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Andersen Syndrome

A form of inherited long QT syndrome (or LQT7) that is characterized by a triad of potassium-sensitive periodic paralysis, VENTRICULAR ECTOPIC BEATS, and abnormal features such as short stature, low-set ears, and SCOLIOSIS. It results from mutations of KCNJ2 gene which encodes a channel protein (INWARD RECTIFIER POTASSIUM CHANNELS) that regulates resting membrane potential.
Also Known As:
Andersen Cardiodysrhythmic Periodic Paralysis; Andersen Cardiodysrythmic Periodic Paralysis; Andersen Tawil Syndrome; Andersen-Tawil Syndrome; Long QT Syndrome 7; Periodic Paralysis, Potassium-Sensitive Cardiodysrhythmic Type; Potassium-Sensitive Periodic Paralysis, Ventricular Ectopy, and Dysmorphic Features; Periodic Paralysis, Potassium Sensitive Cardiodysrhythmic Type; Syndrome, Andersen; Syndrome, Andersen Tawil
Networked: 93 relevant articles (6 outcomes, 8 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Bidirectional tachycardia
2. Cardiac Arrhythmias (Arrythmia)
3. Tachycardia (Tachyarrhythmias)
4. Cardiomyopathies (Cardiomyopathy)
5. Left Ventricular Dysfunction

Experts

1. Horie, Minoru: 6 articles (03/2017 - 06/2011)
2. Márquez, Manlio F: 4 articles (01/2020 - 10/2014)
3. Ohno, Seiko: 4 articles (03/2017 - 06/2011)
4. Shimizu, Wataru: 4 articles (03/2017 - 03/2008)
5. Ptácek, Louis J: 4 articles (10/2012 - 12/2003)
6. Hanna, Michael G: 3 articles (01/2022 - 04/2013)
7. Bienias, Piotr: 3 articles (01/2018 - 01/2014)
8. Kostera-Pruszczyk, Anna: 3 articles (01/2018 - 01/2014)
9. Miszczak-Knecht, Maria: 3 articles (01/2018 - 01/2014)
10. Pruszczyk, Piotr: 3 articles (01/2018 - 01/2014)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Andersen Syndrome:
1. Flecainide (Tambocor)FDA LinkGeneric
2. Inwardly Rectifying Potassium Channels (Inward Rectifier Potassium Channels)IBA
3. Potassium Channels (Potassium Channel)IBA
4. Ion Channels (Ion Channel)IBA
10/10/2017 - "Mutations in the KCNJ2 gene encoding the ion channel Kir2.1 have been linked to the Andersen-Tawil syndrome (ATS). "
10/01/2014 - "The Andersen-Tawil syndrome is a cardiac ion channel disease that is inherited in an autosomal dominant way and is classified as type 7 of the congenital long QT syndromes. "
12/01/2004 - "2. Mutations in cardiac ion channels have been identified as the basis of a wide range of inherited arrhythmia syndromes, including the congenital long QT syndromes, Brugada syndrome, Lenegre syndrome, Andersen's disease and familial atrial fibrillation. "
01/01/2013 - "In this review, we have summarized (1) the significance of unveiling mutations in genes encoding transporter-associated proteins as the cause of congenital LQTS, (2) the technique of catheter ablation applied at the right ventricular outflow tract may be curative for severely symptomatic BrS, (3) mutations with channel function modulated by protein Kinase A-dependent phosphorylation can be the culprit of CPVT mimicry in Andersen-Tawil syndrome (LQT7), (4) ablation of the ion channel anchoring protein may prevent arrhythmogenesis in Timothy syndrome (LQT8), (5) altered intracellular Ca2+ cycling can be the basis of effective targeted pharmacotherapy in CPVT, and (6) the technology of induced pluripotent stem cells is a promising diagnostic and research tool as it has become a new paradigm for pathophysiological study of patient- and disease-specific cells aimed at screening new drugs and eventual clinical application of gene therapy. "
5. Proteins (Proteins, Gene)FDA Link
6. Cyclic AMP-Dependent Protein Kinases (cAMP-Dependent Protein Kinase)IBA
7. Dichlorphenamide (Daranide)FDA Link
8. PotassiumIBA
9. Kir2.1 channelIBA
10. Acetazolamide (Diamox)FDA LinkGeneric

Therapies and Procedures

1. Catheter Ablation
2. Therapeutics
3. Induced Heart Arrest (Cardioplegia)
4. Drug Therapy (Chemotherapy)
5. Defibrillators (Defibrillator)