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哈博扬综合征

Harboyan Syndrome.

作者信息

Javaid Ayesha, Orakzai Abdullah Ahmad

机构信息

Department of Ophthalmology, Khyber Teaching Hospital, Peshawar, Pakistan.

出版信息

J Ayub Med Coll Abbottabad. 2020 Oct-Dec;32(Suppl 1)(4):S701-S703.

Abstract

Harboyan syndrome is a degenerative corneal disorder characterized by congenital hereditary endothelial dystrophy along with progressive, post lingual sensorineural hearing loss. We present the case of a 16-year-old female, who came to us for her follow up after surgical correction for bilateral corneal opacities (bilateral keratplasy) and use of hearing aid for SNHL. Her symptoms resolved significantly. Currently, she's living a healthy life after being treated for Harboyan syndrome. Physical examination and laboratory investigations ruled out other causes (eg: Congenital cataracts, Peters anomaly, Sclerocornea) and the diagnosis of Harboyan syndrome was established.

摘要

哈博扬综合征是一种退行性角膜疾病,其特征为先天性遗传性内皮营养不良以及进行性的舌后感觉神经性听力丧失。我们报告一例16岁女性病例,该患者因双侧角膜混浊接受手术矫正(双侧角膜移植术)并使用助听器治疗感音神经性听力损失后前来我们处进行随访。她的症状明显缓解。目前,她在接受哈博扬综合征治疗后过着健康的生活。体格检查和实验室检查排除了其他病因(如:先天性白内障、彼得斯异常、巩膜角膜),确诊为哈博扬综合征。

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