KEGG   DISEASE: 総排泄腔外反症
エントリ  
H01856                                                             
名称    
総排泄腔外反症;
膀胱腸裂
概要    
Cloacal exstrophy is an extremely rare congenital complex deformity that is associated with anterior abdominal wall defects, reflex and exposure of the cloaca (uninterrupted ureter and bowel), aproctia, a widely separated pubic bone, and defects or dysplasia of external genital organs. Cloacal exstrophy is thought to be related to abnormal development of the cloacal membrane, a transitory structure composed of endoderm and ectoderm that overlies the embryonic cloaca. The result is primarily an abdominal wall defect with failed closure of the lower urinary tract. Cloacal exstrophy has also been referred to as the OEIS complex (omphalocele, exstrophy, imperforate anus and spinal defect), and other malformations of the urogenital, gastrointestinal, skeletal and neurospinal axis frequently coexist.
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
   消化管の構造的発達異常
    LB17  肛門管の構造的発達異常
     H01856  総排泄腔外反症
指定難病 [jp08407.html]
 H01856
リンク   
ICD-11: LB17.3
ICD-10: Q64.1
OMIM: 258040
文献    
  著者
Makiguchi T, Yokoo S, Takaku Y, Morita H, Yasuda M
  タイトル
Squamous cell carcinoma arising from abdominal wall defect lesion complicated with cloacal exstrophy.
  雑誌
Plast Reconstr Surg Glob Open 3:e315 (2015)
DOI:10.1097/GOX.0000000000000286
文献    
  著者
Woo LL, Thomas JC, Brock JW
  タイトル
Cloacal exstrophy: a comprehensive review of an uncommon problem.
  雑誌
J Pediatr Urol 6:102-11 (2010)
DOI:10.1016/j.jpurol.2009.09.011
LinkDB    

» English version

DBGET integrated database retrieval system