Outcome of Living Donor Liver Transplantation for Wilson's Disease in Adults: A Single Center Experience

Choudhary, Narendra S.; Saigal, Sanjiv; Saraf, Neeraj; Rastogi, Amit; Goja, Sanjay; Bhangui, Prashant; Thiagrajan, Srinivasan; Gautam, Dheeraj; Govil, Deepak; Vohra, Vijay; Soin, Arvinder S.

doi:10.1016/j.jceh.2017.11.003

Cited by 10 publications

(12 citation statements)

References 16 publications

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“…The concept of ACLF has recently been applied in pediatric and adult WD patients. (15,36) In this study, WD patients who fulfilled the criteria for ACLF at the time of LT displayed an excellent survival rate, with a comparable postoperative course to patients with ESLD. The sickest WD patients with grade 3 ACLF at the time of LT (2/14, 14%) showed a good post-LT survival probably due to young age, short wait-list time, and the absence of mechanical ventilation at the time of surgery (Supporting Table 1).…”

Section: Discussionmentioning

confidence: 57%

“…The concept of ACLF has recently been applied in pediatric and adult WD patients . In this study, WD patients who fulfilled the criteria for ACLF at the time of LT displayed an excellent survival rate, with a comparable postoperative course to patients with ESLD.…”

Section: Discussionmentioning

confidence: 90%

“…At the time of LT, 5 (20%) patients were not receiving medical treatment, whereas 13 (52%) and 5 (20%) were taking D-penicillamine and zinc sulfate monotherapy, respectively. The median WD prognostic index, (26) Child-Pugh, and MELD scores were 10 (3-14), 11 (5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15), and 27 (6-49), respectively. More than 80% of patients were hospitalized at the time of their LT, necessitating admission to the intensive care unit (ICU) due to multiorgan failure in 6 patients.…”

Section: Characteristics Of Wd Patients At the Time Of Ltmentioning

confidence: 98%

“…Since 1971, liver transplantation (LT) has represented an effective treatment for WD patients with liver involvement because it removes the biochemical deficit and improves quality of life . The absence of postoperative disease recurrence and the young age at LT explain the excellent postoperative survival curves (1‐year graft and patient survival rates of 73%‐86% and 79%‐91%, respectively, depending also on the type of transplantation and the disease phenotype) …”

mentioning

confidence: 99%

“…(6)(7)(8) The absence of postoperative disease recurrence and the young age at LT explain the excellent postoperative survival curves (1-year graft and patient survival rates of 73%-86% and 79%-91%, respectively, depending also on the type of transplantation and the disease phenotype). (9)(10)(11)(12)(13)(14)(15) The spectrum of indications for LT in WD patients remains controversial, (6,13) especially for the mixed or exclusively neuropsychiatric phenotypes. Neuropsychiatric symptoms should theoretically improve after LT due to a normal biliary copper excretion.…”

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confidence: 99%

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Outcomes of Liver Transplant for Adults With Wilson’s Disease

et al. 2020

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Wilson’s disease (WD) is a rare genetic disorder with protean manifestations. Even if liver transplantation (LT) could represent an effective therapeutic option for patients with end‐stage liver disease, it has remained controversial in the presence of neuropsychiatric involvement. This study aimed to examine the frequency of adult LT for WD in Italy, focusing on the disease phenotype at the time of LT. A retrospective, observational, multicenter study was conducted across Italy exploring the frequency and characteristics of adults transplanted for WD between 2006 and 2016. A total of 29 adult WD patients underwent LT during the study period at 11 Italian LT centers (accounting for 0.4% of all LTs performed), and 27 of them were considered in this analysis (male/female, n = 9/18; age at LT, 29 years [19‐60 years]; median Model for End‐Stage Liver Disease score at LT, 27 [6‐49]). Isolated hepatic phenotype was the indication for LT in 17 (63%) patients, whereas 2 (7%) patients underwent LT for neurological impairment on compensated liver disease. Overall 1‐ and 5‐year patient survival was excellent (88% and 83%, respectively). Neuropsychiatric symptoms early after LT completely recovered in only a few patients. In conclusion, WD remains an uncommon, unusual indication for LT in Italy, displaying good post‐LT graft and patient survival. Because isolated neuropsychiatric involvement represents a rare indication to LT, more data are needed to properly assess the value of LT for WD in this subset of patients.

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Section: Discussionmentioning

confidence: 57%

Section: Discussionmentioning

confidence: 90%

Section: Characteristics Of Wd Patients At the Time Of Ltmentioning

confidence: 98%

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confidence: 99%

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confidence: 99%

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Outcomes of Liver Transplant for Adults With Wilson’s Disease

et al. 2020

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Liver transplantation as a treatment for Wilson’s disease with neurological presentation: a systematic literature review

et al. 2022

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Introduction Wilson’s disease (WD) is a potentially treatable, genetic disorder of copper metabolism, with survival similar to healthy populations if controlled. However, in almost 50% of WD patients, neurological symptoms persist despite treatment, and in up to 10% of patients, neurological deterioration is irreversible. International guidelines on WD treatment do not recommend liver transplantation (LT) as a treatment for neurological symptoms in WD. However, such treatment has been assessed in retrospective analyses, case and series reports. We aimed to systematically assess all available evidence on the effectiveness and safety of LT in WD patients with neurological presentation. Methods This systematic literature review was performed according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Studies were identified by searching the PubMed database (up to 6 April 2021) and by screening reference lists. Results Based on the systematic literature review, 48 articles were identified, showing outcomes of LT in 302 WD patients with neurological symptoms. Of these patients, major improvement was found in 215 cases (71.2%), with no difference in neurological status before and after LT in 21 cases (6.9%). There were 29 deaths (9.6%), neurological worsening in 24 cases (7.9%), and 13 cases (4.3%) were lost to follow-up. Conclusions The results suggest that LT is a promising method of WD management in patients with severe, neurological symptoms, particularly if the patient has not responded to pharmacological de-coppering treatment. Further studies of LT in these patients are warranted.

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