Respikam - instructions for use, dose, side effects, contraindications

Active substanceAlpha 1-Proteinase inhibitorAlpha 1-Proteinase inhibitor

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Respicam®

solution d / infusion

Kamada Co., Ltd. Israel

Dosage form: & nbspsolution for infusions

Composition:

1 bottle with 50 ml of solution contains:

Active substance: Inhibitor of alpha1-proteinase [human] (alpha1-antitrypsin) 1000 mg

Excipients: sodium dihydrogen phosphate dihydrate, sodium chloride, water for injection

Description:

Transparent, from colorless to yellow-green color solution. May contain a small precipitate (suspended protein particles).

Pharmacotherapeutic group:Proteinase inhibitor

ATX: & nbsp

B.02.A.B Inhibitors of plasma proteinases

Pharmacodynamics:

Respikam® is a sterile, ready-to-use solution containing a purified alpha 1-proteinase inhibitor (also known as alpha1-antitrypsin (AAT)).

Respikam® is made from blood plasma of healthy people using ion-exchange chromatography. In order to prevent the risk of transfer of pathogens of infectious diseases, each portion of human plasma used to produce the drug is tested for human immunodeficiency virus (HIV), antibodies to hepatitis C (anti-HGS), hepatitis B surface antigen (HBsAg), as well as a serological response to the definition of the causative agent of syphilis.

Respikam ® has a therapeutic effect in case of deficiency of AAT - chronic hereditary, autosomal recessive disease, characterized by the development of severe, slowly progressing panacinous emphysema of the lungs, most often observed in the third or fourth decade of life. It is assumed that the development of this pathological condition is due to an imbalance between elastase (an enzyme capable of destroying the elastin of tissues and produced primarily by neutrophils in the lower respiratory tract) and AAT, the main inhibitor of elastase. As a result of prolonged exposure to elastase, elastin tissue is destroyed and emphysema develops. Approximately 10% of newborns with AAT deficiency have giant cell hepatitis with cholestatic jaundice. In adults, a deficiency of AAT can be accompanied by cirrhosis of the liver.

Deficiency of AAT is a genetic pathology at the gene locus PI, which was found in the chromosome 14q32.1. The concentration of AAT in the blood plasma, associated with PI - type

homozygous inheritance, is usually in the range of 12-18% of the norm, and heterozygous inheritance - is approximately 35% of the norm.Many studies of the effectiveness of substitution therapy were based on maintaining the concentration of AAT in human plasma> 80 mg / dL. This concentration was sufficient to prevent the development of emphysema in these patients.

Pharmacokinetics:

When infused with Respipam ® once a week at a dose of 60 mg / kg body weight in 21 patients, the average value of AAT concentration was 126 mg / dl (before treatment 30 mg / dl). Numerous studies of the effectiveness of substitution therapy have shown that this concentration is sufficient to prevent the development of emphysema in patients with congenital AAT deficiency.

The study found that in the first hours after the infusion, the maximum plasma concentration was 280 mg / dL, with a rapid decrease in the first 2 days, and a gradual subsequent decrease within 7 days. The half-life of the alpha1-proteinase inhibitor was 4.5 days. During 2 years of the study, he did not change, which indicates the lack of antibody formation in response to the introduction of AAT.

Indications:

Congenital deficiency of the alpha 1-proteinase inhibitor in patients with a clinical picture of panacinose emphysema. For prolonged replacement therapy only in patients with phenotypes PiZZ, PiZ(-) or Pi(-)(-).

Contraindications:

Selective immunoglobulin deficiency A (IgA) in the presence of antibodies to IgA.

Hypersensitivity to the constituent components of the drug.

Safety and effectiveness of the drug in children is not established. Use in children is contraindicated.

Pregnancy and lactation:

To date, there is no information about how the Respimcam® drug affects the fetal development of the fetus or the reproductive function of the female body. It is not known whether the Respipak® drug penetrates into breast milk. Therefore, the alpha 1-proteinase inhibitor may be prescribed by a doctor during pregnancy and breastfeeding only in cases of acute necessity, when the expected benefit to the mother exceeds the possible risk to the fetus or the baby.

