Persistence of the intestinal defect in abetalipoproteinaemia after liver transplantation.
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.
Division of Gastroenterology and Nutrition, University Children's Hospital, Zürich, Switzerland.
C P
CP
Braegger
D C
DC
Belli
G
G
Mentha
B
B
Steinmann
D000012:Abetalipoproteinemia / Q000150:complications* / Q000378:metabolism / Q000601:surgery; D000293:Adolescent; D002352:Carrier Proteins / Q000378:metabolism; D005260:Female; D006801:Humans; D007413:Intestinal Mucosa / Q000378:metabolism; D008103:Liver Cirrhosis / Q000209:etiology* / Q000601:surgery; D016031:Liver Transplantation
D002352:Carrier Proteins; C106092:microsomal triglyceride transfer protein