论文部分内容阅读
迄今,全世界已报道14例先天性C_3缺乏症,大多有反复的严重化脓性细菌感染,本文报告以免疫学手段研究的第2例遗传性C_3缺乏症。病例 10岁老挝男孩,足月顺产,新生儿期正常。患肺炎双球菌脑膜炎2次(5月龄、20月龄),大叶性肺炎8次(自生后7月～8岁)。5月龄左侧Todd偏瘫和局灶性癫痫,20月龄发现血尿、蛋白尿。3岁7月龄发现活性溶血补体和纯合子C_3缺乏,5岁8月龄肾活检示膜增殖性肾小球肾炎。曾接种单剂多价肺炎链球菌疫苗。免疫学检查示T和B淋巴细胞活性正常,多种特异性抗体血清滴度阳性(血型物质、白喉、破伤风、脊髓灰质炎、风疹和3、18、19及23型肺炎球菌等)。无自身抗体,血清IgG免疫复
To date, 14 cases of congenital C_3 deficiency have been reported in the world, mostly with recurrent severe suppurative bacterial infections. This article reports the second case of hereditary C_3 deficiency studied by immunological methods. Case 10-year-old Lao boy, full-term pregnancy, normal newborn. Mycoplasma pneumoniae two times (5 months old, 20 months old), lobar pneumonia 8 times (after birth in July to 8 years old). Todd hemiplegia and focal epilepsy on the left side of 5 months of age, hematuria, proteinuria at 20 months of age. 3-year-old 7-month-old found that hemolysis complement and homozygous C_3 deficiency, 5-year-old 8-month-old renal biopsy showed proliferative glomerulonephritis. Have been vaccinated single dose multivalent pneumococcal vaccine. Immunological tests showed normal T and B lymphocyte activity, and positive sera for various specific antibodies (blood group, diphtheria, tetanus, poliomyelitis, rubella and Streptococcus pneumoniae types 3, 18, 19 and 23, etc.). No autoantibodies, serum IgG immune complex