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Glucose‐6‐Phosphate Dehydrogenase Deficiency and Homozygous Sickle Cell Disease in Jamaica
Author(s) -
Gibbs W. N.,
Wardle Jane,
Serjeant G. R.
Publication year - 1980
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/j.1365-2141.1980.tb03812.x
Subject(s) - glucose 6 phosphate dehydrogenase , glucose 6 phosphate dehydrogenase deficiency , dehydrogenase , medicine , glucosephosphate dehydrogenase deficiency , bilirubin , population , hemoglobin s , red cell , heterozygote advantage , hemoglobinopathy , endocrinology , disease , gastroenterology , sickle cell anemia , enzyme , biology , biochemistry , genotype , gene , environmental health
S ummary . The relationship between D‐glucose‐6‐phosphate: NADP oxido‐reduc‐tase (E.C.I.1.1.49; glucose‐6‐phosphate dehydrogenase; G6PD) deficiency and homozygous sickle cell (SS) disease was examined in 120 patients. The proportion of hemizygotes (22‐6%) was slightly more than that observed, and the combined proportions of heterozygotes and homozygotes (28‐3%) were slightly less than would be expected, in the general population, but the differences were not significant. However, the proportion of patients of abnormal G6PD status in the 10‐19 years age group was 41‐7%, significantly more than that found in the 20‐29 years age group (0‐02< P <0‐05), or expected in the general population (P=0‐05). Possible reasons for this are discussed. Difference in G6PD status did not affect the total haemoglobin concentration, reticulocyte count, unconjugated serum bilirubin or Hb F concentration, irreversibly sickled cell counts or plasma haemoglobin concentration, and there was no demonstrable correlation between clinical severity or leg ulceration and abnormal G6PD status.