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Cysteamine bitartrate
Cysteamine bitartrate (Cystagon)
Cysteamine bitartrate
(SIS-tee-ah-meen)
Pregnancy Category: C
Cystagon (Rx)
Classification:
Urinary tract product
Action/Kinetics:
Lowers cystine levels of cells in cystinosis, which is an inherited defect of lysosomal transport. In those with cystinosis, cystine transport out of lysosomes is abnormal, resulting in the formation of crystals which damage the kidney. Other tissues are damaged as well, including the retina, muscles, and CNS. Acts within the cell to convert cystine into both cysteine and cysteine-cysteamine mixed disulfide, which can leave the lysosome in those with cystinosis.
Uses:
Management of nephropathic cystinosis in adults and children.
Contraindications:
Hypersensitivity to cysteamine or penicillamine. Use during lactation.
Side Effects:
CNS:Lethargy, somnolence, depression, encephalopathy, seizures headache, ataxia, confusion, tremor, hyperkinesia, dizziness, jitteriness, nervousness, abnormal thinking, emotional lability, hallucinations, nightmares. GI: N&V;, anorexia, abdominal pain (may be severe), diarrhea, bad breath, dyspepsia, constipation, gastroenteritis, duodenitis, duodenal ulceration. Hematologic: Reversible leukopenia, anemia. Miscellaneous; Decreased hearing, fever, rash, dehydration, hypertension, urticaria.
Laboratory Test Alterations:
Abnormal LFTs.
Overdose Management:
Symptoms: Extension of side effects, respiratory symptoms. Treatment: Support the cardiovascular and respiratory systems. Hemodialysis may be effective in removing the drug from the body.
How Supplied:
Capsule: 50 mg, 150 mg
Dosage
•Capsules
Nephropathic cystinosis.
Initial: New clients should be started on one-fourth to one-sixth of the maintenance dose. The dose is then raised gradually over 4-6 weeks to avoid intolerance. Maintenance, children up to age 12 years: 1.3 g/m2/day (of the free base) given in four divided doses. Maintenance, children over 12 years and over 110 lb: 2 g/day in four divided doses. |
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