新生儿ARCL2B腹股沟疝的外科治疗

E. Aydın, Burak Ayan
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引用次数: 0

摘要

发病率为百万分之一,男女平均分布它可能是先天性的,也可能是后天的。它影响支撑身体框架的结缔组织,为肌肉、关节、器官和皮肤提供结构和力量。基础病理与弹性蛋白纤维有关。它导致皮肤表现为面部老化,皮肤松弛多余,弹性降低;以及皮外表现,如胃肠道或泌尿系统憩室,胃食管反流,膈肌张力不全或疝,脐或腹股沟疝和胼胝体发育不全。2-4在文献中,有许多关于皮肤松弛症及其合并症的报道,但没有一个与患者的每次手术处理有关。据我们所知,这将是英语文献中第一例讨论常染色体隐性皮肤松弛症(ARCL2B)患者的手术治疗的病例。
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Surgical Management of Inguinal Hernia in a Newborn with ARCL2B
world with an incidence of 1 in 1 million and is equally distributed in both sexes.1 It may be congenital or acquired. It affects connective tissue that supports the body’s framework which provides structure and strength to the muscles, joints, organs, and skin. The underlying pathology is related with elastin fibers. It results in skin manifestations such as the aged face, loose redundant skin, and reduced elasticity; and extracutaneous manifestations such as diverticula at the gastrointestinal system or urinary system, gastroesophageal reflux, diaphragmatic atonia or a hernia, umbilical or an inguinal hernia and hypoplasia of corpus callosum.2-4 In the literature, there are many reports about cutis laxa and its comorbidities but none is related with per operative management of the patient. To our knowledge, this will be the first case that discusses the surgical management of an autosomal recessive cutis laxa (ARCL2B) patient in English literature.
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