特发性塌陷性肾小球病二例分析

目的分析和认识小儿特发性塌陷性肾小球病(ICG)的临床特征及病理特点。方法回顾性总结分析二例小儿特发性塌陷性肾小球病的临床资料、病理资料、治疗反应及随访结果。结果二例临床表现为典型的肾病综合征;化验结果有大量蛋白尿、高脂血症、低蛋白血症;病理可见肾小球毛细血管丛节段／全球性塌陷伴上皮细胞增生肥大变性及严重肾小管间质病变;治疗转归二例对激素耐药,其中1例用甲泼尼松和环磷酰胺冲击无效,迅速发展为肾衰竭,半年内死亡;另一例激素耐药。用环孢素A无改善,追踪8个月,持续高血压,蛋白尿无改善。结论小儿特发性塌陷性肾小球病临床以严重的肾病综合征伴迅速进展肾衰竭为特点;病理以肾小球的塌陷、上皮细胞增生肥大变性及严重肾小管间质病变为特点;本病治疗困难,预后不良。

Idiopathic collapsing glomerulopathy in children: report of two cases

OBJECTIVE: Idiopathic collapsing glomerulopathy (ICG) is a clinically and pathologically distinct variant of focal segmental glomerulosclerosis, which is characterized by proteinuria (often nephrotic range) and rapid progression to end-stage renal failure. The typical pathological changes are global/segmental glomerular collapse, hypertrophy and hyperplasia of podocytes and severe tubulointerstitial lesions. Most ICG patients who have been reported in previous published papers are adults. ICG in children is rare. The study aimed to analyze and investigate clinical manifestations, renal histopathological findings, treatment and outcomes of ICG in children. METHODS: Data of two cases of ICG, a 7-year-old boy and a 12-year-old girl, were analyzed. Both of them were Chinese and Han. Clinical characteristics, results of laboratory tests, renal histopathological findings, treatment, outcomes and prognosis of the two children with ICG were retrospectively analyzed. Results were compared with published data. RESULTS: These two children presented typical clinical features of nephrotic syndrome. The quantity of 24 hr urine protein was 7.6 g/d (0.47 g/kg x d for boy) and 10.67 g/d (0.35 g/kg x d for girl). Both of them had hypertension (blood pressure ranged from 130/90 to 150/110 mmHg) and hypercholesterolemia (15.4 mmol/L for the boy and 11.3 mmol/L for the girl). The serum albumin was 12 g/L for girl and 23 g/L for boy. The creatinine clearance rate gradually decreased from normal range to 30 ml/min for the girl. The histopathological changes in renal biopsy of them were focal segmental or global glomerular collapse, hypertrophy and hyperplasia of podocytes and severe tubulointerstitial lesions. These two cases were steroid-resistant and were treated with pulse intravenous methylprednisolone and pulse intravenous cyclphosphamade in one case, who rapidly progressed to end-stage renal failure and died half a year later. Another one was treated with cyclosporine. He showed continuous hypertention and heavy proteinuria for eight months. CONCLUSION: ICG in the 2 children was a severe disease which presented steroid-resistant nephrotic syndrome and rapidly progressive renal failure. The pathological characteristics was global/segmental glomerular collapse, hypertrophy and hyperplasia of podocytes and severe tubulointerstitial lesions. In children with ICG treatment was difficult and the prognosis was poor.