原发性局灶节段性肾小球硬化症患儿21例的临床、病理特点与预后分析

Analysis of Clinical and Pathological Characteristics and Prognosis in 21 Children with Primary Focal Segmental Glomerulosclerosis

目的观察原发性局灶节段性肾小球硬化症(FSGS)患儿的临床表现、肾脏病理特点及其与预后的关系。方法收集重庆医科大学附属儿童医院2003年6月-2008年6月经肾活检确诊为原发性FSGS的21例患儿的临床病理资料,分析其临床表现、实验室指标和肾脏病理特点,随访患儿的预后,并对影响预后的相关因素进行分析。结果21例FSGS患儿临床诊断以肾病综合征最多[15例(71%)],病理分型以非特殊型最多[8例(38%)],病理类型与临床表现无对应关系(P>0.05)。21例中2例失访,余19例随访4～51(22.84±12.13)个月,完全缓解10例(53%),显著缓解4例(21%),部分缓解4例(21%),无效1例(5%)。肾小管间质病变与FSGS的预后相关(P<0.05),是FSGS预后的危险因素(OR>1)。结论1.原发性FSGS患儿临床表现以肾病综合征最多,非特殊型是原发性FSGS的主要病理类型,不同病理类型与临床表现无对应关系。2.原发性FSGS患儿短期预后良好,应加强长期预后的随访观察。3.肾小管间质病变是影响预后的危险因素。

Objective To investigate the relationship between clinical and pathological characteristics and prognosis in children with primary focal segmental glomerulosclerosis （FSGS）. Methods Twenty - one cases of FSGS proven by renal biopsy were retrieved from the archival files in Children＇s Hospital of Chongqing Medical University from Jun. 2003 to Jun. 2008. The clinical features,laboratory data and pathological features were studied retrospectively. Their prognosis were followed up and the prognostic factors were analyzed. Results Of 21 cases, nephrotic syndrome in 15 cases （71% ）was the predominant clinical manifestation, not otherwise specified variant in 8 cases（ 38% ） was the commonest pathological variant. There were no relationship between pathological types and clinical manifestations of FSGS （P 〉 0.05 ）. Two cases were lost to follow up, 19 out of 21 cases were followed up. The follow - up duration was from 4 to 51 months [ mean follow - up duration was （22.84 ± 12.13 ） months ]. At last follow -up, 10 cases （53%） had complete remission,4 cases （21%） had significant remission,4 cases （21% ） had partial remission and 1 case（5% ） had no remission. Tubulointerstitlal lesions were related with prognosis（P 〈0.05 ） ,and it was a risk factor for proguosis（ OR 〉 1 ）. Conclusions 1. The predominant clinical manifestation of primary FSGS is nephrotic syndrome in children. The not otherwise specified variant is the commonest pathological variant in primary FSGS. There is no relationship between pathological types and clinical manifestations of FSGS. 2. Most of the children with primary FSGS have a good short - term prognosis,but their long - term prognosis must be followed up. 3. Tubulointerstitial lesions are risk factors for prognosis.