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Disease of Itenko-Cushing - Review of information

 
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Last reviewed: 24.06.2018
 
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Illness of Itenko-Cushing is one of the most severe neuroendocrine diseases, the pathogenesis of which is caused by the violation of regulatory mechanisms that control the hypothalamic-pituitary-adrenal system. The disease often develops between the ages of 20 to 40 years, but it also occurs in children and people over 50 years of age. Women are 5 times more likely than men.

The disease was named after scientists. In 1924, a Soviet neurologist NM Itenko from Voronezh reported two patients with a lesion of the interstitial-pituitary region. American surgeon Garvey Cushing in 1932 described a clinical syndrome called "pituitary basophilism."

From the Itenko-Cushing's disease it is necessary to distinguish the Itenko-Cushing syndrome. The latter term is used in cases of an adrenal tumor (benign or malignant) or an ectopic tumor of various organs (bronchi, thymus, pancreas, liver).

trusted-source[1], [2], [3], [4], [5]

Causes and pathogenesis

The cause of the disease is not established. In women, Itenko-Cushing's disease often occurs after childbirth. In the history of patients of both sexes there are head injuries, brain concussion, skull injuries, encephalitis, arachnoiditis and other CNS lesions.

Pathogenetic basis of the Itenko-Cushing disease is a change in the mechanism of control of ACTH secretion. Due to a decrease in the dopamine activity responsible for the inhibitory effect on the secretion of CRH and ACTH and an increase in the tone of the serotonergic system, the mechanism of the regulation of the function of the hypothalamic-pituitary-adrenal system and the daily rhythm of the secretion of KRH-ACTH-cortisol are disrupted; the principle of "feedback" with the simultaneous increase in the level of ACTH and cortisol ceases; the reaction to stress is lost - an increase in cortisol under the influence of insulin hypoglycemia.

What provokes Isenko-Cushing's disease?

trusted-source[6], [7], [8], [9]

Symptoms of Itenko-Cushing's disease

In patients, the skin is thin, dry, on the face, in the chest and back of the purple-cyanotic color. The venous pattern on the chest and legs is clearly visible. There is acrocyanosis. On the skin of the abdomen, the inner surfaces of the shoulders, thighs, in the region of the mammary glands appear broad striae of red-violet. Often there is hyperpigmentation of the skin, often in places of friction. On the back, chest and face there are pustular eruptions, hypertrichosis. Hair on the head often falls out, and baldness in women is noted in the male type. There is an increased tendency to furunculosis and development of erysipelas.

There is an excessive deposition of fat in the neck, trunk, abdomen and face, which becomes a "full moon". In the area of the upper thoracic vertebrae, there are fat deposits in the form of a hump. The limbs are thin, lose their normal shape. 

Symptoms of Itenko-Cushing's disease

Diagnosis of Itenko-Cushing's disease

Diagnosis of Itenko-Cushing's disease is based on clinical, radiological and laboratory data.

X-ray methods of investigation are of great importance in diagnosis. With their help, osteoporosis of the skeleton of varying severity is revealed (in 95% of patients). The size of the Turkish saddle can indirectly characterize the morphological state of the pituitary gland, its magnitude. At pituitary microadenomas (about 10% of all cases) the saddle increases in size. Microadenomas can be detected with computer and magnetic resonance imaging (60% of cases) and with surgical adenomectomy (90% of cases).

X-ray examinations of the adrenal glands are performed by various methods: oxygen-suprasarentgenography, angiography, computer and magnetic resonance imaging. Suprarentgenography performed in conditions of pneumoperitoneum is the most accessible way of visualization of the adrenal glands, but it is often difficult to judge their true increase, as they are surrounded by a dense layer of adipose tissue. An angiographic study of the adrenal glands with simultaneous determination of the hormone content in the blood taken from the adrenal veins gives reliable information about the functional state of these glands. But this invasive method is not always safe for patients with Isenko-Cushing's disease.

Diagnosis of Itenko-Cushing's disease

trusted-source[10], [11], [12]

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Treatment of Itenko-Cushing's disease

To treat the disease, pathogenetic and symptomatic methods are used. Pathogenetic methods are aimed at normalization of pituitary-adrenal relationships, symptomatic - to compensate for metabolic disorders.

Normalization of production of ACTH and cortisol is achieved using irradiation of the pituitary gland, surgical adenomectomy or blockers of the hypothalamic-pituitary system. Some patients are removed one or both of the adrenal glands, prescribe inhibitors of biosynthesis of hormones in the cortex of the adrenal glands. The choice of method depends on the severity and severity of the clinical manifestations of the disease.

Treatment of Itenko-Cushing's disease

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