PDF(Pubmed)
Abstract：
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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.
摘要：
■在X连锁鱼鳞病中显示的皮肤鳞屑和相关临床综合征需要多学科护理。鱼鳞病患者面临着麻醉师的众多挑战，需要严格的管理。由于这些患者在围手术期非常脆弱，细致的关心和支持是最大的。
■鱼鳞病是一组遗传疾病，其特征是皮肤表面出现过度角化鳞屑。X连锁鱼鳞病是由类固醇硫酸酯酶（STS）基因突变引起的，它编码类固醇硫酸酯酶。在这里，我们报告了一例X连锁鱼鳞病的6岁儿童。他向我们的手术室介绍了左侧未降睾丸的矫正方法，并顺利接受了手术。围手术期处理X连锁鱼鳞病，细致的计划和有效的麻醉管理至关重要。
■鱼鳞病是一组遗传疾病，其特征是皮肤表面出现过度角化鳞屑。X连锁鱼鳞病是由类固醇硫酸酯酶（STS）基因突变引起的，它编码类固醇硫酸酯酶。在这里，我们报告了一例X连锁鱼鳞病的6岁儿童。他向我们的手术室介绍了左侧未降睾丸的矫正方法，并顺利接受了手术。围手术期处理X连锁鱼鳞病，细致的计划和有效的麻醉管理至关重要。
