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材料和方法83例葡萄糖—6磷酸脱氢酶(G-6-PD)缺陷引起的溶血危象患儿,男60例,女23例。年龄6个月～16岁。入院时进行常规血液学检查,血清和尿游离血红蛋白(Hb)及红细胞内HeinZ小体的检测。红细胞G-6-PD活性由Brewer法检测。Hb电泳在pH8.6的醋酸纤维素膜上进行。常规法检测血清BUN、尿酸和胆红素水平。结果G-6—PD缺陷患者引起溶血危象的主要原因:(1)感染30例(34.3%);(2)药物(抗疟疾药、退热镇痛药、抗生素、铁剂、维生素C等)40例(48.1%);(3)蚕豆5例(6.1%);(4)原因不明8例(10.5%)。G-6-PD活性中度低下者27例(32.53%),重度低下者56例(67.47%)。
MATERIALS AND METHODS: There were 60 males and 23 females with hemolytic crisis caused by the deficit of glucose-6-phosphate dehydrogenase (G-6-PD). Age 6 months to 16 years old. At admission, routine hematology tests, serum and urinary free hemoglobin (Hb), and intrahepatic HeinZ bodies were performed. Erythrocyte G-6-PD activity was assayed by the Brewer method. Hb electrophoresis was performed on a cellulose acetate membrane, pH 8.6. Routines test serum BUN, uric acid and bilirubin levels. Results The main causes of hemolytic crisis in patients with G-6-PD deficiency were: (1) infection in 30 patients (34.3%); and (2) antimalarials, antipyretics and analgesics, antibiotics, iron supplements and vitamin C ) 40 cases (48.1%); (3) 5 cases of Vicia faba (6.1%); (4) 8 cases were unknown reasons (10.5%). There were 27 cases (32.53%) with moderate G-6-PD activity, 56 cases (67.47%) with severe low performance.