目的观察IgA肾病(IgAN)的临床病理特征,探讨肾小管间质损害与小儿IgAN进展的关系。方法收集2004年1月-2008年12月经本院肾活检确诊为原发性IgAN的165例患儿(男107例,女58例;年龄3~17岁)的临床及病理资料,并进行回顾性分析。结果本组患儿肾脏受损的组织学特点为均存在肾小球系膜区病变,肾小球毛细血管襻的病变多为节段性分布;急性重症病变为血管襻纤维素样坏死、新月体形成;慢性化病变则为肾小球毛细血管襻瘢痕形成,导致球性硬化。而肾小管间质也有不同程度损害。肾小管间质无损害组占15.2%,肾小管间质轻度损害组占43.6%,中度损害组占33.9%,重度损害组占7.3%。不同肾小管间质损害程度组年龄、病程比较差异均无统计学意义(Pa〉0.05)。随着肾小管间质损害程度的加重,患儿血压升高,尿蛋白定量增加,血肌酐升高;随着系膜增殖程度及新月体积分的增加,肾小管间质损害程度加重;肾小管间质损害程度与球性硬化呈正相关(r=0.461,P〈0.01)。结论随着小管间质损害程度的加重,IgAN进行性加重。肾小管间质损害与蛋白尿、高血压、肾小球系膜增生、球性硬化、新月体形成密切相关。肾小管间质损害是IgAN进展的重要原因之一。
Objective To observe the characteristics of the clinical and pathological characteristics of immunoglobulin A nephropathy(IgAN),and to analyze the relationship between renal tubulointerstitial lesions(TIL)and progression of IgAN in children.Methods One hundred and sixty-five patients with primary IgAN(107 cases were male,58 cases were female;their ages were 3-17 years old)were diagnosed as primary IgAN combined with clinic and renal biopsy from Jan.2004 to Dec.2008.The clinical pathological features of the children with IgAN were analyzed retrospectively,and the relationships between them were investigated.Results In histological examination,all patients presented mesangial lesions,with or without segmental peripheral loops lesions.The acute severe lesions showed fibrinoid necrosis,crescents,and the chronic lesions were peripheral loops scar and globe sclerosis.Among all the patients,those without TIL accounted for 15.2 %,those with mild TIL was 43.6%,moderate TIL was 33.9%,while severe TIL was about 7.3%.The servrity of TIL was associated with the clinical glomerular pathological features:there was no significant difference in the age and the course of disease for different TIL groups(P0.05).With the increase of the severity of TIL,hypertension,proteinuria became worse and serum creatinine increased;With the increase of the score of mesangial lesions and crescents,TIL became severe.TIL was closely correlated to the score of glomerulosclerosis(r=0.461,P0.01).Conclusions With the increase of the severity of TIL,the condition of IgAN becomes worse.TIL is closely related to the severity of glomeruler and vascular lesions,and these lesions may further lead to the development of TIL.TIL may be one of the key factors for the prognosis of IgAN.