Document Detail

Severe hemolysis and methemoglobinemia following fava beans ingestion in glucose-6-phosphatase dehydrogenase deficiency: case report and literature review.
MedLine Citation:
PMID:  19263080     Owner:  NLM     Status:  MEDLINE    
INTRODUCTION: Reduced concentrations of glucose-6-phospate dehydrogenase (G6PD) render erythrocytes susceptible to hemolysis under conditions of oxidative stress. In favism, the ingestion of fava beans induces an oxidative stress to erythrocytes, leading to acute hemolysis.
DISCUSSION: The simultaneous occurrence of methemoglobinemia has been reported only scarcely, despite the fact that both phenomena are the consequence of a common pathophysiologic mechanism. The presence of methemoglobinemia has important diagnostic and therapeutic consequences. We report a previously healthy boy who presented with combined severe hemolytic anemia and cyanosis due to methemoglobinemia, following the ingestion of fava beans. His condition was complicated by the development of transient acute renal failure. A G6PD-deficiency was diagnosed. We review the literature on the combination of acute hemolysis and methemoglobinemia in favism. Pathophysiologic, diagnostic, and therapeutic aspects of this disorder are discussed.
Marijn Schuurman; Dick van Waardenburg; Joost Da Costa; Hendrik Niemarkt; Piet Leroy
Publication Detail:
Type:  Case Reports; Journal Article; Review     Date:  2009-03-05
Journal Detail:
Title:  European journal of pediatrics     Volume:  168     ISSN:  1432-1076     ISO Abbreviation:  Eur. J. Pediatr.     Publication Date:  2009 Jul 
Date Detail:
Created Date:  2009-05-14     Completed Date:  2009-08-19     Revised Date:  2013-05-20    
Medline Journal Info:
Nlm Unique ID:  7603873     Medline TA:  Eur J Pediatr     Country:  Germany    
Other Details:
Languages:  eng     Pagination:  779-82     Citation Subset:  IM    
Department of Pediatrics, Atrium Medical Centre Heerlen, Heerlen, The Netherlands.
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MeSH Terms
Acute Kidney Injury / etiology,  physiopathology
Cyanosis / etiology,  physiopathology
Favism / enzymology,  genetics
Glycogen Storage Disease Type I / diagnosis*,  physiopathology*,  therapy
Methemoglobinemia / complications*,  etiology*,  physiopathology
Risk Factors
Vicia faba / adverse effects*

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