Addison disease can also be genetic

Addison’s disease can also be genetically determined. With the familial nature of the disease, it is believed that the inheritance of the Addison recessive type. During genesis, antibodies can be not only to the adrenal glands, but also to other organs: the thyroid gland, the mucous membrane of the gastrointestinal tract, the ovaries, etc. Often, these antibodies are also determined by the parents of patients, which suggests that in these cases the hereditary nature of the autoimmune lesions of the adrenal glands. Sometimes, isolated insufficiency of the glomerular zone of the adrenal cortex can occur – isolated hypoaldosteronism. The latter develops due to a congenital defect in aldosterone biosynthesis, which manifests itself in impaired formation of 18-hydroxylase and 18-dehydrogenase, which are involved in the conversion of corticosterone to aldosterone. Addison’s disease of a genetic nature can occur due to congenital insensitivity of the adrenal cortex in ACTH.

The main cause of secondary adrenal insufficiency is considered to be deficiency due to various disorders in the hypothalamic-pituitary system and, in particular, during prolonged therapy with large doses of glucocorticoids due to different chronic tuberculosis — pathogenesis. Deficiency of glucocorticoids (cortisol and corticosterone) leads to adynamia, cardiovascular and gastrointestinal disorders, a sharp decrease in the body’s resistance to adverse factors (infection, intoxication, etc.), a decrease in blood sugar, neutropenia, eosinophilia, lymphocytosis, etc. As a result of insufficient production of adrenal glands of mineralocorticoids (aldosterone), there is a violation of water-salt metabolism (hypo natremia, hypochloremia, hyperkalemia), which leads to dehydration and hypotension A decrease in the production of adrenal hormones by the sex hormones (androgens and estrogens) leads to impotence in men and women to impaired menstrual cycles. The onset of pigmentation is due to the increased deposition of melanin pigment in the papillary dermis and cystic membranes.

Pathological anatomy. Morphological changes in over
renal depend on. causes of disease. In addison disease of tuberculous etiology, necropsy and medulla of both adrenal glands are found at autopsy. They reveal caseosis, tubercular tubercles, foci of disintegration, calcification. In Addison’s disease, which has arisen as a result of primary or secondary atrophy of the adrenal glands, their hypoplasia and degenerative changes are noted, mainly in the puchous and reticular zones of the cortex. The primary atrophy of the adrenal cortex reveals the development of fibrous tissue, round-cell infiltrates consisting of accumulations of lymphocytes and plasmatic cells, as well as hyperplastic islands of hypertrophied cells of the cortex. Often found lymphoid infiltration of the thyroid gland and the development of fibrous tissue in it.

Atrophic changes are also observed in the heart, liver, muscles, and muscles.
Classification. There is no generally accepted classification of Addison eolezni.
According to the clinical course, there are typical forms and typical forms: mineral (like hypoaldosteronism), non-pigmental, etc.

In severity, Addison’s disease can be mild, moderate, and severe.
In the mild form of the disease, the clinical effect (restoration of disturbed metabolic processes, reduction of pigmentation and adynamia, normalization of blood pressure and body weight, restoration of working capacity) is achieved with the help of a diet without hormone replacement therapy. In case of addison disease of moderate severity, hormone replacement therapy (glucocorticoids – cortisone, hydrocortisone, prednisolone, etc.) is required to achieve a clinical effect. ‘

With a severe form of the disease, patients are prone to the development of an addisonic crisis. Their clinical effect can be achieved only with the help of constant replacement therapy with glucocorticoids in combination with mineralocorticoids (deoxycorticosterone acetate, forhydrocortisone, etc.).

Clinic. The disease develops gradually. Patients complain of severe weakness, rapid physical fatigue, darkening of the skin, loss of appetite, nausea, vomiting, diarrhea, weight loss, pain in the muscles of the upper and lower extremities, lower back. The skin of patients most often has a golden brown
(smoky-bronze) color. Pigmentation is especially pronounced on exposed parts of the body (face, palmar folds, back of hands and feet) and areas exposed to friction of clothing (axillary and groin areas, elbows, knees, waist, skin folds). Pigmentation of postoperative cicatrices is noted, as well as increased pigmentation in places of natural pigment deposition (mammary nipples, genitals).
Especially characteristic pigmentmucous membranes in the form of seo-black spots.

Hypothermia is often the case. On the part of the cardiovascular system, hypotension is usually noted (mainly due to a decrease in systolic pressure), a decrease in pulse pressure and minute blood volume. Pulse frequent, small and soft. The size of the heart is reduced, the tones are muffled. On ECG, a low voltage of the teeth is often found, the interval S — T is below the isoelectric line, flattened negative or two-phase wave 7 \ lengthening of the interval P — Q and QRS complex. In the lungs, symptoms of an active or inactive tuberculous process are detected.

On the part of the gastrointestinal tract there are dyspeptic phenomena, attacks of abdominal pain (addisonic gastrointestinal crises), a decrease in the acidity of the gastric juice, a decrease in the external secretion of the pancreas.

In some cases, chronic gastritis develops. Observed combinations of Addison’s disease with gastric ulcer or duodenal ulcer. Sometimes there are multiple superficial ulcerations of the mucous membrane of the gastrointestinal tract. The antitoxic, protein-forming and glycogen-forming functions of the liver are impaired.

Impaired renal function is manifested in the reduction of club-like filtration and reabsorption of sodium and chloride, which especially pronounced during the crisis.

Changes in the sexual sphere are variable. In men in a row in cases of decreased libido and potency, in women the menstrual cycle is disturbed.

Neuropsychiatric disorders are manifested in increased nervous excitability or depression, insomnia. Sometimes psychosis may develop, in some cases paresthesias and convulsions. With a severe form of the disease, an electroencephalogram often reveals a predominance of slow activity such as the 0- and b-frequency range and the absence of an a-rhythm, which indicates a decrease in the activity of the cerebral cortex.

local_offerevent_note February 19, 2019

account_box Admin

Leave a Reply

Your email address will not be published. Required fields are marked *