Simmonds's syndrome

Description:

Simmonds's syndrome (a cachexia pituitary, a panhypopituitarism) is caused by decrease in products of all tropny hormones of a hypophysis. For development of partial hormonal insufficiency 60-70% of a front share have to be struck, and at defeat of 90% the panhypopituitarism forms.

Symptoms of the Syndrome of Simmonds:

Clinical manifestations are variable and depend on extent of loss of these or those tropny hormones.

The disease is observed at women of young and middle age (20-40 years), but casuistic cases of its development in teenagers and women of advanced age are known, it is rare at men. The disease develops slowly. Unmotivated weakness is most characteristic. Women have scanty periods, rare or at all are absent. Body weight decreases. Appearance change is characteristic: the thinned, mummified, dry, wrinkled skin (as tissue paper) alabaster-white or wax color, with reduced greasiness and sweating, a depigmentation of nipples and a crotch, a brittle, "disobedient", dry hair. The destroyed teeth, fragile nails considerably age patients. At survey hair on a pubis and under mice are absent. Patients talk a low, deaf voice, they are disturbed by dizziness, frequent unconscious states, sexual desire is lost, the potentiality decreases. Outside and internal generative organs gradually atrophy. At women mammary glands are reduced. At development of a disease after the delivery reveal an agalaxia, at the same time periods do not renew. In rare instances of the long and erased disease the menstrual cycle though is broken, but remains, and even pregnancy is possible. At men secondary sexual characteristics (sexual, axillary pilosis, moustaches, a beard) disappear, testicles, a prostate, seed bubbles, a penis atrophy, arises oligo-or an azoospermism.

Decrease in products of STG with its universal influence on proteinaceous synthesis leads to the progressing atrophy of smooth and skeletal muscles and internals.


Defect of synthesis of prolactin leads to an agalaxia. The combination of a giperprolaktinemiya and a hypopituitarism is possible at a rupture of a leg of a hypophysis (a syndrome of the isolated hypophysis), rapid growth of a tumor, hypothalamic defeats.

Because of a hypothyroidism there are a chill, drowsiness, slackness, an adynamia, intellectual and physical activity decreases. Cordial reductions are more rare, cardiac sounds become deafs, the ABP decreases.

Because of a hypocorticoidism dispepsichesky frustration develop: a loss of appetite up to full anorexia, nausea, vomiting, abdominal pains. The atony of a GIT and atrophic processes in a mucous membrane with reduction of gastrointestinal and pancreatic secretion are characteristic.

Decrease in level of sex hormones causes osteoporosis.

Mental disturbances are observed at all options of gipotalamo-pituitary insufficiency. Amnesia, a depression and other mental deviations up to hallucinatory paranoid psychosis are characteristic indifference to surrounding. Gipotalamo-gipofizarnaya insufficiency against the background of a tumor of a hypophysis or a hypothalamus is combined with a number of ophthalmologic, radiological and neurologic symptoms of increase in intracranial pressure: headache, decrease in sharpness and restriction of fields of vision.

Course of a disease various. At patients with a cachexia and dominance of symptoms of a hypocorticoidism all symptoms leading without treatment to a lethal outcome quickly progress. However more often the disease develops gradually, gradually, sometimes remaining not diagnosed for many years.

Reasons of the Syndrome of Simmonds:

Lead to insufficiency of products of tropny hormones collagenoses, tumoral and systemic lesions of a hypophysis, an autoimmune hypophysitis, persistent specific infections, hemorrhages, embolisms. Decrease in products of a number of tropny hormones at primary empty Turkish saddle is possible (inborn defect of a diaphragm with invagination of soft covers). Pituitary insufficiency can be also the result of operational or beam impacts on a hypophysis, metastasises or an apoplexy of a hypophysis, especially at various vascular diseases.

Clinical picture. Irrespective of the reasons of development of a hypopituitarism decrease in products of tropny hormones of a hypophysis is the cornerstone of its clinical manifestations that is followed by symptoms of a secondary hypothyroidism, a hypocorticoidism, a hypogonadism.

The back share, as a rule, is not involved in pathological process, and the combination of a panhypopituitarism and not diabetes mellitus is only in rare instances possible. Sometimes not diabetes mellitus is revealed in the course of replacement therapy by glucocorticoids concerning a secondary hypocorticoidism.

Treatment of the Syndrome of Simmonds:

The tumor causing destruction in a hypophysis and a hypothalamus is subject to radical treatment (surgical, beam, administration of radioactive yttrium, a cryolysis). At the diseases caused by acute or persistent infection special or anti-inflammatory drugs are effective.

In replacement therapy use preferential drugs of peripheral closed glands and, more rare, missing tropny hormones of an adenohypophysis. Irrespective of the disease nature at all forms of an organic hypopituitarism treatment has to be systematic, almost always lifelong.

Treatment is begun usually with drugs of bark of adrenal glands. For elimination of a hypocorticoidism peroral drugs, but in hard cases – parenteral are shown. Use a cortisone (75-25 mg/days), or a hydrocortisone (50-200 mg/days), or Prednisolonum (15-5 mg/days). Mineralokortikoida appoint in addition in need of (cortexone acetate, usually in the form of sublingual tablets on 5 mg 1-2 times a day). In recent years the peroral mineralokortikoidny drug "kortinef" (0,0001 g) on 1/4-1 tablet daily is more effective. At physical overworks, infections, injuries and other stresses the dose of corticosteroids should be increased. In 10-15 days of treatment by glucocorticoids add synthetic corticotropin with the shortened polypeptide chain – Synacthenum depot for parenteral administration in a dose of 1 ml (100 PIECES) 1-2 times a week.

Insufficiency of gonads is compensated at women by estrogen and progestins, and at men – drugs of androgenic action. In need of recovery of fertility after preliminary treatment sex hormones and reduction of atrophic processes in generative organs cyclically appoint gonadotrophins. From 75 to 300 PIECES enter a human menopauzny gonadotrophin with preferential follicle-stimulating action (хумегон or pergonal) intramusculary within 10 days under control of content of estrogen; then for the 14th, 16th and 18th day of a cycle – intramusculary the chorionic gonadotrophin (CG) with luteinizing action in a dose from 4500 to 10 000 PIECES and more. In addition to specific influence on the sexual device, sex hormones render positive trophic and anabolic effect. At men with the replaceable purpose apply Testosteroni propionas on 25 mg 2-3 times a week intramusculary or drugs of the prolonged action: Testoenatum (10% solution on 1 ml each 10-15 days), sustanon-250 (on 1 ml of 1 times in 3-4 weeks). Replacement therapy by androgens at young age is alternated to introduction of a chorionic gonadotrophin (profaza) on 500-1500 PIECES intramusculary by 2-3 times a week during 13-15 weeks in combination with pergonal (75-150 PIECES intramusculary 3 times a week).

Drugs of thyroid hormones are appointed in several days after the beginning of corticosteroid therapy as strengthening of exchange processes aggravates a hypocorticoidism. In recent years preferential synthetic drugs of thyroxine, since a dose of 25 mkg a day are recommended. Care at administration of thyroid drugs is defined not only by a hypocorticoidism, but also hypersensitivity of a myocardium of patients with a hypothyroidism to thyroid hormones.