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Cysteamine volume of distribution

Cystagon® has a volume of distribution of 156 L. Cysteamine has a plasma protein binding of 52% and is mostly bound to albumin. The metabolism of cysteamine has not yet been determined. The plasma clearance is about 1.2 L/min.

What is Cysteamine

Cysteamine is a radiation-protective agent that oxidizes in air to form cystamine. It can be given intravenously or orally to treat radiation sickness. The bitartrate and hydrochloride salt forms are indicated for the treatment of neuropathic cystinosis in patients 6 years old and older.. Cysteamine is marketed under several brand names such as Cystaran, Procysbi, and Cystagon.

Cysteamine mechanism of action

The free thiol cysteamine depletes cystinotic leukocytes and other cells of cystine, whose accumulation is considered the cause of organ damage in cystinosis. Cysteamine cleaves the disulfide bond with cystine to produce molecules that can escape the metabolic defect in cystinosis and cystinuria.

Dosage forms of Cysteamine

Indication of Cysteamine

Given intravenously or orally to treat radiation sickness. The bitartrate salts (Cystagon and Procysbi) have been used for the oral treatment of nephropathic cystinosis and cystinurea. The hydrochloride salt (Cystaran) is indicated for the treatment of corneal cystine crystal accumulation in cystinosis patients.

Toxicity of Cysteamine

Symptoms of overdose may include convulsions (seizures), increased thirst and unusual tiredness or weakness.