Cholestatic Syndromes in Childhood and Catch-Up Growth

Abstract

Cholestatic liver diseases frequently result in malnutrition and growth retardation. The pathogenesis of malnutrition is multifactorial and includes reduced calorie intake, fat malabsorption, abnormal protein metabolism, and increased energy expenditure. Poor growth is a typical feature of progressive familial intrahepatic cholestasis (PFIC) and Alagille syndrome and is not directly related to impaired nutrient absorption. Chronic cholestatic liver disease requires intensive nutritional support that includes increased calorie intake and supplementation of lipid-soluble vitamins. If the oral route of supplementation is not sufficient, additional nasogastric tube feeding should be started (e.g., nocturnal feeding). Parenteral nutrition is needed only in selected cases. Intensive nutritional support does not result in good catch-up growth in PFIC and Alagille syndrome, however. Partial biliary diversion is the treatment of choice in selected patients with PFIC and Alagille syndrome but results in improved growth only in PFIC. Liver transplantation is required if liver disease progresses to liver failure – improved nutritional status is achieved within weeks after surgery. Still, patients with Alagille syndrome present with only slight catch-up growth after transplantation, while patients with PFIC and biliary atresia grow significantly.