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首页> 外文期刊>Saudi Journal of Anaesthesia >Anesthetic consideration in dystrophic epidermolysis bullosa
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Anesthetic consideration in dystrophic epidermolysis bullosa

机译：营养不良性大疱性表皮松解术的麻醉考虑

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摘要

Epidermolysis bullosa is a group of inherited rare skin disease, characterized by bullae formation in the skin or mucous membranes. The fundamental abnormality is collagen degeneration leads to splitting of various epidermal layers. Dystrophic epidermolysis bullosa (DEB) is one of the major forms of epidermolysis bullosa. These patients often admitted to the hospital for corrective surgeries, change of dressing, contracture release, and skin grafting. Anesthetic management of these cases is always a challenge. We are reporting a case of 5-year-old boy diagnosed as a case of DEB scheduled for upper lip contracture release, skin grafting and debridement of nonhealing scars under anesthesia. In this case, we have focused mainly on the anesthetic management, preparation of the monitoring, transportation, difficulties in establishing the venous accesses, and airway management.
机译：大疱表皮松解症是一组遗传的罕见皮肤病，其特征是在皮肤或粘膜中形成大疱。基本的异常是胶原变性导致各种表皮层的分裂。营养不良性大疱性表皮松解症（DEB）是大疱性表皮松解症的主要形式之一。这些患者经常因矫正手术，换药，挛缩和植皮而入院。这些病例的麻醉管理一直是一个挑战。我们正在报告一例5岁男孩，被诊断为DEB，计划在麻醉下进行上唇挛缩，皮肤移植和非愈合疤痕清创术。在这种情况下，我们主要关注麻醉管理，监测准备，运输，建立静脉通道的困难以及气道管理。

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