title: Design and validation of a diagnostic score for biliary atresia.
abstract: OBJECTIVE
  The dilemma of early diagnosis of biliary Atresia (BA), particularly distinguishing it from other causes of neonatal cholestasis is challenging. The aim was to design and validate a scoring system for early discrimination of BA from other causes of neonatal cholestasis.
  
  
  METHODS
  A twelve-point scoring system was proposed according to clinical, laboratory, ultrasonographic, and histopathological parameters. A total of 135 patients with neonatal cholestasis in two sets were recruited to design (n=60) and validate (n=75) a scoring system. Parameters with significant statistical difference between BA (n=30) and non-BA (n=30) patients in the design set were analyzed by logistic regression to predict the presence or absence of BA then a scoring system was designed and validated.
  
  
  RESULTS
  The total score ranged from 0 to 37.18 and a cut-off value of >23.927 could discriminate BA from other causes of neonatal cholestasis with sensitivity and specificity of 100% each. By applying this score in the validation set, the accuracy was 98.83% in predicting BA. The diagnosis of BA was proposed correctly in 100% and the diagnosis of non-BA was proposed correctly in 97.67% of patients. By applying this model, unnecessary intraoperative cholangiography would be avoided in non-BA patients.
  
  
  CONCLUSIONS
  This scoring system accurately separates infants with BA and those with non-BA, rendering intraoperative cholangiography for confirming or excluding BA unnecessary in a substantial proportion of patients.
authors.affiliation: Department of Pediatric Hepatology, National Liver Institute, Menofiya University, 32511 Shebin El-koom, Menofiya, Egypt.
authors.forename: Mohamed Abdel-Salam
authors.initials: MA
authors.lastname: El-Guindi
authors.affiliation: Department of Pediatric Hepatology, National Liver Institute, Menofiya University, 32511 Shebin El-koom, Menofiya, Egypt. Electronic address: msira@liver-eg.org.
authors.forename: Mostafa Mohamed
authors.initials: MM
authors.lastname: Sira
authors.affiliation: Department of Pediatric Hepatology, National Liver Institute, Menofiya University, 32511 Shebin El-koom, Menofiya, Egypt.
authors.forename: Ahmad Mohamed
authors.initials: AM
authors.lastname: Sira
authors.affiliation: Department of Pediatric Hepatology, National Liver Institute, Menofiya University, 32511 Shebin El-koom, Menofiya, Egypt.
authors.forename: Tahany Abdel-Hameed
authors.initials: TA
authors.lastname: Salem
authors.affiliation: Department of Radiology, National Liver Institute, Menofiya University, 32511 Shebin El-koom, Menofiya, Egypt.
authors.forename: Osama Lotfy
authors.initials: OL
authors.lastname: El-Abd
authors.affiliation: Department of Pediatric Hepatology, National Liver Institute, Menofiya University, 32511 Shebin El-koom, Menofiya, Egypt.
authors.forename: Hatem Abdel-Sattar
authors.initials: HA
authors.lastname: Konsowa
authors.affiliation: Department of Pathology, National Liver Institute, Menofiya University, 32511 Shebin El-koom, Menofiya, Egypt.
authors.forename: Dina Shehata
authors.initials: DS
authors.lastname: El-Azab
authors.affiliation: Department of Pediatric Hepatology, National Liver Institute, Menofiya University, 32511 Shebin El-koom, Menofiya, Egypt.
authors.forename: Alif Abdel-Hakim
authors.initials: AA
authors.lastname: Allam
mesh_terms: D001656:Biliary Atresia / Q000150:complications / Q000175:diagnosis* / Q000000981:diagnostic imaging; D002758:Cholangiography; D002779:Cholestasis / Q000209:etiology; D015331:Cohort Studies; D047630:Depsipeptides; D003937:Diagnosis, Differential; D042241:Early Diagnosis; D005260:Female; D005670:Fusarium; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008099:Liver / Q000000981:diagnostic imaging / Q000473:pathology; D016015:Logistic Models; D008297:Male; D011237:Predictive Value of Tests; D011446:Prospective Studies; D012680:Sensitivity and Specificity; D014463:Ultrasonography
chemical_list: D047630:Depsipeptides; C014001:fusafungin
keywords: Clay stool; Doppler ultrasonography; Hepatic subcapsular flow; Liver biopsy; Neonatal cholestasis