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进行性原发性虹膜萎缩、钱德勒综合征和虹膜痣(科根-里斯)综合征:一组疾病谱。

Progressive essential iris atrophy, Chandler's syndrome, and the iris nevus (Cogan-Reese) syndrome: a spectrum of disease.

作者信息

Shields M B

出版信息

Surv Ophthalmol. 1979 Jul-Aug;24(1):3-20. doi: 10.1016/0039-6257(79)90143-7.

DOI:10.1016/0039-6257(79)90143-7
PMID:483159
Abstract

Progressive essential iris atrophy, Chandler's syndrome, and the iris nevus (Cogan-Reese) syndrome are considered to be variations of a single disease process, which is characterized by abnormalities of the cornea, anterior chamber angle, and iris. In each variation, the typical patient is a white woman with unilateral disease, negative family history, and an onset of symptoms in early to middle adulthood. Since the membrane theory of Campbell suggests that the disease is a fundamental abnormality of the corneal endothelium, rather than the iris, the term "iridocorneal endothelial syndrome," as proposed by Yanoff, may be an appropriate inclusive term for the spectrum of disease, although further study of the pathogenesis is needed. For each variation of the disease, corneal edema and secondary glucoma are both treated primarily by medical or surgical reduction of the intraocular pressure, although penetrating keratoplasty is occasionally required for cases with advanced corneal edema.

摘要

进行性原发性虹膜萎缩、钱德勒综合征和虹膜痣(科根 - 里斯)综合征被认为是单一疾病过程的不同变体,其特征为角膜、前房角和虹膜异常。在每种变体中,典型患者为白人女性,单侧患病,家族史阴性,症状始于成年早期至中期。由于坎贝尔的膜理论表明该疾病是角膜内皮的基本异常,而非虹膜异常,因此矢野提出的“虹膜角膜内皮综合征”这一术语可能是该疾病谱的一个合适的统称术语,不过仍需对发病机制进行进一步研究。对于该疾病的每种变体,角膜水肿和继发性青光眼主要通过药物或手术降低眼压来治疗,尽管对于角膜水肿晚期的病例偶尔需要进行穿透性角膜移植术。

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Progressive essential iris atrophy, Chandler's syndrome, and the iris nevus (Cogan-Reese) syndrome: a spectrum of disease.进行性原发性虹膜萎缩、钱德勒综合征和虹膜痣(科根-里斯)综合征:一组疾病谱。
Surv Ophthalmol. 1979 Jul-Aug;24(1):3-20. doi: 10.1016/0039-6257(79)90143-7.
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Pathogenesis of Chandler's syndrome, essential iris atrophy and the Cogan-Reese syndrome. I. Alterations of the corneal endothelium.钱德勒综合征、原发性虹膜萎缩和科根-里斯综合征的发病机制。I. 角膜内皮的改变。
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