title: Glucose-6-phosphate dehydrogenase deficiency and sickle cell disease in Brazil.
abstract: The frequency of glucose-6-phosphate dehydrogenase (G-6-PD) deficiency was determined in 54 male patients with sickle cell diseases: 31 sickle cell anemia (SS), 14 sickle cell hemoglobinopathy (SC) and 9 HbS/beta-thalassemia (S/B-thal) by a combination of quantitative assay, fluorescent spot test and electrophoresis. Of the 54 patients tested, 7 were found to be G-6-PD deficient (G-6-PD-) (3 SS, 3 SC and 1 S/B-thal) and 47 G-6-PD normal (G-6-PD+) (6 G-6-PD A and 41 G-6-PD B). All the deficient patients were G-6-PD A-. The frequency of G-6-PD deficiency did not differ significantly from that observed in the general population. Compared to patients who were not G-6-PD-, there were no significant differences in the hemoglobin concentration and reticulocyte count in patients with sickle cell diseases who were G-6-PD-.
authors.affiliation: Department of Clinical Medicine, School of Medical Sciences, State University of Campinas, Unicamp, Brazil.
authors.forename: S T
authors.initials: ST
authors.lastname: Saad
authors.forename: F F
authors.initials: FF
authors.lastname: Costa
mesh_terms: D000293:Adolescent; D000328:Adult; D000755:Anemia, Sickle Cell / Q000150:complications / Q000208:ethnology / Q000235:genetics*; D044383:Black People; D001938:Brazil; D002648:Child; D005787:Gene Frequency / Q000235:genetics; D005838:Genotype; D005955:Glucosephosphate Dehydrogenase Deficiency / Q000150:complications / Q000208:ethnology / Q000235:genetics*; D006801:Humans; D008297:Male; D015995:Prevalence