title: Effect of alpha thalassaemia, G-6-PD deficiency and Hb F on the nature of sickle cell anaemia in south-western Saudi Arabia.
abstract: This study was conducted on sickle cell anaemia (SCA) patients from the south-western province (SWP) of Saudi Arabia to determine the effect of thalassaemias, glucose-6-phosphate dehydrogenase (G-6-PD) deficiency and Hb F level on the clinical presentation of sickle cell anaemia. The results showed that associated alpha-thalassaemia improved the haematological parameter values while associated G-6-PD deficiency and high Hb F level did not play a significant role in amelioration of the disease in these patients. Hb S/beta(0) -thalassaemia cases showed a severe anaemia similar to the SCA patients without alpha-thalassaemia. However, considerable improvement of the haematological parameters were found in patients with S/beta(0)-thalassaemia and associated alpha-thalassaemia. This paper reveals that alpha-thalassaemia may partially ameliorate the clinical manifestations of SCA in Saudi patients from the SWP, while high Hb F level and G-6-PD deficiency do not modify SCA to any statistically significant extent.
authors.affiliation: Medical Biochemistry Department, College of Medicine, King Saud University, Riyadh, Saudi Arabia.
authors.forename: M A
authors.initials: MA
authors.lastname: el-Hazmi
authors.forename: A R
authors.initials: AR
authors.lastname: al-Swailem
authors.forename: H M
authors.initials: HM
authors.lastname: Bahakim
authors.forename: F Z
authors.initials: FZ
authors.lastname: al-Faleh
authors.forename: A S
authors.initials: AS
authors.lastname: Warsy
mesh_terms: D000755:Anemia, Sickle Cell / Q000097:blood / Q000150:complications*; D002648:Child; D002675:Child, Preschool; D005319:Fetal Hemoglobin / Q000032:analysis*; D005955:Glucosephosphate Dehydrogenase Deficiency / Q000150:complications*; D006801:Humans; D012529:Saudi Arabia / Q000208:ethnology; D013789:Thalassemia / Q000150:complications*
chemical_list: D005319:Fetal Hemoglobin