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Published in: European Journal of Pediatrics 7/2009

Open Access 01-07-2009 | Review

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

Authors: Marijn Schuurman, Dick van Waardenburg, Joost Da Costa, Hendrik Niemarkt, Piet Leroy

Published in: European Journal of Pediatrics | Issue 7/2009

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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.
Literature
1.
go back to reference Arese P, Mannuzzu L, Turrini F (1989) Pathophysiology of favism. Folia Haematol Int Mag Klin Morphol Blutforsch 116:745–752PubMed Arese P, Mannuzzu L, Turrini F (1989) Pathophysiology of favism. Folia Haematol Int Mag Klin Morphol Blutforsch 116:745–752PubMed
2.
go back to reference Ash-Bernal R, Wise R, Wright SM (2004) Acquired methemoglobinemia: a retrospective series of 138 cases at 2 teaching hospitals. Medicine (Baltimore) 83:265–273CrossRef Ash-Bernal R, Wise R, Wright SM (2004) Acquired methemoglobinemia: a retrospective series of 138 cases at 2 teaching hospitals. Medicine (Baltimore) 83:265–273CrossRef
3.
go back to reference Beall SN, Moorthy SS (1989) Jaundice, oximetry, and spurious hemoglobin desaturation. Anesth Analg 68:806–807PubMedCrossRef Beall SN, Moorthy SS (1989) Jaundice, oximetry, and spurious hemoglobin desaturation. Anesth Analg 68:806–807PubMedCrossRef
4.
go back to reference Benatti U, Guida L, Grasso M et al (1985) Hexose monophosphate shunt-stimulated reduction of methemoglobin by divicine. Arch Biochem Biophys 242:549–556PubMedCrossRef Benatti U, Guida L, Grasso M et al (1985) Hexose monophosphate shunt-stimulated reduction of methemoglobin by divicine. Arch Biochem Biophys 242:549–556PubMedCrossRef
5.
6.
go back to reference De Flora A, Benatti U, Guida L et al (1985) Favism: disordered erythrocyte calcium homeostasis. Blood 66:294–297PubMed De Flora A, Benatti U, Guida L et al (1985) Favism: disordered erythrocyte calcium homeostasis. Blood 66:294–297PubMed
7.
8.
go back to reference Harvey JW, Keitt AS (1983) Studies of the efficacy and potential hazards of methylene blue therapy in aniline-induced methaemoglobinaemia. Br J Haematol 54:29–41PubMedCrossRef Harvey JW, Keitt AS (1983) Studies of the efficacy and potential hazards of methylene blue therapy in aniline-induced methaemoglobinaemia. Br J Haematol 54:29–41PubMedCrossRef
9.
go back to reference Haymond S, Cariappa R, Eby CS et al (2005) Laboratory assessment of oxygenation in methemoglobinemia. Clin Chem 51:434–444PubMedCrossRef Haymond S, Cariappa R, Eby CS et al (2005) Laboratory assessment of oxygenation in methemoglobinemia. Clin Chem 51:434–444PubMedCrossRef
10.
go back to reference Janssen WJ, Dhaliwal G, Collard HR et al (2004) Clinical problem-solving. Why “why” matters. N Engl J Med 351:2429–2434PubMedCrossRef Janssen WJ, Dhaliwal G, Collard HR et al (2004) Clinical problem-solving. Why “why” matters. N Engl J Med 351:2429–2434PubMedCrossRef
11.
go back to reference Lau HK, Li CH, Lee AC (2006) Acute massive haemolysis in children with glucose-6-phosphate dehydrogenase deficiency. Hong Kong Med J 12:149–151PubMed Lau HK, Li CH, Lee AC (2006) Acute massive haemolysis in children with glucose-6-phosphate dehydrogenase deficiency. Hong Kong Med J 12:149–151PubMed
12.
go back to reference Liao YP, Hung DZ, Yang DY (2002) Hemolytic anemia after methylene blue therapy for aniline-induced methemoglobinemia. Vet Hum Toxicol 44:19–21PubMed Liao YP, Hung DZ, Yang DY (2002) Hemolytic anemia after methylene blue therapy for aniline-induced methemoglobinemia. Vet Hum Toxicol 44:19–21PubMed
