Isolated small bowel transplantation for tufting enteropathy.

PARAMESH A; FISHBEIN T; TSCHERNIA A; LELEIKO N; MAGID M; GONDOLESI GE; KAUFMAN SS.

JOURNAL OF PEDIATRIC GASTROENTEROLOGY AND NUTRITION

LIPPINCOTT WILLIAMS & WILKINS

Lugar: Philadelphia; Año: 2003 vol. 36 p. 138 - 140

0277-2116

Tufting enteropathy is a chronic malabsorptive syndromebeginning in infancy that is characterized histopathologicallyby the presence of ?tufts? of closelypacked surface enterocytes in the bowel, along with featuresof villous atrophy and crypt hyperplasia (1,2). Thesignificance of these tufts is not fully understood, andthere is no effective treatment (3). Patients usually requiretotal parenteral nutrition indefinitely, which canlead to line sepsis, total parenteral nutrition?inducedliver failure, and loss of intravenous access.Only one instance of combined liver/intestinal transplantationfor tufting enteropathy associated with endstageliver disease from total parenteral nutrition hasbeen reported (4). The impact of isolated intestinal transplantationon both developing liver disease and thecourse of the primary disease are unknown. Here wedescribe the first case in which isolated intestinal transplantationwas used to treat tufting enteropathy in a patientwith impending liver failure from total parenteralnutrition.