Bruch's Membrane Calcification in Pseudoxanthoma Elasticum: Comparing Histopathology and Clinical Imaging.
Bruch's Membrane Calcification in Pseudoxanthoma Elasticum: Comparing Histopathology and Clinical Imaging.
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DOI：
10.1016/j.xops.2023.100416
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发表时间：
2024-03
影响因子：
--
通讯作者：
Lengyel, Imre
中科院分区：
文献类型：
--
作者：
Risseeuw, Sara;Pilgrim, Matthew G.;Bertazzo, Sergio;Brown, Connor N.;Csincsik, Lajos;Fearn, Sarah;Thompson, Richard B.;Bergen, Arthur A.;ten Brink, Jacoline B.;Kortvely, Elod;Spiering, Wilko;Ossewaarde-van Norel, Jeannette;van Leeuwen, Redmer;Lengyel, Imre
研究弹力假黄瘤 (PXE) 布鲁赫膜 (BM) 钙化的组织学，并将其与临床视网膜成像相关联。具有临床病理学相关性的实验研究。来自 4 名 PXE 患者的 6 只死后眼睛和来自未使用 PXE 的匿名捐赠者的 1 只对比眼。其中一只眼睛具有用于比较的多模态临床图像集。钙化物用 OsteSense 680RD（一种羟基磷灰石特异性荧光染料）进行标记，并通过共聚焦显微镜进行可视化。扫描电子显微镜结合能量色散 X 射线光谱 (SEM-EDX) 和飞行时间二次离子质谱 (TOF-SIM) 来分析不同解剖位置的元素和离子组成。将尸体组织的结果与 1 名 PXE 患者的临床影像进行比较。将 PXE 眼 BM 中羟基磷灰石的特征和拓扑分布与疾病的临床表现进行比较。对整体和切片 PXE 眼睛的分析显示，在中央和中外周 BM 中存在广泛、汇合的 OsteoSense 标记，并转变为中外周中的斑点标记。这些区域对应于临床成像上的高反射区和等反射区。扫描电子显微镜结合能量色散 X 射线光谱和 TOF-SIM 分析，将 PXE 眼的 BM 中的这些钙化物识别为羟基磷灰石。融合的荧光外观源自胶原蛋白和 BM 弹性层的严重钙化纤维结构。在老年对照眼中也检测到钙化，但这与 PXE 眼明显不同，并在后极呈现小雪花状沉积物。弹性假黄瘤眼在黄斑的内外胶原层和弹性BM层中显示出广泛的羟基磷灰石沉积，并逐渐向中周边变化，这似乎与临床表型相关。老年对照眼 BM 中的雪花状钙化与 PXE 中的广泛钙化明显不同。专有或商业披露可在本文末尾的脚注和披露中找到。
To investigate the histology of Bruch’s membrane (BM) calcification in pseudoxanthoma elasticum (PXE) and correlate this to clinical retinal imaging. Experimental study with clinicopathological correlation. Six postmortem eyes from 4 PXE patients and 1 comparison eye from an anonymous donor without PXE. One of the eyes had a multimodal clinical image set for comparison. Calcification was labeled with OsteSense 680RD, a fluorescent dye specific for hydroxyapatite, and visualized with confocal microscopy. Scanning electron microscopy coupled with energy-dispersive x-ray spectroscopy (SEM-EDX) and time-of-flight secondary ion mass spectrometry (TOF-SIMs) were used to analyze the elemental and ionic composition of different anatomical locations. Findings on cadaver tissues were compared with clinical imaging of 1 PXE patient. The characteristics and topographical distribution of hydroxyapatite in BM in eyes with PXE were compared with the clinical manifestations of the disease. Analyses of whole-mount and sectioned PXE eyes revealed an extensive, confluent OsteoSense labeling in the central and midperipheral BM, transitioning to a speckled labeling in the midperiphery. These areas corresponded to hyperreflective and isoreflective zones on clinical imaging. Scanning electron microscopy coupled with energy-dispersive x-ray spectroscopy and TOF-SIMs analyses identified these calcifications as hydroxyapatite in BM of PXE eyes. The confluent fluorescent appearance originates from heavily calcified fibrous structures of both the collagen and the elastic layers of BM. Calcification was also detected in an aged comparison eye, but this was markedly different from PXE eyes and presented as small snowflake-like deposits in the posterior pole. Pseudoxanthoma elasticum eyes show extensive hydroxyapatite deposition in the inner and outer collagenous and elastic BM layers in the macula with a gradual change toward the midperiphery, which seems to correlate with the clinical phenotype. The snowflake-like calcification in BM of an aged comparison eye differed markedly from the extensive calcification in PXE. Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.
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影响因子：
4.4
作者：
Chang, AA;Zhu, MD;Billson, F
通讯作者：
Billson, F
吲哚菁绿晚相血管造影上年龄相关的散在低荧光斑点：多模态成像及相关因素
影响因子：
4
作者：
Chen L;Zhang X;Liu B;Mi L;Wen F
通讯作者：
Wen F
影响因子：
6
作者：
Chong, NHV;Keonin, J;Hageman, GS
通讯作者：
Hageman, GS
DOI：
10.1136/bjophthalmol-2011-300344
发表时间：
2011-12
期刊：
The British journal of ophthalmology
影响因子：
--
作者：
Curcio CA;Johnson M;Rudolf M;Huang JD
通讯作者：
Huang JD
影响因子：
4.4
作者：
通讯作者：
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