundice.

C) She has biliary atresia; you must consult pediatric gastroenterology.

D) There is an ABO incompatibility between the mother and child.

Answer 13.2.1 The correct answer is "A." Amy most likely has breastfeeding failure jaundice. This occurs within the first several days of birth before the mother's milk supply is adequate. This must be distinguished from breast milk jaundice, which usually occurs later without evidence of dehydration. One sign that Amy is not receiving adequate breast milk feedings—and that this is breastfeeding failure jaundice instead of breast milk jaundice—is the fact that she is 15% below birth weight, having fewer than 6 wet diapers per day and fewer than two to five stools a day. Remember that a well-hydrated infant of Amy's age should lose no more than 10% of birth weight. You should try to evaluate the mother's milk supply by asking about the mother's feeling of engorgement, feeling of breast emptying with feeding, seeing milk on the infant's lips and tongue immediately after feeding, and hearing the infants swallow with feedings. "B" is incorrect. Even though physiologic jaundice peaks at this age, the level of 25.2 mg/dL is higher than would be expected with physiologic jaundice (which should not be higher than 17 mg/dL in a term infant). "C" is incorrect because biliary atresia presents with conjugated hyperbilirubinemia (the liver can appropriately conjugate bilirubin, but there is obstruction to the outflow of the conjugated bilirubin—essentially an obstructive process). "D" is incorrect because ABO incompatibility is unlikely in a mother whose blood type is something other than O (who have both anti-A and anti-B antibodies). In addition, Rh incompatibility is impossible due to the mother being Rh+. However, minor antigen incompatibility remains a possibility.

Question 13.2.2 Which of the following is NOT a risk factor for severe neonatal hyperbilirubinemia?

A) Exclusive breastfeeding.

B) Gestational age ≥41 weeks.

C) Significant birth trauma.

D) Visible jaundice in first 24 hours of life.

E) East Asian race.

Answer 13.2.2 The correct answer is "B." Postdates gestational age is not associated with jaundice. Rather, premature infants are at a greater risk of jaundice. "A" is true, and exclusively formula-fed infants are less likely to have severe hyperbilirubinemia (but this is not a reason to recommend bottle feeding). Cephalohematoma and large bruises result in increased bilirubin production from heme breakdown, so "C" is true. The earlier jaundice occurs, the higher the peak is likely to be, so "D" is also true. Other major risk factors for severe hyperbilirubinemia include a sibling who required phototherapy, East Asian race, and blood group incompatibility.

Amy's CBC is unremarkable and blood type is A+.

Question 13.2.3 Which of the following is the most appropriate initial treatment for this patient (remember the conjugated [direct] fraction is 0.4 mg/dL. The unconjugated [indirect] bilirubin is 21.2 mg/dL)?

A) Admission for exchange transfusion.

B) Admission for IV fluids alone to improve hydration.

C) Admission for phototherapy.

D) Discharge to home with recommendations for formula feeding, light exposure, and follow-up bilirubin tomorrow.

Answer 13.2.3 The correct answer is "C." Amy should be treated with intensive phototherapy given her level of hyperbilirubinemia. "A" is incorrect because phototherapy should be employed first and an exchange transfusion would follow if phototherapy failed to lower the bilirubin level or signs of acute bilirubin encephalopathy developed. "B" is incorrect since phototherapy is the definitive treatment. Adjunctive therapy with intravenous fluids is considered if weight loss exceeds 12% of birth weight, which in this case it does. "D" is incorrect because a serum bilirubin level of 25 mg/dL or higher is a medical emergency and requires immediate hospital admission for phototherapy.

You admit Amy for further management. You confirm Amy's current weight, which is indeed down 15% from birth weight. Children often lose about 10% of their weight after birth.

Question 13.2.4 Beyond what point in time is it considered problematic if the patient has not returned to his or her birth weight?

A) 5 days.

B) 7 days.

C) 10 days.

D) 14 days.

E) None of the above. Don't worry, be happy.

Answer 13.2.4 The correct answer is "D." Children should regain their birth weight by age 2 weeks. Anything beyond this is reason for concern.

Amy does well under your care (and the bili lights) and returns at age 2 months. Mom questions the need for immunizations during the well examination "since we never see these archaic diseases anymore—I mean, come on, I can't even find a chicken pox party." Plus, she's read information on the Internet and has concerns about immunization safety. And wasn't there a study about kids who suffered autism because of thimerosal in a vaccine? And another study that vaccines transformed children into space aliens? Unfortunately, Amy's mother's concerns are not unusual. Parents—and politicians—often harbor misconceptions about vaccinations.

Question 13.2.5 What is a common side effect that Amy might have after her immunizations at her 2-month visit?

A) Fever to 104°F.

B) Autism.

C) Diabetes.

D) Erythema at the site of immunization.

E) Symptomatic shedding of virus in her stool.

Answer 13.2.5 The correct answer is "D." Vaccine side effects, such as low-grade fevers (not to 104°F as in "A"), induration and redness at the site, and fussiness are common. However, they are self-limited. "B" and "C" are incorrect. Autism and diabetes have NOT been linked to vaccines in many large, well-designed studies. "E" is incorrect because the DTaP, HIB, and IPV vaccines are inactivated. Thus, the virus or bacteria is killed and purified for a specific component. The hepatitis B vaccine is constructed with genetic engineering in a yeast cell. Thus, one would not shed virus in the stool. The MMR, oral polio, oral rotavirus, and varicella vaccines are live attenuated vaccines. With these immunizations, the virus has been weakened but can still replicate and be shed in stool. In an immunocompetent host, this usually is not of clinical significance. Thus, "E" is incorrect. Also, oral polio vaccine is no longer used in the United States because the risk of disease from the vaccine is greater than the risk of disease from wild-type polio; IM polio vaccine only, please.

Amy's mother accepts your advice but still has concerns.

Question 13.2.6 What other factually correct information can you share with her about vaccines?

A) Giving a child multiple vaccines at the same time weakens her immune system.

B) The fever and rash side effects of the MMR are from the measles component and usually occur 1 week after the vaccine is given.

C) If Amy receives her MMR at a 9-month visit (before she travels to visit Aunt Tilley who lives in an area where measles has re-surfaced due to lack of vaccination), her MMR immunization would be considered complete after this dose and another one at 5 years old.

D) If Amy had an acute otitis media and fevers of 100.4°F, we should delay her immunizations until she is afebrile.

E) Immunizations are not important, since these diseases are rare in the United States.

Answer 13.2.6 The correct answer is "B." The measles component can cause a fever and rash 5 to 10 days after the immunization. This occurs in 5% to 15% of infants. Up to 25% of adult women may have arthralgias after receiving the vaccine. The MMR dosing schedule includes two doses of MMR. However, the first must be after 1 year of age. It may be given sooner if the child is at risk (such as with travel or with a measles outbreak) but must be repeated after the first birthday. Thus, "C" is incorrect. Minor illnesses should not prevent vaccination. True contraindications include anaphylactic reactions to a vaccine or vaccine constituent, moderate-to-severe febrile illness, and encephalopathy within 7 days of DTaP. Live virus vaccines are contraindicated in immunocompromised patients.

Amy continues her scheduled well child examinations. At one of those visits, she was babbling and crawling around the office. She poked her fingers at the outlets (which thankfully were covered) and used a pincer grasp to pick up a raisin off the floor (ew, gross!).

Question 13.2.7 These behaviors are appropriate for the development of a child at approximately age:

A) 6 months.

B) 9 months.

C) 15 months.

D) 24 months.

Answer 13.2.7 The correct answer is "B." Take a moment to review the developmental milestones in Table 13-1.

Amy continues her scheduled well child examinations. However, at 14 months old, mom brings Amy for a sick visit because she turned blue. After taking a complete history and doing a complete physical examination, you appropriately diagnose breath-holding spells.

Question 13.2.8 Which of the following statements about breath-holding spells is true?

A) The incidence of breath-holding spells for children between 6 months old and 6 years old is 50%.

