Nadpochechnikovy insufficiency – the disease arising owing to insufficient hormonal secretion of bark of adrenal glands (primary) or the gipotalamo-hypophysial system (secondary nadpochechnikovy insufficiency) regulating them. It is shown by characteristic bronze pigmentation of integuments and mucous membranes, sharp weakness, vomiting, ponosa, tendency to faints. Leads to disorder of water and electrolytic exchange and violation of warm activity. Treatment of nadpochechnikovy insufficiency includes elimination of its reasons, replacement therapy by kortikosteroidny medicines, symptomatic therapy.
Nadpochechnikovy insufficiency – the disease arising owing to insufficient hormonal secretion of bark of adrenal glands (primary) or the gipotalamo-hypophysial system (secondary nadpochechnikovy insufficiency) regulating them. It is shown by characteristic bronze pigmentation of integuments and mucous membranes, sharp weakness, vomiting, ponosa, tendency to faints. Leads to disorder of water and electrolytic exchange and violation of warm activity. Extreme manifestation of nadpochechnikovy insufficiency is nadpochechnikovy crisis.
Cortical substance of adrenal glands produces glyukokortikosteroidny (cortisol and ) and mineralokortikoidny () the hormones regulating main types of exchange in fabrics (proteinaceous, carbohydrate, water-salt) and adaptation processes of an organism. Sekretorny regulation of activity of bark of adrenal glands is carried out by a hypophysis and a hypothalamus by means of secretion of AKTG hormones and a kortikoliberin.
Nadpochechnikovy insufficiency unites various etiologichesky and pathogenetic options of a gipokortitsizm - the state developing as a result of hypofunction of bark of adrenal glands and deficiency of the hormones produced by it.
Classification of nadpochechnikovy insufficiency
Insufficiency of bark of adrenal glands can carry a sharp and chronic current.
The sharp form of nadpochechnikovy insufficiency is shown by development of serious condition - the addisonichesky crisis which is usually representing a decompensation of a chronic form of a disease. The current of a chronic form of nadpochechnikovy insufficiency can be compensated, subcompensated or dekompensirovanny.
According to initial violation of hormonal function chronic nadpochechnikovy insufficiency is divided on primary and central (secondary and tertiary).
Primary insufficiency of bark of adrenal glands (1-HH, primary gipokortitsizm, an addisonova or a bronze disease) develops as a result of bilateral damage of adrenal glands, meets more than in 90% of cases, is not dependent on a floor, is more often at mature and advanced age.
Secondary and tertiary nadpochechnikovy insufficiency meet much less often and arise owing to a lack of secretion of AKTG a hypophysis or a kortikoliberina a hypothalamus leading to an atrophy of bark of adrenal glands.
Reasons of nadpochechnikovy insufficiency
Primary nadpochechnikovy insufficiency develops at defeat of 85-90% of tissue of adrenal glands.
In 98% of cases as the reason of primary gipokortitsizm serves the idiopathic (autoimmune) atrophy of a cortical layer of adrenal glands. At the same time for the unknown reasons in an organism the autoimmune antibodies to enzyme 21 to a hydroxylase destroying healthy tissues and cells of adrenal glands are formed. Also at 60% of patients with primary idiopathic form of nadpochechnikovy insufficiency autoimmune defeats of other bodies are noted, is more often – autoimmune tireoidit. Tubercular damage of adrenal glands occurs at 1-2% of patients and is in most cases combined with tuberculosis of lungs.
The rare genetic disease – an adrenoleykodistrofiya serves as the reason of primary nadpochechnikovy insufficiency in 1-2% of cases. Genetic defect of the H-chromosome is resulted by a lack of the enzyme splitting fatty acids. Primary accumulation of fatty acids in fabrics of nervous system and bark of adrenal glands causes their dystrophic changes.
Extremely seldom to development of primary nadpochechnikovy insufficiency bring koagulopatiya, tumoral metastasises into adrenal glands (is more often from a lung or a mammary gland), a bilateral heart attack of adrenal glands, infection HIV-associated, bilateral removal of an adrenal gland.
Serious nagnoitelny illness, syphilis, fungal defeats and adrenal glands, malignant tumors, heart diseases, use of some medicines (anticoagulants, blockers of a steroidgenez, a ketokonazol, a hloditan, a spironolakton, barbiturates) etc. contribute to development of an atrophy of bark of adrenal glands.
Secondary nadpochechnikovy insufficiency is caused by the destructive or tumoral processes of gipotalamo-hypophysial area leading to violation of kortikotropny function, as a result:
- tumors of a hypothalamus and hypophysis: kraniofaringioma, adenomas, etc.;
- vascular diseases: hemorrhages in a hypothalamus or a hypophysis, carotid aneurism;
- granulematozny processes in a hypothalamus or a hypophysis: syphilis, , granulematozny or autoimmune gipofizit;
- destruktivnotravmatichesky interventions: radiation therapy of a hypothalamus and hypophysis, operation, long-term treatment by glucocorticoids etc.
