Scientific editor: Volkov AA, endocrinologist, practical experience since 2015.
September, 2018.
Acromegaly - heavy neuroendocrine pathology. It develops due to chronic excessive production of growth hormone somatotropin (STH) in patients with a complete physiological growth that only in adults. The disease is accompanied by disproportionate growth of bones, cartilage, soft tissue and internal organs.
The development of the disease in children does not lead to acromegaly and in gigantism. But with age man with gigantism, not in treatment is beginning to suffer from the typical symptoms of acromegaly.
Causes of acromegaly
The main causes of acromegaly are the following:
- Producing growth hormone hypophyseal adenoma
- Hereditary and familial forms - syndromes and rejection provoked by genetic defects. Among them:
- somatotrofinoma,
- Rosenthal Syndrome-Klopfer (acromegaly and gigantism combination with folded pachydermia - powerful skin folds and the nape of the neck)
- akromegaloidnogo facial syndrome (thickened lips, eyelids, eyebrows and folds of mucous membranes, big nose "potato"),
- SMTN mutation gene (11q13) - develop somatotrofinoma and subsequent acromegaly,
- Sotos syndrome (moderate neurological and psychic symptoms, accelerated growth with features of acromegaly, high blood levels of valine, leucine and isoleucine),
- gigantism due to excessive secretion somatoliberin.
symptoms of acromegaly
Excessive secretion of growth hormone (one of the causes acromegaly), causes changes in the bones, soft tissues, affecting the metabolism. This is evident in the following symptoms:
- soft-tissue growth and disproportionate increase in bone in adults cause deviations that change the patient's appearance: the increase of the hands and feet (especially the fingertips), coarsening of facial features, thick skin folds, frontal and nasolabial folds, increasing the nose and lower jaw, the appearance of gaps between teeth.
- Increase of heart, lung, liver, spleen, kidney, skin thickening and edema with swelling and soft tissue seal.
- osteoarthritis (Affects the joints of the limbs and spine, which is manifested crunch in the early stages - "looseness" of the joints in the later - restriction of movements in the joints).
- The defeat of the muscles (muscle weakness, cramps).
- Development of cerebrovascular diseases such as atherosclerosis, Vascular endothelial dysfunction.
- Dysfunction of the respiratory system, mainly manifested in the form of sleep apnea syndrome.
- May increase prolactin (hyperprolactinemia), Hypopituitarism (pathology in the anterior pituitary, entailing various endocrine disorders).
- Pathologies of lipid, carbohydrate and mineral metabolism.
Diagnostics
Diagnosis of acromegaly is carried endocrinologist.
As noted by the doctor, due to the slow development of clinical symptoms "diagnosis of the door" is best Becoming a doctor, who had never seen a patient, as he catches the eye disproportionality.
Laboratory studies in acromegaly:
- decrease glucose toleranceExpressed as sugar diabetes observed in 10% of patients
- Increased phosphorus levels in the blood
- Increased urinary excretion of calcium
- Investigation of the synovial fluid did not reveal signs of inflammation
- skull radiography
- radiography of the joints
- X-ray of the spine
- Computer and magnetic resonance imaging
- rheotachygraphy
- Ophthalmologic examination (reveals compression of the optic chiasm)
The diagnosis of acromegaly determine the symptoms.
Diagnosis is recommended to start with the assessment of the level of insulin-like growth factor -1. It is proven that IGF-1 levels are much less susceptible to changes in circadian than STH, due to which the level measuring efficiency disposable above. With increasing IGF-1 levels above normal for a particular age recommended perform additional analysis of the level of growth hormone.
Changes in the content of growth hormone and / or somatomedin C in the blood confirm the diagnosis. The content of growth hormone determined in the morning.
Levels above 10 ng / ml favors acromegaly. In healthy individuals after 1-2 hours after taking 100 g of glucose growth hormone becomes less than 5 ng / ml.
Further evaluation carried prolactin levels to avoid mixed pituitary adenoma, selection combining GH and prolactin exception hypopituitarism performed by evaluating TTG, cortisol, testosterone, and plasma osmolarity urine.
acromegaly treatment
Acromegaly in modern medicine is treated in several ways:
- Removal of pituitary adenoma surgically procedure via transsphenoidal prostatectomy.
- Various kinds of external radiation - radiotherapy, kilocuritherapy hypothalamic-pituitary region, a proton beam irradiation pituitary - very slowly reduce the level of growth hormone; there may be no effect within 3-10 years from start of treatment, and in some cases, normalization does not occur at all. Almost does not apply in cases where it is possible to carry out radical surgery. It used if there is definitive contraindications to surgery or patient's categorical rejection of the intervention.
- Large doses of female sex hormones - estrogen (alone or in combination with male sex hormones - androgens) reduced isolation of growth hormone.
- Bromocriptine at an initial dose of 2.5 mg 4 times a day before meals, with a gradual increase (mean daily dose of 20-30 mg) - As preoperative preparation and after radiation therapy.
- Octreotide of 0.05- 1 mg s.c. 2-3 times a day - with no effect or impossibility of operation, radiation therapy or the use of bromocriptine.
- Drugs long-acting somatostatin.
During therapy with medication is important monitoring the effectiveness of treatment with hormonal studies. Doses are selected by titration for each patient individually. In the absence of the effect of treatment with one drug doctor may change medication, use various combinations.
The prognosis for treatment
The prognosis of acromegaly for life in general favorable, recovery is possible with timely diagnosis and adequate treatment. Ability to work is limited. methods of prevention are unknown.
sources:
- Russian association of endocrinologists. Acromegaly: clinical features, diagnosis, differential diagnosis, treatment. - Clinical guidelines 2014.
