The clinical presentation and prognosis of patients with Congenital Hepatic Fibrosis
MM Kirstein, F Puls, T Krech, J Schlue, ME Dämmrich, S Pischke, HH Kreipe, MP Manns, A Vogel · Zeitschrift für Gastroenterologie · 2013 · Heft 01
Bibliografische Angaben
Zusammenfassung
Background: Congenital hepatic fibrosis (CHF) is a rare developmental disorder caused by ductal plate malformation (DPM). So far, CHF has been described as a multiorgan disorder occurring in child- or young adulthood. In this study, we investigated 17 cases of CHF and their association with other diseases, gender distribution, age of clinical occurrence, form of clinical, morphological and laboratory presentation, long-term course and survival after transplantation. Methods: 17 cases of histopathologically diagnosed CHF were retrospectively identified using clinical, imaging and laboratory findings. Results: The…
