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Jervell-Lange Nielsen Syndrome (Jervell and Lange-Nielsen Syndrome)

A form of long QT syndrome that is associated with congenital deafness. It is characterized by abnormal cardioelectrophysiology involving the VOLTAGE-GATED POTASSIUM CHANNEL. It results from mutation of KCNQ1 gene (Subtype 1 or JLN1) or the KCNE1 gene (Subtype 2 or JLN2).
Also Known As:
Jervell and Lange-Nielsen Syndrome; Cardio-Auditory-Syncope Syndrome; Cardioauditory Syndrome of Jervell and Lange-Nielsen; Deafness, Congenital, and Functional Heart Disease; Jervell And Lange-Nielsen Syndrome 1; Prolonged QT Interval in EKG and Sudden Death; Surdo-Cardiac Syndrome; Cardio Auditory Syncope Syndrome; Cardio-Auditory-Syncope Syndromes; Cardioauditory Syndrome of Jervell and Lange Nielsen; Jervell And Lange Nielsen Syndrome 1; Jervell Lange Nielsen Syndrome; Jervell and Lange Nielsen Syndrome; Surdo Cardiac Syndrome; Surdo-Cardiac Syndromes; Syndrome, Cardio-Auditory-Syncope; Syndrome, Jervell-Lange Nielsen; Syndrome, Surdo-Cardiac; Syndromes, Cardio-Auditory-Syncope
Networked: 45 relevant articles (0 outcomes, 1 trials/studies)

Disease Context: Research Results

Related Diseases

1. Hearing Loss (Hearing Impairment)
2. Cardiac Sudden Death (Sudden Cardiac Arrest)
3. Romano-Ward Syndrome
4. Long QT Syndrome
5. Deafness (Deaf Mutism)

Experts

1. Früh, Andreas: 3 articles (11/2016 - 10/2005)
2. Siem, Geir: 2 articles (11/2016 - 04/2008)
3. Rydberg, Annika: 2 articles (03/2016 - 06/2013)
4. Leren, Trond P: 2 articles (04/2008 - 10/2005)
5. Cai, Cuiyun: 1 article (01/2021)
6. Li, Yihui: 1 article (01/2021)
7. Lin, Xi: 1 article (01/2021)
8. Moreno-Mata, Nicolás: 1 article (01/2021)
9. Moreno-Planas, Javier: 1 article (01/2021)
10. Ramos-Jiménez, Javier: 1 article (01/2021)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Jervell-Lange Nielsen Syndrome:
1. Potassium Channels (Potassium Channel)IBA
2. Ion Channels (Ion Channel)IBA
3. Propranolol (Inderal)FDA LinkGeneric
4. GastrinsIBA
5. DNA (Deoxyribonucleic Acid)IBA
6. Delayed Rectifier Potassium ChannelsIBA
7. Sodium Potassium Chloride Symporter Inhibitors (Loop Diuretics)IBA
8. Voltage-Gated Potassium Channels (Voltage-Gated Potassium Channel)IBA
9. Protein Subunits (Protein Subunit)IBA
10. Protein Isoforms (Isoforms)IBA

Therapies and Procedures

1. Therapeutics
2. Cochlear Implantation
3. Sympathectomy (Sympathectomies)
4. Anesthesia
5. Cochlear Implants (Cochlear Implant)