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Severe hemolysis and methemoglobinemia following fava beans ingestion in glucose-6-phosphatase dehydrogenase deficiency: case report and literature review.

Abstract：

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.

journal_name

Eur J Pediatr

journal_title

European journal of pediatrics

authors

Schuurman M,van Waardenburg D,Da Costa J,Niemarkt H,Leroy P

doi

10.1007/s00431-009-0952-x

subject

Has Abstract

pub_date

2009-07-01 00:00:00

pages

779-82

issue

7

eissn

0340-6199

issn

1432-1076

journal_volume

168

pub_type

杂志文章,评审

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