Persistence of the intestinal defect in abetalipoproteinaemia after liver transplantation

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


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*


  • Carrier Proteins
  • microsomal triglyceride transfer protein