© 1995 by Oxford University Press
research-article |
Glucose-6-phosphate Dehydrogenase Deficiency and Sickle Cell Genes in Bisha
*Medical Biochemistry Department, National Referral and Consulting Unit and WHO Collaborating Center for Haemoglobinopathies, Thalassaemias and Enzymopathies, College of Medicine & University Hospitals, King Saud University Riyadh, Saudi Arabia
**Ministry of Health Riyadh, Saudi Arabia
***Department of Biochemistry, College of Science, King Saud University Riyadh, Saudi Arabia
Correspondence: Professor M. A. F. El-Hazmi, Medical Biochemistry Department (30) & WHO Collaborating Centre for Haemoglobinopathies, Thalassaemias & Enzymopathies, College of Medicine & King Khalid Hospital, PO Box 2925, Riyadh 11461, Saudi Arabia.
This study was conducted on 820 Saudi males and females from Bisha in the western province of Saudi Arabia. Blood samples were analysed to determine the frequency of glucose-6-phosphate dehydrogenase deficiency and haemoglobin S (Hb S) genes, and to investigate interactions between the two genes.
Severe G-6-PD deficiency in this population was due to G-6-PD-Mediterranean; the African variant G-6-PD-A– was not detected. The normal and common form of the enzyme was G-6-PD-B+, occurring at a frequency of 0.8444 and 0.8177 in males and females, respectively. Variants included G-6-PD-A+, G-6-PD-Mediterranean, and G-6-PD-Mediterranean-like at frequencies of 0.0043, 0.0767, and 0.0746, respectively, in males and 0.0057, 0.05413, and 0.0855, respectively, in females.
Sickle cell haemoglobin (Hb S) was encountered in the homozygous (4 per cent) and heterozygous (10 per cent) states at a gene frequency of 0.0860. No interaction between G-6-PD deficiency and Hb S gene was observed. A severe haematological and clinical presentation of the Hb SS disease was encountered in the children from Bisha.