Primary gipokortitsizm is followed by decrease in secretion of hormones of bark of adrenal glands (cortisol and an aldoateron) that leads to a metabolic disorder and balance of water and salts in an organism. At deficiency of an aldosteron the progressing dehydration caused by loss of sodium and a delay of potassium (giperkaliyemiya) in an organism develops. Water and electrolytic frustration cause violations from digestive and cardiovascular systems.
Reduction of level of cortisol reduces synthesis of a glycogen, leading to development of a hypoglycemia. In the conditions of deficiency of cortisol the hypophysis begins is raised to develop AKTG and the melanotsitostimuliruyushchy hormone causing strengthening of pigmentation of skin and mucous. Various physiological stresses (injuries, infections, a decompensation of associated diseases) cause progressing of primary nadpochechnikovy insufficiency.
The secondary gipokortitsizm is characterized only by deficiency of cortisol (as a result of a lack of AKTG) and preservation of production of an aldosteron. Therefore secondary nadpochechnikovy insufficiency, in comparison with primary, proceeds rather easily.
Symptoms of nadpochechnikovy insufficiency
The leading criterion of primary chronic nadpochechnikovy insufficiency is the hyperpegmentation of integuments and mucous membranes which intensity depends on prescription and weight of a gipokortitsizm. In the beginning more dark coloring is got by the open parts of the body subject to solar radiation, - face skin, necks, hands, and also the sites having normal more dark pigmentation - an areola of nipples, genitalia, a scrotum, a crotch, axillary areas. A characteristic sign is the hyperpegmentation of palmar folds noticeable against the background of lighter skin, darkening of the sites of skin more adjoining to clothes. Skin color varies from a subtle shade of suntan, bronze, smoky, dirty skin to diffusion and dark. Pigmentation of mucous membranes (internal surface of cheeks, language, sky, gums, vagina, rectum) bluish-black coloring.
Less often nadpochechnikovy insufficiency a little expressed hyperpegmentation - "a white addisonizm" meets. Quite often against the background of sites of a hyperpegmentation pigment-free light spots – vitiligo, the size from small to large, irregular shape, allocated on more dark skin are found in patients. Vitiligo meets only at autoimmune primary chronic gipokortitsizm.
At patients with chronic nadpochechnikovy insufficiency body weight from moderate weight loss (by 3-5 kg) to a considerable hypotrophy decreases (on 15-25 kg). The adynamy, irritability, a depression, weakness, slackness are noted, up to disability, decrease in sexual desire. It is observed orthostatic (at sharp change of position of a body) the arterial hypotension, unconscious states caused by psychological shocks and stresses. If before development of nadpochechnikovy insufficiency in the patient at the anamnesis there was arterial hypertension, HELL can be within norm. Practically always disorders of digestion - nausea, a loss of appetite, vomiting, pain develop in epigastriya, a liquid chair or locks, anorexia.
At the biochemical level there is a violation proteinaceous (decrease in synthesis of protein), carbohydrate (decrease in level of glucose on an empty stomach and a flat sugar curve after glucosic loading), water-salt (a giponatriyemiya, a giperkaliyemiya) exchanges. At patients the expressed addiction to the use of salty food is noted, up to the use of pure salt, connected with the accruing loss of salts of sodium.
Secondary nadpochechnikovy insufficiency proceeds without hyperpegmentation and the phenomena of insufficiency of an aldosteron (arterial hypotonia, addiction to salty, dyspepsias). Nonspecific symptoms are characteristic of it: the phenomena of the general weakness and the attacks of a hypoglycemia developing in several hours after food.
Complications of nadpochechnikovy insufficiency
The most terrible complication of a chronic gipokortitsizm at not carried out or wrong treatment is nadpochechnikovy (addisonichesky) crisis – a sharp decompensation of chronic nadpochechnikovy insufficiency with coma development. Addisonichesky crisis is characterized by the sharpest weakness (up to a condition of prostration), falling of arterial pressure (up to a collapse and loss of consciousness), unrestrained vomiting and a liquid chair with bystry increase of dehydration of an organism, an acetone smell from a mouth, clonic spasms, heart failure, a bigger pigmentation of integuments.
Sharp nadpochechnikovy insufficiency (addisonichesky crisis) on prevalence of symptoms can proceed in three clinical forms:
- cardiovascular at which blood circulation violations dominate: pallor of skin, , cold snap of extremities, tachycardia, arterial hypotonia, threadlike pulse, collapse, anury;
- the gastrointestinal, reminding signs on symptomatology food toksikoinfektion or clinic of a sharp stomach. There are spastic belly-aches, nausea with unrestrained vomiting, a liquid chair with blood, a meteorizm.
- psychological, with prevalence of a headache, meningealny symptoms, spasms, focal symptomatology, nonsense, block, a stupor.
