Acromegaly is a disease characterized by increased secretion of growth hormone( STH), disproportionate growth of the bones of the skeleton, an increase in the protruding parts of bones and soft tissues, as well as internal organs, a metabolic disorder.
The frequency of acromegaly in men and women is the same, people more often than 30 years old are more likely to get sick. When hypergenerosis of STH occurs at a young age, until the end of growth, proportional accelerated growth of the bones of the skeleton is noted, gigantism develops( normal growth is up to 190 cm).
However, sometimes in children with open growth zones, excessive secretion of this hormone is accompanied by the appearance of acromegaloid traits or the development of acromegaly.
Reasons for
Why does acromegaly develop, and what is it? Acromegaly is a syndrome that develops as a result of excessive production of pituitary somatotropin( growth hormone) after a period of maturation and ossification of the epiphyseal cartilage. The disease is characterized by a gradual pathological growth of bones, internal organs and soft tissues, especially the peripheral parts of the body( limbs, head, face).
Causes of acromegaly in the vast majority of cases in the clinic, the cause of excessive secretion of STH is the pituitary adenoma, located in the zone responsible for the production of growth hormone. Most often, tumor development is provoked by a mutation of the Gs-alpha protein gene. This mutant protein continuously stimulates the enzyme adenylate cyclase, which leads to increased growth of cells that produce growth hormone, and as a consequence, to an increase in its production.
In the absence of benign tumors in the glandular tissues of the pituitary , acromegaly can cause :
- trauma to the skull;
- pathological course of pregnancy;
- acute and chronic infections( eg, measles, influenza, etc.);
- mental trauma;
- tumors localized in the central nervous system;
- malignant neoplasm of the left frontal lobe;
- epidermal encephalitis;
- large cyst cysts formed as a result of head injuries or as a result of transferred infectious diseases;
- congenital or acquired syphilis.
It should be noted that the transferred trauma itself is not the cause of acromegaly, it simply can become a catalyst for the development of this pathology of growth.
Symptoms of acromegaly
Symptoms are slow enough, and their growth occurs gradually. As a rule, for diagnosing this disease in adulthood, it takes about ten years after the first signs of the disease manifest. In the case of acromegaly, symptoms largely depend on the stage.
Specialists identify the following forms of the disease:
- preacromegalic - symptoms are almost absent;
- hypertrophic - most of the signs of the disease show up;
- tumor - tumor growth affects the nearby nerve endings, tissues and organs;
- cachectic is the most difficult stage.
The frequency of occurrence of subjective signs of acromegaly when referring to a doctor is as follows:
- increase in hands and feet - 100%,
- change in appearance - 100%,
- headache - 80%,
- paresthesia - 71%,
- joint and back pain - 69%
- sweating 62%,
- menstrual irregularities 58%,
- general weakness and disability 54%,
- weight gain 48%,
- decreased libido and potency 42%,
- vision impairment 36%,
- drowsinessin the afternoon - 34%,
- hypertrichosis - 29%,
- heartbeat and oyshka - 25%.
When examining a patient, attention is drawn to the coarsening of facial features, an increase in brushes and feet, kyphoscoliosis, changes in hair, skin. As a result of the increase of the superciliary arches, cheek bones and chin, the patient's face acquires a severe appearance. Soft facial tissues are hypertrophied, which leads to an increase in the nose and ears, lips. The skin thickens, deep creases appear( especially on the occiput), as a rule, the surface of the skin is oily( oily seborrhea).
By increasing the internal organs in size and volume, the patient develops muscle dystrophy, which leads to the appearance of weakness, fatigue, and a rapid decrease in working capacity. Rapidly progressing hypertrophy of the heart muscle and heart failure. According to statistics, a third of patients with acromegaly have high blood pressure and disruption of the respiratory center, resulting in frequent attacks of apnea( temporary respiratory arrest).
The tongue and interdental spaces( diastema) are enlarged, prognathism, which breaks the bite, develops. There is a growth of the skull bones, especially facial fibers. Brushes broad, fingers thickened and seem shortened. The skin on the hands is also thickened, especially on the palmar surface. The width of the foot increases, and because of the growth of the calcaneus - and the length, the size of the wearable shoe increases.
Skin of limbs thickened, oily and moist, often with copious hypertrichosis. Paresthesia and numbness often occur. In far-reaching cases of the disease, different degrees of deformation of the skeleton occur.
Diagnosis
Diagnosis is confirmed by examining blood tests for hormones. In all patients with acromegaly, an elevated level of somatotropic hormone is detected in the blood. In addition to laboratory studies, the patient is shown other diagnostic methods:
- radiograph of the skull;
- MRI;
- CT;
- consultation of the endocrinologist.
Diagnosis of a number of cases of acromegaly can not be based solely on changing the appearance of the patient. This is due to the fact that the increase in facial features, limbs and body parts are signs of other diseases - gigantism, osteoarthropathy, Paget's disease.
Therefore, it is extremely important to conduct a number of additional studies with the signs of acromegaly. In particular, the radiograph can reveal an increase in the size of the Turkish saddle, and tomography will help determine the true cause of this. In addition, a number of ophthalmological studies should be carried out.
Acromegalia: photo
How patients with acromegaly look, we offer detailed photos for viewing.
Famous people with such a disease
Chronologically:
- Tiye, Maurice( 1903 -1954) - French professional wrestler, born in the Urals in the family of the French;the preimage of Shrek.
- Keel, Richard( 1939-2014) - American actor with a height of 2.18 m.
- André Gigant( 1946-1993) is a professional French wrestler and actor of Bulgarian-Polish descent.
- Streiken, Karel( born 1948) is a Dutch character actor with a height of 2.13 m.
- Igor and Grishka Bogdanoff( born 1949) are French twins of Russian descent, television personalities and popularizers of space physics.
- McGrory, Matthew( 1973-2005) - American actor with a height of 2.29 m.
- Valuev, Nikolai Sergeevich( born 1973) - Russian boxer-professional and politician.
Read more on Wikipedia.
Treatment of acromegaly
Therapeutic measures for acromegaly are aimed at eliminating the increased secretion of STH by the pituitary gland, reducing the manifestation of clinical symptoms and eliminating symptoms such as headache, violation of visual fields, etc.
This is achieved by the rapid removal of pituitary adenoma, irradiation of the pituitary-hypophysealregion, implantation in the pituitary gland of radioactive yttrium, gold or iridium, cryogenic destruction of the pituitary gland and drug therapy( dopamine agonists and analoguesTina).After the level of somatotropin has decreased, not only the patient's well-being will improve, but also the duration of his life will increase. To date, scientists have proven that a prolonged increase in this hormone leads to an early mortality caused by pulmonary, cardiovascular and cancer diseases.
In any case, timely treatment of acromegaly plays a very important role, since its absence is fraught with early disability of patients in active working age and an increased likelihood of premature death.
Forecast
The absence of acromegaly treatment leads to disability of active and working age patients, increases the risk of premature death. With acromegaly, life expectancy is reduced: 90% of patients do not survive to 60 years. Death usually comes as a result of cardiovascular diseases.
The results of surgical treatment of acromegaly are better with small adenomas. With large tumors of the pituitary gland, the frequency of their relapses sharply increases.