Symptoms of panhypopituitarism

The clinical symptomatology of the disease is very variable and consists of specific symptoms of hormonal insufficiency and polymorphic neurovegetative manifestations.

Pangipopituitarism is much more common in women of young and middle age (20-40 years), but individual cases of the disease are known in both earlier and in old age. The development of the Shien syndrome in a girl 12 years after juvenile uterine bleeding is described.

Symmonds syndrome in the clinical picture is dominated by a steadily increasing loss of body weight, an average of 2-6 kg per month, but with a severe, galloping disease that reaches 25-30 kg. In connection with anorexia, the amount of food consumed is significantly reduced, but the degree and intensity of weight loss in these cases are not adequate to the altered dietary conditions and are determined by primary disturbances of hypothalamic regulation of fat metabolism and a decrease in the level of growth hormone in the body.

In rare cases, along with cachexia, there is no decrease in appetite, but a pathological increase (bulimia). The depletion is usually even, since the subcutaneous fat layer disappears everywhere, the muscles atrophy, the internal organs decrease in volume. Edema, as a rule, does not happen.

Characteristic changes in skin: dryness, wrinkling, peeling in combination with pale yellow-like, waxy color. Sometimes, against a background of general pallor, there are patches of dirty-earthy pigmentation on the face and in the natural folds of the skin. Often there akrozianoz.

Violations of trophic processes lead to fragility and hair loss, their early graying, to atrophic processes in bone tissue with decalcification of bones and the development of osteoporosis. Atrophy of the lower jaw, the teeth collapse and fall out. Rapidly growing phenomenon marasmus, senile involution. Developing a sharp general weakness, apathy, adynamia right up to complete immobility; hypothermia. Patients report dizziness and frequent fainting. Characterized by orthostatic collapse and coma, without specific therapy leading to death.

One of the leading places in clinical symptoms is occupied by sexual disorders caused by a decrease or total loss of gonadotropic regulation of the gonads. These disorders often precede the appearance of all other symptoms. The sexual inclination is lost, the potency decreases, the pubic hair and axillae drop out. External and internal genital organs gradually atrophy. In women, menstruation disappears early and quickly, the mammary glands decrease in volume, the areola nipples are depigmented. With the development of the disease after delivery, lactation is absent, and menstruation is not renewed. In rare cases of a protracted and worn out course of the disease, the menstrual cycle is disturbed, but pregnancy is possible. In men, secondary sexual characteristics disappear (pubic, axillary hair, mustache, beard), testicles, prostate gland, seminal vesicles, penis are atrophied. There is an oligoazoospermia. In rare cases, alopecia can be universal, that is, hair on the head, eyebrows, and eyelashes drop out.

Reducing the production of thyroid-stimulating hormone leads to a rapid or gradual development of hypothyroidism. There is drowsiness, lethargy, adynamia, mental and physical activity decreases. Thyroid hypofunction leads to disruption of metabolic processes in the myocardium. Slow down the cardiac muscle, heart sounds become deaf, blood pressure decreases. Atony of the gastrointestinal tract and constipation develops.

Violation of the water-discharge function, characteristic of hypothyroidism, in patients with intermittent-pituitary insufficiency manifests itself in different ways. With severe depletion, edema usually does not exist, and in patients with Shien syndrome and predominance of symptoms of hypogonadism and hypothyroidism, there is usually no large loss of body weight, and fluid retention is significant. The face becomes puffy, the tongue thickens, dents from the teeth form on its lateral surfaces, there may be hoarseness and a decrease in the voice (edema of the vocal cords). Speech is slowed down, dysarthritic.

The severity of the disease and the nature of its course (rapid or gradual) are largely determined by the degree of decline in the function of the adrenal glands. Heavy hypocorticism reduces the resistance of patients to intercurrent infections and to various stressful situations. Hypocorticism aggravates general weakness, adynamy, hypotension and promotes the development of hypoglycemia.

The latter occurs in connection with a decrease in the level of central, counterinsulatory hormones (ACTH, STG) and biosynthesis of glucocorticoids in the adrenal cortex. Reduction of gluconeogenesis processes mediated by glucocorticoids leads to a drop in blood sugar level to 1-2 mmol / l and to the development of relative hyperinsulinism, which sharply increases the sensitivity of patients to insulin injected from the outside. In some cases, injections of 4-5 U of the drug led to severe hypoglycemic and even coma.

The pathogenesis of the pituitary coma in the finale of Simmonds cachexia or severe panhypopituitarism of another etiology is determined mainly by progressive hypocorticism and hypothyroidism. Comatose state develops, as a rule, gradually, with the increase of adynamia, which goes to stupor, hyponatremia, hypoglycemia, seizures and hypothermia.

Hypocorticism also causes severe dyspeptic disorders: a decrease in appetite, reaching full anorexia, persistent nausea and vomiting after ingestion or out of touch with digestion, abdominal pain resulting from a spasm of smooth bowel musculature. Characteristic and atrophic processes in the mucosa with a decrease in gastrointestinal and pancreatic secretion.

The hypothalamic component in clinical symptoms may manifest as a disorder of thermoregulation more often with hypothermia, but sometimes with subfebrile and vegetative crises with hypoglycemia, chills, tetanic syndrome and polyuria.

Often develops decalcification of bones, osteoporosis, violations of the peripheral nervous system with polyneuritis, polyradiculoneuritis and severe pain syndrome.

Mental disorders are observed in all cases of hypothalamic-hypophyseal insufficiency. Characteristic is a decrease in emotional activity, indifference to the environment, depression and other mental deviations up to a schizophrenic hallucinatory-paranoid psychosis. Hypothalamic-pituitary insufficiency due to a pituitary or hypothalamic tumor is combined with a number of symptoms of increased intracranial pressure: ophthalmic, radiologic and neurological (headaches, decreased acuity and limitation of visual fields).

The course of the disease can be different. In patients with cachexia and the prevalence of symptoms of hypokorticism, there is a progression of all symptoms leading to a fatal outcome in a short time. With Shiena's syndrome, on the contrary, the disease develops gradually, gradually, sometimes remaining unrecognized for many years.

[1], [2], [3], [4]