KEGG   DISEASE: 総排泄腔遺残
エントリ  
H01858                                                             
名称    
総排泄腔遺残
概要    
Persistent cloaca is an uncommon congenital anomaly in which there is a single perineal opening for the urinary, gastrointestinal, and reproductive tracts. It is the most severe form of anorectal malformation encountered in girls. The incidence is approximately 1 in 50,000 live births. Persistent cloaca results from abnormal development of the cloacal membrane before 50 days post fertilization. In addition to a single perineal opening, secondary urogenital or gastrointestinal anomalies are often associated with persistent cloaca. Moreover, anomalies of other organ systems such as cardiac or skeletal malformations are known to coexist. The common channel of persistent cloaca varies from 1 cm to 10 cm. Those cases with a common channel longer than 3 cm are predicted with the higher incidence of complications. For patients with a common channel greater than 3 cm, a laparotomy is usually required.
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
     H01858  総排泄腔遺残
指定難病 [jp08407.html]
 H01858
リンク   
ICD-11: LB17.2
文献    
  著者
Wang C, Li L, Cheng W, Liu S, Diao M, Li X, Qiao G, Zhang Z, Chen Z
  タイトル
A new approach for persistent cloaca: Laparoscopically assisted anorectoplasty and modified repair of urogenital sinus.
  雑誌
J Pediatr Surg 50:1236-40 (2015)
DOI:10.1016/j.jpedsurg.2015.04.016
文献    
  著者
Livingston JC, Elicevik M, Breech L, Crombleholme TM, Pena A, Levitt MA
  タイトル
Persistent cloaca: a 10-year review of prenatal diagnosis.
  雑誌
J Ultrasound Med 31:403-7 (2012)
DOI:10.7863/jum.2012.31.3.403
LinkDB    

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