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巴西伴有严重眼部并发症的史蒂文斯-约翰逊综合征和中毒性表皮坏死松解症的临床情况

Clinical Aspects of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis With Severe Ocular Complications in Brazil.

作者信息

Wakamatsu Tais Hitomi, Dos Santos Myrna Serapião, Barreiro Telma Pereira, Sant'Anna Ana Estela Besteti Pires Ponce, Murta Fabíola, da Costa Alexandre Xavier, Marculino Leonardo Guedes C, de Alcântara Rafael Jorge Alves, de Farias Charles Costa, Gomes José Álvaro Pereira

机构信息

Department of Ophthalmology, Federal University of São Paulo, São Paulo, Brazil.

Moorfields Eye Hospital, London, United Kingdom.

出版信息

Front Med (Lausanne). 2021 Jun 18;8:649369. doi: 10.3389/fmed.2021.649369. eCollection 2021.

Abstract

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are acute and potentially fatal inflammatory vesiculobullous reactions that affect the skin and mucous membranes, and which are most often triggered by particular medications and infections. In Brazil, the drugs most frequently associated with TEN and SJS include cold medicine such as dipyrone and NSAIDs, followed by carbamazepine, phenobarbital, penicillin, and allopurinol. Genetic variations have been found to increase the risk of SJS/TEN in response to triggering factors such as medications. The most closely associated genes found in Brazilian cold-medicine-related SJS/TEN patients with severe ocular complications are HLA-A66:01 in those of mixed African and European ancestry and HLA-B44:03 and HLA-C12:03 in those of solely European ancestry. Our classification system for grading ocular surface complication severity in SJS/TEN patients revealed the most severe complications to be limbal stem cell deficiency and dry eye. Changes to the conjunctival flora have also been observed in SJS/TEN patients. Our group identified bacterial colonization in 95% of the eyes (55.5% of which were gram-positive cocci, 25.5% of which were gram-negative bacilli, and 19% of which were gram-positive bacilli). Several new treatment options in the acute and chronic ocular management of the SJS/TEN patients have been described. This article highlights some Brazilian institutions' contributions to ocular surface care in both the acute phase (including the use of amniotic membrane transplantation) and the chronic phase (such as eyelid margin and fornix reconstruction, minor salivary gland transplantation, amniotic membrane and limbal transplantation, scleral contact lenses, anti-angiogenic eyedrops for corneal neovascularization, cultivated limbal epithelium transplantation, conjunctival-limbal autografting, oral mucosa transplantation, and keratoprosthesis).

摘要

史蒂文斯-约翰逊综合征(SJS)和中毒性表皮坏死松解症(TEN)是急性且可能致命的炎症性水疱大疱性反应,会影响皮肤和黏膜,最常见的诱因是特定药物和感染。在巴西,与TEN和SJS最常相关的药物包括双氯芬酸和非甾体抗炎药等感冒药,其次是卡马西平、苯巴比妥、青霉素和别嘌醇。已发现基因变异会增加因药物等触发因素而患SJS/TEN的风险。在巴西患有严重眼部并发症的与感冒药相关的SJS/TEN患者中,发现与疾病关联最密切的基因在非洲和欧洲混血血统患者中是HLA-A66:01,在纯欧洲血统患者中是HLA-B44:03和HLA-C12:03。我们对SJS/TEN患者眼表并发症严重程度的分级系统显示,最严重的并发症是角膜缘干细胞缺乏和干眼。在SJS/TEN患者中也观察到结膜菌群的变化。我们的研究小组在95%的眼睛中发现了细菌定植(其中55.5%为革兰氏阳性球菌,25.5%为革兰氏阴性杆菌,19%为革兰氏阳性杆菌)。已经描述了SJS/TEN患者急性和慢性眼部治疗的几种新选择。本文重点介绍了一些巴西机构在急性期(包括羊膜移植的应用)和慢性期(如眼睑边缘和穹窿重建、小唾液腺移植、羊膜和角膜缘移植、巩膜接触镜、用于角膜新生血管的抗血管生成眼药水、培养的角膜缘上皮移植、结膜-角膜缘自体移植、口腔黏膜移植和角膜假体)对眼表护理的贡献。

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