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Akromegaliya

Akromegaliya - the pathological increase in separate parts of a body connected with the forward share of a hypophysis raised by production of somatotropny hormone (growth hormone) as a result of its tumoral defeat. Arises at adults and it is shown by integration of features (a nose, ears, lips, the lower jaw), increase in feet and hands, constant headaches and joint pains, violation of sexual and reproductive functions at men and women. The increased growth hormone level in blood causes early oncological, pulmonary, cardiovascular diseases mortality.

    Akromegaliya

    Akromegaliya - the pathological increase in separate parts of a body connected with the forward share of a hypophysis raised by production of somatotropny hormone (growth hormone) as a result of its tumoral defeat. Arises at adults and it is shown by integration of features (a nose, ears, lips, the lower jaw), increase in feet and hands, constant headaches and joint pains, violation of sexual and reproductive functions at men and women. The increased growth hormone level in blood causes early oncological, pulmonary, cardiovascular diseases mortality.

    Akromegaliya begins to develop after the termination of growth of an organism. Gradually, throughout the long period, the symptomatology accrues, and there are appearance changes. On average the akromegaliya is diagnosed 7 years later from the valid onset of the illness. The disease equally occurs among women and men, mainly at the age of 40-60 years. Akromegaliya is rare endocrine pathology and is observed at 40 people on 1 million population.

    Mechanism of development and reason of an akromegaliya

    Secretion of somatotropny hormone (a somatotropin, STG) is carried out by a hypophysis. At children's age somatotropny hormone operates processes of formation of a bone and muscular skeleton and linear growth, and at adults exercises control of a carbohydrate, fatty, water-salt metabolism. Secretion of hormone of growth is regulated by the hypothalamus developing special neurosecrets: (stimulates development of STG) and (slows down development of STG).

    Normal the maintenance of a somatotropin in blood fluctuates within a day, reaching the maximum in hours at dawn. At patients with an akromegaliya not only increase in concentration of STG in blood, but also violation of a normal rhythm of its secretion is observed. Cages of a forward share of a hypophysis owing to various reasons do not submit to the regulating influence of a hypothalamus and begin to breed actively. Growth of cells of a hypophysis leads to developing of a benign ferruterous tumor - the hypophysis adenoma strenuously producing . The sizes of adenoma can reach several centimeters and exceed the size of the gland, squeezing and destroying normal cells of a hypophysis.

    At 45%patsiyentov from an akromegaliy tumor of a hypophysis develop only , at 30% in addition produce Prolactinum, at other 25%, besides, sekretirut lyuteiniziruyushchy, follikulostimuliruyushchy, tireotropny hormones, A-subjedinitsu. In 99% adenoma of a hypophysis serves as the akromegaliya reason. As the factors causing development of adenoma of a hypophysis serve craniocereberal injuries, hypothalamus tumors, a chronic inflammation of bosoms of a nose (sinusitis). A certain part in development of an akromegaliya is assigned to heredity as the disease is more often observed at relatives.

    At children's and teenage age against the background of the continuing growth chronic hyper secretion of STG causes the giantism which is characterized by excessive, but rather proportional increase in bones, bodies and soft fabrics. With completion of physiological growth and ossification of a skeleton violations as an akromegaliya - a disproportional thickening of bones, increase in internals and characteristic metabolic disorders develop. At an akromegaliya there is a hypertrophy of a parenchyma and a stroma of internals: hearts, lungs, pancreas, liver, spleen, intestines. Growth of connecting fabric leads to sclerous changes of these bodies, the threat of development of benign and malignant tumors, including endocrine increases.

    Stages of development of an akromegaliya

    Akromegaliya is characterized by a long, long-term current. Depending on expressiveness of symptomatology in development of an akromegaliya distinguish several stages:

    • Preakromegaliya stage – there are initial, poorly expressed symptoms of a disease. At this stage the akromegaliya is diagnosed seldom, is exclusive on indicators of level of somatotropny hormone in blood and to data of KT of a brain.
    • Hypertrophic stage – the pronounced symptomatology of an akromegaliya is observed.
    • Tumoral stage – to the forefront there are symptoms of a sdavleniye of nearby departments of a brain (increase in intra cranial pressure, nervous and eye violations).
    • Kakheksiya stage – exhaustion as an akromegaliya outcome.

    Akromegaliya symptoms

    Manifestations of an akromegaliya can be caused by surplus of a somatotropin or impact of adenoma of a hypophysis on optic nerves and nearby structures of a brain.

    Excess of hormone of growth causes characteristic changes of appearance of patients with an akromegaliya: increase in the lower jaw, malar bones, nadbrovny arches, a hypertrophy of lips, a nose, the ears leading to coarsening of features. To increase in the lower jaw there is a divergence of interdental intervals and change of a bite. Increase in language is noted (makroglossiya) on which traces of teeth imprint. At the expense of a hypertrophy of language, a throat and vocal chords the voice changes - becomes low and gruffish. Changes in appearance at an akromegaliya happen gradually, is imperceptible for the patient. The thickening of fingers, increase in sizes of a skull, feet and brushes so is observed that the patient is forced to get he, footwear and gloves several sizes bigger, than earlier.

    At an akromegaliya there is a deformation of a skeleton: the backbone is bent, the thorax in a perednezadny size increases, getting a barrel-shaped form, intercostal intervals extend. The developing hypertrophy of connecting and cartilaginous fabrics causes deformation and restriction of mobility of joints, artralgiya.

    At an akromegaliya the excess perspiration and a salootdeleniye caused by increase in quantity and hyperactivity of sweat and sebaceous glands is noted. Skin at patients with an akromegaliya is condensed, thickened, gathers in deep folds, especially in a hairy part of the head.

