Navigation Links
Danon Disease Can Be Quickly Fatal, Study Finds
Date:3/24/2009

Heart experts stress the importance of genetic testing and diagnosis

TUESDAY, March 24 (HealthDay News) -- A rare genetic heart disorder called Danon disease progresses rapidly and is often deadly in young people, according to a new study.

The recently recognized disease is a type of cardiomyopathy, or heart muscle disorder, linked to genetic mutations in the lysosome-associated membrane protein gene (LAMP2). Until now, the natural course of the disease was unclear.

Researchers from the Minneapolis Heart Institute Foundation studied seven people diagnosed with Danon disease when they were 7 to 17 years old. In six of them, diagnosis was made as the result of a heart murmur, family screening and findings on routine electrocardiogram tests or from such symptoms as chest pain and fainting. In one person, atrial fibrillation, an abnormal heart rhythm, led to the diagnosis.

Within nine years of diagnosis, on average, all seven had encountered serious problems. One person had a heart transplant, and four died of acute or progressive heart failure. Their deterioration was often rapid, going from having few or no symptoms to end-stage heart failure within as little as six months.

The other two people in the study had sudden, unexpected, major arrhythmic events, and one of them died suddenly from ventricular fibrillation -- very rapid, uncoordinated contractions of the ventricles -- that was not controlled by an implantable cardioverter defibrillator.

All seven developed problems with contraction of their left heart ventricle, called left ventricular systolic dysfunction. All seven had a defibrillator implanted, but in five of them, the devices failed to prevent lethal tachyarrhythmias -- excessively rapid heartbeat accompanied by irregular heartbeat.

The final electrocardiograms examined by the researchers showed significant enlargement of the left ventricle in all seven, the study reported.

"The clinical course of these seven patients with LAMP2 mutations provides important insights regarding molecular diagnosis as well as the natural history, pathophysiology and clinical implications of this recently recognized genetic cardiomyopathy," the researchers wrote. "LAMP 2 mutations cause a particularly profound and accelerated cardiac disease process characterized by clinical deterioration and early death, perhaps representing one of the most lethal cardiomyopathies in young and usually male patients. Such an outcome occurred in the patients in our study, despite application of the most contemporary treatment strategies."

The finding "establishes the importance of molecular diagnosis and underscores the utility of genetic testing," they concluded.

The study is in the March 25 issue of the Journal of the American Medical Association.

More information

The American Heart Association has more about cardiomyopathy.



-- Robert Preidt



SOURCE: JAMA/Archives journals, news release, March 24, 2009


'/>"/>
Copyright©2009 ScoutNews,LLC.
All rights reserved  

Related medicine news :

1. Graham Packaging On-Site Plant in Zoetermeer Wins Top Performance Award From The Danone Baby Nutrition and Medical Nutrition Divisions
2. Smoking may strongly increase long-term risk of eye disease
3. Pot bellies linked to early signs of cardiovascular disease
4. Anemia and tropical diseases; Is pharmacogenomics ready for the clinic?
5. Radiologists encouraged to look beyond cancer for clinically unseen diseases
6. Use of certain lipid measures not more effective in predicting coronary heart disease
7. Role seen for cannabis in helping to alleviate allergic skin disease
8. New prion protein discovered by Canadian scientists may offer insight into mad cow disease
9. Effectiveness of mouse breeds that mimic Alzheimers disease symptoms questioned
10. New research shows how chronic stress worsens neurodegenerative disease course
11. A new molecular zip code, and a new drug target for Huntingtons disease
Post Your Comments:
*Name:
*Comment:
*Email:
Related Image:
Danon Disease Can Be Quickly Fatal, Study Finds
(Date:1/21/2017)... ... January 21, 2017 , ... Pacifica ... Travis-Teague, the electrifying line-up of events for its annual meeting “Coming Home 2017,” ... community. “Coming Home 2017” will be held on Friday January 27 through ...
(Date:1/21/2017)... ... January 21, 2017 , ... In the United States, 20 million women ... of recovery, they often feel shame for having struggled with an eating disorder as ... the workshop, “Rising Strong in Life After an Eating Disorder” -- to be featured ...
(Date:1/20/2017)... (PRWEB) , ... January 20, 2017 , ... A new ... donate items they no longer use or need, from clothes to couches to dressers ... unwanted items and take them to the nearest Goodwill donation center through February 28th. ...
(Date:1/20/2017)... ... ... Doctor C LLC, a company based out of Arizona that focuses on ... marketing and distribution of its product, The Right C. , The Right C is ... traditional vitamin C supplements. At the trade show, Doctor C had the opportunity to ...
(Date:1/20/2017)... ... January 20, 2017 , ... ATP Science, an ... lives, recently attended the January ECRM Trade Show in Hilton Head, SC, benefiting ... its large range of supplements that keep the body functioning at its peak ...
Breaking Medicine News(10 mins):
(Date:1/21/2017)... 20, 2017 ResMed (NYSE: RMD ), BMC ... Winter Haven, Florida ) today announced they have agreed on ... 3B will be permitted to sell their existing products in exchange ... settlement payment to 3B to close the Florida ... an admission of liability or wrongdoing by any party. ...
(Date:1/21/2017)... , Jan 20, 2017 Research and Markets ... Analysis & Trends - Technology, Route Of Administration, End User - ... ... is poised to grow at a CAGR of around 7.8% over ... This industry report analyzes the global markets for Advanced ...
(Date:1/20/2017)... 2017  Ethicon Endo-Surgery, Inc. announced today ... Inc., a privately held medical device company ... used in operating rooms worldwide. The acquisition ... energy devices with Megadyne,s innovative portfolio of ... in Ethicon,s goal to deliver the most ...
Breaking Medicine Technology: