Acromegaly and Gigantism - Information Review

Acromegaly and gigantism are neuroendocrine diseases that are based on a pathological increase in growth activity.

Gigantism (Greek gigantos - giant, giant; synonym: macrosomia) is a disease that occurs in children and adolescents with incomplete physiological growth, characterized by a relatively proportional epiphyseal and periosteal enlargement of bones, soft tissues and organs that exceeds physiological limits. Pathological growth is considered to be above 200 cm in men and 190 cm in women. After ossification of the epiphyseal cartilages, gigantism usually develops into acromegaly. The leading symptom of acromegaly (Greek akros - extreme, most distant, and megas, megalu - large) is also accelerated body growth, but not in length, but in width, which is manifested in a disproportionate periosteal enlargement of the bones of the skeleton and internal organs, which is combined with a characteristic metabolic disorder. The disease usually develops in adults.

This disease was first 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 a pituitary tumor, which, as S. Benda (1903) established, is a "conglomerate of highly multiplied eosinophilic cells of the anterior lobe of the appendage." In Russian literature, the first report on acromegaly was made in 1889 by B. M. Shaposhnikov.

Causes and pathogenesis of acromegaly and gigantism. The vast majority of cases are sporadic, but cases of familial acromegaly have been described.

The theory of pituitary syndrome was put forward as early as the end of the 19th century. Subsequently, mainly domestic researchers demonstrated the inconsistency of localistic concepts about the exclusive role of the pituitary gland in the pathogenesis of the disease using large clinical materials. It was proven that primary pathological changes in the diencephalon and other parts of the brain play a significant role in its development.

A characteristic feature of acromegaly is increased secretion of growth hormone. However, a direct relationship between its content in the blood and clinical signs of disease activity is not always observed. In approximately 5-8% of cases, with a low or even normal level of somatotropic hormone in the blood serum, patients have pronounced acromegaly, which is explained either by a relative increase in the content of a special form of growth hormone with high biological activity, or an isolated increase in the level of IGF.

Causes and pathogenesis of acromegaly and gigantism

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Symptoms of Acromegaly and Gigantism

Typical complaints of acromegaly include headache, changes in appearance, and enlargement of the hands and feet. Patients are bothered by numbness in the hands, weakness, dry mouth, thirst, joint pain, and limited and painful movements. Due to the progressive increase in body size, patients are forced to frequently change shoes, gloves, hats, underwear, and clothes. Almost all women experience menstrual irregularities, and 30% of men develop sexual weakness. Galactorrhea is observed in 25% of women with acromegaly. These abnormalities are caused by hypersecretion of prolactin and/or loss of the gonadotropic function of the pituitary gland. Complaints of irritability, sleep disturbances, and decreased performance are common.

Headaches may vary in nature, localization, and intensity. Occasionally, persistent headaches are observed, combined with lacrimation, driving the patient to frenzy. The genesis of headaches is associated with increased intracranial pressure and/or compression of the sella turcica diaphragm by a growing tumor.

Symptoms of Acromegaly and Gigantism

Diagnosis of acromegaly and gigantism

When diagnosing acromegaly, one should take into account the stage of the disease, its phase of activity, as well as the form and features of the course of the pathological process. It is advisable to use X-ray examination data and functional diagnostic methods.

X-ray examination of the skeletal bones reveals periosteal hyperostosis with signs of osteoporosis. The bones of the hands and feet are thickened, their structure is usually preserved. The nail phalanges of the fingers are pagoda-shaped thickened, the nails have a rough, uneven surface. Of the other bone changes in acromegaly, the growth of "spurs" on the heel bones is constant, and somewhat less often on the elbows.

Diagnosis of acromegaly and gigantism

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Treatment of acromegaly and gigantism

Treatment of acromegaly should be comprehensive and carried out taking into account the form, stage and phase of disease activity. First of all, it is aimed at reducing the level of growth hormone in the blood serum by suppressing, destroying or removing the active STH-secreting tumor, which is achieved using radiological, surgical, pharmacological methods of treatment and their combination. The correct choice of treatment method and its adequacy are the prevention of the development of subsequent complications. In the presence of complications associated with the loss of tropic functions of the pituitary gland, disruption of the functional activity of various organs and systems, agents that correct neurological, endocrine and metabolic disorders are added to the treatment.

The most common methods of treating the disease include various types of external irradiation (X-ray therapy, tele-y-therapy of the interstitial-pituitary region, irradiation of the pituitary gland with a proton beam). Less commonly used is the implantation of radioactive isotopes into the pituitary gland - gold ( ¹⁹⁸ Au) and yttrium ⁹⁰ I) - in order to destroy tumor cells, as well as cryodestruction of the tumor using liquid nitrogen. Irradiation of the pituitary gland causes perivascular hyalinosis, which occurs 2 or more months after irradiation.

Treatment of acromegaly and gigantism