Mixed methemoglobinemia, congenital and acquired
Keywords:
Methaemoglobinaemia, methylene blue, aniline, cytochrome-b reductaseAbstract
The authors present a case of acute methaemoglobinaemia of mixed aetiology - congenital and acquired. Excessive cutaneous absorption of aniline dye was the causal factor, but an associated moderate defect in cytochrome-b reductase as well as a glucose-6-phosphate dehydrogenase (G6PD) deficiency aggravated the clinicai course of the disease. Therapy with methylene blue was effective, but it caused haemolytic jaundice, probably because of the concomitant glucose-6-phosphate deficiency or because of the haemolytic action of the toxin and/ or possible hemolytic action of methylene blue.
A review of the erythrocyte enzymatic mechanisms that convert methaemoglobin to haemoglobin, clinicai symptoms, aetiology of acute methaemoglobinaemia, referring particularly to aniline as a toxic acquired cause, and to the deficiency of cytocrome-b_, reductase as the most frequent cause of congenital aetiology, is presented. The benefit of methylene blue therapy is considered as well as associated side-effects, particularly in the presence of concomitant G6PD deficiency.
The enzymatic study of relatives is very important for the prevention of similar episodes.
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References
Wright RO, Lewander WJ, Woolf AD. Methemoglobinemia: Etiology. Pharmacology, and Clinicai Management. Ann Emerg Med 1999: 34: 645-656.
Jaffé ER, Hultquist DE. Cytochrome-b5 reductase deficiency and enzymopenic hereditary methemoglobinemia. In: Scriver CR,
Beaudet AL, Valle D et ai, Ed. The Metabolic and Molecular Basis of lnherited Disease 8"' ed. Vol III McGraw Hill New York 2001: 4555-4570.
Curry S. Methemoglobinemia. Ann Emerg Med 1982: 11: 214-221.
Kornberg A et al. In Methods in Enzymology I. Academic Press New York 1955: 323.
Beutler E. In Red Cell Metabolism - A Manual of Biochemical Methods. Grune e Stratton 1971: 70-72.
Board PG. NADH - ferricyanide reductase. a convenient approach to lhe evaluation of NADPH - methaemoglobin reduclase in human erythrocytes. Clin Chim Acta 1981: 109: 233.
Harvey J, Keitt A. Studies of the efficacy and potential hazards of methylene blue therapy in aniline-induced methemoglobinemia. Br J Hematol 1983: 54: 29-41.
Hunter D. Health of the worker in the Twenty Century. In The Diseases of Occupations 6'" ed. Hodder and Stoughton. London 1978: 152.
Funk and Wagnalls. In Standard Dictionary of the English Language. Ed. Mackwardt A et al. 1969: 765.
Maladies causés par les dérivés nitres et aminés toxiques du benzene et de ses homologues - Aniline. In Le dépistage précoce des maladies professionelles. OMS. Geneve 1989: 142-146.
Thienes C, Haley T. Clinicai Toxicology. Lea and Febiger. Philadelphia 1979: 237-239.
Posthumus MD, Van Berkel W. Cytochrome-b5 reductase deficiency, an uncommon cause of cyanosis. Neth J Med 1994: 44: 136-140.
Cohen R, Sachs J, Wicked D et ai. Methemoglobinemia provoked by malarial chemoprophylaxis in Vietnam. New Engl J Med 1968: 279: 1127-1131.
Metz E, Balcerzak S, Sagon L. Mechanism of methylene blue stimulation of the hexose monophosphate shunt in the erythrocytc. J Clin lnvest 1976: 58: 797-802.
Beutler E, Baluda M. Methemoglobin reductase. Studies in the interaction between cell populations and of the role of methylene blue. Blood 1963: 22: 323-333
Nadler J, Green H. Rosenbaun A. lntravenous injection of methylcne blue in man with reference to its toxic symptoms and effect on the clectrocardiogram. Am J Med Sei 1934: 188: 15-21.
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