Persistence of the intestinal defect in abetalipoproteinaemia after liver transplantation

C P Braegger; D C Belli; G Mentha; B Steinmann

doi:10.1007/s004310050882

Persistence of the intestinal defect in abetalipoproteinaemia after liver transplantation

Eur J Pediatr. 1998 Jul;157(7):576-8. doi: 10.1007/s004310050882.

Authors

C P Braegger¹, D C Belli, G Mentha, B Steinmann

Affiliation

¹ Division of Gastroenterology and Nutrition, University Children's Hospital, Zürich, Switzerland.

PMID: 9686820
DOI: 10.1007/s004310050882

Abstract

A 16-year-old girl is described with abetalipoproteinaemia who underwent liver transplantation for hepatic cirrhosis. After this procedure her serum lipoprotein profile was corrected; however, fat malabsorption and steatorrhea persisted because the primary defect, a mutant microsomal triglyceride-transfer protein, remains expressed in the intestine.

Publication types

Case Reports

MeSH terms

Abetalipoproteinemia / complications*
Abetalipoproteinemia / metabolism
Abetalipoproteinemia / surgery
Adolescent
Carrier Proteins / metabolism
Female
Humans
Intestinal Mucosa / metabolism
Liver Cirrhosis / etiology*
Liver Cirrhosis / surgery
Liver Transplantation*

Substances

Carrier Proteins
microsomal triglyceride transfer protein