Natural history of sickle cell anemia in Saudi Arabs. A study of 270 subjects

We studied 270 Saudi Arabs with homozygous sickle cell anemia, using chart review, a register (since 1969), and home visiting in 42 cases. Average follow-up for the total group was 10 years. Seventy-four percent of those diagnosed by age 3 years presented on screening or with merely anemia; 26% pres...

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Bibliographic Details
Published inAnnals of internal medicine Vol. 88; no. 1; p. 1
Main Authors Perrine, R P, Pembrey, M E, John, P, Perrine, S, Shoup, F
Format Journal Article
LanguageEnglish
Published United States 01.01.1978
Subjects
Adolescent
Adult
Aged
Anemia, Sickle Cell - blood
Anemia, Sickle Cell - diagnosis
Anemia, Sickle Cell - epidemiology
Anemia, Sickle Cell - mortality
Black or African American
Black People
Child
Child, Preschool
Female
Follow-Up Studies
Hospitalization
Humans
Infant
Male
Middle Aged
Saudi Arabia
United States
Saudi Arabia
United States
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Summary:We studied 270 Saudi Arabs with homozygous sickle cell anemia, using chart review, a register (since 1969), and home visiting in 42 cases. Average follow-up for the total group was 10 years. Seventy-four percent of those diagnosed by age 3 years presented on screening or with merely anemia; 26% presented with illness, abnormal physical findings, or pain. Compared with American or Jamaican blacks, serious complications occurred only 6% to 25% as frequently; leg ulcers did not occur at all; the mortality under age 15 years was 10% as great; mean levels of blood hemoglobin were higher (10 g/dl), reticulocyte count was lower (5% to 6%), and mean fetal hemoglobin (HbF), which was inversely correlated with reticulocytes, was higher (22% to 26.8%). The high HbF is believed to account for the very mild clinical manifestations.
ISSN:0003-4819
DOI:10.7326/0003-4819-88-1-1