title: Diversity of disorders causing neonatal cholestasis - the experience of a tertiary pediatric center in Germany.
abstract: OBJECTIVE
  Rapidly establishing the cause of neonatal cholestasis is an urgent matter. The aim of this study was to report on the prevalence and mortality of the diverse disorders causing neonatal cholestasis in an academic center in Germany.
  
  
  METHODS
  Clinical chemistry and cause of disease were retrospectively analyzed in 82 infants (male n = 42, 51%) that had presented with neonatal cholestasis to a tertiary medical center from January 2009 to April 2013.
  
  
  RESULTS
  Altogether, 19 disorders causing neonatal cholestasis were identified. Biliary atresia was the most common diagnosis (41%), followed by idiopathic cases (13%), progressive familial intrahepatic cholestasis (PFIC, 10%), cholestasis in preterm infants (10%), α1AT deficiency, Alagille syndrome, portocaval shunts, mitochondriopathy, biliary sludge (all 2%), and others. Infants with biliary atresia were diagnosed with a mean age of 62 days, they underwent Kasai portoenterostomy ~66 days after birth. The majority of these children (~70%) received surgery within 10 weeks of age and 27% before 60 days. The 2-year survival with their native liver after Kasai procedure was 12%. The time span between Kasai surgery and liver transplantation was 176 ± 73 days. Six children (7%), of whom three patients had a syndromic and one a non-syndromic biliary atresia, died prior to liver transplantation. The pre- and post-transplant mortality rate for children with biliary atresia was ~12 and ~17%, respectively.
  
  
  CONCLUSIONS
  Neonatal cholestasis is a severe threat associated with a high risk of complications in infancy and it therefore requires urgent investigation in order to initiate life saving therapy. Although in the last 20 years new causes such as the PFICs have been identified and newer diagnostic tools have been introduced into the clinical routine biliary atresia still represents the major cause.
authors.affiliation: Department for Pediatric Nephrology, Gastroenterology, Endocrinology and Transplant Medicine, Clinic for Pediatrics II, University Children's Hospital Essen, University Duisburg-Essen , Essen , Germany ; Department of Pediatrics and Adolescent Medicine, University Children's Hospital Erlangen, Friedrich-Alexander University , Erlangen , Germany.
authors.forename: André
authors.initials: A
authors.lastname: Hoerning
authors.affiliation: Department for Pediatric Nephrology, Gastroenterology, Endocrinology and Transplant Medicine, Clinic for Pediatrics II, University Children's Hospital Essen, University Duisburg-Essen , Essen , Germany.
authors.forename: Simon
authors.initials: S
authors.lastname: Raub
authors.affiliation: Department of Gastroenterology and Hepatology, University Hospital Essen, University Duisburg-Essen , Essen , Germany.
authors.forename: Alexander
authors.initials: A
authors.lastname: Dechêne
authors.affiliation: Department for Pediatric Nephrology, Gastroenterology, Endocrinology and Transplant Medicine, Clinic for Pediatrics II, University Children's Hospital Essen, University Duisburg-Essen , Essen , Germany.
authors.forename: Michelle N
authors.initials: MN
authors.lastname: Brosch
authors.affiliation: Department for Pediatric Nephrology, Gastroenterology, Endocrinology and Transplant Medicine, Clinic for Pediatrics II, University Children's Hospital Essen, University Duisburg-Essen , Essen , Germany.
authors.forename: Simone
authors.initials: S
authors.lastname: Kathemann
authors.affiliation: Department for Pediatric Nephrology, Gastroenterology, Endocrinology and Transplant Medicine, Clinic for Pediatrics II, University Children's Hospital Essen, University Duisburg-Essen , Essen , Germany.
authors.forename: Peter F
authors.initials: PF
authors.lastname: Hoyer
authors.affiliation: Department of Pediatrics and Adolescent Medicine, University Children's Hospital , Freiburg , Germany.
authors.forename: Patrick
authors.initials: P
authors.lastname: Gerner
keywords: ERCP; Kasai procedure; biliary atresia; neonatal cholestasis; neonatal jaundice