Andersen Syndrome

**Definition:** 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.

**Tree numbers:** - C14.280.067.565.070 - C14.280.123.625.070 - C16.131.240.400.715.070 - C23.550.073.547.070

**Synonyms:** - Andersen-Tawil Syndrome - Andersen Cardiodysrhythmic Periodic Paralysis - Andersen Cardiodysrythmic Periodic Paralysis - Andersen Tawil Syndrome - Long QT Syndrome 7 - Periodic Paralysis, Potassium-Sensitive Cardiodysrhythmic Type - Potassium-Sensitive Periodic Paralysis, Ventricular Ectopy, and Dysmorphic Features