title: Severe hemolysis and methemoglobinemia following fava beans ingestion in glucose-6-phosphatase dehydrogenase deficiency: case report and literature review.
abstract: BACKGROUND
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Pediatrics, Atrium Medical Centre Heerlen, Heerlen, The Netherlands.
authors.forename: Marijn
authors.initials: M
authors.lastname: Schuurman
authors.forename: Dick
authors.initials: D
authors.lastname: van Waardenburg
authors.forename: Joost
authors.initials: J
authors.lastname: Da Costa
authors.forename: Hendrik
authors.initials: H
authors.lastname: Niemarkt
authors.forename: Piet
authors.initials: P
authors.lastname: Leroy
mesh_terms: D058186:Acute Kidney Injury / Q000209:etiology / Q000503:physiopathology; D003490:Cyanosis / Q000209:etiology / Q000503:physiopathology; D005236:Favism / Q000201:enzymology / Q000235:genetics; D005953:Glycogen Storage Disease Type I / Q000175:diagnosis* / Q000503:physiopathology* / Q000628:therapy; D006461:Hemolysis; D006801:Humans; D007223:Infant; D008297:Male; D008708:Methemoglobinemia / Q000150:complications* / Q000209:etiology* / Q000503:physiopathology; D012307:Risk Factors; D031307:Vicia faba / Q000009:adverse effects*