INTRODUCTION: Although liver transplantation is a definitive cure for Wilson's disease (WD), there is limited data about results of living donor liver transplantation (LDLT) in adults. MATERIAL AND METHODS: 18 adults underwent LDLT for WD. The presentations before LDLT were decompensated cirrhosis (n = 16), acute on chronic liver failure (n = 1) and acute liver failure (n = 1). The donors were parents (n = 2), siblings (n = 3), cousin (n = 1), daughter (n = 1), nephew (n = 1), spouse or relatives of spouse (n = 9) and from swap transplantation (n = 1). All genetically related donors were negative for screening of WD. RESULTS: The study cohort comprised of 15 males and 3 females, aged 32 ± 10 years. Severity of liver disease (excluding acute liver failure patient) was as follows; Child's score 10 ± 2, model for end-stage liver disease (MELD) score 18 ± 6. The graft to recipient weight ratio was 1 ± 0.2. The ICU and hospital stay were 5.5 ± 0.9 and 15 ± 5 days. Two patients died in first month after liver transplantation, rest of patients are doing well at median 15 (8-38 months). Two patients had acute cellular rejection that responded to steroids, one had hepatic artery thrombosis and 2 had biliary strictures. Three patients had neurological symptoms; 2 of these patients had partial recovery while one had complete recovery. There was no significant difference between LDLT from genetically related or unrelated donors. CONCLUSION: LDLT for WD in adults is associated with good outcomes.
INTRODUCTION: Although liver transplantation is a definitive cure for Wilson's disease (WD), there is limited data about results of living donor liver transplantation (LDLT) in adults. MATERIAL AND METHODS: 18 adults underwent LDLT for WD. The presentations before LDLT were decompensated cirrhosis (n = 16), acute on chronic liver failure (n = 1) and acute liver failure (n = 1). The donors were parents (n = 2), siblings (n = 3), cousin (n = 1), daughter (n = 1), nephew (n = 1), spouse or relatives of spouse (n = 9) and from swap transplantation (n = 1). All genetically related donors were negative for screening of WD. RESULTS: The study cohort comprised of 15 males and 3 females, aged 32 ± 10 years. Severity of liver disease (excluding acute liver failure patient) was as follows; Child's score 10 ± 2, model for end-stage liver disease (MELD) score 18 ± 6. The graft to recipient weight ratio was 1 ± 0.2. The ICU and hospital stay were 5.5 ± 0.9 and 15 ± 5 days. Two patients died in first month after liver transplantation, rest of patients are doing well at median 15 (8-38 months). Two patients had acute cellular rejection that responded to steroids, one had hepatic artery thrombosis and 2 had biliary strictures. Three patients had neurological symptoms; 2 of these patients had partial recovery while one had complete recovery. There was no significant difference between LDLT from genetically related or unrelated donors. CONCLUSION: LDLT for WD in adults is associated with good outcomes.
Entities:
Keywords:
LDLT, living donor liver transplantation; MELD, model for end-stage liver disease; WD, Wilson's disease; Wilson's disease; complications; living donor liver transplantation; survival
Authors: M A Yagci; A Tardu; S Karagul; I Ertugrul; V Ince; S Kirmizi; B Unal; B Isik; C Kayaalp; S Yilmaz Journal: Transplant Proc Date: 2015-06 Impact factor: 1.066
Authors: Xue-hao Wang; Feng Cheng; Feng Zhang; Xiang C Li; Lian B Kong; Guo Q Li; Jun Li; Xiao F Qian Journal: Transpl Int Date: 2005-06 Impact factor: 3.782
Authors: Tomasz Litwin; Jan Bembenek; Agnieszka Antos; Adam Przybyłkowski; Marta Skowrońska; Iwona Kurkowska-Jastrzębska; Anna Członkowska Journal: Acta Neurol Belg Date: 2022-01-26 Impact factor: 2.396