Composition, form of production and packaging
The tablets covered with a film membrane of dark pink color with a violet hue, round, biconcave.
penicillamine 250 mg
Auxiliary substances: potato starch, lactose monohydrate, povidone, talc, magnesium stearate.
Sheath composition: hypromellose, macrogol-4000, titanium dioxide (E171), dye azorubin (E122).
15 pcs. - blisters (2) - packs of cardboard.
100 pieces. - cans (1) - packs cardboard.
INSTRUCTION FOR THE SPECIALIST.
The product description was approved by the manufacturer for the 2009 print edition.
Kuprenil has a high complexing activity with respect to metals, mainly copper, mercury, arsenic, lead, iron and calcium. The drug reduces the resorption of copper from food and eliminates its excess from the tissues.
Influences on various links of the immune system (suppression of T-helper function of lymphocytes, inhibition of neutrophil chemotaxis and isolation of enzymes from lysosomes of these cells, enhancement of macrophage function).
It has the ability to disrupt collagen synthesis, splitting the cross-links between newly synthesized tropocollagen molecules.
Penicillamine is a pyridoxine antagonist; has an anti-inflammatory effect.
The preparation forms chelates with heavy metal ions - persistent complex compounds, soluble in water and excreted in urine.
Easily absorbed from the digestive tract, reaching Cmax in the blood for 1-3 hours. In the gastrointestinal tract, up to 50% of penicillamine is absorbed.
The drug is metabolized in the liver.
The drug is withdrawn in 2 phases: T 1/2 for the first is 1 hour, for the second - up to 90 hours. About 70-80% of the absorbed drug is excreted as metabolites (cysteine-penicillamine, disulphide) with urine.
- Wilson-Konovalov's disease;
- poisoning with copper, inorganic compounds of mercury, lead (with the exclusion of further exposure to lead through the digestive tract), gold, zinc, iron;
- Cystine nephrolithiasis;
- Systemic scleroderma;
- rheumatoid arthritis.
The drug should be used inside at least 30 minutes before meals, washed down with water, or 2 hours after eating or other medications.
Adults: 1.5 - 2 g / day in divided doses. After achieving remission, the dose can be reduced to 0.75 g or 1 g / day. In patients with a negative balance of copper, the minimum effective dose of penicillamine should be used.
A dose of 2 g / day should be applied for not more than 1 year.
Elderly patients: 20 mg / kg body weight / day in fractional doses. The dose should be selected in such a way as to achieve remission of the disease and to maintain a negative balance of copper.
For children: usually 20 mg / kg of body weight / day in fractional doses. The minimum dose is 500 mg / day
It is best to establish the minimum effective dose after quantitative determination of the concentration of amino acids in the urine by chromatographic method.
Dissolution of cystine stones
Adults: 1-3 g / day in fractional doses. It is necessary to keep the concentration of cystine in the urine below 200 mg / l.
Prevention of cystine lithiasis.
Adults: 0.5-1 g / day until the concentration of cystine in the urine is below 300 mg / l.
Elderly patients: prescribe a minimum dose until the concentration of cystine in the urine is below 200 mg / l.
For children: Assign a minimum dose, which allows you to achieve a cystine concentration in the urine below 200 mg / l.
Attention: during the treatment it is recommended to drink a large amount of liquid - at least 3 liters per day. The patient should drink 500 ml of water before bedtime, and then 500 ml at night, when the urine is more concentrated and more acidic than during the day. Usually, the more liquid the patient drinks, the lower his need for penicillamine.
A diet with a low methionine content is also recommended, so that the synthesis of cystine is as low as possible. In view of the low protein content, such a diet is not recommended for children during growth and pregnant women.
Adults: 1-1.5 g / day in fractional doses until reaching the release of lead in the urine in the range of 0.5 mg / day.
Elderly patients: 20 mg / kg body weight / day in fractional doses until the achievement of lead excretion in the urine within 0.5 mg / day.
For children: 20 mg / kg body weight / day.
Adults: 250 mg / day during the first month of application. Then the dose is increased every 4-12 weeks by 250 mg until remission is achieved. After that, apply the minimum effective dose, which allows you to slow down the symptoms of the disease. If the therapeutic effect is not achieved within 6 months of the drug administration, treatment should be discontinued.
The maintenance dose is usually 500-750 mg / day. Do not exceed the dose of 1.5 grams of medication per day. After achieving remission of the disease lasting 6 months, the dose of the drug should be gradually reduced by 250 mg every 12 weeks.
Elderly patients: the initial dose should not exceed 250 mg / day during the first month of application of the drug. Then the dose can be increased every 4-12 weeks by 250 mg until remission of the disease is achieved. Do not exceed the dose of 1g of the drug per day.
