KEGG   DISEASE: 先天性気管狭窄症
エントリ  
H02600                                                             
名称    
先天性気管狭窄症
概要    
Congenital tracheal stenosis (CTS) is a rare but potentially life-threatening disorder that often leads to severe respiratory insufficiency, particularly in neonates and infants. CTS is characterized by complete cartilaginous rings of the trachea without the membranous portion, resulting in various clinical manifestations. More than half of patients with CTS have other congenital anomalies, particularly cardiovascular disease. Moreover, CTS is often associated with pulmonary agenesis or hypoplasia.
カテゴリ  
先天奇形
階層分類  
ICD-11 による疾患分類 [BR:jp08403]
 20 発達異常
  主に1つの体系に影響する構造的発達異常
   呼吸器系の構造的発達異常
    LA73  気管の構造的発達異常
     H02600  先天性気管狭窄症
指定難病 [jp08407.html]
 H02600
リンク   
ICD-11: LA73.0
MeSH: C536975
OMIM: 603569
文献    
  著者
Yokoi A
  タイトル
Congenital tracheal stenosis: what should we look at for successful tracheoplasty?
  雑誌
Transl Pediatr 7:229-232 (2018)
DOI:10.21037/tp.2018.07.01
文献    
  著者
Hofferberth SC, Watters K, Rahbar R, Fynn-Thompson F
  タイトル
Management of Congenital Tracheal Stenosis.
  雑誌
Pediatrics 136:e660-9 (2015)
DOI:10.1542/peds.2014-3931
LinkDB    

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