Universal reference book for medicines
Name of the drug: RESPIKAM (RESPIKAM)

Active substance: alpha-1-proteinase inhibitor

Type: Alpha-1 proteinase inhibitor used for emphysema

Manufacturer: KAMADA (Israel)
Composition, form of production and packaging
The solution for infusions is
transparent, from colorless to yellow-green, may contain a small precipitate (suspended protein particles).

1 f.

alpha1-proteinase inhibitor 1000 mg

[PRING] sodium dihydrogen phosphate dihydrate, sodium chloride, water d / and.

50 ml - bottles of glass (1) complete with a needle with a filter (in sterile packaging made of paper and PVC) - packs of cardboard.


Description of the drug approved by the manufacturer for the printed edition of 2013.


Respikam is a sterile, ready-to-use solution containing a purified alpha1-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 transmitting pathogens of infectious diseases, each portion of human plasma used to produce the drug is tested for human immunodeficiency virus (HIV), hepatitis C antibodies (anti-HAg), hepatitis B surface antigen (HBsAg), and serological response to the definition of the causative agent of syphilis.
Respicam 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 to fourth decade of life.
It is assumed that the development of this pathological condition is caused by an imbalance between elastase (an enzyme capable of destroying the elastin of tissues and produced mainly 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 in the locus of the PI gene, which was found in chromosome 14q32.1.
The concentration of AAT in the blood plasma associated with the PI type of 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 maintenance of AAT concentration in human blood plasma> 80 mg / dL. This concentration was sufficient to prevent the development of emphysema in these patients.

After infusion of Respikam once a week at a dose of 60 mg / kg of body weight in 21 patients, the average value of AAG 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 AH 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, indicating that there was no antibody formation in response to the administration of AAT.

- 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 (-) (-).


The drug is intended only for intravenous administration


The recommended dose of Respipam for long-term replacement therapy is 60 mg per kilogram of body weight once a week, intravenously drip 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 this, remove the needle with the filter and replace it with a needle intended for injection. Then, transfer the dose to the appropriate plastic container (plastic bag or vial). 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.

Respiratory should be used after the temperature of the drug equals room temperature.

The duration of the course is set by the doctor.


The drug Respikam in 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 treatment with Respikam, 22 cases of side effects were identified in 18 patients, of which only 3 can be considered, possibly associated with reception of Respipam (headache, erythema and dysgeusia) and one, probably related (hyperthermia) with its treatment.

Since the release of the drugs of the alpha 1-proteinase inhibitor, there have been regular reports on the market of other cold-reminding symptoms, allergy-like reactions, chills, the appearance of dyspnoea, rashes, 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.

- selective deficiency of immunoglobulin A (IgA) in the presence of antibodies to IgA;

- hypersensitivity to the components of the drug.

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

To date, there is no information about how the Respimix drug affects the fetal development of the fetus or the reproductive function of the female body.

It is not known whether the Respipam penetrates into breast milk.
Therefore, the alpha 1-proteinase inhibitor may be prescribed by the 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.

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

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 immunoassay of the content of the alpha 1- proteinases).
Despite the inactivation and / or elimination of viral pathogens during the manufacture of the drug, Respipaks can not completely exclude the presence of an unknown infectious agent in it. 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 taken into account in patients with an 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 В° C) for more than 48 hours, the drug is considered to be overdue and not allowed for further use.
Impact on the ability to drive vehicles and manage mechanisms

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.


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

Respiratory should not be confused with other drugs.


On prescription.


Store at a temperature of 2 to 8 В° C.
Do not freeze. Keep out of the reach of children. Shelf life - 2 years.
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