难治性肾病30例临床与病理的关系

Correlation between clinical symptoms and pathological features of the refractory nephrotic syndrome in 30 children

目的 探讨难治性肾病的临床与病理特征之间的关系。方法 对 3 0例小儿难治性肾病的临床特点、病理特征及治疗结果进行分析。结果 小儿难治性肾病临床表现大多为蛋白尿 +血尿占 70 %。最常见病理类型为系膜增殖性肾小球肾炎 ,占 43 .3 % ;其次为局灶节段性肾小球硬化 ,占 2 0 % ;再次为膜增殖性肾小球肾炎占 13 .3 % ,而IgA肾病、微小病变等病理类型少见。临床表现为单纯蛋白尿组病理类型以系膜增殖性肾炎为主 ,临床表现多为对激素依赖、频复发 ;蛋白尿 +血尿组病理特征以局灶节段性肾小球硬化、膜增殖性肾炎最多 ,对激素治疗表现的反应为激素耐药。结论 临床表现为蛋白尿 +血尿的小儿肾病大部分为难治性肾病 ,病理改变多样 ,以系膜增殖性肾小球肾炎最多见。临床及病理分型。

Objective To explore the correlation between clinical symptoms and pathological features of the refractory nephrotic syndrome in children.Methods The clinical symptoms and pathological features of 30 cases with refractory nephrotic syndrome in children were analyzed, as well as the therapeutic results. Results Clinically, most cases of the refractory nephritic syndrome in children were presented with proteinuria and hematuria, about 70 %. Pathologically, mesangioproliferative glomerulonephritis (MsPGN) was frequently seen, 43.3 %. Focal segmental glomerulosclerosis (FSGS) came second in incidence, 20 %. Membranoproliferative glomerulonephrits (MPGN) was about 13.3 % of the total cases, while IgA nephritis and minimal change glomerulonephritis (MCNS) were rarely found. Most cases in simple proteinuria group were pathologically mesangioproliferative glomerulonephritis, which proved to be steroid-dependent with frequent relapse ,but most cases in the proteinuria and hematuria group were focal segmental glomerulosclerosis and membranoproliferative glomerulonephrits in terms of pathology, which appeared steroid-resistant. Conclusions Most cases of children′s refractory nephritic syndrome were clinically proteinuria and hematuria. Its pathological changes could vary, however, mesangioproliferative glomerulonephritis was most common. The clinical symptoms and pathological features of the nephrotic syndrome and its reactions to steroid treatment seem closely related.