Indications and results of liver transplants in Wilson’s disease

Robles, R; Parrilla, Pascual; Sicilia, J; Ramı́rez, Pablo; Bueno, F.S; Rodrı́guez, José Manuel; Luján, Juan; Fernández, José Antonio; López, José Antonio García

doi:10.1016/s0041-1345(99)00414-5

Cited by 14 publications

(8 citation statements)

References 10 publications

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“…The response to the penicillamine/zinc chelation treatment in our series appears to be excellent, even in the presence of severe liver damage, having been successful in 4 children who presented with ALF. Furthermore, unlike other studies,17–19 none of the children on long‐term chelation in the present study, most of whom were taking penicillamine and zinc, has required liver transplantation over a median follow up period of 11.8 (1.45–34.2) years.…”

Section: Discussionmentioning

confidence: 56%

“…The mortality of those patients who present with fulminant hepatic failure, i.e., ALF and encephalopathy, is 100%,11 and they should be immediately listed for urgent transplantation. Although it has been suggested that liver transplantation should also be considered in those patients on chelation therapy who do not achieve normal liver function after 3 months of treatment,17 our data show that, at least in pediatrics, transplantation soon after diagnosis has very little, if any, role to play in WD not presenting with ALF. Thus, none of our patients on long‐term chelation therapy has required a liver transplant during follow‐up, even though some have taken a long time to normalize liver function and others continue to have abnormal transaminases up to 14 years after diagnosis.…”

Section: Discussionmentioning

confidence: 60%

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Wilson's disease in children: 37-Year experience and revised King's score for liver transplantation

et al. 2005

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Wilson's disease (WD) is a rare liver-based disorder of copper metabolism. Prognostic criteria described by our group in 1986 to predict death without transplantation have not been universally validated. The clinical features of 88 children were reviewed, retrospectively in 74 and prospectively in 14. Data from the retrospectively recruited patients that died or survived on long-term chelation were used to evaluate the validity of our old scoring system and to devise a new prognostic index, then assessed in the 14 prospectively recruited patients. Using the old scoring system, 5 children scoring > 7, the cutoff value for death without transplantation, survived, whereas 4 scoring < 7 died (sensitivity 87% and specificity 90%). A new index based on serum bilirubin, international normalized ratio, aspartate aminotransferase (AST), and white cell count (WCC) at presentation identified a cutoff score of 11 for death and proved to be 93% sensitive and 98% specific, with a positive predictive value of 88%. When the new index was evaluated prospectively in 14 patients, it predicted the need for transplantation in only the 4 who required it, although 1 child with a score of 11 survived on medical treatment. In conclusion, the new Wilson Index is more sensitive and specific in predicting mortality without transplantation than the old scoring system, but needs to be validated in a larger number of patients. (Liver Transpl 2005;11:441-448.)

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Section: Discussionmentioning

confidence: 56%

Section: Discussionmentioning

confidence: 60%

Wilson's disease in children: 37-Year experience and revised King's score for liver transplantation

et al. 2005

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“…El presente estudio ninguno de los pacientes con otro tipo de presentación clínica y en terapia quelante crónica requirió trasplante hepático, lo que contrasta con lo descrito por otros autores 8,17 .…”

Section: Discussionunclassified

Enfermedad de Wilson: experiencia pediátrica en Costa Rica

Jiménez

Cambronero

Morales

et al. 2009

Gastroenterología y Hepatología

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“…Currently, only case reports describing LT as a treatment for neurological symptoms in patients with WD have been published, with conflicting results. [67][68][69][70][71][72][73][74][75][76][77][78][79] Stracciari et al reported on 41 patients with WD and neurological symptoms who underwent LT; however, only 5 cases were transplanted because of neurological deterioration while the remaining cases were transplanted because of hepatic indications. [72] In this study, neurological improvement or stabilization was observed in 78% of patients, whereas four patients (10%) died from LT complications.…”

Section: Other Neurological Features Of Wdmentioning

confidence: 99%