Wudy S A, Lenders H, Pirsig W, Mohr W, Teller W M
Department of Pediatrics, University of Ulm, Donau, FRG.
Int J Pediatr Otorhinolaryngol. 1995 Jan;31(1):109-15. doi: 10.1016/0165-5876(94)01074-8.
Pachyonychia congenita is a genetic syndrome of epithelial dysplasia. In infants and young children, laryngeal involvement can present a life threatening complication: obstruction of the patient's airway due to leukokeratosis can lead to severe respiratory distress. This report concentrates on the diagnosis and successful microsurgical management of laryngeal obstruction in the first female case, a 19-month-old girl with pachyonychia congenita.
先天性厚甲症是一种上皮发育异常的遗传综合征。在婴幼儿中，喉部受累可能会出现危及生命的并发症：由于白色角化病导致患者气道阻塞可引发严重的呼吸窘迫。本报告重点介绍了首例女性先天性厚甲症患者（一名19个月大的女孩）喉部梗阻的诊断及成功的显微外科治疗。
