Navigation Links
European-wide study confirms benefits of D-penicillamine and trientine for Wilson disease
Date:3/31/2011

Results from the first ever European-wide retrospective analysis presented today at the International Liver CongressTM have shown both D-penicillamine and trientine continue to be effective treatments, providing positive survival rates in patients with Wilson disease free from a liver transplant.

Wilson disease is a rare genetic storage disorder in which copper is not excreted by the body effectively, leading to excess copper build up, liver failure and damage to the brain (neurological problems). Worldwide the condition, affects approximately one in 30,000 people. If not treated, Wilson disease can be chronically debilitating and life threatening. While current guidelines recommend the use of chelating agents (D-penicillamine, trientine) as first line therapy of symptomatic patients, optimal treatment regimens are yet to be established.

In this retrospective multicentre cohort study the long-term outcomes of treatment with D-penicillamine and trientine were reviewed in 347 patients with the condition. Changes of medication were common in both groups and resulted in a total of 467 analysed treatments (D-penicillamine, n=326, trientine n=141). Following an average follow-up of 16.5 years, both treatments provided a similar and excellent survival rate and prevented disease progression with the need for liver transplantation in over 98% of patients.

Daniele Prati, EASL's Scientific Committee Member and Press Committee Chairman commented: "Not only does this study show that both treatments are effective options for patients with this rare and often debilitating condition, but it is also encouraging to see how European countries can work together and exchange data which is extremely valuable to guide best practice and evaluate existing treatment regimens. Research on rare liver diseases presents remarkable interest."

"To date, a very limited number of drugs for these conditions are marketed, leaving the majority of rare diseases without any effective treatment. Research into rare diseases and possible cures needs to be strongly encouraged."

Follow-up at 48 months showed that liver deterioration occurred in only 8 out of 515 treatments (D-penicillamine n=4, trientine n=4) while neurological deterioration was less frequent in the D-penicillamine group (7/326 treatments) compared to trientine (12/141, p≤=0.05). Improvements in liver function of symptomatic patients was similar between groups when data were stratified between first and second line treatments, with improvement rates during first line therapy in symptomatic patients of 90.7% for D-penicillamine and 92.6% for trientine.


'/>"/>

Contact: Dimple Natali
easlpressoffice@cohnwolfe.com
44-790-013-8904
European Association for the Study of the Liver
Source:Eurekalert

Related biology news :

1. UC Riverside researcher receives $9 million USDA grant to study potato and tomato disease
2. New study shows you can have your candy and eat it too -- without adverse health effects
3. Study: Emissions trading doesnt cause pollution hot spots
4. Butterfly study reveals traits and genes associated with establishment of new populations
5. Study shows hunger hitting closer to home
6. Most states unclear about storage, use of babies blood samples, new study finds
7. GPS study shows wolves more reliant on a cattle diet
8. Russian boreal forests undergoing vegetation change, study shows
9. Spicing up your love life possible, study finds
10. Study finds weight training safe for pregnant women
11. Study predicts large regional changes in farmland area
Post Your Comments:
*Name:
*Comment:
*Email:
(Date:7/20/2017)... , July 20, 2017 Delta (NYSE: DAL ) ... any Delta aircraft at Reagan Washington National Airport (DCA). ... Delta launches biometrics to board ... Delta,s biometric boarding ... Club is now integrated into the boarding process to allow eligible ...
(Date:6/23/2017)... N.Y. , June 23, 2017  IBM (NYSE: ... dairy research, today announced a new collaboration using next-generation ... chances that the global milk supply is impacted by ... Cornell University has become the newest academic institution to ... a food safety initiative that includes IBM Research, Mars, ...
(Date:5/23/2017)... first robotic gym for the rehabilitation and functional motor sense evaluation of ... Italy . The first 30 robots will be available from June ... . The technology was developed and patented at the IIT laboratories and ... thanks to a 10 million euro investment from entrepreneur Sergio Dompè. ... ...
Breaking Biology News(10 mins):
(Date:10/11/2017)... ... ... a basic first aid supply for any work environment, but most personal eye wash can ... a dangerous substance enters both eyes? It’s one less decision, and likely quicker response time ... , “Whether its dirt and debris, or an acid or alkali, getting anything in your ...
(Date:10/11/2017)... ... October 11, 2017 , ... ... will be hosting a Webinar titled, “Pathology is going digital. Is your lab ... digital pathology adoption best practices and how Proscia improves lab economics and realizes ...
(Date:10/11/2017)... LAGUNA HILLS, Calif. , Oct. 11, 2017 ... London (ICR) and University of ... prognostic tool to risk-stratify patients with multiple myeloma (MM), in ... nine . The University of Leeds ... by Myeloma UK, and ICR will perform the testing services ...
(Date:10/11/2017)... ... October 11, 2017 , ... A new study ... in frozen and fresh in vitro fertilization (IVF) transfer cycles. The ... IVF success. , After comparing the results from the fresh and frozen transfer ...
Breaking Biology Technology: