What's on this Page
Cysteamine Brand Names
Cystagon | CYSTARAN | Procysbi
What is Cysteamine
Cysteamine is used orally to treat nephropathic cystinosis. Cysteamine ophthalmic solution is used to treat the corneal cystine crystal accumulation that occurs in patients with cystinosis.
Nephropathic cystinosis is a rare autosomal recessive disease involving the defective transport of cystine across the lysosomal membrane.
The defective transport of cystine out of lysosomes results in free cystine accumulation and crystallization within the lysosomes, which destroys various tissues and damages organs, especially the kidney.
An example of kidney damage includes renal tubular Fanconi Syndrome, which is characterized by polyuria, polydipsia, electrolyte imbalance, dehydration, rickets, and growth failure.
If left untreated, progressive glomerular failure and end stage renal failure, usually occurring by 10 years of age, also are possible.
Other sequelae of untreated cystinosis include photophobia, retinal blindness, hypothyroidism, pulmonary dysfunction, and male hypogonadism.
For patients with cystinosis, treatment with cysteamine early in life has been shown to slow the rate of renal failure progression, increase growth in affected patients, obviate the need for levothyroxine replacement, and decrease corneal cystine deposits.
Indications
- cystinosis
Side Effects
- abdominal pain
- anemia
- anorexia
- arthralgia
- ataxia
- blurred vision
- confusion
- conjunctival hyperemia
- conjunctivitis
- constipation
- cough
- dehydration
- depression
- diarrhea
- diplopia
- dizziness
- drowsiness
- drug-induced body odor
- dyspepsia
- elevated hepatic enzymes
- emotional lability
- encephalopathy
- erythema multiforme
- fatigue
- fever
- GI bleeding
- GI perforation
- halitosis
- hallucinations
- headache
- hearing loss
- hyperkinesis
- hypertension
- increased intracranial pressure
- infection
- influenza
- interstitial nephritis
- lethargy
- leukopenia
- nausea
- nightmares
- ocular irritation
- ocular pain
- osteopenia
- papilledema
- pharyngitis
- pseudotumor cerebri
- rash
- renal failure (unspecified)
- seizures
- toxic epidermal necrolysis
- tremor
- urticaria
- visual impairment
- vomiting
Monitoring Parameters
- CBC
- leukocyte cystine measurements
- LFTs
- ophthalmologic exam
- serum creatinine
- serum electrolytes
Contraindications
- anemia
- bone fractures
- breast-feeding
- contact lenses
- driving or operating machinery
- ethanol ingestion
- GI disease
- hepatic disease
- increased intracranial pressure
- leukopenia
- papilledema
- penicillamine hypersensitivity
- pregnancy
- seizures
- serious rash
Interactions
- Aluminum Hydroxide; Magnesium Carbonate
- Amoxicillin; Clarithromycin; Lansoprazole
- Amoxicillin; Clarithromycin; Omeprazole
- Aspirin, ASA; Citric Acid; Sodium Bicarbonate
- Aspirin, ASA; Omeprazole
- Calcium Carbonate
- Calcium Carbonate; Magnesium Hydroxide
- Calcium Carbonate; Risedronate
- Calcium Carbonate; Simethicone
- Cimetidine
- Dexlansoprazole
- Esomeprazole
- Esomeprazole; Naproxen
- Ethanol
- Famotidine
- Famotidine; Ibuprofen
- H2-blockers
- Lansoprazole
- Lansoprazole; Naproxen
- Lanthanum Carbonate
- Nizatidine
- Omeprazole
- Omeprazole; Amoxicillin; Rifabutin
- Omeprazole; Sodium Bicarbonate
- Pantoprazole
- Proton pump inhibitors
- Rabeprazole
- Ranitidine
- Sodium Bicarbonate