B) If a color change occurs, it occurs after loss of consciousness.

C) Seizure-like activity may occur with breath-holding spells.

D) A child typically takes 60 to 90 minutes to return to her baseline after a breath-holding spell.

E) The evaluation should include an echocardiogram (ECG) and electroencephalogram (EEG).

Answer 13.2.8 The correct answer is "C." A typical breath-holding spell begins with an inciting event (like Santa did not bring the right toy or mom refused to buy a Happy Meal). Breath-holding spells occur in up to 4% of children and 80% start before 18 months. The child begins to cry, holds his or her breath, turns blue, and (may) lose consciousness. After loss of consciousness, some rhythmic jerking of the extremities may occur. The loss of consciousness is brief and the child returns quickly to normal activity (no post-ictal state). The differential diagnosis includes cardiac arrhythmias, seizures, and apnea. If the history is classic for breath-holding spells, no further evaluation is necessary. The treatment is parental reassurance. Parents should be encouraged to ignore the episodes and not to give in to child's requests in attempt to avoid the spells. Iron supplementation may decrease the frequency of spells if anemia or iron deficiency is present.

Question 13.2.9 Between 12 and 15 months of age, Amy should receive all of the following vaccines, as per Centers for Disease Control and Prevention (CDC) recommendations, EXCEPT:

A) MMR.

B) Varicella.

C) Hepatitis A.

D) Rotavirus.

E) Influenza.

Answer 13.2.9 The correct answer is "D." Vaccination schedules and recommendations seem to represent quickly moving targets, so a regular review is required to keep up to date. At 12 to 15 months, the CDC recommends initial MMR, varicella, and hepatitis A vaccinations for all U.S. children. Influenza recommendations are updated annually and have become progressively more inclusive over time. The CDC recommends that all children ages 6 months through 18 years receive the influenza vaccine annually during the appropriate season. If the child is receiving her first year of influenza vaccination and is between 6 months and 8 year of age, she should receive 2 doses about 1 month apart to help boost immunity. Following this, the child may receive one dose of influenza vaccination annually. Of note, rotavirus vaccine should not be administered to children older than 32 weeks, and the first dose should not be given after 15 weeks.

Of course, the vaccine schedule is updated periodically but does not usually change drastically from year to year. We have included the current vaccine schedule in Figure 13-1. For the most up to date version, go to the CDC website at http://www.cdc.gov/vaccines/schedules/hcp/child-adolescent.html. There is also an excellent CDC app for immunization for both the iPhone and Android platforms. It is easier to comprehend than the website.

FIGURE 13-1. Recommended immunization schedule for persons aged 0 through 18 years.

Amy's breath-holding spells resolve by the time she is 3 years old, and she continues to grow and develop normally. Amy's next issue comes when she is 5 years old and in your office for her pre-kindergarten physical.

Question 13.2.10 You notice a new murmur. Of the following, which suggests that this is a benign murmur?

A) Murmur is grade II/VI.

B) Murmur has a thrill.

C) Murmur radiates to the apex.

D) Murmur is diastolic.

E) Murmur is holosystolic.

Answer 13.2.10 The correct answer is "A." Not all murmurs need to be referred to a cardiologist. There are features that can help differentiate pathologic from benign murmurs. A murmur that is diastolic, grades III to VI, pansystolic, or associated with cardiac symptoms is likely pathologic and requires further investigation. A benign murmur typically is systolic, soft (grades I–II, occasionally III), nonradiating, and short. A "benign" murmur is not a diagnosis of exclusion. You should try to make a decision about which innocent murmur it is. See Table 13-2.

On Amy's examination, you hear a grade III/VI systolic murmur in the pulmonic area with fixed splitting of the second heart sound. She is otherwise healthy without any cardiac symptoms.

Question 13.2.11 What is the appropriate next step in management of this issue?

A) Family reassurance.

B) Endocarditis prophylaxis at the dental visit next week.

C) ECG and chest radiograph.

D) Limit physical activity.

E) Refer for immediate operative repair.

Answer 13.2.11 The correct answer is "C." Most likely, Amy has an ASD or atrial septal defect. The murmur is a systolic ejection murmur heard best at the upper left sternal border. The sound you hear is caused by increased flow across the pulmonic valve creating a relative stenosis (more volume needs to get through a relatively fixed outlet) and NOT from flow across the ASD. The increased flow across the pulmonic valve causes a wide, fixed split S2. There may be a mid-diastolic rumbling murmur at the lower left sternal border from increased flow across the tricuspid valve if the ASD is very large. The chest x-ray may demonstrate cardiomegaly with increased pulmonary vascular markings. The ECG can be normal or may show mild right ventricular hypertrophy, right axis deviation, and/or right bundle branch block with the characteristic rsR' pattern in the right precordial leads. Children with an ASD do NOT need endocarditis prophylaxis (except for the first 6 months after their surgical repair) and rarely need to limit their physical activity. Most small ASDs will close spontaneously by age 4 years. Closure by one of a variety of methods is recommended for symptomatic and significant left-to-right shunts with right ventricular enlargement.

Objectives: Did you learn to…

• Describe the causes of neonatal hyperbilirubinemia?

• Manage an infant with breastfeeding failure jaundice?

• Identify risk factors for severe hyperbilirubinemia?

• Recommend vaccines for children in accordance with CDC guidelines?

• Identify vaccine misconceptions, list MMR side effects, and identify contraindications to immunization?

• Recognize stages of infant development?

• Describe and manage breath-holding spells?

• Differentiate benign from pathologic cardiac murmurs in childhood?

The current recommendation for the treatment of breast milk jaundice (NOT breastfeeding failure jaundice as per the above case) is:

A) Continue to breastfeed.

B) Stop breastfeeding and change to a cow's milk formula.

C) Stop breastfeeding and change to soy milk formula.

D) Stop breastfeeding and treat with rehydration solution (e.g., Pedialyte).

The correct answer is "A." Patients with hyperbilirubinemia due to breast milk jaundice should continue to breastfeed. Breast milk jaundice begins on days 5 to 7 of life, peaks by 2 weeks of age, and usually resolves by 10 weeks of age; monitor to make sure that the jaundice remains unconjugated and there is no need to look for a second source. Bilirubin levels will gradually decline while breastfeeding is continued. If breastfeeding is discontinued, serum bilirubin levels decline rapidly. Kernicterus is rare. The etiology is unclear but glucuronidase in breast milk resulting in de-conjugation and increased enterohepatic recirculation of bilirubin has been proposed.

A family has moved into the area and brings their 12-month-old girl in for a well-child check. They bring her medical records with them. Her parents have no concerns, and she has been healthy. Her growth chart concerns you, however. Her length tracks along the 25th percentile. But her weight has gone gradually from around the 25 to 50th percentile at 0 to 6 months old to the 10th percentile at 9 months old, and now is at the 5th percentile at 1 year.

Question 13.3.1 Which of the following most likely explains her pattern of growth?

A) Normal.

B) Familial short stature.

C) Prenatal insult such as exposure to drugs or infection.

D) Hypothyroidism.

E) Inadequate nutrition.

Answer 13.3.1 The correct answer is "E." This patient's pattern of growth is consistent with failure to thrive (now termed "undernutrition"). The criteria for diagnosis include weight less than the 3rd to 5th percentile (depending on the source) or a fall of weight of >2 major percentile lines in 6 months or weight less than 80% of ideal weight for age and/or weight for height less than 5th percentile. The patient meets more than one of these criteria, so "A" is incorrect. In "B," both height and weight would be affected. In this child, only the weight (and not the height) is affected. "C" is incorrect because a child with a prenatal insult tends to be smaller globally in both height and weight, and the weight percentile would not be expected to drop so dramatically from birth. "D" is unlikely because in general, the endocrinologic causes result in "stunting" where a child stops gaining height but continues to gain weight. Thus, "E" is the most likely cause of this patient's failure to thrive.

