Acromegaly (. Pierre Marie, in 1886, French nevropatol, Before it was described Reklingauzenom in 1883, Germany) – characterizes the camping disproportionate and excessive growth of the development of individual cha stey skeleton, soft tissues and vnut nal organs.
Etiology.
The reasons are not clear. The disease mainly develops after 30 years, more often in women.
Pathogenesis.
It occurs hut precise anterior lobe hormone secretion thrust pofiza growth hormone (STH). The content of GH in kro vi comes to 90-150 ng / ml at a rate of 3-6 ng / ml due to hyper plasia eosinophil cells or eozinokletochnoy pituitary adenoma. In some cases, during an autopsy in the pituitary gland, the above changes are not detected. It involves camping, that in these cases there is an aberrant localization of eosinophils cells within the root of the tongue, somewhere or whether the path of the former embryonic Foot pituitary stroke in the body cus -sighted bone in epifaringse .
Electron microscopy The investigations dovaniya recent years show that pituitary adenoma with acromegaly may not only be of eosinophilic cells, but also from the basophil and chromophobe cells. The current view that chromophobic cells do not have the ability to produce hormones has now been rejected.
Adenomas usually are benign , rarely – zlokache governmental. Small tumors do not de form ephippium and annuities genologicheski not recognized. Pain Chiyah tumor compress surround conductive tissue and cause a variety disrupt Nij – headaches, bitemporal narrowing of the visual field, anosemiyu , paresis and paralysis of the oculomotor nerves, exophthalmos, rarely – seizures.
Along with an increase in the secretion of growth hormone in the initial period Zabolev Nia sometimes increased secretion and other tropic pituitary hormones (TSH, ACTH, FSH, LTG, N), whereby there are signs minute reotoksikoza , higher of libido, galactorrhea in women, gynecomastia in men. In the long Shem secretion of tropic Gorm newly suppressed somatotsitami or due to compression, obyzvestvle Niya, scarring or pituitary races pad adenomas, which leads to the converse Nome effects: hypopituitarism , amenorrhea, infertility and hypoplastic external genitalia. Therefore, in women with acromegaly, pregnancy, as a rule, does not occur.
For the implementation of anabolic Skog action STG spent much insulin, and why occurs relative insulin insufficient accuracy, hyperglycemia, hypercholesterolemia .
Clinical symptoms.
Clinic acromegaly enough display stand proliferative.
Distinguish:
- rarely meet schuyusya severe and more frequent chro nical shape .
In the first case, the symptomatology develops rapidly and leads to the death of the patient in 3-4 years. The chronic form lasts 10-30 years, and more. There are also intermediate forms.
Symptoms are distinguished:
- ti pichnuyu form
- oligosymptomatic form
- akromegaloidnuyu odds mu, whereby Often symptomatology is very meager. Usually she is observed in the period of sexual matured Bani, during pregnancy and may persist after birth.
Patients often worried headache, paresthesia, burning pain in the extremities, joints, Naru shenie menstrual cycle. In Pozdov these stages marked nervousness, confusion, decreased intelligence, weakness.
For acromegaly, coarsening of facial features, enlargement of the nose, lower jaw, limbs, thickening and coarsening of the skin are characteristic. Due to the increase in mandibular teeth on it Venue Paiute forward thinning develops prognotizm , tongue thickens. Due to the growth of the calcaneus, the size of the foot increases in length.
Besides external these features noted An increase of the internal organs and – SPLAN hnomegaliya , cardiomegaly . At the onset of the disease, an increase in muscle strength is observed. In future times crease connective tissue between muscle fibers causes them to atrophy. Blood in the initial term period characterized by polycythemia, lymphocytosis. In the terminal stage, anemia. MRC X-ray of the skull ki most discovered vayut increase sella.
In the late stages of acromegaly, pituitary adenomas often malignant , causing the collapse of the bones of the skull, which soon leads to meningoencephalitis . In some cases patients dying out of intercurrent infections uc depletion of, heart failure.
Diagnostics.
Acromegaly diagnosis is based on Kli nical disease symptoms, x- ray study of the skull, Uwe lichenie basal serum level of growth hormone, increase the total protein and inorganic phosphorus.
Differentiate acromegaly sometimes Com ditsya with pachydermoperiostosis , hypo tireozom, constitutional gigantism, hyperparathyroidism – a disease Recklinghausen , deforming osteoarthritis – a disease of Paget .
Patients pahidermoperiosteozom due to thickening and fold STI skin, thickening of phalanges resemble patients with acromegaly, however, they have not increased foot and hand, the lower jaw. Patients with hypothyroidism due to swelling and coarsen Niemi skin resemble pain GOVERNMENTAL acromegaly, particularly in the initial perio de, however certain functions tional state of the thyroid gland is not made difficult Neno, especially after SHORT- Nogo therapy with thyroid hormone in E. When deforming osteoarthritis there is an increase and quench schenie proximal tubes chatyh bones, but not on is observed by an increase in facial skull case. The Turkish saddle may be slightly deformed without magnification. When hyperparathyroidism nablyudastsya deformation vertebrate ka such as acromegaly, osteoporosis with cystic areas and spontaneous fractures. Enlarged limbs and the Turkish saddle are not observed.
Forecast.
With benign adenomas, it is favorable, especially with timely treatment. In malignant forms outcome op redelyaetsya hypophysectomy .
TREATMENT.
- The main method of le cheniya acromegaly is radiotherapy. The course is assigned from 4000 to 1300 p. A single dose of 200-600 p from three fields with 2-day breaks between sessions. If after the first treatment course remission occurs (normalized growth hormone levels in syvo blood ROTKO, improved vision, about present status disappearance heads GOVERNMENTAL pain), the repetition rate is not required. When X-ray is necessary to pay special Atte of on X rays OPU holy and thus change to the memory. Often, after large doses mo Jette develop brain edema, intratumoral hemorrhage with after blowing necrosis, lesions at adjacen to the pituitary tissues.
- In malignant adenomas applied kilocuritherapy pa radio active cobalt.
- In recent years microadenomas pituitary successfully applies Xia dopamine antagonist bromocriptine ( Parlodelum ) on 5 to 20 mg per day for 1 year. Parlodel actually inactivates STH and has little effect on adenoma. Recommended use of Parlodel in com bination with radiotherapy.
- In cases where medikamentoz naya and radiotherapy did not produce the desired direct effect and the tumor continues to grow, have resorted to surgical lecheniyu- hypophysectomy .
- In recent years, with success at changing the implantation of radioactive -inflammatory drugs of gold, sodium , cryotherapy – the destruction thrust pofiza liquid nitrogen selective hypophysectomy ultrasound.
Pre property implant Meto poisons it is that they do not destroy the surrounding tissue. Proposition wives and method of administration antises wrenches to growth hormone (FA La glow 1965), according to which the antiserum off Only cells that produce growth hormone.