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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

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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.

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Abbreviations

AHA:

Acute hemolytic anemia

CHA:

Chronic hemolytic anemia

COHb:

Carboxyhemoglobin

G6PD:

Glucose-6-phosphate dehydrogenase

Hb:

Hemoglobin

MetHb:

Methemoglobin

PK:

Pyruvate kinase

WHO:

World Health Organization

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Conflict of interest

None of the authors have any conflict of interest to be declared.

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Authors and Affiliations

  1. Service de Pédiatrie, AP-HP, Hôpital Louis-Mourier, 178 rue des Renouillers, Colombes, 92700, France

    Marie-Hélène Odièvre, Névéna Danékova, Bettina Mesples & Nathalie Parez

  2. Service des Urgences Pédiatriques, AP-HP, Hôpital Louis-Mourier, 178 rue des Renouillers, Colombes, 92700, France

    Myriam Chemouny & Nathalie Parez

  3. INSERM, U763, Hôpital Robert-Debré, Paris, 75019, France

    Marie-Hélène Odièvre & Jacques Elion

  4. Laboratoire de Génétique Moléculaire et Biochimie, AP-HP, Hôpital Robert-Debré, 48 boulevard Sérurier, Paris, 75019, France

    Nathalie Couque, Rolande Ducrocq & Jacques Elion

  5. Faculté de Médecine, Université Paris Diderot, Paris, 75019, France

    Marie-Hélène Odièvre, Rolande Ducrocq & Jacques Elion

Authors
  1. Marie-Hélène Odièvre
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  2. Névéna Danékova
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  3. Bettina Mesples
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  4. Myriam Chemouny
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  5. Nathalie Couque
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  6. Nathalie Parez
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  7. Rolande Ducrocq
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  8. Jacques Elion
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Corresponding author

Correspondence to Marie-Hélène Odièvre.

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Odièvre, MH., Danékova, N., Mesples, B. et al. Unsuspected glucose-6-phosphate dehydrogenase deficiency presenting as symptomatic methemoglobinemia with severe hemolysis after fava bean ingestion in a 6-year-old boy. Int J Hematol 93, 664–666 (2011). https://doi.org/10.1007/s12185-011-0827-6

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  • DOI: https://doi.org/10.1007/s12185-011-0827-6

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