Taking more history, you discover that the patient eats mostly rice and fruit. She does not like meat (happily supported by one of your editors who is vegetarian!). She is picky. She drinks about 12 oz of whole milk and 12 oz of apple juice each day. She is active and developmentally appropriate. Her review of systems is negative as is her past medical history.

Question 13.3.2 What is the LEAST appropriate way to proceed with evaluation and management of this patient?

A) Allow the child to feed herself whenever she is hungry or thirsty. Parents should offer food and drink frequently throughout the day.

B) Obtain CBC, iron studies, and urinalysis.

C) Limit juice intake.

D) Consult a nutritionist.

E) Prescribe a multivitamin.

Answer 13.3.2 The correct answer (and least appropriate approach) is "A." The goal of management of failure to thrive is to increase caloric intake, identify treatable causes of failure to thrive, and look for effects of her malnutrition. Thus, a CBC, iron studies, electrolytes, bicarbonate, glucose, and urinalysis may be helpful to investigate possible iron deficiency, anemia, or renal tubular acidosis. Juice should be limited (due to its low nutritional value), meals scheduled, and caloric intake boosted. Although children should feed themselves, they take in more calories when given a set schedule for meals and snacks instead of being allowed to graze on food or drink throughout the day as in "A." A nutritionist can help educate the family on appropriate food choices for a toddler and ways to boost calories. A child with a restrictive eating pattern, especially poor in iron-containing foods (such as meats), may benefit from a multivitamin with iron.

Your patient presents to the emergency department (ED) at 14 months old. Mom states that she has had several episodes of emesis over the last 48 hours and today began to have watery, foul-smelling diarrhea (as opposed to the yummy-smelling diarrhea to which we are so accustomed). Mom has lost track of number of episodes of emesis and diarrhea. She cannot tell if the child has had urine output because of the watery diarrhea. On your examination, the temperature is 102.5°F, heart rate 180 bpm, respiratory rate 50, blood pressure 80/50 mm Hg. Her weight is 8 kg. She has dry, cracked lips and dry skin. She lies in her mom's arms and is not very interested in your examination. Her capillary refill is about 4 seconds with some mottling of the extremities.

Question 13.3.3 What is the MOST appropriate next step in the management of this patient's condition?

A) Evaluate for underlying bacterial infection with blood, urine, and stool cultures.

B) Admit to an inpatient unit and begin maintenance IV fluids.

C) Infuse 20 mL/kg normal saline IV over 20 minutes.

D) Begin oral rehydration with Pedialyte.

E) Infuse 10 mL/kg D5/half-normal saline over 2 hours.

Answer 13.3.3 The correct answer is "C." This patient is severely dehydrated (estimated 15%) with signs of compromised tissue perfusion; therefore, she should have parenteral fluid resuscitation quickly with a 20 mL/kg isotonic fluid bolus. Although she might need admission, she should be stabilized in the ED before transfer to the inpatient unit; in addition, starting with maintenance fluids is inappropriate. Oral rehydration is cost-effective in less severe dehydration without compromised tissue perfusion. There are several tables published listing the clinical examination associated with various degrees of dehydration. See Table 13-3 for clues in determining the degree of dehydration.

After completing the initial management, the patient is more interested in her surroundings. Her vital signs are pulse 160 bpm, respiratory rate 36, and blood pressure 80/50 mm Hg. Her lips are still dry and she is irritable. The capillary refill is 2 to 3 seconds. She is more interactive with her mother.

Question 13.3.4 What further treatment is indicated for her dehydration?

A) 80 mL/hr × 2 hours of D5-quarter-NS.

B) 33 mL/hr × 24 hours of D5-half-NS with 20-mEq KCl.

C) 50 mL/hr × 24 hours of D5-quarter-NS.

D) 20 mL/kg NS over 20 minutes.

E) 10 mL/kg D5-half-NS over 2 hours.

Answer 13.3.4 The correct answer is "D." After an initial bolus, the patient should be reassessed. This patient is still moderately dehydrated. Although she has responded to her initial bolus of normal saline, she still has abnormal vital signs as well as signs of dehydration on examination. Therefore, the normal saline bolus should be repeated. Hypotonic solutions (e.g., 1/2 NS) or those containing glucose and/or potassium should never be used as a bolus. Also, potassium (if needed at all) should be withheld until the patient urinates and has better hydration status.

The patient recovers well from her GI illness, but she returns a few months later to your clinic with a new illness. She has had temperatures of 103°F at home for 2 to 3 days. She has had no upper respiratory symptoms. Her oral intake has decreased, but she is maintaining good urine output. She has had no vomiting or diarrhea. Your examination reveals a febrile child who is slightly irritable. She is nontoxic and not dehydrated. Her oral cavity shows increased tonsil size with ulcers on her tongue and lips but not on the tonsillar pillars. Her anterior cervical lymph nodes are enlarged. The rest of her examination is noncontributory.

Question 13.3.5 What is the most likely diagnosis?

A) Streptococcal pharyngitis.

B) Hand, foot, and mouth disease (coxsackie virus).

C) Herpetic gingivostomatitis.

D) Varicella.

E) Infectious mononucleosis.

Answer 13.3.5 The correct answer is "C." Primary oral herpes (herpetic gingivostomatitis) is associated with a relatively high fever and anteriorly placed ulcerations and vesicles (gums, tongue, and lips). Symptoms tend to start relatively abruptly with pain, salivation, refusal to eat, and fever. Herpes gingivostomatitis may recur during life in the form of "cold sores." "B" is incorrect because the patient does not have hand and foot lesions. "D," varicella, can occur in the oral pharynx but would have lesions elsewhere in various stages and respiratory symptoms as well. Infectious mononucleosis is not associated with vesicular lesions or mucosal ulcerations.

Next, you see the patient's sister who is age 10 and presenting for a school physical. Her mother is concerned because this patient has pubic hair, which her mother thinks is premature (and scary…her baby is not growing up yet!). On your examination, she has enlarged areola and a small amount of breast tissue. She also has sparse, dark, but mostly straight pubic hair.

Question 13.3.6 This patient is sexual maturity rating or Tanner stage:

A) 1.

B) 2.

C) 3.

D) 4.

E) 5.

Answer 13.3.6 The correct answer is "B." Breast buds and sparse, downy pubic hair put the patient in Tanner stage 2. Tanner stage 1 is prepubertal. Any sign of puberty moves a child from Tanner 1 to Tanner 2. Tanner stage 5 is adult or fully matured secondary sexual characteristics. See Table 13-4 for details.

The mom has a lot of questions about puberty. She is nervous about beginning to discuss sexual development with her daughter.

Question 13.3.7 What is a developmental fact that you can provide the mom for discussion with her daughter?

A) Typically, in females, the first sign of puberty is pubic hair.

B) At 10 years old, the patient is experiencing premature puberty.

C) The height spurt occurs early during puberty and is almost complete at menarche.

D) Menarche occurs approximately 1 year after breast budding.

E) Schools discuss puberty and relieve mom or dad of that duty (whew!).

Answer 13.3.7 The correct answer is "C." In girls, the growth spurt occurs early and is almost complete at menarche. Females begin puberty about 1 to 2 years before males. "A" is incorrect as thelarche, or breast budding, marks female onset of puberty. "B" is incorrect. The average age of breast budding is 10.9 years. Thus, Sara is appropriate in her pubertal timing. "D" is incorrect. Menarche typically begins 2 years after breast budding. "E" is incorrect. Although schools may discuss puberty, these discussions are often limited in the time and content. It is important for the child to hear her mother's opinions on puberty and sex.

Objectives: Did you learn to…

• Define failure to thrive in a child?

• Evaluate a child with failure to thrive?

• Evaluate a dehydrated child and determine severity of dehydration?

• Manage dehydration in the ED?

• Calculate rehydration fluids for a dehydrated child?

• Generate a differential diagnosis for pharyngitis with blisters?

• Describe changes of puberty and Tanner staging in a female?

Which is not an indication for use of soy formula in an infant?

A) Galactosemia.

B) Congenital lactase deficiency.

C) Cow milk protein intolerance.