13.
go back to reference Luzzatto L (2003) Glucose-6-Phosphate Dehydrogenase Deficiency and Hemolytic Anemia. In: Nathan DG, Orkin SH (eds) Nathan and Oski’s Hematology of Infancy and Childhood, 6th edn. W. B. Saunders, Philadelphia, p 721–742 Luzzatto L (2003) Glucose-6-Phosphate Dehydrogenase Deficiency and Hemolytic Anemia. In: Nathan DG, Orkin SH (eds) Nathan and Oski’s Hematology of Infancy and Childhood, 6th edn. W. B. Saunders, Philadelphia, p 721–742
14.
go back to reference Maddali MM, Fahr J (2005) Postoperative methemoglobinemia with associated G-6-P-D deficiency in infant cardiac surgery—enigmas in diagnosis and management. Paediatr Anaesth 15:334–337PubMedCrossRef Maddali MM, Fahr J (2005) Postoperative methemoglobinemia with associated G-6-P-D deficiency in infant cardiac surgery—enigmas in diagnosis and management. Paediatr Anaesth 15:334–337PubMedCrossRef
15.
go back to reference Mullick P, Kumar A, Dayal M et al (2007) Aniline-induced methaemoglobinaemia in a glucose-6-phosphate dehydrogenase enzyme deficient patient. Anaesth Intensive Care 35:286–288PubMed Mullick P, Kumar A, Dayal M et al (2007) Aniline-induced methaemoglobinaemia in a glucose-6-phosphate dehydrogenase enzyme deficient patient. Anaesth Intensive Care 35:286–288PubMed
16.
go back to reference Nik-Akhtar B, Khakpour M, Rashed MA (1972) Recovery from acute renal failure from favism by means of dialysis. Trans R Soc Trop Med Hyg 66:801–802PubMedCrossRef Nik-Akhtar B, Khakpour M, Rashed MA (1972) Recovery from acute renal failure from favism by means of dialysis. Trans R Soc Trop Med Hyg 66:801–802PubMedCrossRef
17.
go back to reference Rosen PJ, Johnson C, McGehee WG et al (1971) Failure of methylene blue treatment in toxic methemoglobinemia. Association with glucose-6-phosphate dehydrogenase deficiency. Ann Intern Med 75:83–86PubMed Rosen PJ, Johnson C, McGehee WG et al (1971) Failure of methylene blue treatment in toxic methemoglobinemia. Association with glucose-6-phosphate dehydrogenase deficiency. Ann Intern Med 75:83–86PubMed
18.
go back to reference Sarkar S, Prakash D, Marwaha RK et al (1993) Acute intravascular haemolysis in glucose-6-phosphate dehydrogenase deficiency. Ann Trop Paediatr 13:391–394PubMed Sarkar S, Prakash D, Marwaha RK et al (1993) Acute intravascular haemolysis in glucose-6-phosphate dehydrogenase deficiency. Ann Trop Paediatr 13:391–394PubMed
19.
go back to reference Verma M, Aggarwal A (1977) Glucose–6 phosphate dehydrogenase deficiency with methemoglobinemia. Indian Pediatr 14:831–836PubMed Verma M, Aggarwal A (1977) Glucose–6 phosphate dehydrogenase deficiency with methemoglobinemia. Indian Pediatr 14:831–836PubMed
20.
go back to reference Wright RO, Lewander WJ, Woolf AD (1999) Methemoglobinemia: etiology, pharmacology, and clinical management. Ann Emerg Med 34:646–656PubMedCrossRef Wright RO, Lewander WJ, Woolf AD (1999) Methemoglobinemia: etiology, pharmacology, and clinical management. Ann Emerg Med 34:646–656PubMedCrossRef
21.
Metadata
Title
Severe hemolysis and methemoglobinemia following fava beans ingestion in glucose-6-phosphatase dehydrogenase deficiency—case report and literature review
Authors
Marijn Schuurman
Dick van Waardenburg
Joost Da Costa
Hendrik Niemarkt
Piet Leroy
Publication date
01-07-2009
Publisher
Springer-Verlag
Published in
European Journal of Pediatrics / Issue 7/2009
Print ISSN: 0340-6199
Electronic ISSN: 1432-1076
DOI
https://doi.org/10.1007/s00431-009-0952-x

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