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Liver transplantation as a treatment for Wilson's disease with neurological presentation: a systematic literature review
Acta Neurol Belg. 2022 Apr;122(2):505-518. doi: 10.1007/s13760-022-01872-w.Epub 2022 Jan 26.
Tomasz Litwin#1, Jan Bembenek#2, Agnieszka Antos3, Adam Przybylkowski4, Marta Skowronska3, Iwona Kurkowska-Jastrzebska3, Anna Czlonkowska3
Second Department of Neurology, Institute of Psychiatry and Neurology, Sobieskiego 9, 02-957, Warsaw, Poland. firstname.lastname@example.org.
Department of Clinical Neurophysiology, Institute of Psychiatry and Neurology, Warsaw, Poland.
Second Department of Neurology, Institute of Psychiatry and Neurology, Sobieskiego 9, 02-957, Warsaw, Poland.
Department of Gastroenterology and Internal Medicine, Medical University of Warsaw, Warsaw, Poland.
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.