Navigation Links
EASL publishes first European Clinical Practice Guidelines for Wilson's disease
Date:2/7/2012

Geneva, Switzerland: The first European Clinical Practice Guidelines (CPGs) for the diagnosis and management of Wilson's disease are published today by the European Association for the Study of the Liver (EASL) on the EASL website -- www.easl.eu.(1) Developed to assist physicians and healthcare providers in the clinical decision making process, the guidelines describe best practice for the diagnosis and treatment of patients with Wilson's disease -- a rare genetic(2) disorder that, if left untreated, is fatal.

Approximately one in 30,000 people worldwide are affected by Wilson's disease -- a condition in which copper is not excreted by the body effectively, leading to excess copper build up, liver failure and damage to the brain. While Wilson's disease may manifest at any age, the majority of patients present between the ages of 5 and 35.

Lead author Professor Peter Ferenci said: "The clinical presentation of Wilson's disease can vary widely, but it must be considered in any patient who presents with a combination of unexplained liver disease and neurological or neuropsychiatric disorders. In the absence of Kayser-Fleischer rings(3) -- which are typical, but not always present -- the guidelines recommend measurement of urinary copper excretion and hepatic parenchymal copper as diagnostic methods of choice. Notably, age alone should not be the basis for eliminating a diagnosis of Wilson's disease."

The CPGs, based on a systematic review of existing literature, provide best practice diagnosis and treatment protocols with an emphasis on:

  • Clinical presentation and prognosis
  • Diagnostic strategies (e.g. serum ceruloplasmin, basal 24-hour urinary copper excretion, genetic analysis)
  • Importance of family screening
  • Treatment options (e.g. chelating agents, zinc, liver transplantation)

With treatment, prolonged survival has become the norm for Wilson's disease patients. The guidelines recommend chelating agents -- drugs that bind to copper and remove it from the body (D-penicillamine or trientine) -- as the initial treatment for symptomatic patients and that, unless liver transplantation is performed, treatment is maintained for life.

Professor Roderick Houwen added: "Unfortunately, as there are no optimally designed randomized controlled trials conducted in Wilson's disease, there is a lack of high-quality evidence to estimate the relative treatment effects of the available drugs. Our evaluation is mostly based on large case series that have been reported in recent decades, which highlights a clear need to conduct more robust randomized controlled trials to better understand treatment for this rare condition."

Professor Mark Thurz, EASL Secretary General, added: "EASL is dedicated to promoting hepatology research and education to improve the worldwide treatment of liver disease. Its series of Clinical Practice Guidelines aims to promote best practice to drive better clinical outcomes and inform both the scientific community and the wider public of the latest developments in the field. We hope these new Wilson's disease guidelines provide clinicians with the most up-to-date, evidence based methods for the management of affected patients."

The Wilson's disease CPGs will be published in the March issue (Volume 56, No. 3) of the Journal of Hepatology -- EASL's official journal.


'/>"/>

Contact: Travis Taylor
easlpressoffice@cohnwolfe.com
44-207-331-5472
European Association for the Study of the Liver
Source:Eurekalert

Related biology news :

1. Elseviers Maturitas publishes position statement on the role of vitamin D in postmenopausal women
2. Bulletin of the Atomic Scientists publishes Japanese translation of special Fukushima issue
3. Dr. Michael Collins publishes paper on ivory-billed woodpecker
4. ESHRE publishes English textbook for paramedics
5. Census of Marine Life publishes historic roll call of species in 25 key world areas
6. Nano Letters publishes Dr. Yong Shis energy harvesting technology
7. NASA scientist publishes new book: Coming Climate Crisis? Consider the Past, Beware the Big Fix
8. Endangered Species Research publishes theme section on biologging science
9. International Biometric Society co-publishes journal with Springer
10. AIBS publishes Darwin articles open access
11. JAX publishes online tool for exploring autoimmune disease gene networks
Post Your Comments:
*Name:
*Comment:
*Email:
(Date:6/1/2016)... -- Favorable Government Initiatives Coupled With Implementation ... to Boost Global Biometrics System Market Through 2021  ... " Global Biometrics Market By Type, By End ... - 2021", the global biometrics market is projected to ... growing security concerns across various end use sectors such ...
(Date:5/9/2016)... 2016 Elevay is currently known ... freedom for high net worth professionals seeking travel for ... connected world, there is still no substitute for a ... sealing your deal with a firm handshake. This is ... advantage of citizenship via investment programs like those offered ...
(Date:4/26/2016)... , April 27, 2016 ... the  "Global Multi-modal Biometrics Market 2016-2020"  report to ... ) , The analysts forecast the ... CAGR of 15.49% during the period 2016-2020.  ... number of sectors such as the healthcare, BFSI, ...
Breaking Biology News(10 mins):
(Date:6/23/2016)... BEACH, Calif. , June 23, 2016  Blueprint ... new biological discoveries to the medical community, has closed ... co-founder Matthew Nunez . "We have ... us with the capital we need to meet our ... will essentially provide us the runway to complete validation ...
(Date:6/23/2016)... ... June 23, 2016 , ... ClinCapture, the only free validated ... will showcase its product’s latest features from June 26 to June 30, 2016 ... on Disrupting Clinical Trials in The Cloud during the conference. DIA (Drug ...
(Date:6/23/2016)... 22, 2016  Amgen (NASDAQ: AMGN ) ... QB3@953 life sciences incubator to accelerate the development ... laboratory space at QB3@953 was created to help high-potential ... for many early stage organizations - access to laboratory ... Amgen launched two "Amgen Golden Ticket" awards, providing each ...
(Date:6/22/2016)... June 22, 2016 Research and Markets has ... report to their offering. ... 2014 from $29.3 billion in 2013. The market is expected to ... from 2015 to 2020, increasing from $50.6 billion in 2015 to ... forecasts during the forecast period (2015 to 2020) are discussed. As ...
Breaking Biology Technology: