Visual impairment caused by retinal abnormalities in mesangiocapillary (membranoproliferative) glomerulonephritis type II ("dense deposit disease")
Visual impairment caused by retinal abnormalities in mesangiocapillary (membranoproliferative) glomerulonephritis type II ("dense deposit disease")
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DOI：
10.1016/s0272-6386(03)00665-6
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发表时间：
2003-08-01
影响因子：
13.2
通讯作者：
Savige, J
中科院分区：
文献类型：
--
作者：
Colville, D;Guymer, R;Savige, J
II型系膜毛细血管性肾小球肾炎（MCGN）患者通常在成年早期出现血尿、蛋白尿和肾损害，这些特征通常伴有部分脂肪营养不良和补体旁路途径转化酶（C3NeF）自身抗体。MCGN II型的诊断取决于肾小球基底膜（GBM）中“致密沉积物”的显示。大多数患者还具有多个视网膜下白色斑点或玻璃疣，其在组织病理学上与GBM沉积物相同，并且在肾衰竭发展时通过检眼镜检查明显。最初视力和视野得以保留，但荧光素血管造影和视网膜功能的专门测试，如暗适应，视网膜电图和眼电图，可能异常，并会逐渐恶化。在接下来的20年里，视力经常因为视网膜萎缩而恶化，有时是因为视网膜下新生血管膜、黄斑脱离和中心性浆液性视网膜病变。作者描述了一名患有MCGN II型的患者，他在59岁时出现肾功能衰竭和视力受损。他已经有广泛的视网膜萎缩，随后视网膜下膜发展。在MCGN II型中看到的玻璃疣，如部分脂肪营养不良，是诊断这种情况的有用的临床指标。所有II型MCGN患者都应被警告视网膜并发症的风险，并在就诊时和约10年后定期由眼科医生进行复查，以尽量减少视网膜病变并发症造成的视力损失。
Patients with mesangiocapillary glomerulonephritis (MCGN) type II usually present by early adulthood with hematuria, proteinuria, and renal impairment, and these features often are accompanied by a partial lipodystrophy and an autoantibody for the alternative complement pathway convertase (C3NeF). The diagnosis of MCGN type II depends on the demonstration of "dense deposits" in the glomerular basement membrane (GBM). Most patients also have multiple subretinal white spots or drusen that are histopathologically identical with the GBM deposits and evident ophthalmoscopically by the time renal failure develops. Initially visual acuity and visual fields are preserved, but fluorescein angiography and specialized tests of retinal function, such as dark adaptation, electroretinography, and electrooculography, may be abnormal and will worsen progressively. Over the next 20 years, vision often deteriorates because of retinal atrophy, and sometimes because of subretinal neovascular membranes, macular detachment, and central serous retinopathy. The authors describe a patient with MCGN type II who presented with renal failure and impaired vision at the age of 59. He already had widespread retinal atrophy, and subsequently a subretinal membrane developed. The drusen seen in MCGN type II, like the partial lipodystrophy, are a helpful clinical pointer to the diagnosis of this condition. All patients with MCGN type II should be warned of the risk of retinal complications and reviewed by an ophthalmologist at presentation and regularly after about 10 years to minimize the loss of visual acuity from complications of the retinopathy.