title: Secondary sclerosing cholangitis following major burn.
abstract: OBJECTIVE
  Secondary sclerosing cholangitis in critically ill patients (SSC-CIP) is a relatively new previously unrecognized entity which may lead to severe biliary disease with rapid progression to cirrhosis. We present for the first time a case series of patients with rapidly progressive SSC-CIP requiring aggressive intensive care treatment following major burn injury.
  
  
  RESULTS
  SSC-CIP was diagnosed in 4 consecutive patients hospitalized due to major burn injuries at our Intensive Care Unit (ICU). SSC-CIP was diagnosed when ERCP (n = 1) or MRCP (n = 3) demonstrated irregular intrahepatic bile ducts with multiple strictures and dilatations and, when a liver biopsy (n = 3) demonstrated severe cholestasis and bile duct damage. All patients were males; none of whom had pre-existing liver disease. Ages: 18-56 y. All patients suffered from severe (grade 2-3) burn injuries with total burn surface area ranging from 35 to 95%. Mean length of ICU hospitalization was 129.2 ± 53.0 days. All patients required mechanical ventilation (with a mean PEEP of 8.4 ± 2.1 cm H2O) and the administration of catecholamines for hemodynamic stabilization. All patients demonstrated severe cholestasis. Blood cultures and cultures from drained liver abscesses grew hospital acquired multiple resistant bacteria. Liver cirrhosis developed within 12 months. One patient underwent orthotopic liver transplantation. Two patients (50%) died. In conclusion, SSC-CIP following major burn injury is a rapidly progressive disease with a poor outcome. Liver cirrhosis developed rapidly. Awareness of this grave complication is needed for prompt diagnosis and considerations of a liver transplantation.
authors.affiliation: Liver Disease Center, Sheba Medical Center, Ramat Gan and Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel.
authors.forename: Ziv
authors.initials: Z
authors.lastname: Ben-Ari
authors.affiliation: Intensive Care Unit, Sheba Medical Center, Tel Aviv University, Tel Aviv, Israel.
authors.forename: David
authors.initials: D
authors.lastname: Levingston
authors.affiliation: Liver Disease Center, Sheba Medical Center, Tel Aviv University, Tel Aviv, Israel.
authors.forename: Ella
authors.initials: E
authors.lastname: Weitzman
authors.affiliation: Intensive Care Unit, Sheba Medical Center, Tel Aviv University, Tel Aviv, Israel.
authors.forename: Yael
authors.initials: Y
authors.lastname: Haviv-Yadid
authors.affiliation: Liver Disease Center, Sheba Medical Center, Ramat Gan and Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel.
authors.forename: Oranit
authors.initials: O
authors.lastname: Cohen-Ezra
authors.affiliation: Liver Disease Center, Sheba Medical Center, Tel Aviv University, Tel Aviv, Israel.
authors.forename: Peretz
authors.initials: P
authors.lastname: Weiss
authors.affiliation: Liver Unit, Carmel Medical Center, Haifa, Israel.
authors.forename: Eli
authors.initials: E
authors.lastname: Zuckerman
authors.affiliation: Department of Radiology, Sheba Medical Center, Tel Aviv University, Tel Aviv, Israel.
authors.forename: Yael
authors.initials: Y
authors.lastname: Inbar
authors.affiliation: Department of Radiology, Sheba Medical Center, Tel Aviv University, Tel Aviv, Israel.
authors.forename: Michal
authors.initials: M
authors.lastname: Amitai
authors.affiliation: Department of Radiology, Sheba Medical Center, Tel Aviv University, Tel Aviv, Israel.
authors.forename: Uri
authors.initials: U
authors.lastname: Rimon
authors.affiliation: Department of Plastic Surgery, Sheba Medical Center, Tel Aviv University, Tel Aviv, Israel.
authors.forename: Maya
authors.initials: M
authors.lastname: Goder
authors.affiliation: Department of Plastic Surgery, Sheba Medical Center, Tel Aviv University, Tel Aviv, Israel.
authors.forename: Josef
authors.initials: J
authors.lastname: Haik
mesh_terms: D000293:Adolescent; D000328:Adult; D001706:Biopsy; D002056:Burns / Q000150:complications* / Q000175:diagnosis / Q000401:mortality / Q000628:therapy; D049448:Cholangiopancreatography, Magnetic Resonance; D015209:Cholangitis, Sclerosing / Q000175:diagnosis / Q000209:etiology* / Q000401:mortality / Q000628:therapy; D016638:Critical Illness; D018450:Disease Progression; D006801:Humans; D015601:Injury Severity Score; D007362:Intensive Care Units; D007902:Length of Stay; D008297:Male; D008875:Middle Aged; D012307:Risk Factors; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult