儿童IgA肾病的临床与病理分析

目的 探讨IgA肾病患儿临床表现和病理改变的关系及疾病转归.方法 分析30例IgA肾病患儿的临床和病理改变,病理分型采用改良Lee分型法,其中28例进行随访,4例重复肾活检.结果 临床表现孤立性血尿型10例,病理为Ⅰ、Ⅱ级;血尿和蛋白尿型11例,病理为Ⅰ～Ⅲ级;急性肾炎型2例,病理Ⅲ级;肾病综合征型7例,病理为Ⅱ～Ⅳ级,以Ⅱ、Ⅲ级为主.4例行重复肾活检,病理变化为改善、加重和无明显变化,但免疫复合物沉积均有加重.28例随访1年2个月～9年,其中9例预后不乐观;死亡1例;预后不良患儿7例治疗依从性差.结论 IgA肾病临床表现与病理有一定相关性,急性肾炎型、肾病综合征型患儿损伤重、预后欠佳,同时提示临床医师重视IgA肾病患儿随访和健康宣教,提高治疗依从性,以利疾病康复.

Clincopathologic features of IgA nephropathy in children

Objective To study the clinicopathologic features and prognosis of children with IgA nephropathy.Methods We analyzed the clinicopathologic data of 30 IgA nephropathy cases classified by improved Lee pathological typing.Twenty eight cases were followed up,and 4 cases accepted renal biopsy repeatedly.Results There were 10 cases of isolated hematuria with pathologic grades Ⅰ and Ⅱ.Eleven cases had hematuria and proteinuria with pathologic grades from Ⅰ to Ⅲ.Two cases showed acute nephritis with pathologic grades from Ⅱ to Ⅲ.Seven cases showed nephrotic syndrom with pathologic grades from Ⅱ to Ⅳ.Among all the cases,grades Ⅱ and Ⅲ were in majority with proportion of 66.7％.Four cases were biopsied repestedly,and the pathologic changes showed improvement,exacerbation or no change,but immunoglobulin deposition was increased in these cases.We had followed up 28 cases from one year 2 month to 9 years.Nine cases had bad prognosis,in which 1 case died,and 7 cases had poor treatment adherence.Conclusions IgA nephropathy patients were mainly in school children,in which boys were more than girls.Clinical manifestation showed relations with pathology to some extent.The pathologic damage of isolated hematuria in IgA nephropathy had better prognosis relatively.In the patients with pathologic changes of acute nephritis,the damage was more serious,and prognosis was worse.So we should pay more attention to the follow up of the children with IgA nephropathy.We should guide them to have proper diet and to prevent against infectior.Also we should monitor side effects of pharmaceutical drugs and pay close attention to renal outward manifestations for the disease rehabilitation.