D) Parental desire for a vegan diet.

The correct answer is "C." Few indications exist for use of soy-based formula over cow milk-based formula. Soy formula is lactose free and therefore should be used in conditions "A," "B," and "D." Galactosemia is a metabolic disorder characterized by the inability to breakdown galactose. Dietary galactose comes from lactose found in human and cow milk. An extensively hydrolyzed or amino acid formula should be used for cow milk protein intolerance. Infants who are sensitive to cow milk protein have an approximately 10% to 15% chance of also being sensitive to soy proteins. Reflux deserves special note. There is no data supporting the use of soy formula for reflux.

A 2-year-old male presents to your office with his father complaining that the patient has had intermittent crying followed by episodes of profound lethargy during which he is difficult to arouse. The father relates that the patient will sometimes clutch at his abdomen and roll into the fetal position during episodes. These episodes have been going on for about 4 hours. He has had no fever, no vomiting, and no diarrhea. Nobody else at home has been sick. He is developmentally normal and has no significant past medical history. His examination reveals that he is afebrile and lethargic but can be aroused. His abdominal examination is benign. The rest of the examination is unremarkable.

Question 13.4.1 Given his presentation, your clinical suspicion is for:

A) Meningitis.

B) Renal colic.

C) Intussusception.

D) Gastric outlet obstruction.

E) Appendicitis.

Answer 13.4.1 The correct answer is "C." The patient may have intussusception. The combination of abdominal colic plus mental status changes should suggest intussusception. The etiology of the mental status changes is not clear, but mental status changes are one of the classical findings of intussusception. "A," meningitis, should still be a consideration in a patient with mental status changes, but abdominal pain is not usually part of the presentation. In addition, the patient is afebrile and his symptoms are intermittent, making meningitis less likely. "B" is not associated with mental status changes. "D" and "E" usually present with some abnormal findings on abdominal examination.

Question 13.4.2 Which laboratory finding will help you to confirm the diagnosis of intussusception?

A) CBC.

B) Urinalysis.

C) Glucose.

D) Serum lactate.

E) None of the above.

Answer 13.4.2 The correct answer is "E." None of the above will help to make the diagnosis of intussusception. Early in the course, the CBC may be normal and only 75% will have heme positive stools.

Question 13.4.3 You decide that this patient probably has intussusception based on your history and physical examination. The next step in diagnosing this patient is:

A) Upper GI with barium.

B) A plain film of the abdomen.

C) An air enema.

D) An abdominal ultrasound.

E) Administration of IV antibiotics.

Answer 13.4.3 The correct answer is "D." In general, an ultrasound is the diagnostic modality of choice. An ultrasound may show a typical "bull's eye" lesion helping to make the diagnosis. "C," an air enema, is a diagnostic and therapeutic intervention. An air enema can be used to reduce the intussusception obviating the need for surgery. In general, however, a diagnosis is first made by ultrasound. An upper GI will be of no use since intussusception is a distal process. A plain film of the abdomen will most likely be nondiagnostic as is the case in most abdominal processes (except perhaps bowel obstruction or free air).

Well, the ultrasound tech is in the Cayman Islands, so you opt for an air enema, which simultaneously diagnoses and reduces the intussusception. The patient appears well with no signs of perforation. He is eating goldfish crackers by the fistful in the ED and generally making a mess of the examination room. Recommendations vary as to the patient's disposition. Some authors recommend admission since recurrence rate is 10% in the first 24 hours while others would let the child go home. We suspect admission will be the right answer for the test.

Objectives: Did you learn to…

• Recognize the clinical presentation of intussusception?

• Evaluate and treat a child with intussusception?

Benjamin, a 4-month-old male, comes to your office for a well-baby check. His parents have no concerns. Development and feeding are progressing as expected. Looking at him, you think to yourself, "My gosh. Infants have such big heads relative to their bodies." On your examination, his head is >95th percentile, but his weight and height are still at the 50th percentile. His previous head circumferences have all been at the 50th percentile. He is a social infant but is having difficulty supporting his large head.

Question 13.5.1 What is the imaging study LEAST likely to assist in the diagnosis of the macrocephaly?

A) X-ray.

B) CT scan.

C) MRI scan.

D) Ultrasound.

Answer 13.5.1 The correct answer is "A." Because the relative head size is so dramatic, an imaging study is needed, but skull radiographs are not likely to assist you in arriving at a diagnosis. The other modalities are used in this setting, and each has its benefits and detractions. Ultrasound is often used in infants when the anterior fontanelle is still patent and offers the advantage of no radiation exposure. Ultrasound is primarily used to assess fluid-filled spaces (ventricles in hydrocephalus, subdural hematoma, etc.). CT scan is a rapid and readily available test that can detect hemorrhages, intracranial calcifications, skull fractures, Chiari malformations, and more but given the concern for radiation exposure and the risk of future malignancy, it is not the first line imaging technique. CT is also limited in its ability to delineate soft-tissue problems, which is where MRI becomes important.

Question 13.5.2 The ultrasound tech is still on vacation (why can't we have his job?) and your MRI is undergoing scheduled maintenance, so you order a CT scan, which shows subdural hematomas. What is your next step in managing this patient?

A) Admit to the hospital.

B) Examination of eyes in an ophthalmologist's office.

C) "Babygram" to view entire skeleton of infant for fractures.

D) Alert the department of human services within the next week.

E) Send the patient to a neurosurgeon's office for the next available appointment.

Answer 13.5.2 The correct answer is "A." Subdural hematomas in an infant are highly suspicious of abusive head trauma. Your first priority is to protect the child by admission to the hospital. A retinal examination and skeletal survey are important in the evaluation but are secondary to protection of the child. A "babygram" should never be done to evaluate for skeletal trauma. There is a specific procedure for performing a skeletal survey for abuse. Your state agency responsible for investigating child abuse must be notified within 24 hours (although clinicians are encouraged to know their legal responsibilities in the state in which they practice). Protection of the child is most important; if the family flees, calling the police or the appropriate state agency would be the first step. Suspicion of abuse, especially abusive head trauma, is an emergency, and it should not wait for a neurosurgeon's next available appointment.

As you have done in this case, radiological studies should always be obtained in the evaluation of suspected child abuse. However, the cause of fractures has a broader differential than abuse.

Question 13.5.3 Which patient's injuries and/or radiological studies would be pathognomonic of abuse?

A) A 9-month-old male with widened distal radius and ulna with fraying and cupping evident on radiograph.

B) A 21-month-old female with multiple fractures at different ages on radiograph. She has delayed tooth eruption and sparse hair.

C) A colicky 1-month-old male infant with callus on the left clavicle radiograph.

D) A 2-year-old female with a spiral fracture of the distal right tibia.

E) None of the above.

Answer 13.5.3 The correct answer is "E." None of these injuries is diagnostic of abuse. Although each of these children would make you think of possible abuse, none of them have been abused. "A" is a child with rickets. "B" is a child with osteogenesis imperfecta. "C" is a child who sustained a clavicle fracture at birth. "D" is a child with a "toddler's fracture." A toddler's fracture is a spiral tibial fracture found in children age 9 to 36 months (see Chapter 12). These fractures are found in the distal one-third of the tibia.

Benjamin's evaluation shows bilateral subdural hemorrhages and retinal hemorrhages. His skeletal survey is negative. The state agency removes him from the home and places him in foster care. Benjamin then returns for his 6-month well child examination. He has been in foster care since you last saw him. His vision is being followed by an ophthalmologist. He smiles and squeals. His foster mother says, "Looks like he's right-handed. Does that mean that he's advanced for his age?" He rolls both ways. He has started on rice cereal and bananas. He sleeps well. On your examination, he continues to have macrocephaly but a soft fontanelle. His left hand is fisted. The rest of the examination is unremarkable.

Question 13.5.4 What is your assessment and plan for Benjamin?

A) Normal 6 months old: Routine anticipatory guidance and immunizations.

B) Infant with history of head trauma: Delay pertussis vaccination because of the autism risk.

