Bhatta Sunil, Pandit Sukriti, Chaudhary Pratik, Chhetri Naresh Thapa
Department of Anesthesia and Critical Care Nisarga Hospital and Research Center Dhangadhi Nepal.
Department of Anesthesia and Critical Care P.T. Birta City Hospital and Research Center Birtamode Nepal.
Clin Case Rep. 2024 Aug 5;12(8):e9245. doi: 10.1002/ccr3.9245. eCollection 2024 Aug.
Cutaneous scaling and associated clinical syndrome displayed in X-linked ichthyosis mandates multidisciplinary care. Patient with ichthyosis confronts a numerous challenge to an anesthesiologist and demands a rigorous management. As these patients are very vulnerable perioperatively, meticulous care and support are utmost.
Ichthyosis is a group of genetic conditions distinguished by the appearance of hyperkeratotic scales on the skin's surface. X-linked ichthyosis results from a mutation in the steroid sulfatase (STS) gene, which encodes the steroid sulfatase enzyme. Here we report a case of a 6-year-old child with X-linked ichthyosis. He presented to our operation theater for correction of left-sided undescended testis and underwent surgery uneventfully. To handle X-linked ichthyosis perioperatively, meticulous planning and efficient anesthetic administration are critical.
X连锁鱼鳞病所表现出的皮肤脱屑及相关临床综合征需要多学科护理。鱼鳞病患者给麻醉医生带来诸多挑战,需要严格管理。由于这些患者在围手术期非常脆弱,细致的护理和支持至关重要。
鱼鳞病是一组遗传性疾病,其特征是皮肤表面出现角化过度的鳞屑。X连锁鱼鳞病是由类固醇硫酸酯酶(STS)基因突变引起的,该基因编码类固醇硫酸酯酶。本文报告一例6岁X连锁鱼鳞病患儿。他因左侧隐睾矫正入我院手术室,手术顺利进行。为在围手术期处理X连锁鱼鳞病,精心规划和高效的麻醉管理至关重要。