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[家族性胶滴状角膜营养不良]

[Familial form of gelatin drop corneal dystrophy].

作者信息

el Matri L, Bachtobji A, Ghorbal M, Maamri J, Kamoun M, Ouertani A, Bardi R, Triki M F

机构信息

Institut d'Ophtalmologie, Tunis, Tunisie.

出版信息

J Fr Ophtalmol. 1991;14(2):125-9.

PMID:1880340
Abstract

We report 4 cases of gelatin drop dystrophy corneal amyloidosis in two brothers and two sisters of the same family. The age of onset is between 1 and 10 years. The corneal signs are described at different stages of development. Lesions are initially asymmetrical. The typical is a subepithelial nodule starting in the center and then expanding to involve the whole cornea. There is no relationship between extension and outcome of the disease. Diagnostic is confirmed by histological examination. There was no consanguinity between parents and no other cases were found in the family. An immunological study with HLA typing was performed. All patients were treated with lamellar or transfixing keratoplasty with a follow-up of 2 to 5 years.

摘要

我们报告了同一家庭中两兄弟和两姐妹患明胶滴状角膜营养不良性角膜淀粉样变性的4例病例。发病年龄在1至10岁之间。描述了角膜体征在不同发育阶段的情况。病变最初是不对称的。典型表现为始于角膜中央的上皮下结节,随后扩展至累及整个角膜。病变范围与疾病转归之间无关联。通过组织学检查确诊。父母无血缘关系,且家族中未发现其他病例。进行了 HLA 分型的免疫学研究。所有患者均接受了板层或穿透性角膜移植术治疗,随访时间为2至5年。

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