    At an akromegaliya there is an increase in sizes of muscles and internals (heart, a liver, kidneys) to the gradual accruing dystrophy of muscle fibers. Weakness, fatigue, the progressing decrease in working capacity begins to disturb patients. The myocardium hypertrophy which is replaced then a miokardiodistrofiya and the accruing heart failure develops. At a third of patients with an akromegaliya arterial hypertension is observed, almost at 90% - the syndrome sleepy , connected with a hypertrophy of soft tissues of the top airways and violation of work of the respiratory center develops.

    At an akromegaliya sexual function suffers. At most of women at excess of Prolactinum and deficiency of gonadotrophins violations of a menstrual cycle and infertility develop, appears - the allocation from milk nipples which is not caused by pregnancy and childbirth. 30% of men have a decrease in a sexual potentiality. Hyposecretion of antidiuretichesky hormone at an akromegaliya is shown by development of not diabetes.

    In process of increase in a tumor of a hypophysis and a sdavleniye of nerves and fabrics there is an increase in intra cranial pressure, a photophobia, doubling in eyes, pain in cheekbones and a forehead, dizziness, vomiting, decrease in hearing and sense of smell, a sleep of extremities. At the patients suffering from an akromegaliya the risk of development of tumors of a thyroid gland, bodies of digestive tract, uterus increases.

    Akromegaliya complications

    The current of an akromegaliya is followed by development of complications from almost all bodies. Most often the heart hypertrophy, a miokardiodistrofiya, an arterial hypertension, heart failure occurs at patients with an akromegaliya. More than at a third of patients diabetes develops, dystrophy of a liver and emphysema of lungs are observed.

    Hyperproduction of factors of growth at an akromegaliya leads to development of tumors of various bodies, both good-quality, and malignant. Akromegaliya is often accompanied by a diffusion or nodal craw, fibrous and cystous mastopathy, an adenomatozny giperplaziya of adrenal glands, ovaries, myoma of a uterus, intestines polyposes. The developing hypophysial insufficiency (pangipopituitarizm) is caused by a sdavleniye and destruction by a hypophysis tumor.

    Diagnostics of an akromegaliya

    (In 5-6 years from the beginning of a disease) the akromegaliya can be suspected of late stages on the basis of the increase in parts of a body and other external signs noticeable at survey. In such cases the patient goes for consultation of the endocrinologist and testing for performing laboratory diagnostics.

    The main laboratory criteria of diagnostics of an akromegaliya are determination of content in blood:

    • somatotropny hormone in the morning and after the test with glucose;
    • IRF I - an insulinopodobny growth factor.

    Increase in level of a somatotropin decides practically at all patients on an akromegaliya. Oral test with glucose loading at an akromegaliya means definition of a reference value of STG, and then after glucose reception - in half an hour, hour, 1,5 and 2 hours. Normal after glucose reception the level of somatotropny hormone decreases, and at an active phase of an akromegaliya, on the contrary, its increase is noted. Carrying out the glyukozotolerantny test especially informatively in cases of moderate increase in the STG level, or its normal values. Also the test with loading of glucose is used at assessment of efficiency of treatment of an akromegaliya.

    Somatotropny hormone affects an organism through the insulinopodobny growth factors (IGF). Concentration reflects total allocation of STG in plasma of IRF I blood per day. Increase in IRF I in the adult's blood directly indicates development of an akromegaliya.

    At ophthalmologic inspection at patients with an akromegaliya narrowing of visual fields since anatomic visual ways are located in a brain near a hypophysis is noted. At a X-ray analysis of a skull increase in sizes of the Turkish saddle where the hypophysis settles down comes to light. For visualization of a tumor of a hypophysis computer diagnostics and MRT of a brain is carried out. Besides, patients with an akromegaliya are examined regarding identification of various complications: polypose of intestines, diabetes, multinodal craw etc.

    Treatment of an akromegaliya

    At an akromegaliya a main objective of treatment is achievement of remission of a disease by elimination of hyper secretion of a somatotropin and normalization of concentration of IRF I. The modern endocrinology applies the medicamentous, surgical, beam and combined methods to treatment of an akromegaliya.

    For normalization in blood of level of a somatotropin appoint reception of analogs of a somatostatin - a neurosecret of the hypothalamus suppressing growth hormone secretion (an oktreotid, a lanreotid). At an akromegaliya purpose of sex hormones, dopamine agonists is shown (a bromokriptina, a kabergolina). In the subsequent one-time gamma or radiation therapy on area of a hypophysis is usually carried out.

    At an akromegaliya the most effective is surgical removal of a tumor in the skull basis through a wedge-shaped bone. At the small sizes of adenomas after operation at 85% of patients normalization of level of a somatotropin and permanent remission of a disease is noted. At the considerable sizes of a tumor the izlechennost percent as a result of the first operation reaches 30%. The mortality indicator at surgical treatment of an akromegaliya makes from 0,2 to 5%.

    Forecast and prevention of an akromegaliya

    Lack of treatment of an akromegaliya leads to an invalidization of patients active and working-age, increases risk of premature mortality. At an akromegaliya life expectancy is reduced: 90% of patients do not live up to 60 years. Death usually is caused by cardiovascular diseases. Results of expeditious treatment of an akromegaliya it is better at the small sizes of adenomas. At large tumors of a hypophysis the frequency of their recurrence sharply increases.

    For prevention of an akromegaliya it is necessary to avoid head injuries, to sanify the chronic centers of an infection of a nasopharynx. Early identification of an akromegaliya and normalization of level of hormone of growth will allow to avoid complications and to cause permanent remission of a disease.

    Akromegaliya - treatment should be carried out only under the supervision of a doctor. Self-treatment is unacceptable!!!

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