Piqueras A I, White R H, Raafat F, Moghal N, Milford D V
Department of Nephrology, The Children's Hospital, Birmingham, UK.
Pediatr Nephrol. 1998 Jun;12(5):386-91. doi: 10.1007/s004670050471.
We reviewed the clinical and renal biopsy findings in 322 children presenting during the years 1975-1996 with recurrent macro- or continuous microscopic haematuria persisting for > or =6 months, in whom non-glomerular causes were excluded. Family involvement was documented for first-degree relatives. All biopsies were examined by light microscopy, 317 by electron microscopy and 315 by immunofluorescence. Biopsies were classified as IgA nephropathy (78), Alport nephropathy (86), thin basement membrane nephropathy (TMN) (50), miscellaneous glomerulonephritis (32), hilar vasculopathy (28) and normal glomeruli (48). Although microscopic haematuria alone was more frequent in Alport nephropathy and TMN, the pattern of haematuria in individual patients did not predict histology. Of patients with familial haematuria, 79% of biopsies showed either Alport nephropathy or TMN. Hilar vasculopathy was observed both in isolation and in all abnormal histological categories.
我们回顾了1975年至1996年间就诊的322例儿童的临床和肾活检结果,这些儿童反复出现肉眼血尿或持续镜下血尿,持续时间≥6个月,且排除了非肾小球性病因。记录了一级亲属的家族受累情况。所有活检标本均进行了光镜检查,317例进行了电镜检查,315例进行了免疫荧光检查。活检结果分为IgA肾病(78例)、Alport肾病(86例)、薄基底膜肾病(TMN)(50例)、其他肾小球肾炎(32例)、肾门血管病(28例)和正常肾小球(48例)。虽然单纯镜下血尿在Alport肾病和TMN中更为常见,但个体患者的血尿模式并不能预测组织学类型。在家族性血尿患者中,79%的活检显示为Alport肾病或TMN。肾门血管病既可见于孤立病例,也可见于所有异常组织学类型中。