title: A new diagnostic scoring system to differentiate Hirschsprung's disease from Hirschsprung's disease-allied disorders in patients with suspected intestinal dysganglionosis.
abstract: OBJECTIVE
  To create a simple diagnostic scoring system to differentiate Hirschsprung's disease (HD) from Hirschsprung's disease-allied disorders (HAD) in patients with suspected intestinal dysganglionosis (IDs).
  
  
  METHODS
  Between 1998 and 2008, 967 patients with suspected intestinal dysganglionosis underwent surgical treatment at the pediatric surgery department of Tongji Hospital. The diagnosis of HD or HAD was confirmed by postoperative pathological examination. All patients underwent preoperative work-up including barium enema, anorectal manometry, and histochemical acetylcholinesterase staining of rectal mucosa. Known risk factors for IDs were recorded. The predicting score was calculated by summing the scores of the risk factors and three preoperative tests. The sensitivity, specificity, accuracy, positive predictive values, negative predictive values, positive likelihood ratios, and negative likelihood ratios of the predicting score were calculated. The cutoff score for predicting HD was determined using receiver operating characteristic (ROC) analysis. The accuracy of the predicting score was measured by the area under the ROC curve.
  
  
  RESULTS
  Failed or delayed passage of meconium, age <3 years and male gender were risk factors associated with HD. The area under the ROC curve of the predicting score was 0.927 (95 % confidence interval, 0.910-0.944). A predicting score of more than 5 was used as a cutoff for predicting HD. The scoring system achieved 83.1 % sensitivity, 89.5 % specificity, and 85.9 % accuracy in predicting HD.
  
  
  CONCLUSIONS
  Patients with a predicting score of more than 5 are more likely to be diagnosed with HD, whereas a score less than 5 are mostly indicative of HAD.
authors.affiliation: Department of Pediatric Surgery, Tongji Hospital, 1095 Jiefang Avenue, Wuhan 430030, China.
authors.forename: Xiao-juan
authors.initials: XJ
authors.lastname: Wu
authors.forename: Hong-Yi
authors.initials: HY
authors.lastname: Zhang
authors.forename: Ning
authors.initials: N
authors.lastname: Li
authors.forename: Mao-sheng
authors.initials: MS
authors.lastname: Yan
authors.forename: Jia
authors.initials: J
authors.lastname: Wei
authors.forename: Dong-hai
authors.initials: DH
authors.lastname: Yu
authors.forename: Jie-xiong
authors.initials: JX
authors.lastname: Feng
mesh_terms: D019540:Area Under Curve; D002675:Child, Preschool; D003710:Demography; D003937:Diagnosis, Differential; D003955:Diagnostic Tests, Routine; D005260:Female; D005724:Ganglia / Q000473:pathology*; D006627:Hirschsprung Disease / Q000150:complications* / Q000175:diagnosis*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D012372:ROC Curve; D012107:Research Design