A premature infant was demonstrated to have intestinal malrotation. After surgical repair, she experienced repeated episodes of emesis and abdominal distention. The differential diagnosis should include all of the following EXCEPT

Correct Answer: D

Rationale: In this scenario, the correct answer is D) cystic fibrosis. The rationale behind this is that cystic fibrosis is a genetic disorder that primarily affects the lungs and pancreas, leading to issues with respiratory and digestive functions. Intestinal malrotation, on the other hand, is a congenital condition where the intestine fails to rotate properly during fetal development. Following surgical repair for malrotation, complications like adhesions, electrolyte disturbances, and intestinal pseudo-obstruction can arise, leading to symptoms such as emesis and abdominal distention. Adhesions can form post-surgery, causing blockages or kinks in the intestines, leading to symptoms similar to those described. Electrolyte disturbances can occur due to vomiting and can exacerbate abdominal distention. Intestinal pseudo-obstruction is a condition where the intestines are unable to contract and push food through, leading to symptoms of obstruction. Educationally, understanding the differential diagnosis in a case like this is crucial for healthcare providers to provide appropriate care and treatment. Recognizing the potential complications post-intestinal malrotation repair helps in timely intervention and management to prevent further complications and ensure the well-being of the patient.

Correct Answer: C

Rationale: The correct answer is C) Both A and B. Hypoalbuminemia, FUO (Fever of Unknown Origin), tenesmus, sclerosing cholangitis, growth failure, and strictures can be seen in both ulcerative colitis and Crohn's disease, which are the two main types of inflammatory bowel diseases (IBD) affecting the pediatric population. In ulcerative colitis, hypoalbuminemia can occur due to protein loss in the inflamed colon. FUO may be present due to the systemic inflammatory response. Tenesmus, the feeling of incomplete defecation, can be a symptom of the rectal inflammation seen in ulcerative colitis. Sclerosing cholangitis is a rare but serious complication of IBD that can occur in both ulcerative colitis and Crohn's disease. In Crohn's disease, growth failure can result from malabsorption and chronic inflammation affecting the entire gastrointestinal tract. Strictures, which are narrowed areas in the intestines due to inflammation and scarring, are more common in Crohn's disease. Educationally, understanding the clinical manifestations associated with different types of pediatric gastrointestinal disorders is crucial for healthcare providers to make accurate diagnoses and provide appropriate management. Recognizing the similarities and differences between ulcerative colitis and Crohn's disease helps in tailoring treatment plans and monitoring for potential complications in pediatric patients with IBD.

Correct Answer: A

Rationale: Wilson disease is a rare genetic disorder characterized by copper accumulation in various tissues, predominantly the liver and brain. The correct answer, A) cardiomyopathy, is not typically associated with Wilson disease. Cardiomyopathy is more commonly seen in conditions like muscular dystrophy or certain viral infections. Hepatomegaly (B), ascites, and portal hypertension (C) are classic features of Wilson disease due to copper build-up in the liver leading to liver damage and dysfunction. Dystonia and tremor (D) are neurological manifestations of Wilson disease caused by copper accumulation in the basal ganglia of the brain. In an educational context, understanding the clinical manifestations of Wilson disease is crucial for healthcare providers to recognize and manage this condition promptly. By differentiating the associated symptoms, providers can initiate appropriate diagnostic testing and treatment strategies to improve patient outcomes. This question helps reinforce knowledge about the unique presentations of Wilson disease, enhancing diagnostic skills in pediatric GI disorders.

Correct Answer: D

Rationale: In this scenario, the correct answer is D) perform endoscopy to assess the severity of the ingestion. When a child is suspected of ingesting a strong alkali, it is crucial to assess the extent of damage to the gastrointestinal tract. Endoscopy allows direct visualization of the esophagus and stomach to determine the severity of injury and guide appropriate treatment. Option A) administer ipecac is incorrect because inducing vomiting is contraindicated in cases of ingestion of strong alkalis as it can cause further damage to the esophagus. Option B) administer milk is also incorrect as milk is not recommended for alkali ingestions as it may increase the risk of aspiration and does not neutralize the alkali. Option C) administer toast is incorrect as it does not play a role in the management of alkali ingestion and may cause further harm. Educationally, understanding the appropriate management of pediatric GI disorders, including ingestions, is critical for healthcare providers working with children. This question highlights the importance of prompt and appropriate assessment and treatment in cases of toxic ingestions to prevent further harm and guide patient care effectively.

Correct Answer: A

Rationale: In the context of pediatric liver transplantation, it is crucial for healthcare providers to understand the potential complications that can arise post-transplant. In this case, the correct answer is A) Epstein-Barr infection. Epstein-Barr virus (EBV) infection is common after liver transplantation and can lead to post-transplant lymphoproliferative disorder (PTLD), which is a serious complication characterized by uncontrolled lymphocyte proliferation. PTLD can progress to lymphoma if not managed promptly. Option B) Cytomegalovirus (CMV) infection is another common post-transplant complication, but it typically presents with different clinical manifestations and is not directly linked to lymphoma development. Option C) Hepatitis C infection is a concern in liver transplant recipients due to potential reinfection of the new liver, leading to liver damage over time, but it is not directly associated with an increased risk of lymphoma. Option D) Chronic rejection is a complication of liver transplantation characterized by ongoing immune-mediated damage to the transplanted liver. While chronic rejection can result in liver dysfunction, it is not the primary risk factor for lymphoma development post-transplant. Understanding these complications is vital for healthcare providers caring for pediatric patients post-liver transplantation. Early recognition and appropriate management of these complications can significantly impact patient outcomes and quality of life.