發(fā)布時間：2018-10-12 07:36

【摘要】：目的明確青少年特發(fā)性膜性腎病(IMN)的臨床病理特點以及腎臟預后。方法回顧性研究2012年6月至2014年12月腎活檢病理診斷IMN的青少年患者(定義發(fā)病年齡在13~24歲),排除膜性腎病繼發(fā)病因,收集相關的臨床、病理資料。隨機選擇同期年齡大于24歲的IMN患者33例作為對照組。結果青少年IMN組33人,腎活檢時年齡(20±3)歲,男/女比22/11;符合腎病綜合征23例,收縮壓(127±13)mm Hg(1 mm Hg=0.133 k Pa),舒張壓(77±9)mm Hg,24小時尿蛋白中位數(shù)5.14(3.39,9.31)g/d,血肌酐62(52,73)μmol/L,血清抗磷脂酶A2受體抗體陽性率54%。與對照組相比,高血壓發(fā)生率和基線估算腎小球濾過率差異有統(tǒng)計學意義[15.2%比39.3%,χ~2=4.889,P=0.03;125 ml/(min·1.73m~2)比100 ml/(min·1.73m~2),U=137.5,P0.001]。膜性腎病分期:Ⅰ期1例、Ⅱ期21例、Ⅲ期及以上11例;免疫熒光腎小球IgG亞型陽性率分別為:IgG1 46.9%、IgG2 3.1%、IgG3 56.3%、IgG4 87.5%。與對照組相比,青少年組的腎間質纖維化和腎小動脈病變發(fā)生率更低(6.1%比66.7%,χ~2=26.19,P0.001;15.2%比66.7%,χ~2=18.11,P0.001)。青少年組出院后失訪3例,其余30例初始方案選擇:小劑量潑尼松聯(lián)合環(huán)孢素方案20例、潑尼松聯(lián)合環(huán)磷酰胺7例、潑尼松+霉酚酸酯1例、潑尼松單藥2例;中位隨訪時間18(12,24)個月,隨訪終點24小時尿蛋白中位數(shù)0.20(0.10,0.42)g/d,血肌酐69(56.8,81.3)μmol/L,估算腎小球濾過率(117.3±19.2)ml/(min·1.73m~2)。完全緩解17例(56.7%),部分緩解13例(43.4%),完全緩解及部分緩解中位緩解時間分別為3個月和6個月,復發(fā)1例。30例隨訪患者中,完全緩解、部分緩解患者停用免疫抑制劑分別5、4例,其余21例維持治療:環(huán)孢素18例、硫唑嘌呤2例、霉酚酸酯1例。結論青少年IMN腎小球免疫熒光IgG亞型分布及血清抗磷脂酶A2受體抗體陽性率與對照組IMN相似;免疫抑制治療對青少年IMN有效,小劑量環(huán)孢素作為IMN緩解后的維持性治療有效且不增加腎毒性發(fā)生風險。
[Abstract]:Objective to investigate the clinicopathological features and renal prognosis of adolescent idiopathic membranous nephropathy (IMN). Methods from June 2012 to December 2014, the patients with IMN diagnosed by renal biopsy from June 2012 to December 2014 were studied retrospectively. The secondary etiology of membranous nephropathy was excluded and the clinical and pathological data were collected. Thirty-three IMN patients aged more than 24 years were randomly selected as control group. Results 33 adolescent IMN patients were aged (20 鹵3) years at the time of renal biopsy, male / female ratio 22 / 11, systolic blood pressure (127 鹵13) mm Hg (1 mm Hg=0.133 k Pa),) diastolic blood pressure (77 鹵9) mm Hg,24 hour urinary protein median 5.14 (3.39 鹵9.31) g / d, serum creatinine 62 (52773) 渭 mol/L, serum anti-phospholipase A2 receptor antibody positive rate 54. Compared with the control group, the incidence of hypertension and the baseline estimated glomerular filtration rate were significantly different [15.2% vs 39.3%, 蠂 ~ 2 4.889 ml/ (min 1.73m~2) vs 100 ml/ (min 1.73m~2), UG137.5% P0.001]. There were 1 stage 鈪，

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