Abe S, Amagasaki Y, Iyori S, Konishi K, Kato E, Sakaguchi H, Shimoyama K
J Clin Pathol. 1987 Mar;40(3):318-22. doi: 10.1136/jcp.40.3.318.
Eight (two men, six women) cases of adult thin basement membrane syndrome were studied to clarify the clinicopathological characteristics of the disease. The average age at the time of biopsy was 40 years. All the patients had persistent microscopic haematuria, normal renal function, and normal blood pressure, with the exception of one who was hypotensive. Most of them had persistent or transient proteinuria. Renal symptoms were found in four families, although no relative had Alport's syndrome. Renal biopsy findings observed by light and immunofluorescence microscopy did not indicate any important abnormalities, but extensive diffuse thinning of the glomerular basement membrane, ranging from 153 to 213 nm, was a constant finding by electron microscopy. All the patients retained stable renal function at the time of final follow up, indicating a benign prognosis of the syndrome.
对8例（2名男性，6名女性）成人薄基底膜综合征患者进行了研究，以明确该疾病的临床病理特征。活检时的平均年龄为40岁。所有患者均有持续性镜下血尿、肾功能正常、血压正常，但有1例为低血压。他们中的大多数人有持续性或短暂性蛋白尿。在4个家族中发现了肾脏症状，尽管没有亲属患有Alport综合征。光镜和免疫荧光显微镜观察的肾活检结果未显示任何重要异常，但电子显微镜下的一个恒定发现是肾小球基底膜广泛弥漫性变薄，范围为153至213纳米。在最后随访时，所有患者的肾功能均保持稳定，表明该综合征预后良好。
