Pituitary Dwarfism (Dwarfism)

A disease characterized by retarded growth and physical development. Dwarfism is considered to be the height of an adult man below 130 cm, and for an adult woman - below 120 cm.

Etiology. Genetic factors, tumor factors (craniopharyngioma, meningioma, chromophobe adenoma), traumatic, toxic and infectious damage to the interstitial pituitary region are important.

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

Symptoms Growth retardation is detected in the first months of a child’s life, less often during puberty (not only height and body weight are taken into account, but also the dynamics of these indicators). The body retains the proportions characteristic of childhood. There is a lag in differentiation and synostosis of the skeleton with age, and a delay in changing teeth. The skin is dry, pale, wrinkled; poor development of subcutaneous fatty tissue, sometimes excessive fat deposition on the chest, abdomen, and thighs. The muscular system is poorly developed.

Loss of gonadotropic function of the pituitary gland is manifested by signs of insufficiency of sexual development. In male patients, the gonads and penis are reduced compared to age norms, the scrotum is underdeveloped, and there are no secondary sexual characteristics. The majority of female patients also exhibit symptoms of hypogonadism: no menstruation, underdeveloped mammary glands, and secondary sexual characteristics.

Mental development is normal with some juvenile features. A neurological examination may reveal signs of organic damage to the nervous system. Characterized by a decrease in the size of internal organs (splanchnomicria), hypotension and bradycardia are common.

Phenomena of secondary hypothyroidism and secondary hypocortisolism are possible. The sella turcica is, as a rule, reduced, and there is often calcification of its diaphragm; in the presence of a pituitary tumor, enlargement of the sella turcica and destruction of its walls are detected. X-ray examination of the hands and wrist joints reveals a delay in differentiation and ossification of the skeleton.

The basal concentration of growth hormone in the blood serum is reduced or within normal limits, the administration 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 short-term and slight increase in its secretion. To diagnose growth hormone deficiency in children, they also use the determination of its excretion in urine, and determine the level of insulin-like growth factors and proteins that bind them.

Treatment. Replacement therapy with genetically engineered growth hormone preparations is carried out in case of deficiency of endogenous somatotropin of pituitary or hypothalamic-pituitary origin confirmed by clinical, hormonal and instrumental methods. In the treatment of classic growth hormone deficiency in children - 0.07-0.1 U/kg per injection, daily, intravenously, 6-7 injections per week. Treatment is carried out until the growth zones are closed or until socially acceptable growth is achieved.

Patients with clinical manifestations of hypothyroidism are prescribed thyroxine. To stimulate sexual development after the closure of growth plates, sex hormones are prescribed: for women - estrogens and corpus luteum preparations (ethinyl estradiol, etc.), for men - testosterone preparations; for hypofunction of the adrenal cortex - glucocorticoids.