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目的:分析有机溶剂相关性肾病综合征患者的流行病学、临床表现及肾组织病理特点。方法:回顾性分析2004年10月至2008年4月在解放军肾脏病研究所住院经临床及肾活检诊断为有机溶剂相关性肾病综合征18例患者临床资料,分析其接触有机溶剂的种类、临床表现、实验室检查、肾组织病理特点及初步随访结果。结果:(1)18例有机溶剂相关性肾病综合征患者多见于青年男性,其中男性15例,女性3例,平均年龄为(26.1±11.9)岁,所有患者均表现为中-重度水肿,大量混合性蛋白尿,平均尿蛋白(9.67±2.85)g/24h,其中4例患者入院时出现血压升高。(2)肾小管损伤标记物尿NAG酶及RBP均明显升高,其中尿NAG酶平均(67.5±40.4)u/g.cr。所有患者均无镜下血尿、糖尿及氨基酸尿。(3)18例患者血白蛋白均明显降低,平均(17.5±2.5)g/L,总胆固醇水平均明显升高,平均(11.5±2.87)mmol/L。9例患者入院时出现血肌酐升高,平均(130.8±73.7)μmol/L。16例患者血免疫球蛋白IgG显著降低,平均为(3.93±2.61)g/L。(4)所有患者肾活检病理示肾小球病变轻,表现为轻度节段系膜增生性病变或无系膜增生性病变,但均可见足细胞数目减少,13例患者出现节段壁层上皮细胞增生,16例患者出现肾小管返流。所有患者均存在近端肾小管上皮细胞刷状缘脱落,但无间质炎细胞浸润。(5)18例患者对免疫抑制剂治疗均无明显疗效,随访时间1～43月,2例(11.1%)患者死亡,7例(38.89%)患者出现肌酐倍增,其中3例(16.67%)患者进入ESRD期。结论:有机溶剂相关性肾病综合征是一种新发现的肾脏疾病,多见于中青年男性,临床表现为超大量蛋白尿伴近端小管损伤,表现为尿NAG酶,尿RBP升高,混合性蛋白尿,尿渗量正常,无糖尿及氨基酸尿;肾活检病理表现为足细胞数目减少,节段壁层上皮细胞增生,肾小管返流以及近端小管病变;该病对免疫抑制剂治疗反应不佳,预后较差。
OBJECTIVE: To analyze the epidemiology, clinical manifestations and renal pathological features of patients with organic solvent-related nephrotic syndrome. Methods: The clinical data of 18 patients diagnosed as organic solvent-related nephrotic syndrome who were diagnosed as organic solvent-related nephrotic syndrome by inpatient and renal biopsy in the PLA Institute of Nephrology were retrospectively analyzed from October 2004 to April 2008. The types of organosolv exposure, Performance, laboratory tests, pathological features of kidney and initial follow-up results. Results: (1) 18 cases of organic solvent-related nephrotic syndrome more common in young men, including 15 males and 3 females, the average age was (26.1 ± 11.9) years old, all patients showed moderate to severe edema, a large number Mixed proteinuria, the average urinary protein (9.67 ± 2.85) g / 24h, of which 4 patients admitted to hospital when the blood pressure. (2) Urine NAG and RBP were significantly increased in tubular injury markers, urinary NAG enzyme was (67.5 ± 40.4) u / g.cr. All patients had no microscopic hematuria, diabetes and amino aciduria. (3) The serum albumin of 18 patients were significantly lower (mean, 17.5 ± 2.5) g / L and total cholesterol (11.5 ± 2.87) mmol / L, respectively. Nine patients had elevated serum creatinine on admission, with an average of (130.8 ± 73.7) μmol / L. 16 patients with serum immunoglobulin IgG decreased significantly, with an average of (3.93 ± 2.61) g / L. (4) The pathological changes of renal biopsy showed mild glomerular lesions in all patients, showing mild segmental mesangial proliferative lesions or non-mesangial proliferative lesions. However, the number of podocytes was reduced in all patients, and the segmental parietal layers Proliferation of epithelial cells, tubular reflux in 16 patients. All patients had proximal tubular epithelial cell brush border shedding, but no interstitial inflammatory cell infiltration. (5) 18 patients were treated with immunosuppressive agents without any effect. The follow-up time ranged from 1 to 43 months. Two patients (11.1%) died and seven patients (38.89%) had creatinine doubling. Three patients (16.67% Patients enter the ESRD phase. CONCLUSION: Organic solvent-associated nephrotic syndrome is a newly discovered renal disease and is more common in young and middle-aged men. The clinical manifestations include massive proteinuria with proximal tubule injury, manifested as urinary NAG enzyme, increased urine RBP, mixed Proteinuria, normal urinary osmolality, no diabetes and amino aciduria; renal biopsy showed decreased number of podocytes, segmental parietal epithelial cell proliferation, tubular regurgitation and proximal tubule lesions; the disease response to immunosuppressive agents Poor, poor prognosis.