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Blood exchange and transfusion therapy for acute cholestasis in protoporphyria

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Summary

Acute cholestasis is a rare complication of EPP with a high mortality rate despite extensive treatment with corticosteroids, cholestyramine, and antioxidants. A single survivor, reported in the literature, was treated with blood exchange transfusions. We treated two EPP patients with blood exchange and additional blood transfusions which resulted in full clinical and biochemical recovery from the cholestasis and accompanying hepatitis. Recurrences of the cholestasis and hepatitis could repeatedly be corrected by additional blood transfusions.

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Authors and Affiliations

  1. Departments of Gastroenterology and Dermatology, University Hospital Utrecht, The Netherlands

    Hans J. Van Wijk MD,  Jan Van Hattum MD, Harold Baart De La Faille MD, J. Willem O. Van Den Berg PhD, Annie Edixhoven-Bosdijk & J. H. Paul Wilson MD

  2. Department of Internal Medicine II, University Hospital Rotterdam Dijkzigt, The Netherlands

    Hans J. Van Wijk MD,  Jan Van Hattum MD, Harold Baart De La Faille MD, J. Willem O. Van Den Berg PhD, Annie Edixhoven-Bosdijk & J. H. Paul Wilson MD

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  1. Hans J. Van Wijk MD
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  2. Jan Van Hattum MD
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  3. Harold Baart De La Faille MD
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  4. J. Willem O. Van Den Berg PhD
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  5. Annie Edixhoven-Bosdijk
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  6. J. H. Paul Wilson MD
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Van Wijk, H.J., Van Hattum, J., De La Faille, H.B. et al. Blood exchange and transfusion therapy for acute cholestasis in protoporphyria. Digest Dis Sci 33, 1621–1625 (1988). https://doi.org/10.1007/BF01535955

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  • DOI: https://doi.org/10.1007/BF01535955

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