Acromegaly is a disease characterized by increased secretion of growth hormone (STH), a disproportionate growth 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 who are more than 30 years old are more likely to fall ill. 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.
Causes
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 glandacromegaly 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 left frontal lobe;
- epidermal encephalitis;
- cysts of a large tank formed as a result of head injuries or as a result of transferred infectious diseases;
- congenital or acquired syphilis.
It is worth noting 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- the symptoms are practically absent;
- hypertrophic- the main part of the signs of the disease is manifested;
- tumor- Growth of the tumor affects the nearby nerve endings, tissues and organs;
- cachectic- 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 of appearance - 100%,
- headache - 80%,
- paresthesia - 71%,
- pain in the joints and back - 69%,
- sweating - 62%,
- disorders of the menstrual cycle - 58%,
- general weakness and disability - 54%,
- weight gain - 48%,
- decreased libido and potency - 42%,
- impaired vision - 36%,
- drowsiness in the afternoon - 34%,
- hypertrichosis - 29%,
- palpitation and shortness of breath - 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 violation work of the respiratory center, as a result of which often there are attacks of apnea (temporary stop of breathing).
The tongue and interdental spaces (diastema) increase, prognathism develops, which breaks the bite. There is a growth of the skull bones, especially facial fibers. The brushes are broad, the fingers are thickened and appear 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.
The skin of the extremities is thickened, oily and moist, often with abundant hypertrichosis. Paresthesia and numbness often occur. In far-reaching cases of the disease, different degrees of deformation of the skeleton occur.
Diagnostics
The 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:
- radiography 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.
Acromegaly: photo
How patients with acromegaly look, we suggest to view detailed photos.
Famous people with such a disease
In chronological order:
- 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.
- Andre Gigant (1946-1993) is a professional French wrestler and actor of Bulgarian-Polish descent.
- Streiken, Karel (b. 1948) is a Dutch character actor with a height of 2.13 m.
- Igor and Grishka Bogdanovy (Igor et Grichka Bogdanoff; b. 1949) - French twins of Russian origin, TV presenter-popularizers of space physics.
- McGrory, Matthew (1973-2005) - American actor with a height of 2.29 m.
- Valuev, Nikolai Sergeevich (b. 1973) is a Russian professional boxer and politician.
Read more on Wikipedia.
Treatment of acromegaly
Therapeutic measures with acromegaly are aimed at eliminating the increased secretion of STH by the pituitary gland, a decrease in manifestation clinical symptoms and elimination of symptoms of the disease, such as headache, violation of visual fields, etc.
This is achieved through the rapid removal of pituitary adenoma, irradiation of the interstitial-pituitary region, implantation in the pituitary gland of the radioactive yttrium, gold or iridium, cryogenic destruction of the pituitary gland and drug therapy (dopamine agonists and somatostatin analogues). 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 mortality.
Forecast
Absence of acromegaly treatment leads to disability of active and able-bodied 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 recurrence increases sharply.
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