KEGG   DISEASE: 先天性肺静脈狭窄症
エントリ  
H02605                                                             
名称    
先天性肺静脈狭窄症
概要    
Congenital pulmonary venous stenosis is a rare condition with fibromyxoid intimal proliferation leading to obstruction of the large pulmonary veins and of blood flow from the lungs to the left atrium. This results in elevated pulmonary venous pressure and pulmonary edema, pulmonary hypertension, potentially cardiac failure, and death. Clinical signs of the disease include failure to thrive,  dyspnea, hemoptysis, recurrent respiratory tract infections, cyanosis, and subcostal retractions.
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
   循環器系の構造的発達異常
    心臓または大血管の構造的発達異常
     LA86  縦隔静脈の先天異常
      H02605  先天性肺静脈狭窄症
指定難病 [jp08407.html]
 H02605
リンク   
ICD-11: LA86.3
文献    
  著者
Holcomb RG, Tyson RW, Ivy DD, Abman SH, Kinsella JP
  タイトル
Congenital pulmonary venous stenosis presenting as persistent pulmonary hypertension of the newborn.
  雑誌
文献    
  著者
Suntharos P, Prieto LR
  タイトル
Treatment of Congenital and Acquired Pulmonary Vein Stenosis.
  雑誌
Curr Cardiol Rep 22:153 (2020)
DOI:10.1007/s11886-020-01395-x
文献    
  著者
Humpl T, Fineman J, Qureshi AM
  タイトル
The many faces and outcomes of pulmonary vein stenosis in early childhood.
  雑誌
Pediatr Pulmonol 56:649-655 (2021)
DOI:10.1002/ppul.24848
LinkDB    

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