O'Brien C, Duvall-Young J, Brown M, Short C, Bone M
Royal Liverpool University Hospital.
Br J Ophthalmol. 1993 Dec;77(12):778-80. doi: 10.1136/bjo.77.12.778.
The retinal electrophysiology is reported in four patients with type II mesangiocapillary glomerulonephritis and partial lipodystrophy with associated fundus abnormalities and no visual symptoms. The histological hallmark of the condition is that of widespread electron dense deposits in the renal glomerulus and in the choriocapillaris and Bruch's membrane of the eye. Three of the four patients had the typical fundal appearance of multiple, yellow, drusen-like lesions at the posterior pole of the eye with normal visual acuity. These three patients had abnormally low Arden ratios on electro-oculography with normal electroretinography responses. This is the first clinical model of disease known to be isolated to the choriocapillaris and Bruch's membrane causing an electro-oculographic abnormality without any clinically detectable deficit in visual function.
报告了4例II型系膜毛细血管性肾小球肾炎合并部分脂肪营养不良患者的视网膜电生理情况,这些患者伴有眼底异常但无视觉症状。该疾病的组织学特征是在肾小球以及眼脉络膜毛细血管和布鲁赫膜中广泛存在电子致密沉积物。4例患者中有3例在眼后极有典型的眼底表现,即多个黄色、类似玻璃膜疣的病变,视力正常。这3例患者眼电图的阿登比率异常低,而视网膜电图反应正常。这是已知的首个仅累及脉络膜毛细血管和布鲁赫膜的疾病临床模型,可导致眼电图异常,而视觉功能无任何临床可检测到的缺陷。