No increased risk of hepatocellular carcinoma in cirrhosis due to <scp>W</scp>ilson disease during long‐term follow‐up

Meer, Skadi van der; Man, Robert A. de; Berg, Aad P. van den; Houwen, Roderick H. J.; Linn, F.; Oijen, Martijn G.H. van; Siersema, Peter D.; Erpecum, Karel J. van

doi:10.1111/jgh.12716

Cited by 51 publications

(31 citation statements)

References 24 publications

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“…In addition, HCC has been reported in patients with Wilson disease. However, a recent report indicated that the risk of HCC was low in Wilson disease even in cases of cirrhosis [65]. …”

Section: Risk Factors For Hccmentioning

confidence: 99%

Asia–Pacific clinical practice guidelines on the management of hepatocellular carcinoma: a 2017 update

et al. 2017

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There is great geographical variation in the distribution of hepatocellular carcinoma (HCC), with the majority of all cases worldwide found in the Asia–Pacific region, where HCC is one of the leading public health problems. Since the “Toward Revision of the Asian Pacific Association for the Study of the Liver (APASL) HCC Guidelines” meeting held at the 25th annual conference of the APASL in Tokyo, the newest guidelines for the treatment of HCC published by the APASL has been discussed. This latest guidelines recommend evidence-based management of HCC and are considered suitable for universal use in the Asia–Pacific region, which has a diversity of medical environments.

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“…In addition, HCC has been reported in patients with Wilson disease. However, a recent report indicated that the risk of HCC was low in Wilson disease even in cases of cirrhosis [65]. …”

Section: Risk Factors For Hccmentioning

confidence: 99%

Asia–Pacific clinical practice guidelines on the management of hepatocellular carcinoma: a 2017 update

et al. 2017

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“…Copper accumulation in WD is said to be protective against tumorigenesis. The estimated annual risk of HCC from a Dutch study on 140 adults with WD followed up over 15 years was calculated to be 0.09%[ 84 ]. From another multicenter European study of 1186 WD patients, the prevalence of HCC was found to be 0.67%[ 85 ].…”

Section: Pathogenic Risk Factorsmentioning

confidence: 99%

Pediatric hepatocellular carcinoma

Khanna

Verma

2018

WJG

113

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Pediatric hepatocellular carcinoma (HCC) is the second common malignant liver tumor in children after hepatoblastoma. It differs from the adult HCC in the etiological predisposition, biological behavior and lower frequency of cirrhosis. Perinatally acquired hepatitis-B virus, hepatorenal tyrosinemia, progressive familial intrahepatic cholestasis, glycogen storage disease, Alagille’s syndrome and congenital portosystemic shunts are important predisposing factors. Majority of children (87%) are older than 5 years of age. Following mass immunization against hepatitis-B, there has been a drastic fall in the incidence of new cases of pediatric HCC in the Asia-Pacific region. Management is targeted on complete surgical removal either by resection or liver transplantation. There is a trend towards improving survival of children transplanted for HCC beyond Milan criteria. Chemotherapeutic regimens do not offer good results but may be helpful for down-staging of advanced HCC. Surveillance of children with chronic liver diseases with ultrasound and alpha-fetoprotein may be helpful in timely detection, intervention and overall improvement in outcome of HCC.

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“…[88][89][90] Some authors do not advocate routine hepatoma surveillance in cirrhosis related to Wilson's disease. 8,91 In practice however, clinicians will often opt for hepatoma surveillance, especially in the elderly.…”

Section: Initial Therapymentioning

confidence: 99%

Insights into the management of Wilson’s disease

Kathawala

Hirschfield

2017

Therap Adv Gastroenterol

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Abstract:Wilson's disease is a rare, inherited autosomal recessive disease of copper metabolism, in which the causative gene, ATP7B, results in absent or reduced function of the ATP7B transporter important for biliary excretion of copper and incorporation of copper into caeruloplasmin. Affected patients accumulate excessive copper within the liver, brain and other tissues. A disease mainly of children, adolescents and young adults; clinical features vary from the asymptomatic state to chronic liver disease, acute liver failure, and neuropsychiatric manifestations. Diagnosis requires a high index of suspicion and is based on a combination of clinical signs, biochemical tests, hepatic copper content assay and mutation analysis of the ATP7B gene; to date, there are more than 500 mutations of ATP7B in patients with Wilson's disease. Early recognition and treatment can result in an excellent prognosis whereas untreated disease is almost always fatal. Drug therapies include chelating agents, such as penicillamine or trientine, and zinc salts. Liver transplantation is curative correcting the underlying pathophysiology and is traditionally indicated in acute liver failure or end-stage liver disease refractory to medical therapy. This review provides an overview of various aspects of Wilson's disease including molecular basis of the disease, clinical features, diagnostic and management strategies with their current limitations.

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No increased risk of hepatocellular carcinoma in cirrhosis due to Wilson disease during long‐term follow‐up

Cited by 51 publications

References 24 publications

Asia–Pacific clinical practice guidelines on the management of hepatocellular carcinoma: a 2017 update

Asia–Pacific clinical practice guidelines on the management of hepatocellular carcinoma: a 2017 update

Pediatric hepatocellular carcinoma

Insights into the management of Wilson’s disease

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