Sickle cell disease in Saudi Arabs in early childhood. 1981

R P Perrine, and P John, and M Pembrey, and S Perrine

Haemoglobin electrophoresis screening of 2341 infants from the oases of eastern Saudi Arabia, performed in an attempt to detect cases early and then to follow up and give better management to patients with sickle cell disease, showed 20% with S-trait and 43 with sickle cell disease (37 HbSS and 6 S-beta(0) thalassaemia). On follow-up from birth (or from 3 months) for a mean of 3 1/2 years there was more morbidity and mortality than in matched Saudi Arab controls, but these rates were lower than for affected black infants in the US or Jamaica. Serial haemoglobin levels were l g/dl lower than for controls; height and weight increases were the same. Mean fetal haemoglobin level was 32% at 4-5 years. Half of a subsample of 23 cases had evidence of coexistent alpha-thalassaemia which appeared to protect against functional asplenia, judged by the presence of Howell-Jolly bodies; however there was no difference in degree of anaemia or clinical course. Early screening is recommended for infants at risk for sickle cell disease, and comprehensive care should be given even if the infant has the less severe type.

UI MeSH Term Description Entries
D007223 Infant A child between 1 and 23 months of age. Infants
D008297 Male Males
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D002675 Child, Preschool A child between the ages of 2 and 5. Children, Preschool,Preschool Child,Preschool Children
D005260 Female Females
D005319 Fetal Hemoglobin The major component of hemoglobin in the fetus. This HEMOGLOBIN has two alpha and two gamma polypeptide subunits in comparison to normal adult hemoglobin, which has two alpha and two beta polypeptide subunits. Fetal hemoglobin concentrations can be elevated (usually above 0.5%) in children and adults affected by LEUKEMIA and several types of ANEMIA. Hemoglobin F,Hemoglobin, Fetal
D006451 Hemoglobin, Sickle An abnormal hemoglobin resulting from the substitution of valine for glutamic acid at position 6 of the beta chain of the globin moiety. The heterozygous state results in sickle cell trait, the homozygous in sickle cell anemia. Hemoglobin S,Deoxygenated Sickle Hemoglobin,Deoxyhemoglobin S,Hemoglobin SS,Hemoglobin, Deoxygenated Sickle,SS, Hemoglobin,Sickle Hemoglobin,Sickle Hemoglobin, Deoxygenated
D006452 Hemoglobinometry Measurement of hemoglobin concentration in blood. Hemoglobinometries
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000755 Anemia, Sickle Cell A disease characterized by chronic hemolytic anemia, episodic painful crises, and pathologic involvement of many organs. It is the clinical expression of homozygosity for hemoglobin S. Hemoglobin S Disease,HbS Disease,Sickle Cell Anemia,Sickle Cell Disease,Sickle Cell Disorders,Sickling Disorder Due to Hemoglobin S,Anemias, Sickle Cell,Cell Disease, Sickle,Cell Diseases, Sickle,Cell Disorder, Sickle,Cell Disorders, Sickle,Disease, Hemoglobin S,Hemoglobin S Diseases,Sickle Cell Anemias,Sickle Cell Diseases,Sickle Cell Disorder

Related Publications

R P Perrine, and P John, and M Pembrey, and S Perrine
January 1970, Acta haematologica,
R P Perrine, and P John, and M Pembrey, and S Perrine
January 1998, International journal of gynaecology and obstetrics: the official organ of the International Federation of Gynaecology and Obstetrics,
R P Perrine, and P John, and M Pembrey, and S Perrine
April 1988, Journal of tropical pediatrics,
R P Perrine, and P John, and M Pembrey, and S Perrine
January 1987, Acta haematologica,
R P Perrine, and P John, and M Pembrey, and S Perrine
November 2007, Journal of the International Neuropsychological Society : JINS,
R P Perrine, and P John, and M Pembrey, and S Perrine
January 1982, The American journal of pediatric hematology/oncology,
R P Perrine, and P John, and M Pembrey, and S Perrine
January 1985, The American journal of pediatric hematology/oncology,
R P Perrine, and P John, and M Pembrey, and S Perrine
January 1971, Acta haematologica,
R P Perrine, and P John, and M Pembrey, and S Perrine
July 2019, The British journal of ophthalmology,
R P Perrine, and P John, and M Pembrey, and S Perrine
February 2003, Saudi medical journal,
Copied contents to your clipboard!