In 2015 we launched our SmartBanks for the Pediatrics In-Training and Qualifying exams. We’re excited to announce that we’ve upgraded both of those SmartBanks to their 2016 versions to reflect changes made in the ABP blueprints. In addition, we have added hundreds of questions to bring the total in each SmartBank to 800! Sample questions from each SmartBank are featured below. To purchase a subscription to either, click here.
PEDIATRICS QUALIFYING EXAM EXAMPLE QUESTION
Q: A 10-day-old full term female is brought to the ER after 10 episodes of bilious emesis and increasing abdominal distention over the last 24 hours. She has no past medical history. She passed stool within 48 hours of life and has had a soft stool every 1-2 days since discharge. On physical exam, she has mild tachycardia, a distended, tender, tympanic abdomen, and increased tone on digital rectal exam with no retained fecal material. Upon withdrawal of your finger, a large amount of liquid stool and foul smelling gas is released.
Which of the following is the BEST initial step in management of this patient?
A. Barium enema
B. IV antibiotics
C. Stool sample for Clostridium difficile toxin
D. Surgical correction
E. Upper GI series with small bowel follow through
Correct Answer: B. IV antibiotics
Hirschsprung Disease results from failure of ganglion cell precursors to migrate to the distal colon in utero, causing colonic dysmotility and spasm. In 80 percent of cases, the affected segment is limited to the rectosigmoid, but it can extend more proximally 10-15 percent of the time. Up to 95 percent of those with Hirschsprung will fail to pass meconium in the first 48 hours of life, but 5 percent will pass meconium and potentially go undiagnosed. Definitive diagnosis of Hirschsprung disease is made by demonstrating absence of ganglion cells in arectal biopsy and can be treated with pull-through technique.
Enterocolitis is one of the most serious complications of Hirschsprung Disease, as the mortality rate is high. This complication is seen in both undiagnosed Hirschsprung Disease (such as the patient in the vignette) as well as in those with incomplete resection of the affected bowel. Fecal retention causes distention of the bowel to the extent that blood flow is compromised and intestinal perforation can occur. Patients can present with signs of distal obstruction (abdominal distention, bilious emesis), fever, and eventually signs of shock (tachycardia, hypotension, lethargy). Digital rectal exam will demonstrate increased rectal tone without retained stool and explosive expulsion of foul- smelling stool upon removal of the finger (the so-called “blast sign”). Abdominal X-ray will show colonic dilitation.
Initial treatment is with broad-spectrum antibiotics (to address the bacterial overgrowth and risk of peritonitis) and fluid resuscitation.
Answer A: Although barium enema can be used to assist in the diagnosis of Hirschsprung disease, it is contraindicated when the child presents with acute enterocolitis due to the risk of iatrogenic perforation.
Answer C: Acute entercolitis can lead to bacterial overgrowth, causing abdominal distention and foul-smelling stools, but stool testing is secondary to patient stabilization.
Answer D: This patient will likely require surgical correction of her underlying Hirschsprung disease, but only after the acute illness is fully treated and the patient is clinically stable.
Answer E: Bilious emesis can be a sign of small bowel obstruction, for which an upper GI series may be helpful. However, the presence of abdominal distention suggests distal obstruction, and the presence of the “blast sign” and increased rectal tone make Hirschsprung-associated enterocolitis the most likely etiology.
The Bottom Line:
Undiagnosed Hirschsprung disease can present in the neonatal period as acute enterocolitis with abdominal distention and bilious emesis. Patients are at high risk of perforation and shock, and should receive broad-spectrum antibiotics and IV fluid resuscitation as quickly as possible.
For more information: Swenson, O. Hirschsprung’s Disease: A Review. Pediatrics, 2002; 105:914-918. Kliegman R, Stanton B, Geme J, Schor N, Behrman R. Motility Disorders and Hirschsprung Disease. In: Nelson Textbook of Pediatrics. 19th Ed. Philadelphia: Elsevier; 2010:1283-1287.
TRUELEARN INSIGHT // For the test, if you are presented with a newborn that fails to pass meconium in the first 48 hours, you should think: Hirschsprung, cystic fibrosis, anal stenosis.
PEDIATRICS IN-TRAINING EXAM EXAMPLE QUESTION
Q: Darcy is an 8-year-old coming in for academic issues. She has a history of partial seizures that have worsened to grand mal seizures and has been on antiepileptic drugs (AEDs) for approximately 3-years, but she was recently changed from Levetiracetam after her irritable mood became too much for the family. She has been excessively tired and is having problems with remembering items she learned during the school day.
Which medicine is MOST likely to be causing these new academic issues?
C. Valproic Acid
Correct Answer: C. Valproic acid
Many medications impact academic performance. Side effects, particularly sedation, are common and children are particularly sensitive to this. Sedation not only causes falling asleep during the day or missing time, but also can cause irritability and aggression. (Think about how you feel when you’re tired and still being asked to work.) Medications that are necessary but cause sedation are problematic but a part of everyday practice. Working with known side effects is possible and options may include dosing before bedtime or spacing doses. Other side effects besides sedation impact children and their performance. As mentioned in the vignette, levetiracetam, an antiepileptic, are known to cause behavioral changes and aggression completely independent of sedation. Academic performance is also easily impacted by a variety of other factors, such as home life, sleep duration, and caffeine intake.
Valproic acid is an antiepileptic used in the treatment of generalized or grand mal seizures. Valproix acid has significant cognitive impact, such as memory problems, as well as headaches. (There are multiple other side effects to remember for Valproic acid for boards, but for school performance these are key.) Valproic acid inhibits CYP2C9, causing interactions with a variety of medication metabolized through the same enzyme system and so worsening the side effects of other medications as the medication builds up from inappropriate metabolism.
Answer A: Diphenhydramine is an antihistamine used in allergy control, not seizure control. While it is highly sedating and would impact academic performance at least initially, the child would not be on Diphenhydramine for this vignette.
Answer B: Ethosuximide is used to treat absence seizures, not grand mal seizures, and it is certain this child is not on this medication, even though sedation is a common side effect. Nephrotic syndrome and rash, as severe as Stevens-Johnson syndrome.
Answer D: Zonisamide is an antiepileptic used as an adjunctive therapy, particularly in partial seizures, infantile spasm, Lennox-Gastaut syndrome, and generalized tonic clonic seizures. It would be used in conjunction with another agent or after failing several agents. Side effects of note include renal stones, nystagmus, and attentional issues.
Answer E: Tiagabine is an antiepileptic use to treat partial seizures. There are multiple off label uses that specialists may opt to try Tiagabine (such as neuropathic pain or fibromyalgia). While confusion, difficulty speaking, and psychiatric issues are known side effects which would be a reasonable cause of the academic performance, this would not have been prescribed in this case as the patient is having grand mal seizures.
The Bottom Line:
A variety of medications can impact academic performance related to side effects, their known mechanisms, and idiosyncratic reactions. Antiepileptic drugs (AEDs) are a class of drugs that are known to cause sedation as well as cognitive slowing and may have a particular impact on school performance.
For more information: Hay W., Levin M., Sondheimer J, and Deterding R. Current Diagnosis & Treatment: Pediatrics. “Seizure Disorders (Epilepsies).” 20th ed. McGraw-Hill Companies, Inc; 2009:706-723.