Treatment of Itenko-Cushing's disease

Treatment of Isenko-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.

Currently, worldwide, in the treatment of the Itenko-Cushing's disease, transsphenoidal adenomectomy with the use of microsurgical techniques is preferred. This method is considered one of the main methods of pathogenetic therapy of this serious disease, it gives a quick positive clinical result, leads to complete remission of the disease in 90% of patients with restoration of hypothalamic-pituitary-adrenal functions.

In cases of mild and moderate illness, irradiation of the interstitial-pituitary region is applied - remote radiation therapy: gamma-therapy (dose 40-50 Gy) and a proton beam (dose 80-100 Gy per course).

The use of heavy proton particles for irradiation of the pituitary gland makes it possible to increase the dose, significantly reduce the radiation load on surrounding tissues and conduct one session instead of 20-30 with gamma therapy. The advantage of proton therapy is a faster onset of remission of the disease and a high percentage of recovery (90%). Irradiation of the interstitial-pituitary region leads either to the disappearance of the majority of clinical manifestations of the disease, or a part of the symptoms. These changes occur 6 to 12 months after the end of the course of treatment.

Operative removal of both adrenal glands in severe form of the disease is carried out in two stages. After removal of one adrenal and healing, the operating wound proceeds to the second stage - removal of the second adrenal gland with autotransplantation of the adrenal cortex sites in the subcutaneous tissue. Auto-transplantation of the cortex is performed in order to reduce the dose of hormone replacement therapy, which is lifelong in patients after bilateral total adrenalectomy. Approximately one third of patients at different times after removal of the adrenal glands develop Nelson's syndrome, which is characterized by an increase in the pituitary tumor, expressed by hyperpigmentation of the skin and a labile form of adrenal insufficiency. In recent years, in connection with the development of new methods of treatment, the number of patients who underwent adrenal removal was dramatically reduced.

In most cases, with moderate severity of the disease, combined treatment is used: operative removal of one adrenal gland and radiotherapy of the interstitial-pituitary region.

The drug treatment method includes therapy aimed at reducing the function of the pituitary and adrenal glands under the influence of drugs that suppress the secretion of ACTH, and substances that block the biosynthesis of corticosteroids in the adrenal cortex. The first group includes reserpine, difenine, cyproheptadine, bromocriptine (parlodel), to the second - elliptan, chloridant.

Reserpine at a dose of 1 mg / day is prescribed in the subsequent period for 3-6 months to normalize blood pressure, reduce the activity of the pituitary gland. Remission of the disease with such combination therapy occurs at an earlier time. In addition to radiation therapy, cyproheptadine 80-100 mg or parlodel 5 mg / day for 6-12 months is also used. The blockers of the hypothalamic-pituitary system are not recommended to be administered in the form of monotherapy and before the irradiation of the pituitary, since these drugs do not always cause a persistent clinical improvement in the disease and reduce the radiosensitivity of the pituitary adenoma.

Drugs that inhibit the biosynthesis of hormones in the adrenal glands, elliptan and chloridan, are used in addition to other types of treatment. With incomplete remission after radiotherapy or in combination with unilateral adrenalectomy, chloridin is prescribed in a dose of 3-5 g / day until the function of the adrenal cortex is normalized, and then maintain a maintenance dose (1-2 g) for a long time (6-12 months). Eliptene and Chliditan are also used to temporarily normalize the function of the adrenal cortex in preparing severe patients to remove one or two adrenals. Elipten is prescribed in a dose of 1-1.5 g / day.

With Isenko-Cushing's disease, symptomatic therapy is also needed to compensate and correct protein, electrolyte and carbohydrate metabolism, blood pressure and cardiovascular insufficiency. It is necessary to carry out treatment of osteoporosis, purulent complications, pyelonephritis and mental disorders. Anabolic steroids are widely used , retabolil is often used 0.5 g / m every 10-15 days, depending on the severity of dystrophic disorders. For the treatment of hypokalemic alkalosis, it is advisable to combine potassium and veroshpiron preparations. When steroid diabetes is used biguanides, sometimes in combination with sulfonamides. Insulin is prescribed before surgery. Cardiovascular failure requires parenteral therapy with cardiac glycosides or digitalis preparations. The use of diuretics should be limited. With septic manifestations, antibiotics of a broad spectrum of action are prescribed taking into account sensitivity.

Symptomatic treatment of osteoporosis is a very important problem, as changes in the bones can be reversed slowly and not in all patients, especially in adolescence and after 50 years. The treatment of steroid osteoporosis should be approached from three positions; to accelerate the absorption of calcium salts from the intestine, to promote fixation of their bone matrix, as well as to restore the protein component of bone tissue. Increased calcium intake is achieved appointment derivatives of vitamin D ₃, in particular oksidevita, or mixture of alpha-D ₃ -Teva.

For the treatment of steroid osteoporosis, drugs that reduce bone resorption and stimulate bone formation are used.

The first group includes calcitonin preparations and bisphosphonates.

Calcitonins, along with inhibition of bone resorption, also have a pronounced analgesic effect. Currently, the most widely used drug is myacalcic, which is used in two medicinal forms: in ampoules for IM and n / to injections of 100 units and bottles in the form of a nasal spray of 200 units. Rates of treatment with calcitonins are carried out for 2-3 months with the same intervals in treatment, then again prescribe the drug. In the interruptions of the treatment with calcitonin, bisphosphonates are used, most often the domestic kidofon, or alendronate (fosamax). Necessarily in that and other form of treatment add calcium preparations (500-1000 mg per day).

Drugs that stimulate bone formation include compounds containing fluoride salts (ossin, tridin), anabolic steroids.

One of the damaging mechanisms of the effect of excess glucocorticoids on bone tissue is suppression of osteoblast function and decreased bone formation. The use of fluorides, as well as anabolic steroids in steroid osteoporosis, is based on their ability to enhance bone formation.

When immunodeficient state, developed against the background of the Itenko-Cushing disease, it is recommended to treat with thymalin or T-activin, which affect the immune system, accelerating the differentiation and maturation of T-lymphocytes. As a biostimulator, thymalin improves reparative processes, activates hematopoiesis, enhances the production of alpha interferon by segmented leukocytes and γ-interferon T lymphocytes. Treatment is conducted for 20 days 2 times a year.

The prognosis depends on the duration, severity of the disease and age of the patient. With a short duration of the disease, mild form and age under 30, the prognosis is favorable. After successful treatment, recovery is observed.

In cases of moderate, with a long course after the normalization of the function of the adrenal cortex, irreversible cardiovascular disorders, hypertension, renal dysfunction, diabetes mellitus, and osteoporosis often remain.

As a result of bilateral adrenalectomy, chronic adrenal insufficiency develops, therefore, constant replacement therapy and dynamic observation, prevention of Nelson's syndrome development are necessary.

With full regression of the symptoms of the disease, the work capacity is preserved. Patients are advised to avoid night shifts and heavy physical work. After adrenalectomy, disability is often lost.

Prophylaxis of Itenko-Cushing's disease

Prevention of the pituitary form of the Itenko-Cushing disease is problematic, since its cause has not been fully studied. Prevention of functional hypercorticosis in obesity and alcoholism is the prevention of the underlying disease.