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March 1973, Vol 125, No. 3 >
Article | March 1973

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Sydney S. Gellis, MD; Murray Feingold, MD; Sidney J. Sussman, MD; J. L. Naiman, MD
[+] Author Affiliations

Received for publication Sept 18, 1972; accepted Sept 26.

Reprint requests to Reprint Department, AJDC, American Medical Association, 535 N Dearborn St, Chicago 60610 (Joann Kitchen).


Am J Dis Child. 1973;125(3):407-408. doi:10.1001/archpedi.1973.04160030071014.
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Manifestations  Sickle cell anemia, an hereditary hemoglobinopathy, results from an amino acid substitution in the beta chain of the globin portion of the hemoglobin molecule. Under decreased oxygen tension such molecules undergo structural rearrangement with the formation of needle-like "tactoids" which distort the red cell from a biconcave disk to a "sickle" shape. The consequences include a hemolytic anemia and a tendency for the sickle cells to sludge in small blood vessels with resultant tissue hypoxia and necrosis.Manifestations usually do not appear until after four to six months of life. The chronic hemolytic anemia may give rise to listlessness, irritability, jaundice, and splenomegaly. At unpredictable intervals, there are various vasocclusive crises. These most commonly involve the bones and soft tissues resulting in attacks of pain and swelling around joints. In the young infant there is a predilection for involvement of the hands and feet (the "hand-foot syndrome").

REFERENCES

1 +
Pearson HA:  The critically ill child: Sickle cell disease crises and their management . Pediatrics 48:629-635, 1971;.
2 +
Barrett-Connor E:  Bacterial infection and sickle cell anemia . Medicine 50:97-112, 1971;.
3 +
Snyder GB, Wilson CA:  Surgical management of priapism and its sequelae in sickle cell disease . Southern Med J 59:1393-1396, 1966;.
4 +
Watson RJ, et al:  The hand-foot syndrome in sickle cell disease in young children . Pediatrics 31:975-982, 1963;.

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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 +
Pearson HA:  The critically ill child: Sickle cell disease crises and their management . Pediatrics 48:629-635, 1971;.
2 +
Barrett-Connor E:  Bacterial infection and sickle cell anemia . Medicine 50:97-112, 1971;.
3 +
Snyder GB, Wilson CA:  Surgical management of priapism and its sequelae in sickle cell disease . Southern Med J 59:1393-1396, 1966;.
4 +
Watson RJ, et al:  The hand-foot syndrome in sickle cell disease in young children . Pediatrics 31:975-982, 1963;.

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