| Hemolysis in a patient with alkaptonuria and chronic kidney failure. | |
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MedLine Citation:
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PMID: 20207456 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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In alkaptonuria, the absence of homogentisic acid oxidase results in the accumulation of homogentisic acid (HGA) in the body. Fatal disease cases are infrequent, and death often results from kidney or cardiac complications. We report a 24-year-old alkaptonuric man with severe decreased kidney function who developed fatal metabolic acidosis and intravascular hemolysis. Hemolysis may have been caused by rapid and extensive accumulation of HGA and subsequent accumulation of plasma soluble melanins. Toxic effects of plasma soluble melanins, their intermediates, and reactive oxygen side products are increased when antioxidant mechanisms are overwhelmed. A decrease in serum antioxidative activity has been reported in patients with chronic decreased kidney function. However, despite administration of large doses of an antioxidant agent and ascorbic acid and intensive kidney support, hemolysis and acidosis could not be brought under control and hemolysis led to the death of the patient. |
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Authors:
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Anne-Elisabeth Heng; Marie Courbebaisse; Jean Louis Kemeny; Raluca Matesan; Claude Bonniol; Patrice Deteix; Bertrand Souweine |
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Publication Detail:
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Type: Case Reports; Journal Article Date: 2010-03-06 |
Journal Detail:
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Title: American journal of kidney diseases : the official journal of the National Kidney Foundation Volume: 56 ISSN: 1523-6838 ISO Abbreviation: Am. J. Kidney Dis. Publication Date: 2010 Jul |
Date Detail:
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Created Date: 2010-07-12 Completed Date: 2010-07-27 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 8110075 Medline TA: Am J Kidney Dis Country: United States |
Other Details:
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Languages: eng Pagination: e1-4 Citation Subset: IM |
Affiliation:
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Service de Néphrologie Réanimation Médicale, Pole REUNNIRH, Clermont-Ferrand, France. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Alkaptonuria
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complications*,
diagnosis*,
therapy Fatal Outcome Hemolysis* Humans Kidney Failure, Chronic / complications*, diagnosis*, therapy Male Young Adult |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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