Pituitary Nanism (dwarfism)

Gipofizarnyj nanizm (dwarfism) - the disease characterized by a delay of growth and physical development. Dwarf considers the growth of an adult male below 130 cm, the adult woman - below 120 cm.

Etiology. Genetic factors, tumor factors (craniopharyngiomas, meningiomas, chromophobic adenomas), traumatic, toxic and infectious lesions of the interstitial-pituitary region are important.

Pathogenesis. Decrease or loss of somatotropic function of the pituitary gland, biological inactivity of growth hormone or impaired sensitivity of peripheral tissues.

Symptoms. The delay in growth is revealed in the first months of the child's life, less often during puberty (not only the height and body weight, but also the dynamics of these indicators are taken into account). The body retains the proportions characteristic of childhood. There is a lag in the differentiation and synostosis of the skeleton from age, the delay in the change of teeth. Skin is dry, pale, wrinkled; A weak development of subcutaneous fat, sometimes excessive fat deposition on the chest, abdomen, thighs. The muscular system is poorly developed.

The loss of gonadotropic function of the pituitary gland is manifested by signs of a lack of sexual development. In male patients, the sex glands and penis are reduced in comparison with age norms, the scrotum is underdeveloped, there are no secondary sexual characteristics. The majority of female patients are also expressed hypogonadism: there are no menstruation, mammary glands are underdeveloped, secondary sexual characteristics. Mental development is normal with some juvenile features. When a neurological examination can be detected signs of organic damage to the nervous system. Characteristic decrease in the size of internal organs (splanchnomycria), often hypotension, bradycardia. Possible phenomena of secondary hypothyroidism and secondary hypocorticism.

The Turkish saddle is usually reduced, often there is a calcification of its diaphragm; In the presence of a pituitary tumor, an increase in the Turkish saddle, the destruction of its walls, is revealed. Radiographic examination of the wrists and wrist joints indicates a delay in the differentiation and ossification of the skeleton. Basal concentration of growth hormone in the blood serum is reduced or within the normal range, the introduction of insulin (insulin test) or arginine is not accompanied by an increase in the secretion of growth hormone, in some cases there may be a brief and insignificant increase in its secretion.

Treatment. To stimulate growth, intermittent courses of therapy with anabolic steroids (methylandrostenediol-1-1.5 mg / kg per day under the tongue, methandrostenolone-0.1-0.15 mg / kg / day, phenobolin-1 mg / kg per month, Monthly dose is given for 2-3 doses, retabolil - 1 mg / kg per month). Human somatotropin (with a low level of endogenous somatotropin) at 4 units intramuscularly 3 times a week for 2 months with interruptions for 2 months. Patients with clinical manifestations of hypothyroidism are prescribed thyroidin, thyreog, thyrecombe, thyroxine. To stimulate sexual development, after closing the growth zones, sex hormones are prescribed: for women - estrogens and yellow body preparations (sinestrol, microfollin, estradioladipropionate, pregnin, progesterone, infekundin), chorionic gonadotropy; Men-chorionic gonadotropin and testosterone preparations (testosterone propionate, testanate, sustanon-250, omnmemia), with adrenal cortex hypofunction - prednisolone, cortisone, deoxycorticosterone acetate.

When the function of the central nervous system is prescribed glutamic acid, cerebrolysin, aminalon. In some cases, the dehydration (furosemide, veroshpiron, hypothiazide), resorption (biyohinol, aloe) therapy is carried out. Diet with increased protein, vitamins.