Clinicopathologic Findings in Three Siblings With Geographic Atrophy.
Clinicopathologic Findings in Three Siblings With Geographic Atrophy.
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DOI：
10.1167/iovs.64.3.2
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发表时间：
2023-03-01
影响因子：
4.4
通讯作者：
中科院分区：
文献类型：
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作者：
年龄相关性黄斑变性（AMD）是全球老年人失明的主要原因。临床影像学和组织病理学研究是了解疾病病理的关键。本研究结合3例地域性萎缩（GA）兄弟的临床观察，随访20年，并进行组织病理学分析。对于三兄弟中的两个人，临床图像是在2016年拍摄的，即死亡前两年。利用免疫组织化学、平片和横断面、组织学和透射电镜对GA眼的脉络膜和视网膜与年龄匹配的对照组进行比较。脉络膜凝集素（UEA）染色显示血管面积和血管直径百分比明显减少。在一个供体中，组织病理学分析显示两个不同的区域有脉络膜新生血管（CNV）。重新评估扫描源光学相干断层扫描血管造影（SS-OCTA）图像显示两兄弟CNV。UEA凝集素也显示萎缩区视网膜血管的显著减少。视网膜下胶质膜，由胶质纤维酸性蛋白和/或静脉蛋白阳性的过程组成，占据与视网膜色素上皮（RPE）相同的区域，在所有三个AMD供体中脉络膜萎缩。SS-OCTA在2016年成像的两名供体中也显示了假定的钙化性肾盂。免疫组化分析和茜素红S染色证实，脑组织内钙被胶质细胞包裹。本研究证明了临床组织病理学相关性研究的重要性。它强调需要更好地了解绒毛膜毛细血管和RPE、胶质反应和钙化结节之间的共生关系如何影响GA的进展。
Age-related macular degeneration (AMD) is a leading cause of blindness among the elderly worldwide. Clinical imaging and histopathologic studies are crucial to understanding disease pathology. This study combined clinical observations of three brothers with geographic atrophy (GA), followed for 20 years, with histopathologic analysis. For two of the three brothers, clinical images were taken in 2016, 2 years prior to death. Immunohistochemistry, on both flat-mounts and cross sections, histology, and transmission electron microscopy were used to compare the choroid and retina in GA eyes to those of age-matched controls. Ulex europaeus agglutinin (UEA) lectin staining of the choroid demonstrated a significant reduction in the percent vascular area and vessel diameter. In one donor, histopathologic analysis demonstrated two separate areas with choroidal neovascularization (CNV). Reevaluation of swept-source optical coherence tomography angiography (SS-OCTA) images revealed CNV in two of the brothers. UEA lectin also revealed a significant reduction in retinal vasculature in the atrophic area. A subretinal glial membrane, composed of processes positive for glial fibrillary acidic protein and/or vimentin, occupied areas identical to those of retinal pigment epithelium (RPE) and choroidal atrophy in all three AMD donors. SS-OCTA also demonstrated presumed calcific drusen in the two donors imaged in 2016. Immunohistochemical analysis and alizarin red S staining verified calcium within drusen, which was ensheathed by glial processes. This study demonstrates the importance of clinicohistopathologic correlation studies. It emphasizes the need to better understand how the symbiotic relationship between choriocapillaris and RPE, glial response, and calcified drusen impact GA progression.
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DOI：
10.1097/iae.0000000000002182
发表时间：
2018-10
期刊：
Retina (Philadelphia, Pa.)
影响因子：
--
作者：
Li M;Huisingh C;Messinger J;Dolz-Marco R;Ferrara D;Freund KB;Curcio CA
通讯作者：
Curcio CA
影响因子：
4.4
作者：
Curcio CA;Zanzottera EC;Ach T;Balaratnasingam C;Freund KB
通讯作者：
Freund KB
影响因子：
13.7
作者：
Li, Miaoling;Dolz-Marco, Rosa;Freund, K. Bailey
通讯作者：
Freund, K. Bailey
影响因子：
4.4
作者：
McLeod DS;Grebe R;Bhutto I;Merges C;Baba T;Lutty GA
通讯作者：
Lutty GA
影响因子：
4.4
作者：
Edwards MM;McLeod DS;Bhutto IA;Grebe R;Duffy M;Lutty GA
通讯作者：
Lutty GA