C) Infant with history of head trauma: Perform Denver Developmental Screening Test and document findings; repeat in 3 months.

D) Hypertonic left upper extremity: Monitor progress at next well child check.

E) Hypertonic left upper extremity: Refer for further evaluation and treatment of his delay.

Answer 13.5.4 The correct answer is "E." Benjamin has a risk of developmental delays due to his abusive head injury. He should be monitored closely for delays. One way to monitor for delays is by performing a screening test, such as the Denver Developmental Screening Test. However, Benjamin has concerns that go beyond screening. His tightly fisted left hand with corresponding favoring of his right hand suggests spastic hemiplegia. Early hand preference is a worrisome sign. Typically hand preference is not definite before 18 to 24 months. Hand preference should prompt the physician to examine the other extremity. In this case, there is hypertonicity resulting from his trauma. Any child with concern of developmental delay should be referred for further evaluation. Some local and state agencies offer a free service to families to evaluate development and offer therapy. Do NOT watch and wait with a suspected delay. The earlier intervention begins, the better the outcome.

Objectives: Did you learn to…

• Recognize and evaluate macrocephaly in an infant?

• Generate a differential diagnosis of macrocephaly in an infant?

• Determine when child abuse should be included in the differential diagnosis?

• Describe some aspects of developmental delay and its management?

A mother brings her 18-month-old son in for a routine visit but is concerned that he is not as verbal as he was 3 months ago. She reports that he eats well. He just started walking. You wish to screen him for a pervasive developmental disorder (which includes autism, Rett syndrome, Asperger syndrome, childhood disintegrative disorder, and others).

What is the most appropriate office tool to use with this patient given his age and presenting symptoms?

A) Ages and Stages Questionnaire (ASQ).

B) Conner's Rating Scale—Revised Short Form.

C) Denver Developmental Screening Test.

D) Modified Checklist for Autism in Toddlers (M-CHAT).

E) Parents Evaluation of Developmental Status (PEDS).

The correct answer is "D." The Modified Checklist for Autism in Toddlers (M-CHAT for short) is a validated office-screening tool for autism spectrum disorders. It would be the most appropriate tool of the options given. Of note, the AAP recommends screening all children for autism spectrum disorders using a standard screening tool such as the M-CHAT at ages 18 and 24 months. "A" is incorrect as the Ages and Stages Questionnaire is a broad-based developmental screening tool, as is "C", the Denver Developmental Screening Test. "B" is incorrect because this test is used to detect ADHD. Note that the USPSTF does not recommend for or against autism screening between 18 and 30 months noting that there is insufficient evidence to take a position on such screening. However, most practitioners follow the AAP recommendations.

A 7-year-old male presents to your clinic with his mother with a complaint of enuresis. Evidently, this child has never been completely continent at night, wetting his bed several times per week. This has become somewhat of a problem for him now that his friends are having sleepover birthday parties. Plus, his mother confides that she's tired of paying for pull-ups and cleaning sheets. His incontinence is monosymptomatic (meaning no overactive bladder symptoms or daytime wetting).

Question 13.6.1 What percent of 7-year-old continue to have enuresis?

A) 1%.

B) 10%.

C) 20%.

D) 40%.

E) 75%.

Answer 13.6.1 The correct answer is "B." About 10% continue to have enuresis at age 7 years which tapers to 1% to 2% at age 15 years or greater. Even though this would suggest that it is a variant of normal, there is extreme social pressure on children age 7 to remain dry at night. Parental expectations may depend on their experience with other children. For example, if an older sibling was dry at night at age 3 or if a younger sibling is dry while an older child has enuresis, parents may not accept the bed wetting as normal. Age 7 is considered the cutoff for "normal" enuresis; therefore, beyond age 7 years, it is considered a problem.

Because he has had no period of nocturnal dryness, this patient has primary enuresis.

Question 13.6.2 Which of the following is likely to be part of this child's history?

A) A family history of enuresis.

B) A stressful event in the family such as the birth of a new child or parental divorce.

C) Increased fluid intake over the past 2 months.

D) History of urinary tract infections.

Answer 13.6.2 The correct answer is "A." Enuresis is divided into primary and secondary enuresis. Primary enuresis occurs in cases where there is never a consistent period of dryness at night. Secondary enuresis occurs when there is a period of dryness (by convention, 6 months) before the patient develops enuresis. "B," "C," and "D" would more likely be seen in children with secondary enuresis. Primary enuresis tends to be a familial trait.

This patient's examination is essentially normal including neurologic evaluation.

Question 13.6.3 Further evaluation of this patient should include all of the following EXCEPT:

A) Asking about a history of bowel problems.

B) Assessment of growth and development.

C) Investigation into family history of nocturnal enuresis.

D) Spine MRI to rule out pathologic lesion.

E) Urinalysis.

Answer 13.6.3 The correct answer is "D." Patients with enuresis who have an otherwise normal neurologic examination need not have an MRI done. If on examination this child had neurologic findings, an MRI would be indicated. All of the remaining options are part of a thorough evaluation of nocturnal enuresis. Of particular note is the assessment of growth and development (is this child neurologically delayed leading to enuresis?). Attention to bowel problems is also important. Fecal impaction can lead to incontinence. Asking about snoring can help to identify obstructive sleep apnea, which can be associated with enuresis.

You find no indication of an underlying cause, and you decide that this is primary enuresis. The parents are desperate for some sort of intervention to fix the problem, since it is becoming a major source of anxiety in the home and of teasing at school (of course anything can be a source of teasing at school).

Question 13.6.4 Which of the following should constitute the INITIAL treatment of this patient's primary enuresis?

A) Patient education and motivational training (e.g., rewards for staying dry).

B) Over-learning.

C) Enuresis alarm.

D) Nasal desmopressin (DDAVP).

E) Oral desipramine.

Answer 13.6.4 The correct answer is "A." All of the above have been found to be useful in the treatment of enuresis; however, the best initial approach includes education. Patients and parents should be informed of how common this condition is, how to reduce fluid intake in the evening without getting dehydrated, and how to schedule voiding. Motivational training plays a role as well. The other interventions are secondary. "B," over-learning, is thought to help prevent relapses in patients who have been successful with an enuresis alarm. Once continence is achieved with the alarm, the child drinks a set amount of fluid before bedtime. The amount is successively increased once dryness is achieved until a maximum is reached. The idea is that the patient is conditioned to respond to his increasing bladder capacity.

"C," an enuresis alarm, is an effective treatment but relatively expensive and requires significant motivation on the part of the family. The enuresis alarm increases success of the other options, and when used in combination with other modalities, it has shown the best long-term results. "D" and "E" are incorrect in this scenario although they are used to treat enuresis. Relapse is more common when pharmacologic therapy is discontinued than with the other modalities. However, medications are effective as a short-term treatment option but are felt to be second tier and used when the urine alarm fails or is impractical (think sleepover or summer camp). Of note, "D" is wrong for another reason: nasal DDAVP is no longer approved for nocturnal enuresis due to problems with hyponatremia. Oral DDAVP still carries the enuresis indication, but it can cause hyponatremia as well—it just occurs less often.

Objectives: Did you learn to…

• Define primary and secondary enuresis?

• Describe some of the epidemiologic characteristics of enuresis?

• Evaluate a patient with enuresis?

• Develop a management strategy for enuresis?

Delayed meconium passage occurs in which of the following conditions?

A) Encopresis.

B) Hirschsprung disease.

C) Cystic fibrosis.

D) Hyperthyroidism.

E) B and C.

The correct answer is "E." Seventy percent of newborns will pass meconium within the first 12 hours of life. More than 90% of newborns pass meconium within the first 24 hours. In those with delayed passage of meconium (generally defined as >24 hours of life), consider Hirschsprung disease, cystic fibrosis, hypothyroidism, sepsis, intrauterine narcotic exposure, or imperforate anus.

