Document Detail

Hemolysis in a patient with alkaptonuria and chronic kidney failure.
MedLine Citation:
PMID:  20207456     Owner:  NLM     Status:  MEDLINE    
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.
Anne-Elisabeth Heng; Marie Courbebaisse; Jean Louis Kemeny; Raluca Matesan; Claude Bonniol; Patrice Deteix; Bertrand Souweine
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Publication Detail:
Type:  Case Reports; Journal Article     Date:  2010-03-06
Journal Detail:
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:
Created Date:  2010-07-12     Completed Date:  2010-07-27     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8110075     Medline TA:  Am J Kidney Dis     Country:  United States    
Other Details:
Languages:  eng     Pagination:  e1-4     Citation Subset:  IM    
Service de Néphrologie Réanimation Médicale, Pole REUNNIRH, Clermont-Ferrand, France.
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MeSH Terms
Alkaptonuria / complications*,  diagnosis*,  therapy
Fatal Outcome
Kidney Failure, Chronic / complications*,  diagnosis*,  therapy
Young Adult

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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