Acromegaly and Gigantism: An Overview of Information

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

[1], [2], [3], [4], [5], [6], [7], [8]

Symptoms of acromegaly and gigantism

Typical complaints for acromegaly include headache, changes in appearance, an increase in the size of brushes, feet. Patients are concerned about numbness in the hands, weakness, dry mouth, thirst, joint pain, limitation and painful movements. In connection with the progressive increase in body size, patients are often forced to change shoes, gloves, hats, underwear and clothing. Almost all women are disrupted by the menstrual cycle, 30% of men develop sexual weakness. Galactorrhea is noted in 25% of women with acromegaly. These abnormalities are due to hypersecretion of prolactin and / or loss of gonadotropic pituitary gland function. Complaints of irritability, sleep disturbances, and reduced capacity for work are frequent.

Headache can be different in nature, localization and intensity. Occasionally, persistent headaches are observed, combined with lacrimation, leading the patient to frenzy. The genesis of headaches is associated with increased intracranial pressure and / or compression of the diaphragm of the Turkish saddle with a growing tumor.

Symptoms of acromegaly and gigantism

Diagnosis of acromegaly and gigantism

When diagnosing acromegaly, it is necessary to take into account the stage of the disease, the phase of its activity, as well as the shape and features of the pathological process. It is expedient to use the data of X-ray study and methods of functional diagnostics.

When radiographing the bones of the skeleton, the phenomena of periosteal hyperostosis with signs of osteoporosis are noted. The bones of the hands and feet are thickened, their structure is usually preserved. The nail phalanges of the fingers are pagodically thickened, the nails have a rough, uneven surface. Of the other bone changes with acromegaly, the growth of spurs on the calcaneus is constant, somewhat less often on the elbows.

Diagnosis of acromegaly and gigantism

[9], [10], [11]

Treatment of acromegaly and gigantism

Treatment of acromegaly should be comprehensive and conducted 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 STG-secreting tumor, which is achieved with the help of radiological, surgical, pharmacological methods of treatment and their combination. The correctness of the choice of the method of treatment 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, a violation of the functional activity of various organs and systems, the means that correct neurological, endocrine and metabolic disorders are connected to treatment.

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

Treatment of acromegaly and gigantism