Blood exchange and transfusion therapy for acute cholestasis in protoporphyria

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...

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Bibliographic Details
Published in:Digestive diseases and sciences 1988-12, Vol.33 (12), p.1621-1625
Main Authors: VAN WIJK, H. J, VAN HATTUM, J, BAART DE LA FAILLE, H, VAN DEN BERG, J. W. O, EDIXHOVEN-BOSDIJK, A, WILSON, J. H. P
Format: Article
Language:English
Subjects:
Acute Disease
Adult
Biological and medical sciences
Blood Transfusion
Cholestasis - etiology
Cholestasis - therapy
Exchange Transfusion, Whole Blood
Female
Humans
Liver Diseases - complications
Male
Medical sciences
Metabolic diseases
Other metabolic disorders
Pigments (porphyrias, hyperbilirubinemias...)
Porphyrias - complications
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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.
ISSN:0163-2116
1573-2568
DOI:10.1007/BF01535955