Sickle Cell Anemia


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Related to Sickle Cell Anemia: sickle cell trait, thalassemia, Sickle cell crisis

Sickle Cell Anemia

 

one of the forms of hereditary hemolytic anemia. The distinguishing characteristic of sickle cell anemia is the sickle shape of erythrocytes during hypoxia. The disease was described in 1910 by the American physician J. B. Herrick.

Sickle cell anemia is caused by the pathological hemoglobin S, in which the amino acid valine is substituted for glutamic acid in one portion of the protein chain. The discovery of this fact in 1949 by L. Pauling and his co-workers laid the basis for the study of human molecular diseases. Sickle cell anemia occurs in Equatorial Africa; it has not been observed among the indigenous populations of America, Australia, and northern Europe.

During sickle cell anemia, erythrocytes have an increased resistance to invasion by malarial plasmodia. Homozygotic children suffer from severe forms of the disease, including hemolytic crises with jaundice, fever, progressive anemia, and abdominal pains, and usually die during early childhood or during adolescence. Heterozygotic children are usually only potential carriers of sickle-shaped erythrocytes and do not suffer from the disease. However, under hypoxia-producing conditions, for example, a jet flight, they may suffer from hemolytic crises, thromboses of cerebral vessels, and infarcts of internal organs.

Sickle cell anemia is treated with aspirin and anticoagulants in cases of crises and with transfusion in cases of severe anemia. The preventive treatment of crises in heterozygotic individuals is important.

A. I. VOROBEV

References in periodicals archive ?
Central sensitization associated with low fetal hemoglobin levels in adults with sickle cell anemia. Scand J Pain 2017; 17:279-286.
In sickle cell anemia, hypoxaemia and tissue hypoperfusion are the key notes.
"Sickle Cell Anemia." Color Atlas of Hematology: An Illustrated Field Guide Based on Proficiency Testing.
Sickle cell anemia trait prevalence was found 10.0% in Adana and 13.6% in Mersin-the cities in Mediterranean region-(4-7).
Sickle cell pain crises, which are responsible for the patient's back and chest pain, are known as the hallmarks of sickle cell anemia. (2) These episodes of pain, such as acute chest syndrome, can be explained by a difficult mechanism resulting in red cell adherence to the vascular endothelium and activation of coagulation factors.
Lakshmanan Krishnamurti at the Children's Hospital of Pittsburgh developed a gentler, easier technique for treating sickle cell anemia with adult stem cells.
Iron deficiency in sickle cell anemia in Nigerian children.
When the vitamin is deficient, sickle cell anemia may also show a response to vitamin supplements.
For the study, 79 children aged 2-18 years with sickle cell anemia from 25 medical centers in North America were randomized to receive either standard supportive care with periodic blood transfusions or be withdrawn from periodic blood transfusions if their transcranial Doppler velocity had returned to a low risk range (defined as less than 170 cm/sec).
(2) Figure 1 is a regional map that displays the major initial geographic distribution of people with sickle cell anemia. (5)
Symposium on Sickle Cell Anemia and Its Prevalence among Blacks in some Southern States of the USA
The association of sickle cell anemia with the black "race" came through mandated screening programs reflecting social bias and prejudice.