GLUCOSE-6-PHOSPHATE-DEHYDROGENASE DEFICIENCY AND HOMOZYGOUS SICKLE-CELL DISEASE IN JAMAICA
GLUCOSE-6-PHOSPHATE-DEHYDROGENASE DEFICIENCY AND HOMOZYGOUS SICKLE-CELL DISEASE IN JAMAICA
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DOI：
10.1111/j.1365-2141.1980.tb03812.x
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发表时间：
1980-01-01
影响因子：
6.5
通讯作者：
SERJEANT, GR
中科院分区：
文献类型：
--
作者：
GIBBS, WN;WARDLE, J;SERJEANT, GR
本文研究了120例d -葡萄糖-6-磷酸：NADP氧化还原酶（葡萄糖-6-磷酸脱氢酶；G6PD）缺乏与纯合子镰状细胞病（SS）的关系。半合子比例（22.6%）略高于观察值，杂合子和纯合子组合比例（28.3%）略低于一般人群的预期值，但差异不显著。10-19岁年龄组G6PD状态异常的患者比例为41.7%，显著高于20-29岁年龄组（0.02 < P < 0.05）或一般人群的预期（P = 0.05）。讨论了可能的原因。G6PD状态的差异不影响总Hb浓度、网状红细胞计数、非结合血清胆红素或Hb F浓度、不可逆镰状细胞计数或血浆Hb浓度，临床严重程度或腿部溃疡与G6PD异常状态之间没有明显的相关性。
The relationship between D-glucose-6-phosphate:NADP oxido-reductase (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. The proportion of patients of abnormal G6PD status in the 10-19 yr age group was 41.7%, significantly more than that found in the 20-29 yr age group (0.02 < P < 0.05) or expected in the general population (P = 0.05). Possible reasons for this were discussed. Difference in G6PD status did not affect the total Hb concentration, reticulocyte count, unconjugated serum bilirubin or Hb F concentration, irreversibly sickled cell counts or plasma Hb concentration, and there was no demonstrable correlation between clinical severity or leg ulceration and abnormal G6PD status.