Penicillamine, a chelating agent to treat Wilson’s disease - Gafacom (For health. For knowledge)

Penicillamine, a chelating agent to treat Wilson’s disease

Penicillamine is a chelating agent used in the treatment of Wilson’s disease. It is also used to reduce cystine excretion in cystinuria and to treat patients with severe, active rheumatoid arthritis unresponsive to conventional therapy. It is 3-mercapto-D-valine. It is a white, or practically white, crystalline powder, freely soluble in water, slightly soluble in alcohol, and insoluble in ether, acetone, benzene, and carbon tetrachloride. Although its configuration is D, it is levorotatory
It is also used to reduce cystine excretion in cystinuria


Class: Chelating Agent

Indications

Treatment of Wilson's disease, cystinuria; adjunctive treatment of severe, active rheumatoid arthritis
Penicillamine is used in adults only to treat chronic active hepatitis – a type of liver disease




Canadian labeling: Additional use (not in U.S. labeling): Treatment of chronic lead poisoning

Action

Antirheumatic effect, probably resulting from enhanced lymphocyte function. Chelates heavy metals, including copper, mercury, lead, and iron, into complexes that are excreted by the kidneys. Forms a soluble complex with cystine that is readily excreted by the kidneys.

Therapeutic Effects: Decreased disease progression in rheumatoid arthritis. Decreased copper deposition in Wilson’s disease. Decreased cystine renal calculi formation.

Do not take Penicillamine Tablets if you:

• are allergic (hypersensitive) to penicillamine or any of the other ingredients in these. Signs of an allergic reaction include rash, itching, swelling of the face, fainting and breathingproblems.
• have severe kidney problems
• have lupus erythematosus (an auto-immune disorder)
• have a history of suffering from blood disorders after taking penicillamine e.g. agranulocytosis (a low white cell blood count), aplastic anaemia (symptoms include feeling tired, breathless, having pale skin and more susceptible to infection) or thrombocytopenia (a blood disorder which causes bleeding into your skin, bruising and bleeding more than normal)

Pregnancy

Penicillamine can cause fetal harm when administered to a pregnant woman. Penicillamine has been shown to be teratogenic in rats when given in doses six times higher than the highest dose recommended for human use. Skeletal defects, cleft palates, and fetal toxicity (resorptions) have been reported.

There are no controlled studies on the use of penicillamine in pregnant women. Although normal outcomes have been reported, characteristic congenital cutis laxa and associated birth defects have been reported in infants born of mothers who received therapy with penicillamine during pregnancy. Penicillamine should be used in women of childbearing potential only when the expected benefits outweigh the possible hazards

Taking with food and drink

Penicillamine should be taken on an empty stomach, and at least half an hour (one hour for children with Wilson’s disease or cystinuria) before a meal, with a drink of water.

Driving and using machines

Penicillamine may cause dizziness, confusion and problems with your vision. If you are affected by any of these side effects do not drive or use machines.

Dosage for adults

Rheumatoid arthritis
Take 125mg - 250mg a day for the first month. Your doctor will increase the dose slowly up to 500mg - 750mg daily (in divided doses). Your doctor may prescribe a dose as high as 1500mg. If there is no improvement in your condition after 12 months, your doctor might stop your Penicillamine treatment. If you stay well for six months your doctor may reduce your dose.

Wilson’s disease
Take 1500mg - 2000mg daily in divided doses. Do not take more than 2000mg in one day. Your doctor may reduce your dose to 750mg - 1000mg daily once your condition is controlled. A dose of 2000mg daily should not be continued for more than 12 months.

Cystinuria
To treat cystinuria take 1000mg - 3000mg daily in divideddoses. To prevent cystinuria take 500mg - 1000mg before going to bed.

Lead poisoning
Take 1000mg - 1500mg daily in divided doses until your doctor tells you that the amount of lead in your urine is normal.

Chronic active hepatitis
Start by taking 500mg daily in divided doses. Your doctor will increase the amount of tablets you take over 3 months to 1250mg daily.

Dosage for children

The dose may depend on the weight of the child. As the smallest available tablet is 125mg, it might be too large for very small children.

Rheumatoid arthritis
15mg to 20mg a day for each kilogram of body weight. You will start with a low dose for the first month and increase gradually.

Wilson’s disease
For children under 12 years 20mg a day for each kilogram of body weight in two or three separate doses given 1 hour before meals. For older children the usual dose is 0.75g to 1g daily.
Cystinuria
Start with 20 - 30 mg for each kilogram of body weight, in two or three separate doses given 1 hour before meals. Your doctor may change your dose depending on the results of tests on your urine.

Lead poisoning
15mg - 20mg a day for each kilogram of body weight, in 2 to 3 separate doses.



Reference
https://www.webmd.com/drugs/2/drug-7560/penicillamine-oral/details
https://reference.medscape.com/drug/cuprimine-depen-penicillamine-343728
https://www.ncbi.nlm.nih.gov/books/NBK513316/
https://pubchem.ncbi.nlm.nih.gov/compound/Penicillamine
https://www.drugbank.ca/drugs/DB00859
https://www.medicines.org.uk/emc/medicine/26390/SPC
https://www.medicalnewstoday.com/articles/322202
https://www.medicinenet.com/penicillamine/article.htm
Penicillamine, a chelating agent to treat Wilson’s disease Penicillamine, a chelating agent to treat Wilson’s disease Reviewed by gafacom on July 01, 2020 Rating: 5

No comments:

Powered by Blogger.