Acromegaly

Acromegaly is a disease, with a disproportionate growth of the bones of the skeletonand soft tissues, organs and near metabolic disorders. In the basis of acromegaly lies excessive production of growth hormone (HGH eosinophilic cells of the anterior pituitary. The vast majority of patients akromegaliei found adenoma of the pituitary gland.
Acromegaly is observed relatively rare, more often occurs in Mature age. The disease develops slowly.
the face of a patient with acromegalyEmerge General weakness, fatigue, headache, paresthesia , and burning pain in the hands, muscle weakness. Gradually increase legs, nose, lips, chin (see figure). The lower jaw is pushed forward towards the top. Increase the intervals between teeth. The language does not fit in my mouth. The voice becomes rough and hoarse. Disturbed function of sexual glands (menstrual disorders in women, sexual impotence in men), thyroid (Hypo - or hyperthyroidism). May develop diabetes. In case of compression pituitary tumors of the optic chiasm disorders arise view (narrowing of the field of vision, decreased visual acuity). On the radiograph: increase in the size of the skull, the Turkish saddle, lower jaw; thickening of bones of the feet and hands. At onset, can help in the diagnosis comparison view of a patient with photos of previous years, radiology, ophthalmology research, the definition of somatotropic hormone in the blood.
Forecast for benign for acromegaly against life and disability favorable.
Treatment. To suppress excessive growth hormone secretion conduct x - ray or telehomecare on the pituitary region. In addition, use the method of radial gipofizektomii (implantation in the pituitary gland of radioactive yttrium-90 or radioactive gold). At infringement of function of sexual glands given substitutive hormone therapy: women nominated estrogen drugs yellow body, men - androgens. In cases of persistent headache intramuscularly 5 ml of 25% solution of magnesium sulfate, intravenous 10 ml of 10% of the RA target of sodium chloride. Sharp narrowing of the vision and the absence of remission after radiation therapy are an indication for surgery (removal of pituitary tumors). Patients with acromegaly must be under medical supervision.

Acromegaly (acromegalia; from the Greek. akron-konechnostei megas, megalos-large) is a disorder characterized by the growth of bones and soft tissues, increase of limbs and splanchnomegaly.
The etiology is unknown. The most characteristic symptoms of acromegaly are developing under the influence of excessive entering the body of growth hormone that is caused by the hyperactivity eosinophilic cell of the anterior pituitary gland in connection with BPH or hyperplasia them. A number of symptoms caused by secondary violations of other endocrine glands that are functionally connected to the pituitary gland, as well as changes adjacent to the pituitary diencephalic region.
Acromegaly is observed relatively rare; usually begins at the age of 20-30 years. Children and adolescents rarely get sick.
Pathological anatomy. A tumor of the anterior pituitary gland that lies at the basis of acromegaly is usually a benign adenoma of eosinophilic cells, rarely is malignant. It can grow hiazmu, brain, sphenoid bone. Sometimes the tumor develops in the sphenoid bone or in the middle of cranio-faringealny channel. In some cases, along with pituitary adenoma described parathyroid adenomas, thyroid, pancreas, cortical layer of the adrenal glands. In akromegalia observed splanchnomegaly, sclerosis of the pancreas, the thyroid hyperplasia, goiter, parathyroid glands.
Sometimes develops hyperplasia lymph nodes. Changes bones and joints in patients with open the epiphyseal growth zones are expressed in the development of proportional gigantism.
With the development of acromegaly in adults there is a resumption of growth of bones and cartilage differing degrees in different parts of the skeleton.
In the skull emphasized are the natural features of its structure (Century A. Rusakov): when the elongated shape of the skull takes dolichocephalic form, with wide - brachycephalics and so on increase of the size of the outstanding parts of the face, hands, feet due to growth not only of bones and soft tissues.
Changing the shape of the lower jaw, extracting teeth, progeny is caused by the growth and reconstruction of the mandibular bone. In the spine thickens front and side of the vertebral bodies so that their wider than it is tall. In the cervical-thoracic spine is celebrated kyphosis, in the lumbar - compensatory lordosis. In the later stages of acromegaly occurs deforming spondylosis. Ribs are extended, front departments them to dilate, formed the so-called acromegalics rosary. Tubular bones, especially the hands and feet, become broader, develops deforming arthrosis.
The muscles in the early stages of the disease is exaggerated, then atrophy. The skin thickens, sweat and sebaceous gland is exaggerated.

Fig. 1. Face, brush and stop a patient with acromegaly (right - hand and foot healthy person).