Your next patient is a 3-year-old female with a rash. After playing outside (could playing out-side be a red herring? The editors think so …), her father found spots on her legs, and she did not want to walk because her knees hurt. On examination, she is afebrile with normal vitals and slightly irritable but otherwise interactive. She has slight nasal drainage, which her father says is a residual from a cold last week. Her legs and buttocks have palpable purpura. Her knees are mildly swollen and are painful with range of motion. Her CBC (including platelets) and coagulation studies are normal, and her urinalysis is significant for 2+ hematuria.

The most likely diagnosis in this patient is:

A) Acute exposure to lawn chemicals.

B) Henoch–Schönlein purpura (HSP) AKA "Immunoglobulin A vasculitis".

C) Juvenile idiopathic arthritis (JIA).

D) Meningococcemia.

E) Rocky Mountain spotted fever.

The correct answer is "B." This is classic HSP, an IgA-mediated, generally self-limited, leukocytoclastic vasculitis. The symptoms and signs of HSP are a rash (typically nonthrombocytopenic purpura), abdominal pain (from submucosal hemorrhage and edema), arthritis/arthralgia, and renal disease. Typically, the vasculitis follows an upper respiratory infection or streptococcal pharyngitis, as in this case. Treatment for HSP is supportive.

"A," lawn chemical exposure, usually results in nothing acute but may cause acute cholinergic symptoms if organophosphates are used. "C," JIA, is not likely because the rash of systemic onset JIA is evanescent salmon-pink rash on the trunk and axilla that classically occurs when the patient spikes a fever. "D," meningococcemia, is incorrect because the patient is afebrile, alert, and nontoxic appearing without laboratory evidence of disseminated intravascular coagulopathy. "E" is unlikely because Rocky Mountain spotted fever presents with headache, high fever, petechial rash involving the palms and soles, and the patients will appear toxic. In addition, patients with Rocky Mountain spotted fever have thrombocytopenia when their petechiae appear.

A 7-month-old fully immunized female presents to the office with fever. Mom reports she has "not been herself" and felt "a bit warm on the forehead." Vitals reveal a temperature of 39.2°C. Physical examination reveals a mildly toxic infant. You perform a complete history and physical but are unable to identify a source of the fever.

Question 13.7.1 What is the first step in your approach to this child?

A) Obtain a urinalysis and urine culture.

B) Admit for observation and perform blood, urine, and CSF for culture.

C) Give an intramuscular dose of ceftriaxone.

D) Order acetaminophen 30 mg/kg and discharge the patient if the temperature comes down.

E) Admit for IV fluids overnight.

Answer 13.7.1 The correct answer is "A." At this age, occult serious bacterial infection is less likely than during the neonatal period especially when vaccinated against Haemophilus influenzae type b and Streptococcus pneumoniae. In a well-appearing child older than 90 days with a fever without a focal source, most experts recommend a screening urinalysis and urine culture as the initial diagnostic test. "B" is overly aggressive, and "C" is not indicated and can make cultures more difficult to interpret should this child become sicker. "D" might be appropriate although the dose of acetaminophen is wrong (it should be 15 mg/kg/dose by mouth every 4–6 hours). Since you are not seeing signs of dehydration, "E" is inappropriate.

Question 13.7.2 What is the most appropriate manner in which to obtain a urine specimen in this child?

A) Midstream "clean-catch."

B) Bag specimen from sterile plastic bag taped to the perineal region.

C) Bladder catheterization.

D) Suprapubic aspiration.

E) C or D.

Answer 13.7.2 The correct answer is "C." The gold standard test for the diagnosis of urinary tract infection is a quantitative urine culture. While the urinalysis and microscopy may suggest UTI, no component, or combination of components, is as sensitive or specific as culture. Therefore, if a child is likely to require antimicrobial therapy, one should attempt to obtain urine for culture in a manner not likely to cause contamination. The midstream "clean-catch" may be obtained in older children who are toilet trained; making certain that the perineum is cleansed first. Bag specimens are prone to contamination and should be avoided in toxic patients who are likely going to need antibiotics. Suprapubic aspiration is generally not necessary though it is the gold standard.

Question 13.7.3 The patient has 20 WBC/hpf on UA. Without knowing the culture and susceptibility results, which antibiotic would be the LEAST appropriate choice for the treatment of a UTI in this child?

A) Trimethoprim/sulfamethoxazole (TMP/SMX).

B) Amoxicillin.

C) Cefdinir.

D) Cefixime.

Answer 13.7.3 The correct answer is "B." The majority of urinary tract infections are due to Gram-negative organisms such as Escherichia coli in children and the resistance rate to amoxicillin is high (up to 40%). Thus, amoxicillin is not a good choice for treating a UTI in children—or anybody, for that matter. The usual choice for empiric oral antibiotic treatment of UTI in children includes sulfonamides or third-generation cephalosporins. Caution should be used with empiric use of TMP/SMX due to increasing resistance of E. coli to this agent. Fluoroquinolones may be considered in adolescents or children older than 1 year with a complicated UTI; fluoroquinolones do seem to be safe in children but should be saved for situations where other drugs are sub-optimal. The duration of treatment should be 7 to 10 days for uncomplicated UTI. If the child appears clinically ill suggesting pyelonephritis, initial parenteral treatment with ceftriaxone would be an appropriate first-line agent.

Her urine culture reveals Escherichia coli susceptible to trimethoprim/sulfamethoxazole, and you treat her appropriately.

Question 13.7.4 Following her initial treatment, what diagnostic study should be performed next?

A) Abdominal supine and upright radiographs.

B) Renal ultrasound and voiding cystourethrogram (VCUG).

C) Spiral CT of abdomen and pelvis.

D) Urology consultation for urodynamic studies.

E) None of the above.

Answer 13.7.4 The correct answer is "E." In febrile infants with UTI, it is important to look for anatomic abnormalities of the urinary tract. Ultrasound imaging (not VCUG) is recommended following a first febrile UTI to identify those at risk for recurrence and subsequent damage to renal parenchyma (male or female). So, "B" would be correct if not for the inclusion of VCUG. Urinary tract ultrasonography should be performed at the earliest convenient time. Ultrasound is useful in the evaluation for kidney size and hydronephrosis, but it is limited in the evaluation of vesicoureteral reflux (VUR), a common abnormality associated with recurrent UTI. VUR is evaluated with a VCUG or radionuclide cystogram (RNC). VCUG should be done after a first febrile UTI only when there is evidence of renal abnormality on the ultrasound including scaring, hydronephrosis, abnormal anatomy, etc. Routine VCUG is NOT recommended. Thus, choice "B" is incorrect.

For some unknown reason, a VCUG is done (you said "Is it time for tea?" and it got transcribed as "do a VCUG") and your patient is found to have grade II reflux (see Table 13-5).

Question 13.7.5 Which of the following would be the most appropriate initial management of her grade II reflux?

A) Prescribe prophylactic antibiotics.

B) Refer for surgical intervention.

C) Perform GFR to assess renal scarring.

D) Observe for now with repeat VCUG in 6 months.

E) None of the above.

Answer 13.7.5 The correct answer is "E." Since the last edition of this book things have just gotten more muddled. It seems (most) everyone agrees that children with grade I–II reflux with bladder and bowel dysfunction benefit from prophylactic therapy. In those with grade I-II reflux and no bladder or bowel dysfunction the benefit is less clear. It gets a bit trickier with grade III–IV. Overall, children with III-IV reflux seem to have about half the number of infections with prophylactic therapy (typically with TMP/SMX); the benefit is most pronounced in those with a febrile first episode and bowel or bladder dysfunction. However, there is no difference in renal scarring and 63% develop resistant organisms (vs. 19% in the placebo group) (N Eng J Med. 2014;370;2367–2376 and N Eng J Med. 2014;370: 2440–2441). Have a discussion with the parents. And promptly treat any UTIs to prevent renal scarring (particularly a risk in those with a temperature of >39°C or a non E. coli organism). Mild reflux tends to resolve with maturity. Renal scarring is best detected by radioisotope scanning with 99 m-technetium dimer captosuccinate. Doing a GFR looks at kidney function and does not assess for renal scarring. Therefore, "C" is incorrect. "D" is incorrect because a VCUG was not indicated in the first place!

