Corneal Dystrophies, Hereditary

角膜营养不良,遗传性
  • 文章类型: Practice Guideline
    自体血清滴眼液提供润滑并促进上皮愈合。它们已成功用于治疗眼表疾病,如干眼病,几十年来持续的上皮缺损和神经营养性角膜病变。制备自体血清滴眼液的方法有很大差异,最终浓度和使用持续时间存在于已发表的文献中。在这次审查中,简化的准备建议,运输,描述了自体血清的储存和使用。总结了在房水缺乏的干眼症中使用这种方式的证据,以及基于专业知识的基本原理。
    Autologous serum eye drops provide lubrication and promote epithelial healing. They have been successfully used in the management of ocular surface disorders such as dry eye disease, persistent epithelial defects and neurotrophic keratopathy for many decades. A great deal of variation in the methods of preparation of autologous serum eye drops, the end concentration and the duration of use exists in published literature. In this review, simplified recommendations for preparation, transport, storage and use of autologous serum are described. Evidence for the use of this modality in aqueous deficient dry eye disease is summarized, along with expertise-based rationale.
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  • 文章类型: Journal Article
    背景:神经营养性角膜病变(NK)是一种相对罕见的,由诸如单纯疱疹或带状疱疹性角膜炎对三叉神经的眼科分支的损害引起的未被诊断的退行性角膜疾病,颅内占位性病变,糖尿病,或神经外科手术。随着时间的推移,上皮破裂,角膜溃疡,角膜融化(变薄),穿孔,可能会出现视力丧失。逆转眼表毁伤的最好机会是在NK最早阶段。然而,患者通常很少出现症状,并且诊断通常会延迟。提高对NK原因的认识,关于何时以及如何筛查NK的共识,以及如何治疗NK的建议是必要的。
    方法:一个由11名成员组成的专家小组使用一种经过验证的方法(RAND/UCLA修改的Delphi小组)就何时筛选以及如何最好地诊断和治疗NK达成共识。临床医生回顾了有关NK诊断和管理的文献,然后对735种情况进行了详细的评估。在646种情况下,小组成员评估了是否需要角膜敏感性测试;在20种情况下,他们认为特定的测试和检查足以诊断和分期NK;在69种情况下,他们评估了NK治疗的适当性。小组成员评级用于制定临床建议。
    结果:对94%的方案达成了一致。基于这一共识,当我们强烈建议或可能考虑进行角膜敏感性测试时,我们会出现不同的情况。我们还提出了对怀疑有NK的患者进行诊断测试的建议以及NK的治疗选择。
    结论:这些专家建议应通过临床数据进行验证。这些建议代表了专家的共识,根据已发表的文献和经验,并可能通过帮助改善NK患者的诊断和治疗来改善预后。
    BACKGROUND: Neurotrophic keratopathy (NK) is a relatively uncommon, underdiagnosed degenerative corneal disease that is caused by damage to the ophthalmic branch of the trigeminal nerve by conditions such as herpes simplex or zoster keratitis, intracranial space-occupying lesions, diabetes, or neurosurgical procedures. Over time, epithelial breakdown, corneal ulceration, corneal melting (thinning), perforation, and loss of vision may occur. The best opportunity to reverse ocular surface damage is in the earliest stage of NK. However, patients typically experience few symptoms and diagnosis is often delayed. Increased awareness of the causes of NK, consensus on when and how to screen for NK, and recommendations for how to treat NK are needed.
    METHODS: An 11-member expert panel used a validated methodology (a RAND/UCLA modified Delphi panel) to develop consensus on when to screen for and how best to diagnose and treat NK. Clinicians reviewed literature on the diagnosis and management of NK then rated a detailed set of 735 scenarios. In 646 scenarios, panelists rated whether a test of corneal sensitivity was warranted; in 20 scenarios, they considered the adequacy of specific tests and examinations to diagnose and stage NK; and in 69 scenarios, they rated the appropriateness of treatments for NK. Panelist ratings were used to develop clinical recommendations.
    RESULTS: There was agreement on 94% of scenarios. Based on this consensus, we present distinct circumstances when we strongly recommend or may consider a test for corneal sensitivity. We also present recommendations on the diagnostic tests to be performed in patients in whom NK is suspected and treatment options for NK.
    CONCLUSIONS: These expert recommendations should be validated with clinical data. The recommendations represent the consensus of experts, are informed by published literature and experience, and may improve outcomes by helping improve diagnosis and treatment of patients with NK.
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  • 文章类型: Journal Article
    Neurotrophic keratitis is a degenerative disease of the cornea caused by impairment in the trigeminal nerve, which can lead to dry eyes, corneal epithelial defects and corneal ulcers. The common causes include herpes virus infection, craniocerebral and ophthalmic surgery injury, and diabetes. Due to the reduced or lack of corneal sensitivity, patients\' symptoms are mild, while severe corneal stroma melting or even perforation may occur. And the conventional symptomatic treatment is not effective. In view of the current lack of clinical understanding of this disease, there are a large number of problems of misdiagnosis and mistreatment. To improve the level of diagnosis and treatment, the consensus of experts on diagnosis and treatment of neurotrophic keratitis, which can be used as a reference for ophthalmologists in clinical work, is formulated by the Cornea Group of Chinese Ophthalmological Society. (Chin J Ophthalmol, 2021, 57:90-94).
    神经营养性角膜炎是由三叉神经损伤引起的角膜退行性病变,常见原因包括疱疹病毒感染、颅脑和眼科手术损伤、糖尿病等,可导致干眼、角膜上皮缺损和角膜溃疡。因角膜知觉减退或缺失,患者自觉症状轻,就诊时多已出现严重的角膜基质融解甚至穿孔,且常规对症治疗效果不佳。鉴于目前临床对本病的认识不足,存在大量误诊误治问题,为了提高临床诊疗水平,中华医学会眼科学分会角膜病学组经过充分讨论制定本共识,以供眼科医师在临床工作中参考使用。(中华眼科杂志,2021,57:90-94).
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    文章类型: Journal Article
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