21例儿童孤立性蛋白尿的病理组织学特征及预后

Pathologic features and prognosis of 21 children with isolated proteinuria

目的：探讨孤立性蛋白尿（IP）患儿的病理组织学特征、治疗及预后。方法：对21例IP患儿进行肾组织病理活检，根据其病理类型分别给予相应治疗，并进行0．5～10年的随访。结果：21例患儿肾活检结果示，系膜增生性肾炎（MsPGN）13例（轻度系膜增生3例，中度系膜增生6例，重度系膜增生4例），轻微病变型（MCN）2例，IgA肾病（IgAN）3例（病理分级Ⅰ级1例，Ⅱ级2例），局灶性硬化性肾小球肾炎（FSGS）2例，毛细血管内增生性肾小球炎（EnPGN）1例。间质-小管病变主要见于MsPGN和FSGS两种病理类型，主要表现为间质纤维化及炎性细胞浸润、肾小管萎缩、上皮细胞空泡变性。21例中除1例EnPGN外，余20例均给予了药物治疗。21例中症状消失无复发15例，蛋白尿持续存在者3例（病理表现为MsPGN和FSGS），蛋白尿持续不消并伴肾功能损害（病理表现为重度MsPGN）者2例，血压增高者（病理表现为FSGS）1例。结论：本病表现为持续性蛋白尿者均有不同程度的肾脏病理损害，大多数经治疗可有良好预后，严重的MsPGN和FSGS患儿预后较差，对此应以护肾为主，适当应用延缓肾功能损伤的药物。

Objective To discuss the pathologic features, treatment and prognosis of the children with isolated proteinuria （IP）. Methods Twenty-one children with IP were enrolled according to their renal biopsy and were followed up for 0. 5 to 10 years. Results Renal biopsy was performed in all children. Among them 13 were mesangial proliferation glomerulonephritis （MsPGN） （ including 3 minor, 6 moderate, and 4 severe ones）, 2 minimal change nephritis （ MCN）, 3 IgA nephropathy （IgAN） （1 in Grade Ⅰ and 2 in Grade Ⅱ ）, 2 focal segmemtal glomerulosclerosis （FSGS） and 1 endocapillary proliferative glomerulonephritis （EnPGN）. Interstitial changes could be found in MsPGN and FSGS mostly, presenting interstitial fibrosis, infiltration of inflammatory cells, atrophy of renal tubule, and the vacuolar degeneration of epithelia. All children accepted the medical treatment except the EnPGN case. Fifteen children recovered with no relapse; proteinuria persisted in 3 severe MsPGN and FSGS cases; 2 got the impaired renal function accompanied by persistent proteinuria; and i had hypertension. Conclusion The different degrees of renal damage can be found in all IP children who have persistent proteinuria. Most patients can get good outcome after aggressive therapies. However, the prognosis of those with severe MsPGN and FSGS was not so optimistic, and some reno-protective treatments should be given to postpone the deterioration of the renal function.