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July 1958, Vol 96, No. 1 >
Article | July 1958

Sickle-Cell Anemia in Infancy FREE

SANFORD L. LEIKIN, M.D.; JAMES W. McCOO, B.S.
[+] Author Affiliations

Submitted for publication Jan. 21, 1958.

This work was supported in part by a grant from the Mead Johnson Company.


AMA Am J Dis Child. 1958;96(1):51-57. doi:10.1001/archpedi.1958.02060060053009.
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References
Comments

Sickling can be produced in the blood of young Negro babies with sickle-cell trait. The percentage of cells showing this change is low but will increase as the infant matures. The homozygous state is present from birth; however, recognition of the symptomatic condition is rare in infants under the age of 4 months. Investigation of the literature reveals only four case reports in infants less than 3 months of age. These cases predate the period when electrophoretic and biochemical procedures could be applied. This article concerns case reports of two infants with sickle-cell anemia who were studied with these methods.

Methods  The erythrocyte and leukocyte counts were made by routine methods. Hemoglobin was determined as cyanmethemoglobin with a Klett-Summerson photoelectric colorimeter.1 The hematocrit values were determined by the Wintrobe method. Tests for sickling were performed by the sealed cover-slip method, with use of sodium metabisulfite as a reducing agent.

REFERENCES

1 +
Crosby, W. H.; Munn, J. I., and Furth, F. W.:  Standardizing a Method for Clinical Hemoglobinometry , U. S. Armed Forces M. J. 5:693, 1954;.
2 +
Smith, E. W., and Conley, C. L.:  Filter Paper Electrophoresis of Human Hemoglobins with Special Reference to Incidence and Clinical Significance of Hemoglobin C , Bull. Johns Hopkins Hosp. 93:94, 1953;.
3 +
Tiselius, A., and Kabat, E.:  Electrophoretic Study of Immune Sera and Purified Antibody Preparation , J. Exper. Med. 69:119, 1934;.
4 +
Singer, K.; Singer, L., and Chernoff, A. I.:  Studies on Abnormal Hemoglobins: I. Their Demonstration in Sickle Cell Anemia and Other Hematologic Disorders by Means of Alkali Denaturation , Blood 6:413, 1951;.
5 +
Weinstein, T. F.; Spurling, C. L.; Klein, H., and Necheles, T. F.:  Radioactive Sodium Chromate for the Study of Survival of Red Blood Cells: Part III , Blood 9:1155, 1954;.
6 +
Ashby, W.:  Determination of Length of Life of Transfused Blood Corpuscles in Man , J. Exper. Med. 29:267, 1919;.
7 +
Cohen, S. M.; Miller, B. M., and Orris, H. W.:  Fatal Sickle Cell Anemia in a One Month Old Infant , J. Pediat. 30:468, 1947;.
8 +
Frazier, C. A., and Rice, C. E.:  Neonatal Sickle Cell Anemia , J.A.M.A. 143:1065, 1950;.
9 +
Johnson, F. B., and Townsend, E. W.:  Sickle-Cell Anemia: Report of 30 Cases , South. M. & Surg. 99:377, 1937;.
10 +
Wollstein, M., and Kreidel, K. V.:  Sickle Cell Anemia , Am. J. Dis. Child. 36:998, 1928;.
11 +
Watson, R. J.:  Seminar on Premature and New-Born Infants , Pediatrics 20:147, 1957;.
12 +
Watson, R. J.:  The Significance of the Paucity of Sickle Cells in New-Born Negro Infants , Am. J. M. Sc. 215:419, 1948;.
13 +
Switzer, P. K.:  The Incidence of the Sickle Cell Trait in Negroes in the Sea Island Area of South Carolina , South. M. J. 43:48, 1950;.
14 +
Schneider, R. G., and Haggard, M. E.:  Sickling, a Quantitatively Delayed Genetic Character , Proc. Soc. Exper. Biol. & Med. 89:196, 1955;.
15 +
Diggs, L. W.; Ahmann, C. F., and Bibb, J.:  The Incidence and Significance of the Sickle Cell Trait , Ann. Int. Med. 7:269, 1933;.
16 +
Scott, R. B.; Crawford, R. P., and Jenkins, M.:  Incidence of Sicklemia in the Newborn Negro Infant , Am. J. Dis. Child. 75:842, 1948;.
17 +
Harris, J. W.:  Studies on the Destruction of Red Blood Cells: VIII. Molecular Orientation in Sickle Cell Hemoglobin Solutions , Proc. Soc. Exper. Biol. & Med. 75:197, 1950;.
18 +
Singer, K., and Singer, L.:  Studies on Abnormal Hemoglobins: VIII. The Gelling Phenomenon of Sickle Cell Hemoglobin; Its Biologic and Diagnostic Significance , Blood 8:1008, 1953;.
19 +
Zuelzer, W. W.; Neel, J. V., and Robinson, A. R.:  Abnormal Hemoglobins , in Progress in Hematology , edited by L. M. Tocantins, New York, Grune & Stratton, Inc., 1956;, Vol. 1, p. 108.
20 +
Giblett, E. R.; Motulsky, A. G.; Casserd, F.; Houghton, B., and Finch, C. A.:  Studies on the Pathogenesis of Splenic Anemia , Blood 11:1118, 1956;.

