Acromegaly is a disease characterized by an increase in the secretion of somatotropic hormone (STH), disproportionate growth of the bones of the skeleton, an increase in protruding parts of the bones and soft tissues, as well as internal organs, metabolic disturbances.
The frequency of acromegaly in men and women is the same, more often people over the age of 30 are affected. With the emergence of hypertrophic hyperglycemia at a young age, until the growth is complete, proportional accelerated growth of the skeleton bones is noted, gigantism develops (growth up to 190 cm is considered normal).
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.
Why does acromegaly develop, and what is it? Acromegaly is a syndrome that develops due to the overproduction of somatotropin by the pituitary gland (growth hormone) after a period of maturation and ossification of the epiphyseal cartilage. The disease is characterized by gradual pathological growth of bones, internal organs and soft tissues, especially the peripheral parts of the body (limbs, head, face).
The causes of acromegaly in the vast majority of cases in the clinic causing excessive secretion of growth hormone is a pituitary adenoma, located in the area responsible for the production of growth hormone. Most often, tumor development is triggered by mutation of the Gs-alpha protein gene. This mutant protein continuously stimulates the enzyme adenylate cyclase, this leads to increased growth of cells producing somatotropin, and as a result, to an increase in its production.
In the absence of benign tumors in the glandular tissues of the pituitary gland, acromegaly can cause:
- skull injuries;
- pathological course of pregnancy;
- acute and chronic infections (eg, measles, influenza, etc.);
- mental injury;
- tumors localized in the central nervous system;
- malignant neoplasms of the left frontal lobe;
- epidermal encephalitis;
- large tank cysts resulting from head injuries or as a result of infectious diseases;
- congenital or acquired syphilis.
It is worth noting that the trauma suffered is not in itself the cause of acromegaly, it can simply become a catalyst for the development of this growth pathology.
Symptoms of Acromegaly
Symptoms appear rather slowly, and their growth occurs gradually. As a rule, to diagnose this disease in adulthood takes about ten years after the first signs of the disease. In the case of acromegaly, the symptoms largely depend on the stage.
Experts identify the following forms of the disease:
- preacromegalic - symptoms are practically absent;
- hypertrophic - the main part of the signs of the disease;
- tumor - the growth of the tumor affects the nearby nerve endings, tissues and organs;
- kahekticheskaya - 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 disorders - 58%,
- general weakness and disability - 54%,
- weight gain - 48%,
- decrease in libido and potency - 42%,
- visual impairment - 36%,
- daytime sleepiness - 34%,
- hypertrichosis - 29%,
- heartbeat and shortness of breath - 25%.
On examination of the patient, the coarsening of the facial features, an increase in the hands and feet, kyphoscoliosis, and changes in hair and skin attract attention. As a result of an increase in the superciliary arches, zygomatic bones and chin, the face of the patient takes on a stern look. The soft tissues of the face hypertrophy, which leads to an increase in the nose and ears, lips. The skin thickens, deep folds appear (especially on the back of the head), as a rule, the surface of the skin is oily (oily seborrhea).
By increasing the internal organs in size and volume in a patient, muscular dystrophy increases, which leads to the appearance of weakness, fatigue, and a rapid decline in working capacity. Cardiac muscle hypertrophy and heart failure progresses rapidly. According to statistics, one-third of patients with acromegaly have increased blood pressure and impaired functioning of the respiratory center, with the result that often apnea attacks (temporary respiratory arrest) occur.
The tongue and interdental spaces (diastema) increase, prognathism develops, which breaks the bite. Marked growth of the bones of the skull, especially the facial. The hands are wide, 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 limbs is thickened, oily and moist, often with abundant hypertrichosis. Often there are paresthesias and numbness of the fingers. In advanced cases of the disease, varying degrees of skeletal deformity occur.
The diagnosis is confirmed by examining blood tests for hormones. All patients with acromegaly revealed a high content of somatotropic hormone in the blood. In addition to laboratory tests, the patient is shown other diagnostic methods:
- radiography of the skull;
- CT scan;
- consultation of the endocrinologist.
The diagnosis of a number of cases of acromegaly cannot be based solely on a change in the patient's appearance. This is due to the fact that an 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 series of additional studies with signs of acromegaly. In particular, a radiograph may 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 ophthalmologic examinations should be performed.
As acromegaly sufferers look like, we offer for viewing detailed photos.
Famous people with this disease
In chronological order:
- Tille, Maurice (1903−1954) - French professional wrestler, born in the Urals in a French family; Shrek's prototype.
- Kiel, Richard (1939-2014) - American actor height 2.18 m.
- Andre Gigant (1946-1993) - professional French wrestler and actor of Bulgarian-Polish origin.
- Streiken, Karel (born 1948) is a Dutch characteristic actor 2.13 m tall.
- Igor and Grishka Bogdanov (Igor et Grichka Bogdanoff; b. 1949) - French twins of Russian origin, TV presenters, popularizers of space physics.
- McGrory, Matthew (1973-2005) - American actor with a growth of 2.29 meters.
- Valuev, Nikolai Sergeevich (born 1973) is a Russian professional boxer and politician.
Read more on wikipedia.
Therapeutic measures in acromegaly are aimed at eliminating increased secretion of growth hormone by the pituitary gland, reducing the manifestation of clinical symptoms and eliminating such symptoms of the disease as headache, visual field disturbances, etc.
This is achieved by prompt removal of pituitary adenoma, irradiation of the interstitial-pituitary area, implantation of radioactive yttrium, gold or iridium into the pituitary, cryogenic destruction of the pituitary and drug therapy (dopamine agonists and somatostatin analogues). After the somatotropin level decreases, not only will the patient feel better, but his life span will also increase. To date, scientists have proven that a prolonged increase in this hormone leads to early mortality caused by pulmonary, cardiovascular and oncological 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 increase in the likelihood of premature mortality.
Lack of treatment of acromegaly leads to disability of patients of active and working age, increases the risk of premature mortality. Acromegaly reduces life expectancy: 90% of patients do not live to 60 years. Death usually occurs as a result of cardiovascular disease.
The results of surgical treatment of acromegaly are better with small adenomas. With large pituitary tumors, the frequency of their relapses sharply increases.