Acromegaly and gigantism are related to neuroendocrine diseases, which are based on a pathological increase in growth activity.
Gigantism (Greek gigantos - giant, giant syn: macrosomia) - a disease that occurs in children and adolescents with incomplete physiological growth, characterized by exceeding the physiological boundaries by a relatively proportionate epiphysial and periosteal increase in bones, soft tissues and organs. Pathological is considered to be height above 200 cm in men and 190 cm in women. After ossification of the epiphyseal cartilage, gigantism, as a rule, passes into acromegaly. The leading sign of acromegaly (Greek akros - extreme, the most distant, and megas, megalu - large) is also the accelerated growth of the body, but not in length, but in width, which manifests itself in a disproportional periosteal increase in the bones of the skeleton and internal organs, a characteristic metabolic disorder. The disease, as a rule, develops in adults.
For the first time this disease was described by P. Marie in 1886, and a year later O. Minkowski (1887) proved that the basis of P. Marie's disease is an increase in the hormonal activity of the pituitary tumor, which, according to S. Benda (1903), represents a conglomerate of highly multiplying eosinophilic cells in the anterior lobe of the epididymis. " In domestic literature the first report on acromegaly was made in 1889 by BM Shaposhnikov.
The causes and pathogenesis of acromegaly and gigantism. The vast majority of cases are sporadic, but cases of familial acromegaly have been described.
At the end of the XIX century, the theory of the pituitary syndrome was put forward. Subsequently, mainly domestic researchers on a large clinical material showed the inconsistency of localistic concepts of the exceptional role of the pituitary gland in the pathogenesis of the disease. It was proved that a primary role in its development is played by primary pathological changes in the interstitial and other parts of the brain.
A characteristic feature of acromegaly is an increased secretion of growth hormone. However, there is not always a direct correlation between its content in the blood and the clinical signs of disease activity. Approximately 5-8% of cases, with a small or even normal level of somatotropic hormone in the blood serum, show pronounced acromegaly in patients, which is explained either by a relative increase in the content of a specific form of growth hormone possessing great biological activity, or by an isolated increase in the level of IGF.
The causes and pathogenesis of acromegaly and gigantism
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