31例儿童原发性局灶节段性肾小球硬化临床、病理分析

发布时间：2018-05-29 08:24

  本文选题：儿童 + 原发性　； 参考：《吉林大学》2017年硕士论文

【摘要】：目的:探讨儿童原发性局灶节段性肾小球硬化(FSGS)的临床、病理关系。方法:回顾性分析31例2007-2017年在吉林大学第一医院住院诊断为FSGS患儿的一般资料、临床资料、病理资料及治疗,并进行随访。结果:原发性FSGS占同期肾活检5.9%,平均起病年龄7.74±4.73岁,平均诊断年龄9.58±4.36岁;其中男19例,女12例,男女比例1.58:1。21例(68%)临床表现为肾病综合征,其中12例(39%)肾炎型肾病,9例(29%)单纯型肾病;4例(12%)表现为孤立性蛋白尿,3例(10%)表现为肾小球肾炎,3例(10%)表现为孤立性血尿。组织病理分型中,非特殊型16例(52%),顶端型10例(32%),塌陷型3例(10%),细胞型2例(6%),无门部型。28例于我院治疗,其中20例临床表现肾病综合征均给予激素治疗,其中激素敏感6例,激素耐药10例,激素依赖4例;14例联合免疫抑制剂治疗,6例使用环磷酰胺,5例使用他克莫司,1例环磷酰胺联合他克莫司,1例他克莫司联合霉酚酸酯,1例环磷酰胺、他克莫司及霉酚酸酯。随访时间2个月-120个月,完全缓解率80%,部分缓解率10%,未缓解率10%。结论:1.儿童原发性FSGS最常见的临床表现为肾病综合征。2.儿童原发性FSGS的最常见的病理类型为非特殊型。3.激素联合免疫抑制剂可提高儿童原发性FSGS的缓解率。
[Abstract]:Objective: to investigate the clinical and pathological relationship of primary focal segmental glomerulosclerosis (FSGS) in children. Methods: the general data, clinical data, pathological data and treatment of 31 patients with FSGS diagnosed in the first Hospital of Jilin University from 2007 to 2017 were retrospectively analyzed and followed up. Results: primary FSGS accounted for 5.9% of renal biopsy in the same period, with an average onset age of 7.74 卤4.73 years and an average diagnostic age of 9.58 卤4.36 years. There were 12 cases of nephritis nephropathy (9 cases) simple nephropathy (n = 12) solitary proteinuria (n = 3) glomerulonephritis (n = 3) and glomerulonephritis (n = 10) and isolated hematuria (n = 12), isolated proteinuria (n = 3) and glomerulonephritis (n = 10). Among the histopathological types, 16 cases were non-special type, 10 cases were apical type, 3 cases were collapse type, 3 cases were cave-in type, 2 cases were cellular type, and 28 cases were without portal type. Among them, 20 cases of nephrotic syndrome were treated with hormone, among which 6 cases were hormone sensitive. 10 cases of steroid resistance, 4 cases of hormone dependence and 14 cases of hormone dependence combined with immunosuppressive therapy. 6 cases were treated with cyclophosphamide, 5 cases were treated with tacrolimus and 1 case with tacrolimus, 1 case with tacrolimus combined with mycophenolate mofetil, 1 case with cyclophosphamide. Tacrolimus and mycophenolate mofetil. Follow up for 2 months-120 months, complete remission rate 80, partial remission rate 10, non-remission rate 10. Conclusion 1. The most common clinical manifestation of primary FSGS in children is nephrotic syndrome. The most common pathological type of primary FSGS in children was non-special type. 3. Hormone combined with immunosuppressive agents can improve the remission rate of primary FSGS in children.
【学位授予单位】：吉林大学
【学位级别】：硕士
【学位授予年份】：2017
【分类号】：R726.9

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