Upper Airway Complications of Junctional Epidermolysis Bullosa
Upper Airway Complications of Junctional Epidermolysis Bullosa
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DOI：
10.1016/j.jpeds.2011.09.029
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发表时间：
2012-04-01
影响因子：
5.1
通讯作者：
Elluru, Ravindhra G.
中科院分区：
文献类型：
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作者：
Ida, Jonathan B.;Livshitz, Irina;Elluru, Ravindhra G.
目的探讨大疱性结缔组织表皮松解症（JEB）患者上气道病理的发生率。研究设计我们对2004年至2010年间在一家三级医疗机构的跨学科大疱性表皮松解症中心就诊的所有JEB患者进行了回顾性图表回顾。结果共发现JEB患者25例，其中12例在耳鼻喉科就诊，年龄2个月~ 15岁，男8例，女4例。在12例患者中，8例因上呼吸道症状接受了硬喉镜检查和支气管镜检查；其中7例患者出现喉部病变，5例接受手术治疗，症状成功缓解。此外，这些患者都没有任何短期或长期的手术并发症。在这些病例中使用了严格的规程和精确的问题聚焦冷手术技术来保护皮肤和粘膜。结论在注意事项适当的情况下，对乙脑合并喉气管疾病患者行内镜喉部手术是安全有效的。内窥镜喉部手术是可行的，当指征这些患者。[J] .中华儿科杂志；2012;16:657-61。
Objective To assess the incidence of upper airway pathology in patients with junctional epidermolysis bullosa (JEB).Study design We conducted a retrospective chart review of all patients with JEB who came to an interdisciplinary epidermolysis bullosa center at a tertiary care institution between 2004 and 2010.Results Twenty-five patients with JEB were identified, and 12 patients were seen in the otolaryngology clinic (age range, 2 months-15 years; 8 male, 4 female). Of the 12 patients, 8 underwent rigid laryngoscopy and bronchoscopy for upper respiratory tract symptoms; 7 of these patients displayed laryngeal pathology, and 5 of them underwent surgical intervention with successful resolution of symptoms. Furthermore, none of these patients had any short-or long-term complications from their surgery. A strict protocol and a precise problem-focused cold surgical technique were used in these cases to protect skin and mucus membranes.Conclusion With appropriate precautions, endoscopic laryngeal surgery can be safe and effective in patients with JEB and larygnotracheal disease. Endoscopic laryngeal surgery is feasible when indicated for these patients. (J Pediatr 2012;160:657-61).