Outcome and development of symptoms after orthotopic liver transplantation for Wilson disease

Abstract

Survival after OLT for Wilson disease with end-stage liver disease is excellent. Overall, neuropsychiatric symptoms improved after transplantation, substantiating arguments for widening of the indication for liver transplantation in symptomatic neurologic Wilson disease patients with stable liver function.

Wilson’s disease: A review of what we have learned

“…Moreover, both adults and children transplanted for chronic liver disease had better long term survival than patients transplanted for FW, although the difference was not statistically significant [107] . Neurological, and especially psychiatric [108] involvement may show little improvement with transplantation, however [27] . In a multicenter Italian study, 37 cases of patients who underwent liver transplantation, 8 for FW and 29 for WD-related chronic liver disease, were analyzed, demonstrating decreased survival in patients who previous to transplant had neuropsychiatric manifestations, in spite of neurological improvement after transplantation [103] .…”

Right lobe liver transplantation in patient with fulminant form of the Wilson’s disease from AB0-incompatible relative donor

“…Час-то клиника фульминантной формы болезни Вильсо-на-Коновалова возникает у пациентов с уже цирро-тически измененной печенью [3,4].…”

“…Концентрация меди в печени может быть ценным диагностическим критерием, однако в связи с коа-гулопатией и тромбоцитопенией у пациентов с ос-трой печеночной недостаточностью возможно при-менение лишь трансъюгулярной биопсии печени. У пациентов с ранее диагностированной болезнью Вильсона-Коновалова при развитии фульминант-ной формы помимо вышеперечисленных симпто-мов наблюдаются желтуха и тяжелая коагулопатия с тромбоцитопенией [4][5][6].…”

Neurological manifestations in Wilson’s disease –possible treatment options for symptoms

“…[60] Based on the known WD etiology, LT has been suggested as a treatment option for WD since 1971, as it is supposed to ultimately treat the disruption of copper metabolism in the liver and prevent further copper deposition in other organs. [1][2][3][67][68][69][70][71] Because WD is usually responsive to pharmacotherapy, this option is (according to hepatological WD guidelines) reserved only for patients with acute liver failure or decompensated liver cirrhosis (based on appropriate scales -Nazer index, Kings College Wilson Disease Prognostic Index). [1,2] However, as mentioned in the introduction section, some patients with WD and neurological symptoms do not respond to or may even deteriorate during pharmacological anti-copper therapy.…”