Radiographic and Other Studies

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1. Radiologic studies. Long bone films for possible congenital infection; abdominal ultrasound with Doppler flows of hepatic and portal vessels; hepatobiliary scintigraphy (HIDA, DiSIDA) to evaluate hepatobiliary flow; MRCP/ERCP.

2. Other. Liver biopsy; exploratory laparotomy with intraoperative cholangiogram; bone marrow for suspected storage diseases.

V. Plan. A few of the previously listed disorders are amenable to medical or surgical treatment. For most, however, management is directed toward identifying and treating the complications of cholestasis.

A. Malnutrition. Secondary to malabsorption or steatorrhea and decreased intake.

1. Provide age-appropriate nutritional supplements that are relatively high in medium-chain triglycerides (about 40-60% of fat calories).

2. If oral intake is inadequate, patient may require nasogastric tube feedings.

3. Fat-soluble vitamin replacement. Follow levels of vitamin A, 25-hydroxyvitamin D, and vitamin E, as well as PT.

4. Closely monitor weight, anthropometrics, and linear growth.

5. Follow serum calcium, phosphorus, magnesium, zinc, and iron levels; may need replacement.

B. Pruritus. Various medications can be tried, including bile-acid binding agents (cholestyramine, colestipol, antacids), phenobarbital, rifampin, ursodeoxycholic acid, antihistamines, and carba-mazepine. Efficacy is not uniform. Biliary diversion surgery is occasionally used in severe pruritus. Intractable pruritus may be an indication for transplantation.

C. Hyperlipidemia and Xanthomas. More common with intrahepatic cholestasis than extrahepatic disease. Severe hyperlipidemia in Alagille disease is associated with atherosclerosis and renal lipi-dosis. Treat with bile-acid binding resins such as cholestyramine or ursodeoxycholic acid. No significant effect is seen with dietary saturated fat or cholesterol restriction, or with cholesterol-lowering agents.

D. Disease Progression. Monitor closely for progression of liver dysfunction with laboratory studies (CBC, electrolytes, glucose, BUN, creatinine, ALT, AST, alkaline phosphatase, GGTP, total and direct bilirubin, albumin, PT, ammonia levels, lipid profile, prealbumin) and physical exams.

E. Complications. Manage expectantly for complications such as ascites, infections, bleeding, and encephalopathy.

F. General Care. Support patient and family, and promote maintenance of general pediatric care, especially immunization. All live viral vaccines should be given, in an accelerated schedule if needed, prior to transplantation.

G. Referral. Provide early referral to a pediatric liver transplantation center if patient's diagnosis suggests chronic liver disease. Surgical referral for portoenterostomy in patients with presumed extrahepatic biliary atresia should occur before 6-8 weeks of age for optimal results.

VI. Problem Case Diagnosis. The 6-week-old male infant had cholestasis, hepatosplenomegaly, and recent onset of poor feeding with slow weight gain. Abdominal ultrasound confirmed organomegaly but did not detect a gallbladder. HIDA scan revealed no excretion after 24 hours. Liver biopsy and subsequent intraoperative cholan-giogram showed biliary atresia. Kasai procedure was performed before 8 weeks of age, and infant had good bile drainage postoperatively.

VII. Teaching Pearl: Question. Until what age is cholestatic jaundice considered physiologic?

VIII. Teaching Pearl: Answer. Although a moderate, indirect hyperbilirubinemia may be normal in neonates (so-called physiologic jaundice), direct hyperbilirubinemia (cholestasis) is always pathologic.

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