The clinical picture and over. The disease develops slowly, and for a long time neither the patients nor others may not notice. The first most often there are complaints about headaches paroxysmal or permanent nature, usually in fronto-parietal or temporal areas. Headache caused by increased vnutricerepnogo pressure, may be accompanied by dizziness, nausea, vomiting, epileptoid seizures. The growth of bones and soft tissues occurs gradually causes the slow change of the appearance of the patient. The greatest changes occur in the bones of the skull, hands and feet (Fig. 1). Due to the disproportionate growth of the bones of the skull shape, especially his face, greatly disturbed: act eyebrows and zygomatic bones, increase the occiput and the circumference of the skull. The lower jaw protrudes forward towards the upper jaw (progname), which is especially characteristic of acromegaly; increase the intervals between teeth, changed bite. Due to growing cartilages and the soft tissues of the nose becomes thick; increase of the ear. Lips, especially the bottom, thicken. The skin on the neck and skull thickens, forms a deep folds. Fingers thicken, hands and feet often grow only in width (wide type), but if acromegaly is developing at an unclosed the epiphyseal growth zones, hands and feet may grow long long type"). Chest increases in volume due to thickening of the ribs and expansion of intercostal spaces, the lower edge of her acquire the deployed position. Clavicle and the chest thicken. On the bones, especially in places of attachment of muscles, formed exostosis. Hair thicken, become rough and thicker; often there is abundant hair growth. In women, patients with acromegaly may develop rich verily hypertrichosis with the advent beard and moustache, due to secondary dysfunction adrenal cortex (see Virilism).
As a rule, increases the size of the internal organs (splanchnomegaly). The increase of the tongue (macroglossia) leads to changes in the speech, she becomes muffled. There are disorders and other endocrine glands, often sexual impotence, infertility, menstrual cycle disorder). Often there is hyperplasia of the thyroid gland with a tendency to the formation of the nodes, sometimes in violation of its functions (myxedema, thyrotoxicosis).
Often when acromegaly will develop diabetes in some cases with insulin resistance, rarely insipidus; the latter may occur as a result of loss of antidiuretic function of the posterior lobe of the pituitary gland and on the grounds of violation of the regulation of water-salt exchange in connection with the pressure of the tumor on interstitial the brain.
Due to increased intracranial pressure in a significant number of patients come visual disturbances (hemianopsia, decrease of visual acuity), often caused by the pressure of the tumor growing from the pits of the Turkish saddle, on the optic nerves, less congestion in them. Changes of the vegetative nervous system usually expressed symptoms of increased excitability: "hot flashes", hyperhidrosis, the propensity to tachycardia, unstable blood pressure, and other
Specific for acromegaly psychoses not. Often, patients with acromegaly marked apathy, lethargy, lethargy, drowsiness, sometimes mental decline.
The most frequent complications of acromegaly: reduction of vision to complete blindness on the soil of optic atrophy of cerebral blood circulation, bleeding in the brain.
Acromegaly is developing in childhood and adolescence, according to some clinical signs different from acromegaly adults: because the disease occurs until completion of the sexual development and the closure of epiphyseal growth zones, it is combined with gigantism or sophianism.


Diagnosis of acromegaly is easy. Radiography of the Turkish saddle precise localization adenomatous process in the pituitary gland, causing the disease (see below). Systematic monitoring of the functions of the eye and ocular fundus. Hemianopsia often precedes achromatopsia (change of color vision), therefore the study of the field of view in color.
Progressive changes in the fundus of the eye and eye functioning properly allow to determine the method of treatment.
The prognosis depends on the nature of the process in the pituitary gland. Cause of death can be intercurrent infection, resistance to which patients akromegaliei reduced.
Treatment. The most radical methods are the delete operation pituitary tumors (see the Pituitary gland, surgical treatment of tumors) and radiotherapy (see below). Hormonal treatment is indicated in the secondary disorders of the functions of the other endocrine glands. Frequently used drugs to sexual hormones: men-testosterone propionate (intramuscularly in a dose of 25-50 mg a day, the course of 20-30 injection) or methyltestosterone (sublingual 5-10 mg 3 times daily), women - estrogenic drugs and progesterone on the same principle and in the same doses as in the treatment of amenorrhea (see). Other complications is being treated by tireoidina, thiuragyl, mercazole, micro-doses of iodine, insulin (see Hypothyroidism, Diabetes mellitus, diffuse toxic Goiter).
X-ray diagnostics. If acromegaly is characterized by the thickening of calvarium, vastanie brow and occiput, increased facial bones, especially the lower jaw (Fig. 2). Pronounced pneumatische pneumatic bones of the skull.
When pituitary tumors Turkish saddle is enlarged, the bottom of the pituitary fossa descended into the lumen of the sinus main bone, back lengthened. Entrance to the Turkish saddle may not be extended if the enlarged pituitary gland is not left up outside the aperture of the saddle. In this case chiasm phenomena are missing. Thinning bottom of one or both of the front wedge processes or usura the top of the pyramid of the temporal bone indicates that the tumor is large, and allows to judge about the direction of its growth. When carotid angiography in patients with acromegaly may be offset siphon the internal carotid artery in the side, front or back.
The bones of the skeleton if acromegaly is enlarged. Expressed kyphoscoliosis of the spine.
When disease development is, after the period of ossification of bones of the limbs thickened, especially in the epiphyses where there osteophytes. If acromegaly before the end of the period of ossification of bones of the legs extended, the growth of the patient exceeds the norm.
Radiotherapy if acromegaly is used as an independent method or in combination with surgical treatment. The Central location of the tumor in the skull makes it preferable to use the ramp and rotary machines. In the presence of the x-ray apparatuses with fixed tube profitable maximum number of fields and narrow tube (4X4 cm). Use three or five fields: two temporal and two on the median line - nineline and verplanke. Rarely used, and the fifth field of a nape. When 200-190 kV and 10 to 20 mA and filters 1 Cu + 1 Al single dose of 100-200 R a session on the surface of the skin daily. Total dose for a course is brought to 4000-4500-6000 R.
Repeated courses (if control over the condition of skin, hair, blood, fundus, features, eyes, endocrine system and metabolism) through the following periods of time: a second course in two months after the first, the third three months after the second, the fourth - four months after the third course.
Positive results of treatment is to restore menstruation or ordering of the menstrual cycle, sexual function, improvement of fields and visual acuity improvement of metabolism. Negative results point to the continued growth of the tumor.