Jervell and Lange-Nielsen Syndrome
National Organization for Rare Disorders, Inc.
It is possible that the main title of the report Jervell and Lange-Nielsen Syndrome is not the name you expected.
Jervell and Lange-Nielsen syndrome (JLNS) is a rare inherited disorder characterized by deafness present at birth (congenital) occurring in association with abnormalities affecting the electrical system of the heart. The severity of cardiac symptoms associated with JLNS varies from case to case. Some individuals may have no apparent symptoms (asymptomatic); others may develop abnormally increased heartbeats (tachyarrhythmias) resulting in episodes of unconsciousness (syncope), cardiac arrest, and potentially sudden death. Physical activity, excitement, fright, or stress may trigger the onset of these symptoms. Fainting during the aforementioned activities is also a classic sign of JLNS. JLNS is usually detected during early childhood and is inherited as an autosomal recessive genetic disorder. More than half of the untreated cases of JLNS result in death before the age of 15.
Anton Jervell and Fred Lange-Nielsen provided the first complete description of the electrical conduction problem in the heart called long QT syndrome (LQTS) in 1957. LQTS refers to the QT-interval measured on the electrocardiogram that indicates that the heart muscle is taking longer than usual to recharge between beats. It predisposes those affected to tachyarrhythmias called torsade de pointes (TdP) which leads to syncope and may cause sudden cardiac death.
American Academy of Audiology
- 11480 Commerce Park Drive
- Suite 220
- Reston, VA 20191
- Tel: 703-790-8466
- Fax: 703-790-8631
- Tel: 800-222-2336
- Email: firstname.lastname@example.org
- Website: http://www.audiology.org
American Heart Association
- 7272 Greenville Avenue
- Dallas, TX 75231
- Tel: (214)784-7212
- Fax: (214)784-1307
- Tel: (800)242-8721
- Email: Review.email@example.com
- Website: http://www.heart.org
Fetal Hope Foundation
- 9786 South Holland Street
- Littleton, CO 80127
- Tel: (303)932-0553
- Tel: (877)789-4673
- Email: firstname.lastname@example.org
- Website: http://www.fetalhope.org
Genetic and Rare Diseases (GARD) Information Center
- PO Box 8126
- Gaithersburg, MD 20898-8126
- Tel: (301)251-4925
- Fax: (301)251-4911
- Tel: (888)205-2311
- Website: http://rarediseases.info.nih.gov/GARD/
Hearing Health Foundation
- 363 Seventh Avenue, 10th Floor
- New York, NY 10016-3904
- United States
- Tel: (212)257-6140
- Fax: (212)257-6139
- Tel: (866)454-3924
- Email: email@example.com
- Website: http://hearinghealthfoundation.org/home
International Long QT Syndrome Registry
- Heart Research Follow-up Program
- Box 653
- Rochester, NY 14642-8653
- Tel: (585)276-0016
- Fax: (585)273-5283
- Email: firstname.lastname@example.org
NIH/National Heart, Lung and Blood Institute
- P.O. Box 30105
- Bethesda, MD 20892-0105
- Tel: (301)592-8573
- Fax: (301)251-1223
- Email: email@example.com
- Website: http://www.nhlbi.nih.gov/
- Ronnerweg 2
- Nidau, 2560
- Tel: 41794741535
- Email: firstname.lastname@example.org
- Website: http://www.qtsyndrome.ch/index.html
Sudden Arrhythmia Death Syndromes Foundation
- 508 E. South Temple
- Suite 202
- Salt Lake City, UT 84102
- Tel: (801)531-0937
- Fax: (801)531-0945
- Tel: (800)786-7723
- Email: email@example.com
- Website: http://www.sads.org
For a Complete Report
This is an abstract of a report from the National Organization for Rare Disorders (NORD). For a full-text version of this report, go to www.rarediseases.org and click on Rare Disease Database under "Rare Disease Information".
The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only.
It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report.
This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.
Last Updated: 1/16/2013
Copyright 2013 National Organization for Rare Disorders, Inc.
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