以肾病综合征为表现的IgA肾病临床与病理24例分析

Clinical features and pathology of IgA nephrosis in 24 children with manifestation of nephrotic syndrome

为探讨以肾病综合征为表现的IgA肾病的临床与病理和免疫分型的关系,24例经肾活检确诊为IgA肾病,其中应用泼尼松中长程治疗8例,常规使用泼尼松联合CTX冲击治疗10例,泼尼松与雷公藤多苷(30mg/d)联合治疗2例,失访4例。结果根据Lee修改的Meadow标准,Ⅰ级3例(12.5％),Ⅱ级7例(29.2％),Ⅲ级8例(33.3％),Ⅳ级6例(25.0％)。免疫荧光分型:IgA型10例(41.7％),IgA+IgG型2例(8.3％),IgA+IgM型6例(25.0％),IgA+IgM+IgG型6例(25.0％)。20例获随访,8例对激素不敏感;2例激素联合雷公藤多苷治疗后缓解;另10例激素加CTX冲击治疗,8例有效,2例无效,此2例随访病程中出现肾功能不全。提示以肾病综合征为表现的IgA肾病的病理改变均较重,以Ⅲ级～Ⅳ级改变为主。免疫荧光分型中以IgA型多见。多数对激素不敏感。

The present study tried to explore the relationship between clinical features and pathology of IgA nephrosis. IgA nephrosis was diagnosed by renal biopsy in 24 children who manifested nephrotic syndrome clinically. Long-term single prednison was given in 8 cases, prednison combined CTX in a way of the impact therapy in 10 cases, and prednison combined Glucosida tripteryge tota(GTT)in 2 cases, respectively. Another 4 cases were lost in follow-up study. The results showed that it was pathologically classified as grade Ⅰ in 3 cases(12. 5%),grade Ⅱ in 7 cases(29. 2%) .grade 3333 in 8 cases(33. 3%)and grade Ⅳ in 6 cases(25. 0%)based on Lee's Meadow standard. It was also classified by immunofluorescence as type IgA in 10 cases(41. 7%),type IgA+IgG in 2 cases(8. 3%),type IgA+IgM in 6 cases(25. 0%)and type IgA+IgM + IgG in 6 cases(25. 0%). Among 20 follow-up cases, 8 cases were no response to prednison, 2 cases released after the treatment of prednison combined GTT.and 8 cases were effect and 2 cases were failure to prednison combined CTX, respectively, It is concluded that renal tubular damage of IgA nephrosis is serious. Grades Ⅲ and Ⅳ pathologically and type IgA immunologically are common in children with IgA nephrosis clinically. Prednison is usually not useful for the treatment of IgA nephrosis in the most of these patients.