title: Clinical significance of glucose-6-phosphate dehydrogenase deficiency in Nigerian patients with sickle cell disease.
abstract: A total of 75 (45 males, 30 females) patients with sickle cell disease (SCD) at the university of Maiduguri Teaching Hospital were screened for G-6-PD deficiency and their steady state levels of haematocrit, reticulocyte count and irreversibly sickled cells (ISC) as well as the occurrence of vaso-occlusive crises were analysed with respect to their G-6-PD status during a 3 year period (1998-2000). None of the female patients was G-6-PD deficient, while (15.6%) male patients had the enzyme deficiency. The patients with G-6-PD deficiency had significantly lower mean level of haematocrit (0.221/l) with higher level of reticulocyte count (15%) as compared to their non-G-6-PD deficient counter parts who had mean values of 0.271/l and 9% for haematocrit count respectively. However, there was no significant difference in mean levels of ISC between G-6-PD deficient (6%) and non-G-6-PD deficient (5%) patients with SCD. Patients with G-6-PD deficiency had significantly lower episodes of vaso-occlusive crises (2.1 episodes/patient). This data would suggest that co-inheritance of G-6-PD deficiency in SCD reduces frequency of crises and improves prognosis.
authors.affiliation: Department of Haematology, University of Maiduguri Teaching Hospital, PMB 1414, Maiduguri, Nigeria.
authors.forename: S G
authors.initials: SG
authors.lastname: Ahmed
authors.forename: U A
authors.initials: UA
authors.lastname: Ibrahim
mesh_terms: D000208:Acute Disease; D000328:Adult; D000755:Anemia, Sickle Cell / Q000097:blood / Q000150:complications* / Q000453:epidemiology / Q000235:genetics; D001809:Blood Viscosity; D018450:Disease Progression; D005260:Female; D005955:Glucosephosphate Dehydrogenase Deficiency / Q000097:blood / Q000150:complications* / Q000453:epidemiology* / Q000235:genetics; D006400:Hematocrit; D006461:Hemolysis; D006784:Hospitals, Teaching; D006801:Humans; D008297:Male; D008403:Mass Screening; D009549:Nigeria / Q000453:epidemiology; D015995:Prevalence; D011379:Prognosis; D012156:Reticulocytes; D017678:Sex Distribution