title: Development and Validation of a Novel Fibrosis Marker in Biliary Atresia during Infancy.
abstract: OBJECTIVE
  Most biliary atresia (BA) patients suffer from liver fibrosis and often require liver transplantation. The aim of this study was to develop and validate a novel fibrosis marker for BA patients aged <1 year-the infant BA liver fibrosis (iBALF) score-subsequent to the previously reported fibrosis marker for BA patients aged ≥1 year.
  
  
  METHODS
  From three institutions for pediatric surgery, BA patients and their native liver histology examinations performed at the age of <1 year were retrospectively identified and assigned to a development cohort (58 patients and 73 examinations) or validation cohort (92 patients and 117 examinations) according to their institutions. Histological fibrosis stages (F0-F4), blood test results, and clinical information at the time of liver histology examination were reviewed. The iBALF score was determined using multivariate ordered logistic regression analysis and was assessed for its associations with histological fibrosis stages.
  
  
  RESULTS
  The iBALF score equation was composed of natural logarithms, including serum total bilirubin level, blood platelet counts, and days of age. The score revealed a strong correlation with fibrosis stage (r=0.80 and 0.73 in the development and validation cohorts, respectively; P<0.001). The areas under the receiver-operating characteristic curves for diagnosing each fibrosis stage were 0.86-0.94 in the development cohort and 0.86-0.90 in the validation cohort (P<0.001), indicating good diagnostic power. In addition, no patient with an iBALF score >6 (equivalent to F4) at the initial surgery survived with their native liver at 1 year of age (n=9).
  
  
  CONCLUSIONS
  The iBALF score that was developed was a good noninvasive marker of native liver fibrosis for BA patients aged <1 year.
authors.affiliation: Department of Pediatric Surgery, Keio University School of Medicine, Tokyo, Japan.|Department of Pediatric Surgery, Saitama City Hospital, Midori-ku, Saitama-shi, Japan.
authors.forename: Hirofumi
authors.initials: H
authors.lastname: Tomita
authors.affiliation: Department of Surgery, National Center for Child Health and Development, Okura Setagaya-ku Tokyo, Japan.
authors.forename: Yasushi
authors.initials: Y
authors.lastname: Fuchimoto
authors.affiliation: Department of Pediatric Surgery, Keio University School of Medicine, Tokyo, Japan.
authors.forename: Akihiro
authors.initials: A
authors.lastname: Fujino
authors.affiliation: Department of Pediatric Surgery, Keio University School of Medicine, Tokyo, Japan.
authors.forename: Ken
authors.initials: K
authors.lastname: Hoshino
authors.affiliation: Department of Pediatric Surgery, Keio University School of Medicine, Tokyo, Japan.
authors.forename: Yohei
authors.initials: Y
authors.lastname: Yamada
authors.affiliation: Department of Pathology, Keio University School of Medicine, Shinjuku-ku, Japan.
authors.forename: Yohei
authors.initials: Y
authors.lastname: Masugi
authors.affiliation: Department of Pathology, Keio University School of Medicine, Shinjuku-ku, Japan.
authors.forename: Michiie
authors.initials: M
authors.lastname: Sakamoto
authors.affiliation: Department of Transplant Surgery, National Center for Child Health and Development, Okura Setagaya-ku Tokyo, Japan.
authors.forename: Mureo
authors.initials: M
authors.lastname: Kasahara
authors.affiliation: Department of Surgery, National Center for Child Health and Development, Okura Setagaya-ku Tokyo, Japan.
authors.forename: Yutaka
authors.initials: Y
authors.lastname: Kanamori
authors.affiliation: Department of Pathology, National Center for Child Health and Development, Okura Setagaya-ku Tokyo, Japan.
authors.forename: Atsuko
authors.initials: A
authors.lastname: Nakazawa
authors.affiliation: Department of Pediatric Surgery, Saitama City Hospital, Midori-ku, Saitama-shi, Japan.
authors.forename: Fumiko
authors.initials: F
authors.lastname: Yoshida
authors.affiliation: Department of Pathology, Saitama City Hospital, Midori-ku, Saitama-shi, Japan.
authors.forename: Seiya
authors.initials: S
authors.lastname: Akatsuka
authors.affiliation: Department of Pediatric Surgery, Saitama City Hospital, Midori-ku, Saitama-shi, Japan.
authors.forename: Miwako
authors.initials: M
authors.lastname: Nakano
authors.affiliation: Department of Pediatric Surgery, Keio University School of Medicine, Tokyo, Japan.
authors.forename: Tatsuo
authors.initials: T
authors.lastname: Kuroda