交界性大疱性表皮松解症中的急性喉梗阻
Acute laryngeal obstruction in junctional epidermolysis bullosa.
作者信息
Davies H, Atherton D J
机构信息
Department of Respiratory Medicine, Hospital for Sick Children, London, United Kingdom.
出版信息
Pediatr Dermatol. 1987 Aug;4(2):98-101. doi: 10.1111/j.1525-1470.1987.tb00760.x.
A boy with junctional epidermolysis bullosa died from acute laryngeal obstruction at the age of 29 months, having been hoarse since early infancy. Post mortem studies showed gross narrowing of the laryngeal airway by cystic dilatations of the ducts of the seromucinous glands, and replacement of the laryngeal epithelium by oedematous granulation tissue. Laryngeal involvement appears to occur not infrequently in junctional epidermolysis bullosa, and contributes to its considerable mortality.
一名患有交界型大疱性表皮松解症的男孩在29个月大时死于急性喉梗阻，自婴儿早期就一直声音嘶哑。尸检研究显示，浆液黏液性腺导管的囊性扩张导致喉气道明显狭窄，喉上皮被水肿性肉芽组织取代。喉受累在交界型大疱性表皮松解症中似乎并不少见，并导致了相当高的死亡率。
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