Biliary atresia This disorder is the prototypic biliary tract disorder in infancy, accounting for ^50% of all liver transplants in the pediatric age group and ~10% of all liver transplants. It presents with cholestasis in early infancy; there is a palliative surgical procedure called the Kasai hepatic portoenterostomy that, if performed before 60 days of age, may at least delay disease progression. In ^20-25% of infants in whom the procedure is done in a timely fashion, liver transplantation may never be necessary. Severe steatorrhea and malnutrition are common and malabsorption of the fat-soluble vitamins is profound, sometimes requiring parenteral administration (particularly of vitamin K) to achieve sufficiency. Nutritional deficiency disorders such as osteoporosis are common.
Primary sclerosing cholangitis This disorder most commonly presents in association with ulcerative colitis and less commonly with Crohn's disease or as an isolated entity. The nutritional management of the disorder is essentially like that of other cholestatic disorders; in patients with Crohn's disease of the small bowel, aggressive administration of an elemental diet rich in medium-chain triglycerides may be beneficial. It is accepted, however, that endoscopic interventions should be used as needed in the case of significant biliary obstruction. For prevention of severe osteoporosis, supplementation with vitamin D and calcium is needed. Vitamin K and alendronate may be beneficial in increasing bone mineral density. Serum levels of the fat-soluble vitamins should be monitored in high-risk patients and vitamins replaced as appropriate.
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