Long QT syndrome 1

Common Name(s)

Long QT syndrome 1

Romano-Ward syndrome is the most common form of inherited long QT syndrome. Symptoms include arrhythmia, fainting, cardiac arrest, and sudden death. There are six different types of this syndrome, long QT 1 through 6. Each type is caused by a change in a different gene. The most prevalent form of long QT syndrome is long QT type 1. Long QT type 1 is caused by changes in the KCNQ1 gene. Romano-Ward syndrome is inherited in an autosomal dominant fashion.
 

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Condition Specific Organizations

Following organizations serve the condition "Long QT syndrome 1" for support, advocacy or research.

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Long QT syndrome 1" returned 43 free, full-text research articles on human participants. First 3 results:

A novel KCNQ1 nonsense variant in the isoform-specific first exon causes both jervell and Lange-Nielsen syndrome 1 and long QT syndrome 1: a case report.
 

Author(s): Motoi Nishimura, Marehiko Ueda, Ryota Ebata, Emi Utsuno, Takuma Ishii, Kazuyuki Matsushita, Osamu Ohara, Naoki Shimojo, Yoshio Kobayashi, Fumio Nomura

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According to previous KCNQ1 (potassium channel, voltage gated, KQT-like subfamily, member 1) gene screening studies, missense variants, but not nonsense or frame-shift variants, cause the majority of long QT syndrome (LQTS; Romano-Ward syndrome [RWS]) 1 cases. Several missense variants ...

Last Updated: 31 Dec 1969

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Patients With Long-QT Syndrome Caused by Impaired hERG-Encoded Kv11.1 Potassium Channel Have Exaggerated Endocrine Pancreatic and Incretin Function Associated With Reactive Hypoglycemia.
 

Author(s): Louise Hyltén-Cavallius, Eva W Iepsen, Nicolai J Wewer Albrechtsen, Mathilde Svendstrup, Anniek F Lubberding, Bolette Hartmann, Thomas Jespersen, Allan Linneberg, Michael Christiansen, Henrik Vestergaard, Oluf Pedersen, Jens J Holst, Jørgen K Kanters, Torben Hansen, Signe S Torekov

Journal: Circulation. 2017 May;135(18):1705-1719.

 

Loss-of-function mutations in hERG (encoding the Kv11.1 voltage-gated potassium channel) cause long-QT syndrome type 2 (LQT2) because of prolonged cardiac repolarization. However, Kv11.1 is also present in pancreatic α and β cells and intestinal L and K cells, secreting glucagon, ...

Last Updated: 31 Dec 1969

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Stop-codon and C-terminal nonsense mutations are associated with a lower risk of cardiac events in patients with long QT syndrome type 1.
 

Author(s): Martin H Ruwald, Xiaorong Xu Parks, Arthur J Moss, Wojciech Zareba, Jayson Baman, Scott McNitt, Jorgen K Kanters, Wataru Shimizu, Arthur A Wilde, Christian Jons, Coeli M Lopes

Journal: Heart Rhythm. 2016 Jan;13(1):122-31.

 

In long QT syndrome type 1 (LQT1), the location and type of mutations have been shown to affect the clinical outcome. Although haploinsufficiency, including stop-codon and frameshift mutations, has been associated with a lower risk of cardiac events in patients with LQT1, nonsense ...

Last Updated: 31 Dec 1969

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Reviews from the PubMed Database

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The terms "Long QT syndrome 1" returned 0 free, full-text review articles on human participants.

 
 
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Clinical Trial Information This information is provided by ClinicalTrials.gov

Comparison Between Epinephrine and Exercise Test in QT Long Syndrome Patients
 

Status: Recruiting

Condition Summary: Long QT Syndrome Type 1 or 2

 

Last Updated: 4 Oct 2016

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