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Congenital Methemoglobinemia in Pregnancy

George Pepper, M.D.; Hyman G. Weinstein, M.S.; Paul Heller, M.D.
[+] Author Affiliations

Chicago

From the departments of obstetrics and gynecology, and medicine, University of Illinois College of Medicine, and the Research Laboratory, Veterans Administration West Side Hospital.


JAMA. 1961;177(5):328-330. doi:10.1001/jama.1961.73040310021012
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CONGENITAL methemoglobinemia1-4 is a rare, genetically determined abnormality which is of clinical importance mainly because of the marked cyanotic appearance of the carriers of this defect, thus giving rise to the suspicion of the presence of a serious disease. The cyanosis, however, is only rarely associated with other clinical manifestations. This discrepancy is the most important clinical clue to the diagnosis. If, however, this abnormality coexists with another unrelated clinical condition in the course of which cyanosis may occur, it may be mistaken for a sign of a grave complication. An example of such a clinical situation is described in the following report which shows that a person with congenital methemoglobinemia tolerates pregnancy well.

Report of a Case  A 27-year-old woman, Gravida 4, Para 3, born in Puerto Rico, was first seen at the University of Illinois prenatal clinic during the eighth month of pregnancy, complaining of slight shortness

REFERENCES

Gibson, Q. H.:  Reduction of Methaemoglobin in Red Blood Cells and Studies of Case of Idiopathic Methaemoglobinaemia . Biochem J 42:13-23, 1948;.
Gibson, Q. H., and Harrison, D.:  Familial Idiopathic Methaemoglobinaemia , Lancet 2:941-943 ( (Dec. 27) ) 1947;.
Scott, E. M., and Griffith, I. V.:  Enzymic Defect of Hereditary Methemoglobinemia: Diaphorase , Biochem Biophys Acta 34:584-586 ( (Aug.) ) 1959;.
Gerald, P. S.:  Hereditary Methemoglobinemias  in Metabolic Basis of Inherited Disease , edited by J. B. Stanbury, J. B. Wyngaarden, and D. S. Fredrickson, New York: McGraw-Hill Book Company, 1960;, pp. 1068-1085.
Evelyn, K. A., and Malloy, H. T.:  Microdetermination of Oxyhemoglobin, Methemoglobin, and Sulfhemoglobin in Single Sample of Blood , J Biol Chem 126:655-662 ( (Dec.) ) 1938;.
Huennekens, F. M., et al.:  Erythrocyte Metabolism. IV. Isolation and Properties of Methemoglobin Reductase , J Biol Chem 227:261-272 ( (July) ) 1957;.
Gerald, P. S.:  Electrophoretic and Spectroscopic Characterization of Hgb M , Blood 13:936-949 ( (Oct.) ) 1958;.
Scott, E. M., and Hoskins, D. D.:  Hereditary Methemoglobinemia in Alaskan Eskimos and Indians , Blood 13:795-802 ( (Aug.) ) 1958;.
Scott, E. M.:  Relation of Diaphorase of Human Erythrocytes to Inheritance of Methemoglobinemia , J Clin Invest 39:1176-1179 ( (July) ) 1960;.
Hörlein, H., and Weber, G.:  Über Chronische familiäre Methämoglobinämie und Eine Neue Modifikation des Methämoglobins , Deutsch Med Wschr 73:476-478 ( (Oct. 15) ) 1948;.
Gerald, P. S., and George, P.:  Second Spectroscopically Abnormal Methemoglobin Associated with Hereditary Cyanosis , Science 129:393-394 ( (Feb. 13) ) 1959;.

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Gibson, Q. H.:  Reduction of Methaemoglobin in Red Blood Cells and Studies of Case of Idiopathic Methaemoglobinaemia . Biochem J 42:13-23, 1948;.
Gibson, Q. H., and Harrison, D.:  Familial Idiopathic Methaemoglobinaemia , Lancet 2:941-943 ( (Dec. 27) ) 1947;.
Scott, E. M., and Griffith, I. V.:  Enzymic Defect of Hereditary Methemoglobinemia: Diaphorase , Biochem Biophys Acta 34:584-586 ( (Aug.) ) 1959;.
Gerald, P. S.:  Hereditary Methemoglobinemias  in Metabolic Basis of Inherited Disease , edited by J. B. Stanbury, J. B. Wyngaarden, and D. S. Fredrickson, New York: McGraw-Hill Book Company, 1960;, pp. 1068-1085.
Evelyn, K. A., and Malloy, H. T.:  Microdetermination of Oxyhemoglobin, Methemoglobin, and Sulfhemoglobin in Single Sample of Blood , J Biol Chem 126:655-662 ( (Dec.) ) 1938;.
Huennekens, F. M., et al.:  Erythrocyte Metabolism. IV. Isolation and Properties of Methemoglobin Reductase , J Biol Chem 227:261-272 ( (July) ) 1957;.
Gerald, P. S.:  Electrophoretic and Spectroscopic Characterization of Hgb M , Blood 13:936-949 ( (Oct.) ) 1958;.
Scott, E. M., and Hoskins, D. D.:  Hereditary Methemoglobinemia in Alaskan Eskimos and Indians , Blood 13:795-802 ( (Aug.) ) 1958;.
Scott, E. M.:  Relation of Diaphorase of Human Erythrocytes to Inheritance of Methemoglobinemia , J Clin Invest 39:1176-1179 ( (July) ) 1960;.
Hörlein, H., and Weber, G.:  Über Chronische familiäre Methämoglobinämie und Eine Neue Modifikation des Methämoglobins , Deutsch Med Wschr 73:476-478 ( (Oct. 15) ) 1948;.
Gerald, P. S., and George, P.:  Second Spectroscopically Abnormal Methemoglobin Associated with Hereditary Cyanosis , Science 129:393-394 ( (Feb. 13) ) 1959;.
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