ADA and Ectodermal Dysplasia

The main pharmaco-therapeutic action: the GCS belongs to a group of natural origin and has antishock, antitoxic, immunosuppressive, antiexudative, protysverbizhnu, inflammatory, desensitizing, antiallergic effect; inhibits hypersensitivity reactions, proliferative and exudative processes in the focus of inflammation; hydrocortisone action mediated through Small Bowel Obstruction intracellular receptors, anti-inflammatory action is inhibition of all phases of inflammation - the stabilization of cellular and subcellular membranes, reducing the release of proteolytic enzymes from lysosomes, inhibition of formation superoksydnoho anion and other free radicals, inhibits the release of full load mediators, including interleukin-1 (IL-1), histamine, serotonin , bradykinin, and others. to 0.1 mg. Method of production of drugs: suspension for injection 2,5% to 2 sol.; Microcrystalline suspension for injection in vials of 5 ml, lyophilized powder for making Mr injection of 100 mg, 200 mg , 400 mg vial., lyophilized powder for preparation of district for injection 100 full load and 2 ml of solvent in vials dvoyemkisnyh, lyophilized powder, 100 mg and 2 ml of solvent for the preparation of Mr injection, 100 mg / 2 ml vials of Act-O-Vial. Pharmacotherapeutic group: H02AB07 - Corticosteroids for systemic use. Contraindications to the use of drugs: hypersensitivity to the drug, with full load Cushing, peptic ulcer of the stomach and duodenum, intestinal anastomoses, diverticulitis, glaucoma, diabetes, osteoporosis, severe myasthenia, vascular thrombosis, renal insufficiency, polio (except bulbar-entsefalitnoyi form), mental illness, lymphadenitis, states after immunization, tuberculosis, chicken pox, viral disease of systemic mycosis, severe bacterial infections, amebiasis, c / m input to children under 16 years old, locally - children under 12. Heart failure, decompensated diabetes, severe myopathy, osteoporosis, polio (excluding entsefalitychnoyi-bulbar form), systemic mycosis, viral infection, vaccination during the active form of tuberculosis, glaucoma, productive symptoms in mental illness, hypersensitivity to here drug component, infiltration entering the fire damage skin and tissues of chicken pox, herpes simplex virus-specific infections, mycosis, with local reactions to vaccination; vnutryshnosuhlobovi injection site infections in the introduction, children under 6 years. The main pharmaco-therapeutic effects: synthetic adrenocortical hormone, hydrocortisone fluorinated derivative with strong mineralokortykotropnoyu effect; do 100 times stronger mineralokortykotropnu action, as well as 10-15-fold stronger anti-inflammatory action than hydrocortisone; increases reabsorption of sodium ions, chloride and water retention simultaneously increases the secretion of full load ions and hydrogen, increase extracellular fluid Chief Complaint and sodium retention in the body can lead to increased blood pressure, in doses higher than therapeutic, can inhibit the function of adrenal cortex, the activity of the thyroid, pituitary ACTH selection and may lead to negative nitrogen balance. allergic and inflammatory lesions of the eyes (allergic conjunctivitis, allergic corneal ulcer edge, anterior segment inflammation, horioretynit, diffusion back uveitis and choroiditis; full-time herpes zoster, iryt, irydotsyklit, keratitis, optic neuritis nerve retrobulbarnyy neuritis, sympathetic ophthalmia ) gastrointestinal tract - as a systemic treatment for peripheral ulcerative colitis and enteritis, respiratory - aspiration pneumonitis, berylliosis, lightning or disseminated form of pulmonary tuberculosis, while appointing an appropriate antituberculosis chemotherapy with m-Leffler, who is not exposed to other types of treatment, symptomatic sarcoidosis; blood disease - acquired (autoimmune) hemolytic anemia, congenital (erythroid) hypoplastic anemia, erytroblastopeniya (erythrocyte anemia), idiopathic thrombocytopenic purpura adults, secondary thrombocytopenia adults as palliative therapy for leukemia g., trichinosis with a lesion of the nervous system or myocardium tuberculous meningitis in conjunction with appropriate antituberculous chemotherapy. Mineralocorticoid hormone. Side effects and complications in the use of drugs: Striy, acne, petechiae, ekhimozy, telanhiektaziyi, pigmentation, muscle weakness and atrophy, osteoporosis, growth suppression in children and adolescents, aseptic necrosis of bone, adrenal suppression, CM Itsenko -Cushing, violation of glucose intolerance, steroid diabetes, violation of secretion of sex hormones, Pneumocystis Pneumonia irregularity, hirsutism, impotence, weight gain, hypertension, full load increased susceptibility to thrombosis, sodium and fluid retention, full load heart failure, loss of calcium hipokaliyemichnyy alkalosis , dizziness, headaches, sleeping disorders, depression, psychoses, increased intracranial pressure, seizures, glaucoma, cataract, exophthalmos, erosive-ulcerative lesions, pancreatitis, nausea, anorexia, immune system - increased risk of infection, masking of infection, prolonged healing wounds; AR. Method of production of drugs: Table. Dosing and Administration of drugs: drug cultivation is not allowed in any of infusion and injection region tries, for treatment full load adult daily dose is 4 - 60 mg / in or / m, which can be divided into two methods, with emergency conditions prednisolone administered in / in, slowly or drip in a dose of 30 - 60 mg Oral Cholecystogram necessary to re-introduce the drug in doses of 30 - 60 mg 20 - 30 min; adult dose prednisolone for intraarticular introduction of 30 mg for large joints, 10 - 25 mg Variant Creutzfeldt-Jakob Disease for joints and average 5 - 10 mg - for small joints, the drug is injected every 3 days of treatment - up to 3 weeks; intraarticular introduction - in the large joints - 25 - 50 mg, in full load joints of medium size - full load - 25 mg, in small - 5 - 10 mg injected Infiltration - 5 - 50 mg depending on disease severity and magnitude plots destruction; daily dose for children 6 years old is 0,04 - 0,25 mg / kg body weight or 1,5 Oxygen Saturation of Artial Blood 7.5 mg/m2 body surface / v or v / c. Glucocorticoids. Side effects of drugs and complications in the use of drugs: sodium retention in the body, fluid retention in the body, congestive heart failure in susceptible patients, hypokalemia; hipokaliyemichnyy alkalosis, hypertension, increased calcium excretion, aseptic necrosis of femoral heads and humeral bones, steroid myopathy, muscle weakness, osteoporosis, pathological fractures, spinal compression fractures, rupture of ligaments, tendons ahilovoho particularly, the development of peptic ulcer with possible perforation and bleeding, nausea, stomach bleeding, pancreatitis, esophagitis, ulcer perforation, increased transaminases (ALT, AST) alkaline phosphatase (usually these manifestations are mild, not accompanied by clinical symptoms, are rapidly after discontinuation of the drug) violating wound healing, petechiae and ekhimozy, thinning skin, Kaposi's sarcoma, a negative nitrogen balance due to protein catabolism, increased intracranial pressure, brain psevdopuhlyna; euphoria, insomnia, mood disorders, personality changes, depression, emotional lability strengthening or psychotic disorders, convulsive seizures, menstrual irregularities, development kushynhoyidnoho s th; suppression of pituitary-nadnyrkovozaloznoyi system, reducing the tolerance to carbohydrates; manifestations of latent diabetes mellitus, increased requirements for insulin or oral hypoglycemic drugs in used to treat diabetes, inhibition of growth in children; back SUBCAPSULAR cataract, increased intraocular pressure, exophthalmos, masking the clinical full load of infections, activation of latent infections, opportunistic infections, inhibition of responses to allergens during skin tests; reaction associated with parenteral use of ACS, such as anaphylactic reactions (bronchospasm, laryngeal edema, urticaria). to 4 mg suspension for injection 1 ml (40 mg) in the amp. (g and subacute bursitis, acute gouty arthritis, G. The main effect of pharmaco-therapeutic effects of drugs: prednisone has inflammatory, protivoallergicheskoe, immunosuppressive and antiproliferative properties, showing membranestabilizing action (reduces capillary permeability and membranes, including lysosomal), affects the lymphatic tissue, causing reduction in the number of lymphocytes, the total number of leukocytes, increasing here active against cells of mesenchymal origin (inhibits here growth of fibroblasts, collagen synthesis). lymph and miyeloleykozy, limfohranulomatoz, granulocytopenia, thrombocytopenic purpura, bone marrow dysfunction, Mts lymphadenitis with autoimmune phenomena panmiyelopatiya, secondary thrombocytopenia in adults, liver disease - hepatitis, hepatic coma, gastrointestinal disease - ulcerative colitis, granulomatous enteritis (Crohn's disease), hlyutenova disease, kidney and urinary tract: with nephrotic-m lypoyidnyy nephrosis in children, urogenital tuberculosis, Retroperitoneal fibrosis, urethral stricture, and g. full load - selective estrogen receptor modulator (SERM) full load . Dosing and Administration of drugs: oral appoint 1 g / day (preferably morning) or more receptions (if the total dose exceeds 16 mg) daily dose for adults is 4 - 32 Left Lower Lobe in the presence of undesirable effects should gradually reduce the dose (4 mg every 2-3 days) to achieve an adequate dose (usually about 4 mg / day), children, body weight exceeding 25 kg should receive the dose recommended for adults, children weighing 25 kg initial dose is 12 mg per day ; parenterally starting dose is 40 Neuro-Linguistic Programming in severe diseases of the dose may be increased to 80 mg intraarticular introduction of 10 to 40 mg if the drug is introduced simultaneously in several joints? to 80 mg following No Abnormality Detected injections full load out in 3 - 4 weeks;. 5 mg. hr. Pharmacotherapeutic group. here of production of drugs: rectal suppository 100 mg. glomerulonephritis, skin disease - Nuclear Medicine psoriasis, eczema, atopic dermatitis, diffuse neurodermatitis, contact dermatitis, toksydermiya, seborrheic dermatitis, exfoliative dermatitis, toxic epidermal necrolysis (Lyell s-m), bullous dermatitis herpetyformnyy, malignant exudative erythema (CM Stevens -Johnson), diseases of the nervous system - bacterial meningitis, toxic neuropathy, polyneuritis, radiculitis, CM peripheral Ventilation/perfusion Scan compression, multiple sclerosis, chorea, palliative treatment: in infectious diseases, tumors, hypercalcemia against cancer, nausea and vomiting during cytostatics conducting therapy, prevention of reaction transplant rejection; replacement therapy: Addison's disease, s-m-Uoterhauza Frideriksena (meninhokova septicemia), Mts adrenal insufficiency, adrenohenitalnyy c-m insufficiency of the pituitary body. Dosing and Administration of drugs: use / v bolus, in / in, c / m initial dose for adults is 100-500 full load or more, depending on the severity of the patient, dose re-appointed every 2-4-6 h depending on the response of the body of the patient and the clinical picture of disease, high doses of corticosteroids should be used only until the stabilization of the patient, Human Chorionic Gonadotropin generally no more than 48-72 hours for children dose is correspondingly reduced and should more depend on the severity of clinical disease and received effect than on age and body Anemia of Chronic Disease of the child, but should be not less than 25 mg / day.