Objectives: Did you learn to…

• Evaluate an older (>3 months old) infant with fever?

• Diagnose and treat a child with an initial urinary tract infection?

• Recognize that urinary tract infection is often a marker for urinary tract abnormalities in children?

• Manage a patient with VUR?

Anthony is a 2,300-g male infant born at 35 weeks' gestation to a 23-year-old G2P2 mother. The mother did not seek prenatal care and routine screening labs were not available. She presented to the ED in active labor shortly following spontaneous rupture of membranes at home. Upon transfer to the obstetric ward, she was noted to be febrile (38.8°C) and received intravenous antibiotics. Five hours later, the infant was delivered via spontaneous vaginal delivery.

Question 13.8.1 Management of this newborn infant whose mother probably has chorioamnionitis (not simply GBS screen positive) should include which of the following?

A) Empiric therapy with ampicillin and gentamicin.

B) Empiric therapy with ampicillin.

C) Empiric therapy with vancomycin.

D) Empiric therapy with ceftriaxone

E) Empiric therapy with ampicillin and erythromycin.

Answer 13.8.1 The correct answer is "A." Maternal chorioamnionitis is an important risk factor for early onset sepsis in the newborn. The 2010 CDC guideline on the prevention of perinatal group B streptococcal disease recommends that all infants born to mothers with chorioamnionitis have screening labs and blood cultures at birth followed by minimum of 48 hours of antibiotic therapy. Antibiotic therapy may be extended and/or modified depending on the clinical course and culture results. A lumbar puncture for CSF culture and analysis should be performed if the child is septic appearing. Ampicillin and gentamicin as empiric therapy cover the most likely organisms including GBS and Gram-negative pathogens, respectively.

Anthony is started on appropriate antibiotics. Examination of this neonate reveals hepatosplenomegaly, lymphadenopathy, petechiae, and white mucocutaneous patches in the infant's mouth. His CBC reveals hemoglobin of 12 g/dL and a platelet count of 85,000/mm³ with evidence of hemolysis on the peripheral smear.

Question 13.8.2 The most likely pathogen infecting this infant is:

A) Rubella.

B) Cytomegalovirus.

C) Toxoplasma gondii.

D) Treponema pallidum.

E) Candida albicans.

Answer 13.8.2 The correct answer is "D." Neonatal manifestations of congenital syphilis include hepatosplenomegaly, lymphadenopathy, jaundice, rash, hemolytic anemia, and thrombocytopenia. Unfortunately, these findings overlap considerably with many of the other congenital TORCH (toxoplasmosis, rubella, CMV, herpes) infections. Abnormalities more specific to congenital syphilis include white, patchy mucocutaneous lesions (think "condylomata lata"), edema, rhinitis (snuffles), osteochondritis, and pseudoparalysis. Congenital syphilis is caused by transplacental transmission of the spirochete T. pallidum ("D"). Intrauterine infection can result in stillbirth, hydrops fetalis, or prematurity.

In addition to the laboratory data obtained above, you order an ultrasound of the patient's head, which appears normal.

Question 13.8.3 Intracerebral calcifications are CLASSICALLY associated with which congenital infection(s)?

A) Cytomegalovirus.

B) Toxoplasmosis.

C) T. pallidum.

D) Rubella.

E) A and B.

Answer 13.8.3 The correct answer is "E." The clinical manifestations of congenital cytomegalovirus and toxoplasmosis are often similar. Infants are typically asymptomatic at birth, but a significant number ultimately develop visual impairment, learning disabilities, and developmental and intellectual delays months to years later. Those infants who are symptomatic at birth may demonstrate intrauterine growth retardation, hepatosplenomegaly, jaundice, hemolytic anemia, and thrombocytopenia. Intracerebral calcifications also occur in both CMV and toxoplasmosis. The calcifications tend to be periventricular in CMV (remember this by the fact that the calcifications look like a "C" in CMV) and more dispersed throughout the cortex in toxoplasmosis (remember by noting the "X" in both cortex and toxoplasmosis). Additional central nervous system abnormalities include microcephaly, chorioretinitis, and sensorineural hearing loss.

Objectives: Did you learn to…

• Appropriately manage the neonate born to a mother who has evidence of chorioamnionitis?

• Recognize that many congenital TORCH infections may have overlapping signs and symptoms?

• Recognize that many infants with congenital TORCH infections are asymptomatic at birth?

A small for gestational age newborn infant is found to have hepatosplenomegaly, jaundice, and thrombocytopenia. Cardiac examination reveals a grade II/VI continuous murmur heard best at the left upper sternal border. An ophthalmologic examination demonstrates microphthalmia and cataracts.

The result of which maternal prenatal screening lab is likely to have been concerning in the above case?

A) VDRL.

B) Rubella immunity status.

C) HIV status.

D) Varicella zoster immunity status.

E) GBS cultures.

The correct answer is "B." Cardiac, ophthalmologic, auditory, and neurologic findings predominate in the symptomatic infant with congenital rubella. Up to 85% of infants infected during the first 12 weeks of gestation will have some form of congenital defect. This decreases to 5% when the primary infection occurs after the third to fourth month of gestation. Ophthalmologic findings include microphthalmia, cataracts, glaucoma, and salt and pepper retinopathy. Infants are often microcephalic and develop sensorineural hearing deficits, meningoencephalitis, and developmental delay. Deafness, cataracts and cardiac anomalies are the classic findings with congenital rubella. Additional findings of hepatosplenomegaly, thrombocytopenia, and osteitis may be present. A characteristic "blueberry muffin" appearance may be present due to the combination of jaundice and extramedullary (skin) hematopoiesis. Syphilis and GBS infections are described above and they do not typically cause the ophthalmologic findings described in this case. "C" is incorrect because congenital HIV infection is commonly asymptomatic. Evidence of immune deficiency may present later in the child's life with failure to thrive, generalized lymphadenopathy, hepatosplenomegaly, and recurrent infections. "D" is incorrect because congenital herpes infections do not cause cardiac abnormalities.

Which of the following cardiac lesions is likely to be found in an infant with congenital rubella?

A) Patent ductus arteriosus (PDA).

B) Tetralogy of Fallot.

C) Transposition of great vessels.

D) Coarctation of aorta.

E) Bicuspid aortic valve.

The correct answer is "A." The most common cardiac lesions associated with congenital rubella are a PDA and peripheral pulmonary artery stenosis. PDA is characterized by a continuous "machine-like" murmur heard best over the left upper sternal border. Tetralogy of Fallot and transposition of great vessels are classically cyanotic heart defects that have no known association with congenital infections. Coarctation of the aorta and bicuspid aortic valve both cause harsh systolic murmurs. The murmur of coarctation radiates to the back. Both coarctation and bicuspid aortic valve are commonly found in females with Turner syndrome.

A term newborn infant is noted to have a solitary, tense bulla located on the dorsum of his wrist. The underlying skin is nonerythematous. Pregnancy was uncomplicated; however, the infant was delivered via cesarean section for failure to progress. The infant appears to be a vigorous feeder and is noted to frequently suck on his hands and wrists while in the nursery. He is otherwise asymptomatic.

Question 13.9.1 Which of the following is MOST appropriate at this time?

A) Start acyclovir.

B) Perform lumbar puncture and send a sample of the CSF for HSV PCR.

C) Perform a Tzanck smear of the bulla fluid.

D) Obtain CBC and blood cultures.

E) Observation with no intervention at this time.

Answer 13.9.1 The correct answer is "E." Nothing needs to be done at this time. The appearance and location of the bulla is consistent with a sucking blister. A solitary bulla or blister on normal-appearing skin located on the upper limbs may be present at birth from in utero sucking. Often when presented with the affected extremity, the infant will demonstrate the sucking behavior in that location. Such blisters are often mistaken for herpes simplex, but the solitary nature and location help to establish the correct diagnosis—although there may be multiple blisters at times. Additional history of cesarean section makes HSV less likely in this scenario. Nonetheless, a sucking blister is a diagnosis of exclusion, and other more serious diagnoses should be ruled out by history and examination and further testing as indicated.

