title: Unusual Indications for a Liver Transplant: A Single-Center Experience.
abstract: OBJECTIVE
  This study sought to evaluate the efficacy of liver transplant for unusual liver diseases.
  
  
  METHODS
  The results of 476 patients who underwent liver transplant from 1988 to January 2015 were retrospectively analyzed. Two hundred forty-five of them were adult patients and 231 of them were pediatric. Thirty-one patients had unusual liver disease.
  
  
  RESULTS
  Of the 31 patients with unusual liver disease, 9 (29%) were adult and 22 (71%) were pediatric patients. In the pediatric group, indications for liver transplant were Alagille syndrome (n = 5), Crigler-Najjar syndrome type 1 (n = 5), glycogen storage disease (n = 3), oxalosis (n = 3), familial hypercholesterolemia (n = 2), alpha-1-antitrypsin deficiency (n = 2), Caroli disease (n = 1), and cystic neuroblastoma metastasis (n = 1). Six patients (27.2%) had acute rejection episodes and were successfully treated with pulse steroids. In 2 patients, retransplant was performed for chronic rejection. Three patients died during follow-up (13.6%): 2 because of sepsis and 1 because of cranial hemorrhage. In the adult group, indications for liver transplant were neuroendocrine tumor metastasis (n = 1), liver angiosarcoma (n = 1), familial hypercholesterolemia (n = 2), alveolar hydatid disease (n = 2), cystic fibrosis (n = 1), congenital hepatic fibrosis (n = 1), and oxalosis (n = 1). Four patients (44.4%) had acute rejection episodes and were successfully treated with pulse steroid therapy. One patient died due to the recurrence of primary disease (liver angiosarcoma) during follow-up (11.1%).
  
  
  CONCLUSIONS
  Advances in liver transplant and our understanding about unusual liver disease have led to significant improvements in managing these diseases. Liver transplant effectively treats the underlying defect and the complications of portal hypertension, or risk of malignancy for those disorders, in which the liver is affected.
authors.affiliation: Department of General Surgery, Baskent University, Ankara, Turkey.
authors.forename: Aydincan
authors.initials: A
authors.lastname: Akdur
authors.forename: Mahir
authors.initials: M
authors.lastname: Kirnap
authors.forename: Ebru H
authors.initials: EH
authors.lastname: Ayvazoglu Soy
authors.forename: Figen
authors.initials: F
authors.lastname: Ozcay
authors.forename: Gokhan
authors.initials: G
authors.lastname: Moray
authors.forename: Gulnaz
authors.initials: G
authors.lastname: Arslan
authors.forename: Mehmet
authors.initials: M
authors.lastname: Haberal
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D058625:End Stage Liver Disease / Q000175:diagnosis / Q000209:etiology / Q000401:mortality / Q000601:surgery*; D005260:Female; D006084:Graft Rejection / Q000276:immunology / Q000517:prevention & control; D006085:Graft Survival; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007223:Infant; D016031:Liver Transplantation / Q000009:adverse effects / Q000401:mortality; D008297:Male; D012189:Retrospective Studies; D012307:Risk Factors; D013997:Time Factors; D016896:Treatment Outcome; D014421:Turkey; D055815:Young Adult
chemical_list: D007166:Immunosuppressive Agents