Unsuspected glucose-6-phosphate dehydrogenase deficiency presenting as symptomatic methemoglobinemia with severe hemolysis after fava bean ingestion in a 6-year-old boy.

Abstract

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
• Journal: International journal of hematology (Int J Hematol) Vol. 93 Issue 5 Pg. 664-666 (May 2011) ISSN: 1865-3774 [Electronic] Japan
• PMID: 21479984 (Publication Type: Case Reports, Journal Article)

Chemical References

• Hemolytic Agents
• Glucosephosphate Dehydrogenase

Topics

• Algeria
• Child
• Eating
• Favism (complications, diagnosis, physiopathology)
• Glucosephosphate Dehydrogenase (blood, genetics)
• Glucosephosphate Dehydrogenase Deficiency (complications, diagnosis, physiopathology)
• Hemolysis (drug effects)
• Hemolytic Agents (administration & dosage, adverse effects)
• Humans
• Male
• Methemoglobinemia (complications, diagnosis, physiopathology)
• Mutation
• Vicia faba (adverse effects)