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首页> 外文期刊>Saudi Journal of OphthalmologybElectronic resource >Amyloid corneal deposition in corneal buttons of congenital hereditary endothelial dystrophy (CHED) – A clinical and histopathological case series
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Amyloid corneal deposition in corneal buttons of congenital hereditary endothelial dystrophy (CHED) – A clinical and histopathological case series

机译：先天性遗传性内皮营养不良（CHED）角膜纽扣中的淀粉样角膜沉积-临床和组织病理学病例系列

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Purpose To determine the frequency, pathology and clinical relevance of amyloid deposited in corneas of CHED. Methods Clinical and histopathological case series. Results Amyloid subepithelial deposition was found in 5 (6.6%) corneal buttons of 75 patients with histopathologically confirmed CHED diagnosis. Clinical findings included history of parental consanguinity, poor vision (ranging from counting fingers from one foot to 3/200), corneal edema, and central whitish subepithelial corneal nodules in all the five cases and positive family history in 4 of 5 cases. The patients underwent PKP at a mean age of 15years (range 3–22years). Histological findings included attenuated endothelium (6/6) thickened Descemet’s membrane (6/6), stromal edema (2/6), and subepithelial amyloid deposits (6/6). All patients improved from vision point of view. To date, no recurrence of the amyloid has been seen in the grafts. Conclusion Considering the consanguinity, family history, early onset, and bilaterality, this study supports our hypothesis that the amyloid is primary in nature in our patients and indicates a new subtype of autosomal recessive CHED that require further chemical and genetic analysis. This subtype has the same prognosis for PKP as all CHED patients, if not better.
机译：目的确定沉积在CHED角膜中的淀粉样蛋白的频率，病理学和临床意义。方法临床和组织病理学病例系列。结果75例经病理组织学确认为CHED的患者中，有5例（6.6％）角膜纽带发现淀粉样上皮下沉积。临床发现包括所有五个病例的父母血缘病史，视力差（手指数从一只脚到3/200不等），角膜水肿和中央白色上皮下角膜结节以及5例中有4例阳性家族史。患者平均年龄为15岁（3至22岁）。组织学发现包括内皮细胞减薄（6/6），Descemet膜增厚（6/6），基质水肿（2/6）和上皮下淀粉样沉积（6/6）。从视力的角度来看，所有患者均得到改善。迄今为止，在移植物中未见淀粉样蛋白复发。结论考虑到血缘关系，家族病史，早期发作和双侧性，本研究支持我们的假设，即淀粉样蛋白在患者中是原发性的，并表明常染色体隐性遗传性CHED的新亚型需要进一步的化学和遗传分析。如果不是更好的话，这种亚型与所有CHED患者的PKP预后相同。

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