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本文观察了14例临床或病理诊断的肝糖原累积症-Ⅰ型(GSD),年龄3月-19岁,5例(36%)伴有肾脏改变.其中1例,8岁,仅血β_2-M轻度升高,4例大于12岁.4例中2例表现为轻度蛋白尿,3例内生肌酐清除率降低,3例β_2-M轻度升高,1例伴继发性Fanconi综合征和肾性佝倭病表现.1例(14岁)进行了肾活检,光镜下示系膜基质轻度增生,肾小管胞浆内有糖原物质沉积.观察结果表明GSD-Ⅰ型病人的肾脏改变较为普遍,应密切追随肾脏情况,以期经过合理饮食治疗后能延缓本病晚期肾功能恶化.
In this paper, 14 cases of clinically or pathologically diagnosed hepatic glycogenosis-type I (GSD), aged from 3 months to 19 years old and 5 cases (36%) with renal changes were observed, including 1 case, 8 years old, -M was mildly elevated in 4 cases, and more than 12 cases in 4 cases, 2 cases showed mild proteinuria in 4 cases, 3 cases had decreased creatinine clearance, 3 cases had slight increase of β_2-M, 1 case had secondary Fanconi syndrome and renal disease caused by Toyo disease.One case (14 years old) underwent renal biopsy, under the light microscope showed a slight proliferation of mesangial matrix, tubular glycogen deposition within the cytoplasm.The results show that GSD-Ⅰ Type of kidney changes in patients is more common, should closely follow the kidney, in order to delay the delay of this disease after advanced renal dysfunction.