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Country-Specific Mortality and Growth Failure in Infancy and Yound Children and Association With Material Stature

Use interactive graphics and maps to view and sort country-specific infant and early dhildhood mortality and growth failure data and their association with maternal

References

1 +
Crosby, W. H.; Munn, J. I., and Furth, F. W.:  Standardizing a Method for Clinical Hemoglobinometry , U. S. Armed Forces M. J. 5:693, 1954;.
2 +
Smith, E. W., and Conley, C. L.:  Filter Paper Electrophoresis of Human Hemoglobins with Special Reference to Incidence and Clinical Significance of Hemoglobin C , Bull. Johns Hopkins Hosp. 93:94, 1953;.
3 +
Tiselius, A., and Kabat, E.:  Electrophoretic Study of Immune Sera and Purified Antibody Preparation , J. Exper. Med. 69:119, 1934;.
4 +
Singer, K.; Singer, L., and Chernoff, A. I.:  Studies on Abnormal Hemoglobins: I. Their Demonstration in Sickle Cell Anemia and Other Hematologic Disorders by Means of Alkali Denaturation , Blood 6:413, 1951;.
5 +
Weinstein, T. F.; Spurling, C. L.; Klein, H., and Necheles, T. F.:  Radioactive Sodium Chromate for the Study of Survival of Red Blood Cells: Part III , Blood 9:1155, 1954;.
6 +
Ashby, W.:  Determination of Length of Life of Transfused Blood Corpuscles in Man , J. Exper. Med. 29:267, 1919;.
7 +
Cohen, S. M.; Miller, B. M., and Orris, H. W.:  Fatal Sickle Cell Anemia in a One Month Old Infant , J. Pediat. 30:468, 1947;.
8 +
Frazier, C. A., and Rice, C. E.:  Neonatal Sickle Cell Anemia , J.A.M.A. 143:1065, 1950;.
9 +
Johnson, F. B., and Townsend, E. W.:  Sickle-Cell Anemia: Report of 30 Cases , South. M. & Surg. 99:377, 1937;.
10 +
Wollstein, M., and Kreidel, K. V.:  Sickle Cell Anemia , Am. J. Dis. Child. 36:998, 1928;.
11 +
Watson, R. J.:  Seminar on Premature and New-Born Infants , Pediatrics 20:147, 1957;.
12 +
Watson, R. J.:  The Significance of the Paucity of Sickle Cells in New-Born Negro Infants , Am. J. M. Sc. 215:419, 1948;.
13 +
Switzer, P. K.:  The Incidence of the Sickle Cell Trait in Negroes in the Sea Island Area of South Carolina , South. M. J. 43:48, 1950;.
14 +
Schneider, R. G., and Haggard, M. E.:  Sickling, a Quantitatively Delayed Genetic Character , Proc. Soc. Exper. Biol. & Med. 89:196, 1955;.
15 +
Diggs, L. W.; Ahmann, C. F., and Bibb, J.:  The Incidence and Significance of the Sickle Cell Trait , Ann. Int. Med. 7:269, 1933;.
16 +
Scott, R. B.; Crawford, R. P., and Jenkins, M.:  Incidence of Sicklemia in the Newborn Negro Infant , Am. J. Dis. Child. 75:842, 1948;.
17 +
Harris, J. W.:  Studies on the Destruction of Red Blood Cells: VIII. Molecular Orientation in Sickle Cell Hemoglobin Solutions , Proc. Soc. Exper. Biol. & Med. 75:197, 1950;.
18 +
Singer, K., and Singer, L.:  Studies on Abnormal Hemoglobins: VIII. The Gelling Phenomenon of Sickle Cell Hemoglobin; Its Biologic and Diagnostic Significance , Blood 8:1008, 1953;.
19 +
Zuelzer, W. W.; Neel, J. V., and Robinson, A. R.:  Abnormal Hemoglobins , in Progress in Hematology , edited by L. M. Tocantins, New York, Grune & Stratton, Inc., 1956;, Vol. 1, p. 108.
20 +
Giblett, E. R.; Motulsky, A. G.; Casserd, F.; Houghton, B., and Finch, C. A.:  Studies on the Pathogenesis of Splenic Anemia , Blood 11:1118, 1956;.

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