20例儿童狼疮性肾炎临床与病理分析

目的探讨儿童狼疮性肾炎(LN)的临床特点与肾脏病理类型之间的关系。方法回顾性分析20例狼疮性肾炎患儿的临床表现、实验室检查和病理检查资料。结果临床分型以肾病综合征型为最常见,占8例(40%);孤立性血尿或(和)蛋白尿2例(10%),急性肾炎型4例(20%),急进性肾炎型2例(10%),慢性肾炎型1例(5%),肾小管间质损害型2例(10%),亚临床型1例(5%)。肾外症状以发热(65%)、贫血(35%)、皮肤损害(50%)、关节炎(35%)等多见;实验室检查:补体C3降低17例(85%),抗核抗体(ANA)阳性16例(80%),抗ds-DNA阳性13例(65%),血浆白蛋白降低8例(40%),转氨酶增高4例(20%),Hb降低13例(65%),白细胞减少11例(55%),血小板减少9例(45%),BUN增高7例(35%),D二聚体阳性9例(45%),Coob’s试验阳性3例(15%),球蛋白增高4例(20%)。ESR增高3例(65%)。20例肾活检均异常,病理类型以Ⅳ型LN最多,10例(50%),其次是V型3例(15%),Ⅰ型、Ⅱ型和Ⅲ型各1例(分别占5%),Ⅳ+Ⅴ型2例(10%),Ⅲ+Ⅴ型2例(10%)。大多数患儿免疫荧光多呈"满堂亮",2例呈寡免疫复合物沉着。结论儿童狼疮性肾炎的临床表现及病理类型有其自身特点,肾脏病理学检查对狼疮性肾炎的诊断治疗具有十分重要的指导作用。

The analysis of clinic and pathology in lupus nephritis in 20 children

Objective To explore the relationship of clinical characteristic and renal biopsy in pediatric lupus nephritis.Methods To analyze retrospectively the clinic manifestations,laboratory and pathological data in 20 cases of children with lupus nephritis.Results The clinic type with nephritic syndrome was the most common,accounting for 8 cases(40%),isotated hematuria or(and)proturia were 4 cases(10%),acute nephritis were 4 cases(20%),rapidly progressive glomerulonephritis were 2 cases(10%),chronic nephritis was 1 cases(5%),renal tubular interstitial damage were 2 cases(10%),subclinical type was 1 case(5%).There were more extrarenal symptoms with fever(65%),anemia(35%),skin lesion(50%),arthritis(35%).In laboratory data,there were complement C3 decreased in 17 cases(85%),antinuclear antibody(ANA) positive in 16 cases(80%),ds-DNA positive in 13(65%),plasma albumin decreased in 8 cases(40%),transaminase increased in 4 cases(20%),Hb decreased in 13 cases(65%),leukocyte reduction in 11 cases(55%),thrombocytopenia in 9 cases(45%),blood urea nitrogen increased in 7 cases(35%),D two dimmer positive in 9 cases(45%),Coob’s test positive in 3 cases(15%),globulin increased in 4(20%),ESR increased in 3(15%).20 cases of renal biopsy were abnormal,in which type Ⅳ was the most,accounting for 50%(10 cases),followed by type V(3 cases)accounted for 15%,type Ⅰ,type Ⅱ and type III(1 cases each) accounted for 5% each,type IV plus V(2 cases)accounted for 10%,type III plus V(2 cases) accounted for 10%.In most renal pathology of the patients,immune fluorescence was "a bright",2 cases showed less immune complex deposit.Conclusion The clinic manifestations and pathological types of Lupus nephritis in children have their own characteristics,the renal pathology examination has a very important guiding role to the diagnosis and treatment of lupus nephritis.