steven-johnson syndrome (sjs)

  • 文章类型: Case Reports
    毒性表皮坏死松解症(TEN)是一种罕见的致命的皮肤粘膜起泡疾病,可以有各种潜在的触发因素。受侵蚀性起泡影响的体表面积(BSA)的百分比是将其与史蒂文·约翰逊综合征(SJS)区分开来的原因,两者具有相同的潜在发病机理,并且被认为存在于连续的疾病中,TEN是两者中更严重的。药物是TEN/SJS的最常见原因,通常会在八周内导致成人和儿童患病;然而,中位暴露窗口为4天至4周.非甾体抗炎药,别嘌呤醇,抗惊厥药包括拉莫三嗪,苯妥英,左乙拉西坦和卡马西平,抗菌磺胺类药物,和抗病毒奈韦拉平是经常引起TEN/SJS的药物的例子。这里,我们报告了一例苯妥英钠诱导的TEN病例,强调了患者对免疫调节治疗的良好反应,尽管BSA100%参与.
    Toxic epidermal necrolysis (TEN) is a rare fatal mucocutaneous blistering disorder that can have varied underlying triggers. The percentage of body surface area (BSA) that is impacted by erosive blistering is what separates it from Steven Johnson syndrome (SJS), both of which have the same underlying pathogenesis and are thought to exist on a continuum of disease with TEN being the more serious of the two. Medications are the most frequent cause of TEN/SJS and typically cause disease in both adults and children within eight weeks; however, the median exposure window is four days to four weeks. Nonsteroidal anti-inflammatory drugs, allopurinol, anticonvulsants including lamotrigine, phenytoin, levetiracetam and carbamazepine, antimicrobial sulfonamides, and the antiviral nevirapine are examples of medications that frequently cause TEN/SJS. Here, we are reporting a case of phenytoin-induced TEN highlighting the patient\'s excellent response to immunomodulating treatment despite 100% involvement of the BSA.
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  • 文章类型: Case Reports
    Steven-Johnson综合征(SJS)和中毒性表皮坏死松解症(TEN)是一系列严重皮肤不良反应的一部分,继发于感染或药物诱导。尽管使用抗癫痫药物(ASM)是SJS/TEN发展的危险因素,在一些国家,初级保健医生不熟悉这些病例。我们报告了一例SJS与ASM相关的9岁女孩,有难以控制的癫痫史,他们需要调整和改变药物。
    Steven-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are part of a spectrum of severe cutaneous adverse reactions, secondary to infections or drug-induced. Although the use of antiseizure medications (ASMs) is a risk factor for the development of SJS/TEN, primary care physicians are not familiar with these cases in some countries. We report a case of SJS associated with ASMs in a nine-year-old girl with a history of difficult-to-control epilepsy, who required adjustment and change in medications.
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  • 文章类型: Journal Article
    Steven-Johnson综合征(SJS)是一种罕见且严重的多形性红斑渗出性。SJS中的多系统参与以及对暴露于麻醉药物的疾病沉淀的怀疑使麻醉成为一项具有挑战性的任务。麻醉期间的关注点是粘膜病变和需要特殊护理,以防止在气道管理期间对口咽和喉造成伤害,以及用于麻醉的药物。在文学中,很少有孤立病例报告或病例系列。这里,我们分析了SJS来进行眼科麻醉的病例,考虑到表达方式等因素,沉淀因素,相关疾病,麻醉类型,麻醉修改,麻醉期间使用的各种药物。
    回顾性分析了在18个月内需要眼科检查或手术(局部或全身麻醉)等干预措施的497例SJS患者的电子病历。对记录进行了审查,以获得相关细节,如麻醉剂,肌肉松弛剂,吸入剂,和镇痛药.还注意到与监测和插管有关的问题。对数据进行分析并以频率和百分比表示。
    患者年龄介于9个月至72岁之间。许多手术是在全身麻醉下进行的(441),尽管少数手术需要局部(球周阻滞)麻醉(56)。用于全身麻醉的药物为七氟醚,异氟烷,异丙酚,硫喷酮,维库溴铵,阿曲库铵和用于疼痛管理的药物是芬太尼,曲马多,布托啡诺,和扑热息痛.对非甾体抗炎药(NSAIDs)和扑热息痛敏感的患者未给予相同的治疗。没有患者对用于麻醉的不同药物有不利反应。
    找出促成因素,了解病理生理学及其对麻醉的影响将有助于在罕见的SJS病例中成功管理麻醉。
    UNASSIGNED: Steven-Johnson Syndrome (SJS) is a rare and severe form of erythema exudative multiforme. Multisystem involvement in SJS and the suspicion of precipitation of the disease with exposure to anesthetic drugs makes anesthesia a challenging task. The concerns during anesthesia are the mucosal lesions and special care that is required to prevent injury to the oropharynx and larynx during airway management and also the drugs used for anesthesia. In the literature, very few isolated case reports or case series are available. Here, we have analyzed the cases of SJS coming for ophthalmic anesthesia, taking into consideration factors like mode of presentation, precipitating factors, associated diseases, types of anesthesia, anesthetic modifications, and various drugs used during anesthesia.
    UNASSIGNED: The electronic medical records of 497 cases of SJS who required interventions like ophthalmic examination or surgery (either under local or general anesthesia) over a period of 18 months were analyzed retrospectively. The records were reviewed to obtain the concerned details like anesthesia-inducing agents, muscle relaxants, inhalational agents, and analgesics. The problems concerned with monitoring and intubation were also noted. The data were analyzed and presented as frequency and percentage.
    UNASSIGNED: Patient age ranged between 9 months and 72 years. Many surgeries were conducted under general anesthesia (441) although a few required local (peribulbar block) anesthesia (56). The drugs administered for general anesthesia were sevoflurane, isoflurane, propofol, thiopentone, vecuronium, and atracurium and those administered for pain management were fentanyl, tramadol, butorphanol, and paracetamol. The patients who were sensitive to nonsteroidal anti-inflammatory drugs (NSAIDs) and paracetamol were not administered the same. None of the patients reacted adversely to the different drugs used for anesthesia.
    UNASSIGNED: Identifying the precipitating factors, understanding the pathophysiology and its implications for anesthesia will help in successfully managing anesthesia in the rare cases of SJS.
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    文章类型: Journal Article
    Toxic epidermal necrolysis (TEN) and Steven-Johnson syndrome (SJS) are characterized by extensive necrosis and cleavage of the epidermis from the dermis akin to a superficial or partial thickness burn. Sepsis is the usual cause of mortality but much of the pathophysiologic process results from an outpouring of cytokines and matrixmetalloproteinases (MMPs) which have a destructive effect on the extracellular matrix and may play a part in the epidermal/dermal cleavage seen with this disease. Recent attention has been focused on the modulation of proteases in an attempt to decrease the MMP-mediated destruction. Nanocrystalline silver (NCS) is one such agent that has good anti-microbial efficacy, but is also effective in modulating MMP levels. Twelve cases of confirmed TEN that were treated with NCS were analyzed with a view to assessing efficacy and setting logical guidelines for managing this condition, particularly in relation to immunosupressed patients. From this study important issues have been highlighted for discussion.
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