Itsenko- Cushing’s disease is a rare severe neuroendocrine disease, manifested by increased production of hormones of the adrenal cortex, which is caused by excessive secretion of ACTH caused by pituitary cell hyperplasia or a tumor.
In 1924, the Soviet neuropathologist N. M. Itsenko described a disease with a peculiar clinical course. The cause of the pas Tologoi he has seen changes in hypothalamic structures, in particular Nosta Tuber cinereum . In 1932 the American neurosurgeon Cushing , a closer look at this Patolo Gia, the cause of the disease explained basophilic pituitary adenoma. In the future, with the development of the endocrine ogy, it became clear that A direct -governmental cause of the disease is the overproduction of adrenal corticosteroids, in particular Nosta hydrocortisone.
Regardless of the cause of the disease, there are:
- Itsenko- Cushing’s disease and
- Itsenko- Cushing’s syndrome .
When Diseases Itsenko- Cushing initial violation occurs in the hypothalamic centers where enhanced secretion of releasing – factor adrenocorticotropic hormone (ACTH-RH), whereby hyperplasia occurs basophilic pituitary cells until manner Bani basophilic adenoma. Withdrawn Chiva ACTH production resulting schaya to hyperplasia nadpochech nicknames with giperprodutsiey cortisol.
Itsenko- syndrome Cushing’s is to fight disease state Clinically ski is quite similar to Itsenko- Disease Cushing’s , the only difference is that while in the patho logical link of the hypothalamus and hypo nat do not participate. Itsenko- Syndrome Cushing mo Jette develop as soil hormone producing tumors of the adrenal cortex, tumors and other sites (most bronchi, thymus and pancreas), sell tsiruyuschih ACTH or corticosteroids, and also due to long-term use of corticosteroid prep comrade.
Etiology.
The reason is not known. Defined fief role hereditary constitutional factor. Women suffer about 5 times more likely her husband ‘s rank. Children suffer from relatively re same. Pathogenesis. As the disease Itsenko- Cushing , and Itsenko- syndrome Cushing are the results that hypersecretion of hydrocortisone and other corticosteroids . There is a certain parallelism between the amount of hydrocortisone secretion and the clinical picture of the disease.
According to the feedback law, hyper production of steroid hormones should suppress the secretion of ACTH and AKTG- RF. However, when disease Itsenko- Cushing inhibition of these hormones is not prois walks. This phenomenon explains the NA Yudaev decrease sensitivity Nosta relevant hypothalamic centers to steroids that an agreed or genetic factors, or by raising the threshold Chuv ity of these centers as an effect of Vie lasting impact on them ste high concentration roidov.
NA Yudaev gives another explanation: under the influence of unknown factors decreases receptor sensitivity of hypothalamic centers otvetst governmental Product AKTG- Russian Federation, on the normal or increased amounts of steroids is not able to inhibit the release of AKTG- RF. Disturbed circadian rhythm isolation of ACTH and feedback mechanism that likely Obus catching a change of biogenic amines in the CNS metabolism. Hyper production of steroid hormones in leads to the disintegration of proteins, increasing glycogenesis and lipogenesis . In the results are reduced tolerance to carbon waters.
Pathological anatomy.
Often in the pituitary gland is installed hyper plasia basophil cells acti vatsiey their functions. Adenoma with approximately 80-90% of cases are basophilic , eosinophilic rare-cell or consisting of chromophobe cells, but in most FNF benign. According to the skull on the pictures only in 10-20% of cases are detected from Menenius sella bone . The adrenal glands occurs expressed naya bilateral hyperplasia. The cortical layer thickens 2-3 times. When Itsenko- Syndrome Cushing pituitary remains Xia intact , Adrenal adenoma Cove thus are generally unilateral: glyukokortikosteromy benign or zlokache governmental, weighing from 20 to 2500 g The heart – necrobiotic Ocha rod without any traces of coronary atherosclerosis. In the light – stagnant changes often patchy pneumatic Research Institute; liver-stagnation distrofiche skie changes fatty infiltration tion; stomach – atrophy of mucus stand without ulceration. The bones WHO arises osteoporosis, muscle, CCA cially abdominals, as well as in the limbs and in the back-atrophic effects. The ovaries -ATP raphy sclerosis and cortical layer is schiesya cause amenorrhea and complimentary Dia. Atrophy of the endometrium. In men, atrophy of the testes , impaired sperm pathogenesis. The pancreas – degenerative changes in the island kah Langerhans . Atrophy of the epidermis of the skin , degeneration of elastin in the curl. Vasodilation gives the skin a purplish cyanotic view of mation of red stripes on Telestra .
Clinic.
The disease occurs in all age groups for both fishing, more often in women vozra ste 20-40 years. Characterized by the appearance of the patients – face is round Bagram in-reddish color, women – hair loss male pattern with a slight hypertrichosis of the face and body.
Uneven obesity: pre property accumulation of fat in the upper half of the torso, neck. The limbs, on the contrary, are thin. The skin on the body is dry, thin, capillaries shine through. Due to protein catabolism appear on the skin stretch marks, often on ne Independent user-inner surface of the shoulder, on the sides of the abdomen, buttocks, poor pax.
As a result of the incretion of androgens in the epidermis there is the set set of folliculitis, acne, according to a pigment, and, as a consequence of the action of ACTH and MSH, secre tion which has also been increased. Zhi here increases arterial blood pressure is above the maxi -formal parameters than normal, and the maximum content of ACTH reaches 200-400 mg / l. In this case, the circadian rhythm is disrupted, giving chaotic oscillations is always vayas high.
Differential diagnosis .
The diagnosis of Cushing’s not before are of particular difficulty, but differential diagnosis of diseases Itsenko- Cushing from corticosteroma , particularly from benign, quite difficult.
- X-ray of the skull with the disease Itsenko- Cushing , though not always, but revealed a pituitary adenoma signs that are eliminated on corticosteroma . Imaging of the adrenal glands when Kor tikosteromah not always, but often reveals unilateral tumor, and in diseases Itsenko- Cushing revealed Uwe lichenie adrenal glands.
- Valuable diagnostic Meto house is scanning nadpochech nicknames radioactive yodholesterinom. Contents in daily urine 17-CC and 17-ACS increased in both cases, but in malignant voobrazovaniyah adrenal secre tion of these hormones may increase 10-20 times.
- Differential di agnostics can help The functional -functional tests:
- ACTH loading in Itsenko- Cushing’s disease increases the secretion of 17-KS and 17-ACS. When corticosteroma , by virtue of their autonomy, much of increase of these metabolites occurs.
- Dexamethasone in Itsenko- Cushing’s disease inhibits the secretion of adrenal steroids, which does not happen with corticosteroids . Youth dispituitarism with basophilism in which also revealed sympto Matica, characteristic of the disease Itsenko- Cushing – pink stretch marks , obesity – in the input -governing most cases about go with age, but sometimes they can grow and move in disease Itsenko- Cushing This circadian rhythm ACTH is not disturbed, the test with dexamethasone is positive.