Russell S R, Mullins R F, Schneider B L, Hageman G S
University of Iowa Center for Macular Degeneration, Department of Ophthalmology and Visual Sciences, The University of Iowa, Iowa City 52242-1091, USA.
Am J Ophthalmol. 2000 Feb;129(2):205-14. doi: 10.1016/s0002-9394(99)00345-1.
To determine whether basal laminar drusen differ in their location, ultrastructure, or composition from drusen associated with aging and age-related macular degeneration.
A paraffin-embedded block from an eye of a patient with basal laminar drusen was obtained. Sections were examined immunohistochemically using a battery of antibodies and lectins directed against drusen-associated proteins and glycoconjugates, respectively. Thin sections were examined by electron microscopy and compared with eyes with age-related macular degeneration.
Drusen in the eye with basal laminar drusen are located between the basal lamina of the retinal pigment epithelium and the inner collagenous layer of Bruch membrane, just as they are in age-related macular degeneration. Two distinct ultrastructural phenotypes are observed in the eye with basal laminar drusen; their substructure is indistinguishable from drusen phenotypes in age-related macular degeneration. Both basal laminar drusen and drusen associated with age-related macular degeneration are bound by the lectins Ricinis communis agglutinin and Arachis hypogea agglutinin (after neuraminidase digestion) and by antivitronectin, anti-HLA-DR, anti-serum amyloid P, and anti-C5 antibodies, but not by antibodies directed against basement membrane-associated heparan sulfate proteoglycan, laminin, fibrinogen, or collagen type IV.
These data support the notion that cuticular or basal laminar drusen are similar to, and perhaps indistinguishable from, drusen associated with age-related macular degeneration and are not nodular or diffuse thickenings of Bruch membrane, as previously suggested. Thus, we suggest basal laminar drusen is a misnomer. This clinical phenotype should be identified as "early adult onset, grouped drusen" or by the eponym "Gass syndrome." Features of basal laminar drusen, such as uniform drusen size, clustered distribution, and angiographic features, do not appear to be related to differences in drusen location, composition, or substructure.
确定基底膜性玻璃膜疣在位置、超微结构或组成上是否与衰老及年龄相关性黄斑变性相关的玻璃膜疣不同。
获取一名患有基底膜性玻璃膜疣患者眼部的石蜡包埋组织块。切片分别使用一系列针对玻璃膜疣相关蛋白和糖缀合物的抗体及凝集素进行免疫组织化学检查。薄切片通过电子显微镜检查,并与年龄相关性黄斑变性患者的眼睛进行比较。
患有基底膜性玻璃膜疣的眼睛中的玻璃膜疣位于视网膜色素上皮的基底膜与布鲁赫膜的内胶原层之间,与年龄相关性黄斑变性中的情况相同。在患有基底膜性玻璃膜疣的眼睛中观察到两种不同的超微结构表型;其亚结构与年龄相关性黄斑变性中的玻璃膜疣表型无法区分。基底膜性玻璃膜疣和年龄相关性黄斑变性相关的玻璃膜疣均与蓖麻凝集素和花生凝集素(经神经氨酸酶消化后)以及抗玻连蛋白、抗 HLA-DR、抗血清淀粉样蛋白 P 和抗 C5 抗体结合,但不与针对基底膜相关硫酸乙酰肝素蛋白聚糖、层粘连蛋白、纤维蛋白原或 IV 型胶原的抗体结合。
这些数据支持这样一种观点,即表皮或基底膜性玻璃膜疣与年龄相关性黄斑变性相关的玻璃膜疣相似,甚至可能无法区分,且并非如先前所述是布鲁赫膜的结节状或弥漫性增厚。因此,我们认为基底膜性玻璃膜疣这一名称并不恰当。这种临床表型应被识别为“早发性成人型、成簇玻璃膜疣”或采用其专用名称“加斯综合征”。基底膜性玻璃膜疣的特征,如玻璃膜疣大小均匀、成簇分布及血管造影特征,似乎与玻璃膜疣的位置、组成或亚结构差异无关。