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Using heparin therapy to reverse protein-losing enteropathy in a patient with CDG-Ib

Nature Clinical Practice Gastroenterology & Hepatology volume 5, pages 220–224 (2008)Cite this article

Abstract

Background A 22-year-old female presented with edema, diarrhea, hypoalbuminemia and pancytopenia. She had previously been diagnosed with congenital disorder of glycosylation type Ib, and had a history of congenital hepatic fibrosis, portal hypertension and esophageal varices. In the past she had refused mannose therapy because of associated diarrhea and abdominal pain.

Investigations Laboratory examinations, abdominal ultrasonography, bacterial and viral cultures of blood, urine and stools, double-balloon enteroscopy and fecal excretion test using 51Cr-labeled albumin.

Diagnosis Protein-losing enteropathy.

Management Infusion of albumin followed by intravenous and subcutaneous therapy with unfractionated heparin.

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Figure 1: Graph showing the change in the patient's fecal α-1 antitrypsin concentration in response to heparin therapy.

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Acknowledgements

The authors are grateful to Dr Tom Kennedy of the University of Utah for his advice on heparin therapy. HHF and LB are supported by NIH Grants R01DK55615, R21HL078997 and The Children's Hearts Fund. Désirée Lie, University of California, Irvine, CA, is the author of and is solely responsible for the content of the learning objectives, questions and answers of the Medscape-accredited continuing medical education activity associated with this article.

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Authors and Affiliations

  1. YS Liem is a Resident, FWG Leebeek is a Consultant Hematologist, AAM Zandbergen is an Internist and JHP Wilson is a Professor of Internal Medicine at the Erasmus University Medical Center, Rotterdam, The Netherlands.,

    Ylian S Liem, Frank WG Leebeek, Adrienne AM Zandbergen & JH Paul Wilson

  2. L Bode is a Staff Scientist and HH Freeze is a Professor and Co-Director of the Tumor Microenvironment Program and the Glycobiology and Carbohydrate Chemistry Group I at the Burnham Institute for Medical Research, La Jolla, CA, USA.,

    Lars Bode & Hudson H Freeze

Authors
  1. Ylian S Liem
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  2. Lars Bode
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Correspondence to JH Paul Wilson.

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The authors declare no competing financial interests.

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Liem, Y., Bode, L., Freeze, H. et al. Using heparin therapy to reverse protein-losing enteropathy in a patient with CDG-Ib. Nat Rev Gastroenterol Hepatol 5, 220–224 (2008). https://doi.org/10.1038/ncpgasthep1061

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