Question 13.9.2 The FALSE statement regarding neonatal herpes simplex virus infection is:

A) The majority of infants with neonatal HSV are born to mothers with symptomatic herpetic lesions.

B) The incidence of neonatal transmission is higher when the pregnant woman experiences primary infection versus secondary reactivation.

C) Most neonatal HSV infections are caused by HSV 2.

D) Intrauterine exposure accounts for the minority of perinatal HSV infection.

E) Herpetic skin lesions are often present in infants with CNS or disseminated HSV.

Answer 13.9.2 The correct answer is "A." Less than one-third of infants with neonatal HSV are born to mothers with active genital lesions. In fact, most cases of neonatal HSV are found in infants of asymptomatic mothers who are shedding the virus. Neonatal herpes simplex virus often results in serious morbidity and mortality. Infants with HSV present with one of the following three disease patterns with overlapping features: localized disease involving the skin, eyes, and mouth; CNS disease; or disseminated infection. Encephalitis may occur with or without skin involvement; therefore, HSV infection should be suspected in any infant from birth to approximately 4 weeks of age that presents with fever, seizure, or mental status abnormalities such as lethargy, irritability, or poor feeding.

The other options are correct. A woman who contracts a primary infection during the pregnancy has a greater than 30% chance of transmitting the virus to her infant ("B"). There is less than 2% transmission if she experiences a secondary reactivation intrapartum. The majority of neonatal HSV is due to infection with HSV 2 ("C"). Only 5% of congenital infections occur in utero ("D"), as the majority occurs via exposure to infected cervical secretions during birth. Skin lesions ("E") are present in 60% of CNS cases and about 75% of disseminated cases.

Objectives: Did you learn to…

• Differentiate between a herpes lesion and a sucking blister?

• Recognize rates and modes of transmission of herpes in neonates?

• Identify some of the clinical manifestations of neonatal herpes?

A bunch of parents in your neighborhood decide to have a "chickenpox" party (prompting you to consider moving to a place where people believe in modern medicine). They want to synchronize their kids' chickenpox outbreaks with their busy schedules (heaven forbid we should get immunized—after all, it isn't natural!). One of the mothers, who has never had chickenpox herself, finds out that she is pregnant the day after being exposed to chickenpox. She is not yet symptomatic (from the pregnancy or the chickenpox).

Question 13.10.1 The best advice for this potential mother about her pregnancy is:

A) Consider termination since it is likely that the child will have congenital varicella.

B) Get treated within 96 hours of exposure with varicella immune globulin.

C) Get treated within 96 hours of exposure with varicella immune globulin plus varicella vaccine.

D) Get treated within 96 hours of exposure with IVIG plus varicella vaccine.

Answer 13.10.1 The correct answer is "B." Women who are varicella-susceptible (nonimmune), pregnant, and exposed to chickenpox should be treated with varicellaimmune globulin (VariZIG) within 96 hours of exposure. If VariZIG is unavailable, IVIG may be given instead. The vaccine should be avoided during pregnancy (thus "C" and "D" are incorrect). It is a live, attenuated, virus and still carries some risk to the fetus (although the degree of risk is unknown). It is safe to give the vaccine to mom in the postpartum period and is indicated if the mother is screened to be varicella nonimmune during her pregnancy. The risk of congenital varicella syndrome if the fetus is exposed in the first trimester is about 2%. The risk of neonatal infection is much higher if the mother is infected within 5 days of birth, and 30% of these cases result in severe neonatal varicella infection.

Question 13.10.2 You can let the mother know that:

A) Varicella immune globulin has been shown to reduce the risk of congenital varicella.

B) Varicella immune globulin is aimed mostly at attenuating the case in the mother should she be infected.

C) Varicella immune globulin has not been shown to prevent congenital varicella.

D) A and B.

E) B and C.

Answer 13.10.2 The correct answer is "B." Varicella immune globulin is aimed at reducing the symptoms in the mother and attenuating her case of varicella. Both "A" and "C" are false. There are no good data either way about how varicella immune globulin will affect the child's outcome vis-à-vis congenital varicella.

Question 13.10.3 Manifestations of intrauterine infection with varicella zoster include all of the following defects EXCEPT:

A) Optic atrophy.

B) Congenital cataracts.

C) Cardiac abnormalities.

D) CNS abnormalities.

E) Limb hypoplasia.

Answer 13.10.3 The correct answer is "C." Cardiac abnormalities are not seen with varicella zoster exposure in utero. In addition to the findings above, patients with congenital varicella zoster will have cutaneous scarring called cicatrix.

Glory!! She found a chickenpox party! Now that all of these children have been infected with varicella zoster, the question arises about how long they should be kept out of school.

Question 13.10.4 These children are considered infectious until:

A) 5 days after the first lesion appears.

B) 10 to 14 days after the first lesion appears.

C) New lesions are no longer forming.

D) All lesions are crusted over.

E) They are no longer febrile.

Answer 13.10.4 The correct answer is "D." Patients are considered infectious until all lesions are crusted over. Lesions need not have healed entirely, only crusted over.

Objectives: Did you learn to…

• Offer treatment for varicella exposure to a pregnant nonimmune female?

• Recognize the uses of varicella immune globulin?

• Identify symptoms of congenital varicella infection?

A 3-year-old child presents to your office with a history of bright red cheeks. Over the past several days, she has had mild fever with mild muscle aches. Except for the rash, the child is now asymptomatic.

Question 13.11.1 The most likely cause of this patient's illness is:

A) Parvovirus B-19.

B) Herpes virus 6.

C) Rubeola.

D) Rubella.

E) Influenza virus.

Answer 13.11.1 The correct answer is "A." A mild systemic illness followed by red, "slapped cheeks" is typical of erythema infectiosum or "fifth disease," caused by parvovirus B-19. The red, slapped cheeks are generally followed by a lacy, reticular rash on the extremities and trunk. This is generally a self-limited illness in children.

Question 13.11.2 Which of the following is NOT a complication of parvovirus B-19 infection—either congenital or acquired?

A) Aplastic anemia.

B) Birth defects, including CNS and limb disease.

C) Hydrops fetalis.

D) Inflammatory arthritis.

E) Intrauterine fetal death.

Answer 13.11.2 The correct answer is "B." Parvovirus B-19 causes all of the above except for birth defects. Fetal death, anemia, or nonimmune-mediated hydrops fetalis are potential outcomes of intrauterine exposure (one-third of infected women will pass the virus to their fetus). Most infants infected in utero are born normally at term, including those with evidence of hydrops earlier in the pregnancy. A small subset will acquire chronic or prolonged postnatal infection of unknown significance. "A," transient aplastic crisis, can occur in those with chronic hemolytic conditions such as sickle cell anemia. "D," inflammatory arthritis, is common in adults and adolescents with females more commonly affected.

The question of whether or not to exclude this child from preschool is raised. The patient still has the rash. There are a couple of pregnant teachers at the preschool.

Question 13.11.3 You can tell the child's mother that:

A) This child is infectious and should be excluded from preschool until the rash resolves.

B) Since almost all adult women are already immune, there is no need to worry about transmission to the teachers.

C) Since this virus is an enterovirus, careful hygiene in the school will prevent spread.

D) This child can be allowed back into preschool and is no longer infectious.

Answer 13.11.3 The correct answer is "D." Once the rash is present, this child is no longer infectious and can be allowed back into school. "B" is incorrect because only about 50% of adult women are immune. "C" is incorrect. First, this is not an enterovirus. Even though URI symptoms are often absent, transmission is mostly respiratory with vertical transmission of mom to fetus, and hematogenous spread (via transmissions) being much less common.

Objectives: Did you learn to…

• Recognize the clinical manifestations of parvovirus B-19 infection ("fifth disease")

• Identify complications of parvovirus B-19 infection?