Addisonichesky crisis difficult gives in to knocking over and can cause death of the patient.
Diagnostics of nadpochechnikovy insufficiency
Diagnostics of nadpochechnikovy insufficiency there begin with assessment of the anamnesis, complaints, fizikalny data, clarifications of the reason of a gipokortitsizm. Carry out ultrasonography of adrenal glands. Existence of kaltsinat or centers of tuberculosis in adrenal glands testifies to primary nadpochechnikovy insufficiency of tubercular genesis; at blood are present at the autoimmune nature of a gipokortitsizm an autoantitela to nadpochechnikovy to an antigenu21-hydroxylase. In addition for identification of the reasons of primary nadpochechnikovy insufficiency MPT or KT of adrenal glands can be required. For the purpose of establishment of the reasons of secondary insufficiency of bark an adrenal gland of KT and MPT of a brain.
At primary and secondary nadpochechnikovy insufficiency decrease in blood of cortisol and reduction of daily allocation of free cortisol and 17-OKC with urine is observed. Increase in concentration of AKTG, for secondary – its reduction is characteristic of primary gipokortitsizm. At doubtful data for nadpochechnikovy insufficiency carry out the stimulation test with AKTG, defining the maintenance of cortisol in blood in half an hour and hour after introduction of adrenokortikotropny hormone. Increase in level of cortisol less than on the 550th nmol/l (20 mkg/dl) testifies to insufficiency of adrenal glands.
Apply test of the insulin hypoglycemia causing normal considerable emission of AKTG and the subsequent increase in secretion of cortisol to confirmation of secondary insufficiency of bark of adrenal glands. At primary nadpochechnikovy insufficiency in blood the giponatriyemiya, a giperkaliyemiya, , an eozinofiliya and a leykopeniye are defined.
Treatment of nadpochechnikovy insufficiency
The modern endocrinology possesses effective methods of treatment of nadpochechnikovy insufficiency. The choice of a method of treatment depends first of all on the cause of a disease and pursues two aims: elimination of the reason of nadpochechnikovy insufficiency and replacement of hormonal deficiency.
Elimination of the reason of nadpochechnikovy insufficiency includes drug treatment of tuberculosis, fungal diseases, syphilis; antineoplastic radiation therapy on area of a hypothalamus and hypophysis; expeditious removal of tumors, aneurisms. However in the presence of irreversible processes in adrenal glands, the gipokortitsizm remains and demands lifelong performing replacement therapy by hormones of bark of adrenal glands.
Treatment of primary nadpochechnikovy insufficiency is carried out by glucocorticoid and mineralokortikoidny medicines. At easy manifestations of a gipokortitsizm the cortisone or a hydrocortisone are appointed, at more expressed - a combination of Prednisolonum, a cortisone of acetate or a hydrocortisone from mineralokortikoida (a dezoksikortikosterona , DOKSA - a dezoksikortikosterona acetate). Efficiency of therapy is estimated on indicators of arterial pressure, gradual regress of a hyperpegmentation, a body weight increase, improvement of health, disappearance of dyspepsia, anorexia, muscular weakness etc.
Hormonal therapy at patients with secondary nadpochechnikovy insufficiency is carried out only by glucocorticoids since secretion of an aldosteron is kept. At various stressful factors (injuries, operations, infections, etc.) doses of corticosteroids increase by 3-5 times, at pregnancy insignificant increase in a dose of hormones is possible only in the second trimester.
Purpose of anabolic steroids () at chronic nadpochechnikovy insufficiency is shown to both men, and women by courses to 3 times a year. Observance of the diet enriched with protein, carbohydrates, fats, sodium salts, vitamins B and With, but with restriction of salts of potassium is recommended to patients with a gipokortitsizm. For knocking over of the phenomena of addisonichesky crisis carry out:
- rehydration therapy by isotonic NaCl solution of 1,5-2,5 l a day in combination with 20% glucose solution;
- intravenous replacement therapy by a hydrocortisone or Prednisolonum with gradual decrease in a dose in process of subsiding of the phenomena of sharp nadpochechnikovy insufficiency;
- symptomatic therapy of the diseases which led to a decompensation of chronic nadpochechnikovy insufficiency (is more often antibacterial therapy of infections).
Forecast and prevention of nadpochechnikovy insufficiency
In case of timely purpose of adequate hormonal replacement therapy course of nadpochechnikovy insufficiency rather favorable. The forecast at patients in many respects decides on a chronic gipokortitsizm by prevention and treatment of nadpochechnikovy crises. At the infections accompanying a disease, injuries, surgeries, stresses, gastrointestinal frustration immediate increase in a dose of the appointed hormone is necessary.
Active identification and statement on the dispensary account at the endocrinologist of patients with nadpochechnikovy insufficiency and faces of risk group is necessary (it is long accepting corticosteroids at various chronic diseases).