Children: usually 15-20 mg / kg body weight / day. The initial dose is 2.5-5.0 mg / day, it can be increased gradually every 4 weeks for 3-6 months to the minimum effective dose, but not more than 500 mg.
For 250 mg / day during the first month of application. Then the dose is increased every 4-12 weeks by 250 mg to 1 g / day, followed by a decrease to 250-500 mg / day. The effect is estimated after 6-12 months of the drug.
Anorexia, nausea, vomiting, diarrhea, aphthous stomatitis, glossitis; intrahepatic cholestasis, pancreatitis; skin rash; epidermal necrolysis; complete loss or distortion of taste sensations, reversible polyneuritis (due to a deficiency of pyridoxine); nephritis, hematuria; eosinophilia, thrombocytopenia, leukopenia; anemia (aplastic or hemolytic), agranulocytosis; Allergic alveolitis, interstitial pneumonitis, diffuse fibrosing alveolitis, Goodpasture's syndrome; myasthenia gravis, polymyositis, dermatomyositis, polyneuritis; fever; lupus-like reactions (arthralgia, myalgia, erythematous rash, the appearance of antinuclear antibodies and antibodies to DNA);increased mammary glands with the development of galactorrhea (in women); alopecia.
- kidney failure;
- violations of hemopoiesis;
- pregnancy and the period of breastfeeding (with the exception of Wilson-Konovalov disease in a dose of up to 1 g / day);
- Children under 3 years of age (for this dosage form);
- hypersensitivity to penicillamine and other components of the drug.
It should not be used in combination with gold preparations, aminoquinoline and cytostatic drugs, phenylbutazone.
With caution: when performing surgical interventions, anemia, proteinuria.
PREGNANCY AND LACTATION
Contraindication: pregnancy and the period of breastfeeding (with the exception of Wilson-Konovalov disease in a dose of up to 1 g / day).
APPLICATION FOR FUNCTIONS OF THE LIVER
Contraindication: renal failure.
APPLICATION FOR CHILDREN
Contraindication: children under 3 years (for this dosage form).
APPLICATION IN ELDERLY PATIENTS
A dose adjustment is required (see Dosage regimen).
When using Kuprenil requires constant medical supervision: a complete blood test (including platelet count) and urine every 3 days, then every week; control of kidney function; general and neurological examinations every month. In case of appearance of side effects, which the patient is obliged to inform the doctor, the drug should be immediately canceled, and after their termination it can be reapplied, at the doctor's discretion, starting from the lowest dose.
Precautions should be taken with the simultaneous use of Kuprenil and anti-inflammatory or other agents that can cause impaired bone marrow function.
In the case of withdrawal of gold preparations, due to lack of effect, Kuprenil can be used after 6 months.
Patients with impaired renal function should accordingly reduce the dose of the drug and apply it with caution.
If a patient is recommended treatment with iron preparations, a two-hour interval should be observed when applying Kuprenil.
Due to the influence of penicillamine on collagen and elastin, any surgical interventions (including dental) during the period of drug use should be performed with caution.
During treatment, urine tests and clinical blood tests should be monitored once every 2 weeks during the first 6 months of treatment, then monthly; 1 time in 6 months control the function of the kidneys and liver. With Wilson-Konovalov's disease or cystinuria, pyridoxine is administered concomitantly with penicillamine (due to dietary restrictions used to treat these diseases); with prolonged treatment, these patients should regularly perform X-ray or ultrasound examination of the kidneys and urinary tract. When using Kuprenil it is necessary to compensate for the lack of vitamin B 6 . In case of signs of pyridoxine deficiency in patients with rheumatoid arthritis, and in the event that these signs do not pass by themselves, 25 mg of pyridoxine per day is additionally prescribed. In the case of development of fever, the increase of proteinuria, the appearance of hematuria, lung damage, liver, expressed hematologic or neurologic disorders, myasthenia, hematuria, lupus-like reactions or other serious adverse reactions, the drug is canceled and, if necessary, glucocorticosteroids are prescribed. In the case of isolated proteinuria, if it does not increase and does not exceed 1 g / day, penicillamine treatment is continued, in other cases - canceled.
Impact on the ability to drive vehicles and manage mechanisms
The drug does not cause disturbances in psychophysical functions, ability to drive vehicles and service moving mechanical devices.
There have been no cases of overdose. The antidote is unknown. Treatment is symptomatic.
Kuprenil is a vitamin B 6 antagonist.
The drug can not be used with drugs that can cause a violation of bone marrow function.
The preparation forms persistent complex compounds with heavy metals.
Increase the risk of side effects antimalarial drugs, levamisole and phenylbutazone.
Antacids reduce the absorption of the drug.
TERMS OF RELEASE FROM PHARMACY
The drug is released by prescription.
TERMS AND CONDITIONS OF STORAGE
Store at temperatures between 15 В° C and 25 В° C, out of the reach of children. Shelf life - 3 years.