The effects of glucose-6-phosphate dehydrogenase deficiency on the haematological parameters and clinical manifestations in patients with sickle cell anaemia.
This study was conducted on 81 children with sickle cell anaemia (SCA) from the south-western province of Saudi Arabia. Ten of these children had associated glucose-6-phosphate dehydrogenase (G-6-PD) deficiency due to the presence of the phenotype 'G-6-PD Mediterranean'. Slightly lower, statistically non significant, values of red blood cell count, total haemoglobin, packed cell volume and red cell indices were found in SCA patients with G-6-PD deficiency. However, certain clinical features were improved in these patients. Comparison was also made with results published from the eastern province of the country, where SCA is reported to be mild. It is suggested that G-6-PD deficiency due to G-6-PD 'Mediterranean' worsens certain manifestations and improves others in SCA patients with a severe form of SCA, while improvement in haematological parameters is observed in SCA patients with mild SCA.
el-Hazmi
M A
MA
Medical Biochemistry Department, College of Medicine, Riyadh, Saudi Arabia.
Warsy
A S
AS
D000755:Anemia, Sickle Cell / Q000097:blood*; D002648:Child; D002675:Child, Preschool; D004906:Erythrocyte Count; D004909:Erythrocyte Indices; D004911:Erythrocyte Volume; D005955:Glucosephosphate Dehydrogenase Deficiency / Q000503:physiopathology*; D006452:Hemoglobinometry; D006801:Humans; D010641:Phenotype; D012529:Saudi Arabia