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Unsuspected glucose-6-phosphate dehydrogenase deficiency presenting as symptomatic methemoglobinemia with severe hemolysis after fava bean ingestion in a 6-year-old boy.
MedLine Citation:
PMID:  21479984     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
We report the occurrence of symptomatic methemoglobinemia in a previously healthy boy, who presented with severe acute hemolysis after fava bean ingestion. The methemoglobinemia revealed a previously unrecognized glucose-6-phosphate dehydrogenase (G6PD) deficiency. We discuss the pathophysiology of severe methemoglobinemia when associated with acute hemolysis, favism, and the common African G6PD A-variant [G6PD, VAL68MET, ASN126ASP]. In conclusion, screening for G6PD deficiency must be considered in symptomatic methemoglobinemia, especially in young boys, when associated with intravascular hemolysis.
Authors:
Marie-Hélène Odièvre; Névéna Danékova; Bettina Mesples; Myriam Chemouny; Nathalie Couque; Nathalie Parez; Rolande Ducrocq; Jacques Elion
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2011-4-12
Journal Detail:
Title:  International journal of hematology     Volume:  -     ISSN:  1865-3774     ISO Abbreviation:  -     Publication Date:  2011 Apr 
Date Detail:
Created Date:  2011-4-11     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9111627     Medline TA:  Int J Hematol     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Affiliation:
Service de Pédiatrie, AP-HP, Hôpital Louis-Mourier, 178 rue des Renouillers, Colombes, 92700, France, marie-helene.odievre@lmr.aphp.fr.
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