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Hyperkalemic periodic paralysis (HYPP, HyperKPP) is an inherited autosomal dominant disorder that affects sodium channels in muscle cells and the ability to regulate potassium levels in the blood. It is characterized by muscle hyperexcitability or weakness which, exacerbated by potassium , heat or cold, can lead to uncontrolled shaking followed ...
Hyperkalemic periodic paralysis is an autosomal dominant clinical condition where there is a mutation in gene located at 17q23 that regulates the production of protein SCN4A. SCN4A is an important component of sodium channels in skeletal muscles.
Treatment of hypokalemic periodic paralysis focuses on preventing further attacks and relieving acute symptoms. Avoiding carbohydrate-rich meals, strenuous exercise and other identified triggers, and taking acetazolamide or another carbonic anhydrase inhibitor, may help prevent attacks of weakness.
In 2015, the medication was approved in the US under the name Keveyis as an orphan drug for the treatment of primary hypokalemic and hyperkalemic periodic paralysis. [ 1 ] [ 5 ] Cost
This means people with both hyperkalemic periodic paralysis and paramyotonia congenita can have attacks with fluctuations of potassium up or down. Andersen-Tawil syndrome (Online Mendelian Inheritance in Man (OMIM): 170390), a form of periodic paralysis that includes significant heart rhythm problems, fainting and risk of sudden death ...
Liddle's syndrome, also called Liddle syndrome, [1] is a genetic disorder inherited in an autosomal dominant manner that is characterized by early, and frequently severe, high blood pressure associated with low plasma renin activity, metabolic alkalosis, low blood potassium, and normal to low levels of aldosterone. [1]
Thyrotoxic periodic paralysis (TPP) is a rare condition featuring attacks of muscle weakness in the presence of hyperthyroidism (overactivity of the thyroid gland). Hypokalemia (a decreased potassium level in the blood) is usually present during attacks.
Hyperkalemic periodic paralysis: Voltage-gated sodium channel Hypokalemic periodic paralysis: Voltage-gated sodium channel or. voltage-dependent calcium channel (calciumopathy) Lambert–Eaton myasthenic syndrome: Voltage-gated calcium channel Long QT syndrome main type Romano-Ward syndrome: various, by type Malignant hyperthermia: Ligand-gated ...
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