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Persistence of the intestinal defect in abetalipoproteinaemia after liver transplantation

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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.

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Authors and Affiliations

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

    C. P. Braegger

  2. Paediatric gastroenterology unit, HUG, Geneva, Switzerland , , , , , , CH

    D. C. Belli

  3. Transplantation Unit, HUG, Geneva, Switzerland, , , , , , CH

    G. Mentha

  4. Division of Metabolic and Molecular Diseases, University Children's Hospital, Steinwiesstrasse 75, CH-8032 Zürich, Switzerland, E-mail: binges@kispi.unizh.ch, Fax: -41-1-2667167, , , , , , CH

    B. Steinmann

Authors
  1. C. P. Braegger
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  2. D. C. Belli
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  3. G. Mentha
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  4. B. Steinmann
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Received: 1 September 1997 / Accepted in revised form: 8 December 1997

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Braegger, C., Belli, D., Mentha, G. et al. Persistence of the intestinal defect in abetalipoproteinaemia after liver transplantation. Eur J Pediatr 157, 576–578 (1998). https://doi.org/10.1007/s004310050882

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  • DOI: https://doi.org/10.1007/s004310050882

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