Included in the formulation
АТХ:B.06.A.B Other hematological preparations
Pharmacodynamics:Hema heme by negative feedback mechanism eliminates heme deficiency, inhibits the activity of δ-aminolevulinic acid synthase, a key enzyme for the synthesis of porphyrins, resulting in a decrease in the synthesis of porphyrins and toxic metabolic heme precursors. Thus, gem contributes to the normalization of the level of hemoproteins and respiratory enzymes, by correcting the pathophysiological disorders that accompany porphyria.
In acute intermittent and mixed (variegate) porphyria infusions of hemin normalize urinary excretion of δ-aminolevulinic acid and porphobilinogen - two substances whose cumulation underlies the pathogenesis of the disease. It has no effect on blood clotting and fibrinolysis.
Pharmacokinetics:
After intravenous infusion, the total plasma clearance is 3.7 ± 1.2 ml / min, Vd - 3.4 ± 0.9 l.
The half-life period is 9-11 hours, with repeated administration it increases to 18 hours.
Elimination by the kidneys.
Indications:
It is used to treat acute attacks with a hereditary, variant, intermittent form of hepatic porphyria.
IV.E70-E90.E80.2 Other porphyria
IV.E70-E90.E80 Disorders of porphyrin and bilirubin metabolism
Contraindications:
Individual intolerance, children under 18 years.
Carefully:Dysfunction of the liver, increased sensitivity to the components of the drug.
Pregnancy and lactation:Recommendations for FDA - category is not defined. Used in pregnancy and lactation only for life.
Dosing and Administration:Intravenously drip, 3 mg / kg 1 time per day, duration of infusion - 15 minutes. The course of treatment is 4-7 days.
The highest daily dose: 3 mg / kg.
The highest single dose: 3 mg / kg.
Side effects:Allergic reactions.
Overdose:Cases of overdose are not described.
Treatment is symptomatic.
Interaction:Incompatible with barbiturates.
Special instructions:In 1 ampoule (10 ml) of hemin, 1 g of 96% ethanol is contained, which can harm patients with liver disease, alcoholism, epilepsy, damage or brain diseases, as well as pregnant women and children. The presence of ethanol in the gemin can alter or enhance the effect of other drugs.
Gemin should not be used as a preventive therapy, because a long-term course of regular infusions leads to a glut of the body.