Kilinç Y, Kümi M
Department of Paediatrics, Medical Faculty of Cukurova University, Adana, Turkey.
Acta Paediatr Scand. 1990 Nov;79(11):1075-9. doi: 10.1111/j.1651-2227.1990.tb11386.x.
Clinical and laboratory evaluation of 60 boys with hemizygous, 12 girls with homozygous, and 11 girls with heterozygous erythrocyte glucose-6-phosphate dehydrogenase deficiency was made during haemolytic crisis. The main clinical symptoms were paleness, dark urine and oliguria. Only one patient needed peritoneal dialysis. Coexistence of glucose-6-phosphate dehydrogenase deficiency associated with haemoglobinopathy was found to be higher than expected (32 out of 83 cases). Also, the high prevalence of glucose-6-phosphate dehydrogenase deficiency among females with homozygous and heterozygous disease was surprising. The precipitating factors of haemolysis were variable. Rather than antimalarial and antipyretic-analgesic drugs, infections seemed to be the main haemolytic factor.
在溶血危象期间，对60名半合子男孩、12名纯合子女孩和11名杂合子红细胞葡萄糖-6-磷酸脱氢酶缺乏症女孩进行了临床和实验室评估。主要临床症状为面色苍白、深色尿和少尿。只有一名患者需要腹膜透析。发现葡萄糖-6-磷酸脱氢酶缺乏症与血红蛋白病并存的情况高于预期（83例中有32例）。此外，纯合子和杂合子疾病女性中葡萄糖-6-磷酸脱氢酶缺乏症的高患病率也令人惊讶。溶血的诱发因素各不相同。溶血的主要因素似乎是感染，而非抗疟药和解热镇痛药。