Dosing and Administration:

The drug is intended only for intravenous administration

Adults

The recommended dose of Respipam ® for long-term substitution therapy is 60 mg per kilogram of body weight once a week, intravenously dripping at a rate of 0.08 ml / kg / min or faster. The duration of infusion of Respipam® should not exceed 30 minutes. Observing the rules of asepsis, the necessary dose of the drug is collected in a syringe from the vial using a special needle with a filter (supplied with the drug).After that, remove the needle with the filter and replace it with a needle for injection. Then transfer the dose to the appropriate plastic container (plastic bag or bottle). Infusion of the drug is made using a system designed for intravenous drip solutions (dropper).

Immediately prior to administration, parenteral medications should be checked for impurities and the clarity of the solution.

Respiqam® should be used after the temperature of the drug equals room temperature.

The duration of the course is set by the doctor.

Side effects:

The drug Respikam® at a dose of 60 mg / kg per week is well tolerated. During the study there were no reports of serious adverse events or deaths. There were no deviations from the initial value of the main indicators of the state of the organism or laboratory parameters. There were no changes from the initial value of the indicators on the electrocardiogram (ECG). During the period of the clinical study and within 6 months after it there were no changes in the virological parameters.

In the study of the safety of Respipam® treatment, 22 cases of side-effects were identified in 18 patients, of which only 3 can be considered, possibly related to the use of Respipam® (headache, erythema and dysgeusia) and one, probably related (hyperthermia) with its treatment.

Since the release of the drugs of the alpha1 proteinase inhibitor, there have been regular reports on the market of other cold-like symptoms, allergic reactions, chills, the appearance of dyspnea, rash, tachycardia and, rarely, a drop in blood pressure. Also, rare cases of short-term increase in blood pressure, accompanied by pain in the chest, were recorded.

Overdose:

At present, no cases of drug overdose have been reported. Antidote to the drug does not exist.

Interaction:

Respikam® should not be mixed with other medicines.

Special instructions:

The "threshold" concentration of the alpha 1-proteinase inhibitor in blood plasma, designed to provide sufficient anti-elastic activity in the lungs of patients with alpha 1 antitrypsin deficiency, is 80 mg / dl (this value is based on data obtained by standard immunological analysis of the content of the alpha 1- proteinases).Despite the inactivation and / or elimination of viral pathogens during the manufacture of Respipam®, the presence of an unknown infectious agent in it can not be completely ruled out. In patients receiving infusions of blood or plasma preparations, signs and / or symptoms of a viral disease, for example hepatitis C, may develop. Before prescribing the drug, the doctor must correlate the risk and benefit.

Possible increase in plasma volume after infusion of Respipam ®, which should be considered in patients with increased volume of circulating fluid.

Do not re-apply the remnants of the drug after performing the infusion.

Unused bottles of the drug that were taken from the refrigerator should be returned to the refrigerator for storage for 6 hours. If the vial with the drug remained at room temperature (25 ° C) for more than 48 hours, the drug is considered to be overdue and not allowed for further use.

Effect on the ability to drive transp. cf. and fur:

The effect of the drug on the performance of potentially hazardous activities requiring increased concentration of attention and speed of psychomotor reactions has not been established.

Form release / dosage:Solution for infusions 20 mg / ml.

Packaging:50 ml infusion solution in colorless hydrolytic glass bottles (USP) type I, sealed with rubber stoppers and crimped aluminum caps with a plastic cap (flip-off).
One bottle together with the instructions for use and a filter needle (in a sterile package) is placed in a cardboard box.

Storage conditions:

Store at a temperature of 2 to 8 ° C. Do not freeze.

Keep out of the reach of children.

Shelf life:30 months. Do not use after the expiration date.

Terms of leave from pharmacies:On prescription

Registration number:LSR-003707/10

Date of registration:04.05.2010

The owner of the registration certificate:Kamada Co., Ltd.Kamada Co., Ltd. Israel

Manufacturer: & nbsp

KAMADA, Ltd. Israel

Representation: & nbspGENPHA LTD.GENPHA LTD.Russia

Information update date: & nbsp25.08.2015

Illustrated instructions

Instructions

Respicam® (Respikam®)