Index of drugs

Long-term treatment of growth disorders in children and adolescents aged 2-18 years with severe primary deficiency of insulin-like growth factor-1 (primary IGFD). Severe primary IGFD is defined as: standard deviation rate ≤-3.0 and baseline IGF-1 <2.5 percentile for sex and age and GH within normal limits, no secondary forms of IGF-1 deficiency, such as malnutrition, underactivity thyroid or chronic treatment with pharmacological doses of anti-inflammatory steroids. The group with severe primary IGFD includes patients with mutations of the GH receptor gene, the poreceptor signal pathway and IGF-1 gene damage; they do not have GH deficiency and therefore they can not be expected to respond adequately to exogenous GH treatment. It is recommended that the diagnosis be confirmed by the IGF-1 production test.

1 vial contains 40 mg mecasermin. The drug contains benzyl alcohol (9 mg / ml).

Mecasermin is a human insulin-like growth factor-1 (rhIGF-1) obtained by recombinant DNA technology. The amino acid sequence of the drug is identical to the sequence of human endogenous IGF-1. Endogenous human IGF-1 stimulates the growth and metabolism of epiphyseal cartilage, acts mitogenously, causes organ growth (during treatment of IGF-1 deficient rats with rhIGF-1, body and organ growth was achieved). In addition, IGF-1 inhibits Glucose production in the liver and stimulates peripheral glucose consumption. It inhibits the secretion of insulin. It affects the growth and maintenance of bone mass and increases bone density. Bioavailability after administration reaches about 100%. IGF-1 is metabolised by both the liver and the kidneys. T_0,5 IGF-1 after a single subcutaneous administration of 0.12 mg / kg u is 5.8 hours.

Hypersensitivity to the components of the drug or auxiliary substances. Suspicion or presence of an active neoplastic process. It must not be given to premature babies or newborns due to the benzyl alcohol content.

The drug should not be used intravenously. Before starting treatment, thyroid deficiencies or nutritional status should be compensated. The drug is not a substitute for GH treatment. The drug should not be used to stimulate growth in patients with overgrown bone roots. Because it may have an insulin-like hypoglycaemic effect, it should be given shortly before or after a meal or snack. Special attention is required for young children, children with hypoglycemia in the interview and children who receive food irregularly. At the beginning of treatment, especially until a well-tolerated dose is established, patients should avoid any high-risk activities within 2-3 hours after dosing. If a patient with severe hypoglycaemia is unconscious or otherwise unable to eat properly, it may be necessary to administer glucagon injections. Patients who have previously experienced hypoglycaemia should have access to glucagon. During the first order, the patient should be instructed about signs and symptoms and treatment of hypoglycaemia, including glucagon injections. Before starting treatment, echocardiography should be performed in all patients. Patients terminating the treatment should also have an echocardiogram. Patients who have had echocardiographic abnormalities or cardiovascular symptoms should have regular echocardiogram. It is recommended to examine the fundus at the beginning and periodically during treatment and regularly check for lymphoid tissue hypertrophy. In patients whose growth is rapid, the femoral head may be peeled off and scoliosis progressed; patients who have started limping or complaining of hip or knee pain should be examined. If symptoms of hypersensitivity or signs of cancer are present, treatment should be discontinued. It contains benzyl alcohol, which may cause toxic and alleged anaphylactoid reactions in infants and children up to 3 years of age. The medicine contains less than 1 mmol sodium / vial, hence it is considered essentially 'sodium-free'.

There are no adequate data on the use of mecasermin in pregnant women.The treatment should not be started until a negative pregnancy test result is obtained. Women of childbearing potential should use effective contraception during treatment. The drug should not be used during pregnancy unless clearly necessary. Breast-feeding is not recommended during breast-feeding.

Very common: thymic hypertrophy, headache, hearing loss, hypertrophy of the tonsils, snoring, hypoglycaemia, hypertrophy at the injection site. Common: cardiac murmur, abnormal tympanometry, abnormal echocardiography, increased ALT, increased AST, weight gain; cardiomegaly, ventricular hypertrophy, atrial hypertrophy, tachycardia, paroxysmal tachycardia, mitral regurgitation, tricuspid regurgitation; congenital deformity of the mandible, a pigmented trait; lymphadenopathy; convulsions, febrile seizures, benign intracranial hypertension, loss of consciousness, hyperthyroidism syndrome, dizziness, tremors, restless legs syndrome; macular edema, decreased visual acuity, myopia; ear leaking, abnormal ears, middle ear disorders, eardrum disorders, ear pain, ear congestion, middle ear fluid; hypertrophy of the pharyngeal throat, hypertrophy of nasal turbinates, dyspnea, disorders of the nasal mucosa, obstructive airways disorders, abnormal breathing, nasal congestion, breathing through the mouth; vomiting, nausea, abdominal pain, epigastric pain, bloating, dysphagia; kidney stones, hydronephrosis, renal colic; skin hypertrophy, acrochordon (soft fibroma), abnormal consistency of the hair; joint pain, limb pain, myalgia, scoliosis, spinal deformity, connective tissue disorders, muscle cramps, pain in the side, musculoskeletal stiffness; hypoglycaemic seizures, hyperglycemia, hyperlipidemia, obesity; fever infection, upper respiratory tract infection, otitis media, exudative otitis media, chronic otitis media, external otitis, pharyngitis, tonsillitis, ear infection, oral candidiasis; hyperplasia of the mucous membrane, hypertrophy, pain, bruising, fibrosis, edema, infiltration, pigmentation changes at the injection site, mucosal edema, asthenia, slowing, chest discomfort; gynecomastia, ovarian cyst; depression, anxiety, nervousness, abnormal behavior, orientation disorders; excision of the tonsils, excision of the pharyngeal tonsil, assumption of tympanostomy. Not known: systemic hypersensitivity reactions (anaphylaxis, generalized urticaria, angioneurotic edema, dyspnea), alopecia, local hypersensitivity reactions at the injection site (pruritus, urticaria).

Treatment with the preparation should be carried out by a physician experienced in the diagnosis and treatment of patients with growth disorders.
Subcutaneously. Children> 2 years and young people. The dose should be chosen individually. The starting dose is 0.04 mg / kg. 2 times daily in subcutaneous injection. If there are no serious side effects, after a week or more the dose can be gradually increased by 0.04 mg / kg. up to a maximum dose of 0.12 mg / kg administered twice a day. Doses> 0.12 mg / kg given twice daily have not been evaluated in children with severe primary IGFD. If the recommended dose is not tolerated by the patient, a lower dose should be considered. The therapeutic effect should be assessed on the basis of the rate of growth. The smallest dose associated with a significant acceleration of growth for one patient is 0.04 mg / kg. 2 times a day. If no response has been obtained for one year, treatment should be reconsidered.
The medicine should be given as an injection under the skin shortly before or after a meal or snack. If hypoglycaemia occurs, the dose should be reduced. Use of the preparation should be discontinued if the patient is unable to eat food for any reason. The dose of mecasermin may not be increased to supplement one or more missed doses. At each subsequent injection, the injection site should be changed.