Liver transplantation for children with Wilson disease: comparison of outcomes between children and adults

Arnon, Ronen; Annunziato, Rachel A.; Schilsky, Michael L.; Miloh, Tamir; Willis, Asha; Sturdevant, Mark; Sakworawich, Arnond; Suchy, Frederick J.; Kerkar, Nanda

doi:10.1111/j.1399-0012.2010.01327.x

Cited by 88 publications

(67 citation statements)

References 27 publications

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“…Outcomes for graft and patient survival for WD are excellent for patients who undergo transplantation for acute liver failure or for liver failure from chronic liver disease. 12 Whether LT is indicated in patients with predominantly neurologic manifestations is controversial. This is due to the unpredictability of resolution of neurologic manifestations posttransplantation in LT recipients with neurologic manifestations and data suggesting that posttransplantation survival is worse in patients with neurologic involvement, as well as the scarcity of donor organs.…”

Section: Liver Transplantationmentioning

confidence: 99%

Wilson disease: Clinical manifestations, diagnosis, and treatment

Schilsky

2014

Clinical Liver Disease

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Section: Liver Transplantationmentioning

confidence: 99%

Wilson disease: Clinical manifestations, diagnosis, and treatment

Schilsky

2014

Clinical Liver Disease

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“…In a study analyzing UNOS results on 170 children and 400 adults who underwent liver transplantation for FW or end-stage liver disease in the United States between 1987 and 2008, Arnon et al [102] found that both one-and five-year survival rates were similar between children and adults (90.1% and 89% vs 88.3% and 86%, respectively, P = 0.53, 0.34). 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] .…”

Section: Liver Transplantationmentioning

confidence: 79%

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

Rodríguez–Castro

Hevia‐Urrutia

Sturniolo

2015

WJH

140

123

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Wilson's disease (WD), which results from the defective ATP7B protein product, is characterized by impaired copper metabolism and its clinical consequences vary from an asymptomatic state to fulminant hepatic failure, chronic liver disease with or without cirrhosis, neurological, and psychiatric manifestations. A high grade of suspicion is warranted to not miss cases of WD, especially less florid cases with only mild elevation of transaminases, or isolated neuropsychiatric involvement. Screening in first and second relatives of index cases is mandatory, and treatment must commence upon establishment of diagnosis. Treatment strategies include chelators such as D-penicillamine and trientine, while zinc salts act as inductors of methallothioneins, which favor a negative copper balance and a reduction of free plasmatic copper. As an orphan disease, research is lacking in this field, especially regarding therapeutic strategies which are associated with better patient compliance and which could eventually also reverse established injury.

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“…74 In children, metabolic liver diseases are the second most common indication for liver transplantation after biliary atresia. Fifteen percent of children enrolled in the studies in the pediatric liver transplantation (SPLIT) registry underwent liver transplantation for metabolic diseases and 7.6% of them represented WD.…”

Section: Metabolic Liver Diseasesmentioning

confidence: 99%

Current and Future Burden of Chronic Nonmalignant Liver Disease

Udompap

Kim

2015

Clinical Gastroenterology and Hepatology

117

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Disease burden is an important indicator of the state of health of a population. It can be measured as the frequency (e.g. incidence and prevalence) of a condition or its effects including: fatal and non-fatal health loss from disease (e.g. disability-adjusted life years (DALYs)) as well as the financial costs (e.g. direct health care costs and indirect health care expenditures related to lost income due to premature death). Accurate disease burden information is essential for policy-making such as prioritization of health interventions and allocation of resources. Chronic Liver Disease (CLD) causes substantial health and economic burden in the US, where nearly two million deaths annually are attributable to CLD. In the recent past, overall mortality rate of CLD has been increasing. Viral hepatitis and alcoholic liver disease are thought to be the most common etiologies of chronic liver diseases. More recently, the prevalence of non-alcoholic steatohepatitis (NAFLD) is rapidly increasing and non-alcoholic steatohepatitis (NASH) has become a leading indication for liver transplantation. In this work, we assemble available data on the burden of CLD in the United States, focusing on non-malignant complications, whereas the impact on mortality and healthcare expenses of hepatocellular carcinoma (HCC), an important consequence of CLD is discussed elsewhere.

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Liver transplantation for children with Wilson disease: comparison of outcomes between children and adults

Abstract: LT is an excellent treatment option for patients with WD. Patients transplanted for CLD had higher patient survival rates than patients with FHF.

Cited by 88 publications

References 27 publications

Wilson disease: Clinical manifestations, diagnosis, and treatment

Wilson disease: Clinical manifestations, diagnosis, and treatment

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

Current and Future Burden of Chronic Nonmalignant Liver Disease

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