本文選題：兒童 + 原發(fā)性��； 參考：《吉林大學(xué)》2017年碩士論文

【摘要】：目的:探討兒童原發(fā)性局灶節(jié)段性腎小球硬化(FSGS)的臨床、病理關(guān)系。方法:回顧性分析31例2007-2017年在吉林大學(xué)第一醫(yī)院住院診斷為FSGS患兒的一般資料、臨床資料、病理資料及治療,并進(jìn)行隨訪。結(jié)果:原發(fā)性FSGS占同期腎活檢5.9%,平均起病年齡7.74±4.73歲,平均診斷年齡9.58±4.36歲;其中男19例,女12例,男女比例1.58:1。21例(68%)臨床表現(xiàn)為腎病綜合征,其中12例(39%)腎炎型腎病,9例(29%)單純型腎病;4例(12%)表現(xiàn)為孤立性蛋白尿,3例(10%)表現(xiàn)為腎小球腎炎,3例(10%)表現(xiàn)為孤立性血尿。組織病理分型中,非特殊型16例(52%),頂端型10例(32%),塌陷型3例(10%),細(xì)胞型2例(6%),無門部型。28例于我院治療,其中20例臨床表現(xiàn)腎病綜合征均給予激素治療,其中激素敏感6例,激素耐藥10例,激素依賴4例;14例聯(lián)合免疫抑制劑治療,6例使用環(huán)磷酰胺,5例使用他克莫司,1例環(huán)磷酰胺聯(lián)合他克莫司,1例他克莫司聯(lián)合霉酚酸酯,1例環(huán)磷酰胺、他克莫司及霉酚酸酯。隨訪時(shí)間2個(gè)月-120個(gè)月,完全緩解率80%,部分緩解率10%,未緩解率10%。結(jié)論:1.兒童原發(fā)性FSGS最常見的臨床表現(xiàn)為腎病綜合征。2.兒童原發(fā)性FSGS的最常見的病理類型為非特殊型。3.激素聯(lián)合免疫抑制劑可提高兒童原發(fā)性FSGS的緩解率。
[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 鹵4.73 years and an average diagnostic age of 9.58 鹵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.
【學(xué)位授予單位】：吉林大學(xué)
【學(xué)位級(jí)別】：碩士
【學(xué)位授予年份】：2017
【分類號(hào)】：R726.9

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