title: Ultrasonography evaluation of infants with Alagille syndrome: In comparison with biliary atresia and neonatal hepatitis.
abstract: OBJECTIVE
  To evaluate the ultrasonography (US) features of Alagille syndrome (ALGS), as compared with biliary atresia (BA) or neonatal hepatitis (NH).
  
  
  METHODS
  Our study included 23 ALGS, 75 BA and 70 NH patients. The initial US images were retrospectively reviewed for gallbladder (GB) morphology with systemic classification, GB length and luminal area, presence of triangular-cord (TC) sign and hypertrophied hepatic-artery. The presence of anomalies associated with ALGS was evaluated. The diagnostic values of each finding and their combinations were evaluated.
  
  
  RESULTS
  Both ALGS (57%) and BA (79%) were more frequently associated with abnormal GB shapes than NH (19%, all P<0.001). The short and small GBs were more frequently observed in ALGS and BA than in NH (all P<0.001). None in the ALGS and NH showed TC sign, while 41% in the BA did (all P<0.001). Hypertrophied hepatic-artery was noted less frequently in both ALGS (13%) and NH (14%) than in BA (83%, all P<0.001). The combination of US criteria with associated anomalies increased the positive-predictive-value for ALGS.
  
  
  CONCLUSIONS
  Abnormal shaped GB with absence of the TC sign and hypertrophied hepatic-artery and presence of associated anomalies can be a differential point of ALGS.
authors.affiliation: Department of Radiology, Seoul National University Hospital, 101 Daehak-ro, Jongno-gu, Seoul 03080, Republic of Korea.
authors.forename: Hyun-Hae
authors.initials: HH
authors.lastname: Cho
authors.affiliation: Department of Radiology, Seoul National University Hospital, 101 Daehak-ro, Jongno-gu, Seoul 03080, Republic of Korea; Department of Radiology, Seoul National University College of Medicine, 103 Daehak-ro, Jongno-gu, Seoul 03080, Republic of Korea; Institute of Radiation Medicine, Seoul National University Medical Research Center, 103 Daehak-ro, Jongno-gu, Seoul 03080, Republic of Korea. Electronic address: kimws@snu.ac.kr.
authors.forename: Woo Sun
authors.initials: WS
authors.lastname: Kim
authors.affiliation: Department of Radiology, Seoul National University Hospital, 101 Daehak-ro, Jongno-gu, Seoul 03080, Republic of Korea; Department of Radiology, Seoul National University College of Medicine, 103 Daehak-ro, Jongno-gu, Seoul 03080, Republic of Korea.
authors.forename: Young Hun
authors.initials: YH
authors.lastname: Choi
authors.affiliation: Department of Radiology, Seoul National University Hospital, 101 Daehak-ro, Jongno-gu, Seoul 03080, Republic of Korea; Department of Radiology, Seoul National University College of Medicine, 103 Daehak-ro, Jongno-gu, Seoul 03080, Republic of Korea; Institute of Radiation Medicine, Seoul National University Medical Research Center, 103 Daehak-ro, Jongno-gu, Seoul 03080, Republic of Korea.
authors.forename: Jung-Eun
authors.initials: JE
authors.lastname: Cheon
authors.affiliation: Department of Radiology, Seoul National University Hospital, 101 Daehak-ro, Jongno-gu, Seoul 03080, Republic of Korea.
authors.forename: So Mi
authors.initials: SM
authors.lastname: Lee
authors.affiliation: Department of Radiology, Seoul National University Hospital, 101 Daehak-ro, Jongno-gu, Seoul 03080, Republic of Korea; Department of Radiology, Seoul National University College of Medicine, 103 Daehak-ro, Jongno-gu, Seoul 03080, Republic of Korea; Institute of Radiation Medicine, Seoul National University Medical Research Center, 103 Daehak-ro, Jongno-gu, Seoul 03080, Republic of Korea.
authors.forename: In-One
authors.initials: IO
authors.lastname: Kim
authors.affiliation: Department of Radiology, SMG-SNU Boramae Medical Center, Seoul 07061, Republic of Korea.
authors.forename: Su-Mi
authors.initials: SM
authors.lastname: Shin
authors.affiliation: Department of Pediatrics, Seoul National University Hospital, Seoul National University College of Medicine, Republic of Korea.
authors.forename: Jae Sung
authors.initials: JS
authors.lastname: Ko
authors.affiliation: Department of Pediatrics, Seoul National University Hospital, Seoul National University College of Medicine, Republic of Korea.
authors.forename: Jin Soo
authors.initials: JS
authors.lastname: Moon
mesh_terms: D000015:Abnormalities, Multiple / Q000175:diagnosis; D016738:Alagille Syndrome / Q000000981:diagnostic imaging*; D001656:Biliary Atresia / Q000000981:diagnostic imaging*; D003937:Diagnosis, Differential; D005260:Female; D005704:Gallbladder / Q000000981:diagnostic imaging; D006499:Hepatic Artery / Q000000981:diagnostic imaging; D006505:Hepatitis / Q000000981:diagnostic imaging*; D006801:Humans; D006984:Hypertrophy; D007223:Infant; D007231:Infant, Newborn; D007567:Jaundice, Neonatal / Q000000981:diagnostic imaging; D041781:Jaundice, Obstructive / Q000000981:diagnostic imaging; D008297:Male; D011169:Portal Vein / Q000000981:diagnostic imaging; D011237:Predictive Value of Tests; D012189:Retrospective Studies; D014463:Ultrasonography / Q000379:methods*
keywords: Alagille syndrome; Biliary atresia; Gallbladder; Hepatitis; Ultrasonography