【摘要】 目的探讨狼疮性肾炎(LN)男性患儿的临床特征。方法回顾分析1993年1月至2007年10月在北京大学第一医院儿科确诊为LN的24例男性患儿临床资料及随访结果。结果24例男性LN患儿,起病年龄6～14岁,平均(11.2±1.9)岁;确诊时平均病程为(9.3±24.8)个月,4例曾较长时间误诊为其他疾病。本组病例中以肾脏受累为首发表现者10例(41.7%),2例(8.3%)仅有肾脏损害的表现。LN临床分型主要见于肾病综合征(45.8%),其次为孤立性蛋白尿和血尿(20.8%)伴有蛋白尿者23例(95.8%),24h尿蛋白定量0.87～16.98g,肾功能损害者5例(20.8%)。19例行肾穿刺活检,其病理以Ⅳ型狼疮性肾炎为主,占57.9%,其次为Ⅴ型(15.8%);免疫荧光以IgG和C3沉积为主,呈"满堂亮"者13例,占68.4%。所有患儿均接受肾上腺皮质激素联合免疫抑制剂的治疗,16例平均3.4个月后临床缓解,缓解率66.7%;随访中有6例反复,占25%,2例发生肾功能不全(1例肾移植)。结论男性患儿LN临床类型以肾病综合征为主,肾病理以Ⅳ型LN为主,易误诊、漏诊。早期诊断和治疗、定期随访是力争LN良好预后的关键。更多还原

【Abstract】 Objective To analyze the clinical charactersistic of boys with lupus nephritis(LN).Method Retrospective analysis was used to analyze the clinical data,renal pathological data and follow-up survey data of 24 boys with LN.Results A total of 24 boys suffered from LN at the average age of 11.2±1.9 years old(ranging from 6.0 to 14 years old).The course of disease before diagnosis was 9.3±24.8months(ranging from 1 to 120 months);4 cases of them were misdiagnosed for a long time.Among them,kidney damage was the first manifestation in ten cases(41.7%),and 2 cases(8.3%) displayed only LN;23 cases(95.8%) were accompanied by proteinuria,and 24 hours uria protein ranged from 0.872 to 16.98g;5 of 23 cases(20.8%) were accompanied by kidney function damage.For clinical types,nephrotic syndrome was the main one(45.8%),and the second one was isolated proteinuria and hematuria(20.8%).Renal needle biopsies were performed in 19 cases.Class ⅣLN(57 9 %) was the most frequent pathological findings,and the second was Class Ⅴ(15.8%).In immunofluorescence detection,much aggradation of IgG and C3 was found;and "A full-house pattern" was found in 68.4% of biopsies.All patients accepted the therapy of adrenal cortical hormone and immunodepressive.Sixteen cases obtained clinical remission after average 3.4 months,and the remission rate was 66.7%.Six cases(25%) relapsed during follow-up survey,and two cases suffered from renal inadequacy(one was performed kidney transplantation).Conclusion The main clinical manifestation is nephrotic syndrome in the group,and the main pathologic type is Class Ⅳ.Lower morbidity and atypical clinical symptom induced misdiagnosis and missed diagnosis For this reason,the key of improving the prognosis of LN is early diagnosis,therapy,and regular follow-up survey.更多还原