CUSHING SYNDROME ( H. W. Cushing , amer. neurosurgeon, 1869 — 1939; syndrome; synonym Itsenko — Cushing a syndrome; not recommended term cushingoid) — the clinical syndrome caused by a hormonal and active tumor of cortical substance of an adrenal gland or a tumor from malrelated cortical substance of an adrenal gland. To. the page can be also caused (less often) by hormonal and active malignant new growths of other internals capable to cosecrete AKTG-like substance in blood (in particular cancer of a lung); this syndrome can be caused by long administration of corticosteroid drugs or AKTG with to lay down. the purpose at not endocrine diseases.
is the cornerstone of development of a syndrome excess products a tumor of corticosteroid hormones (see. Corticosteroids ) or AKTG that conducts to a so-called hypercorticoidism. Surplus of corticosteroids leads to strengthening of a catabolism of proteins, especially muscular and bone tissue, disturbance of structure of a proteinaceous matrix of a bone and development of osteoporosis; strengthening of processes of a gluconeogenesis in a liver — to disturbance carbohydrate metabolism (see).
A pathogeny To. page it is in many respects similar to a pathogeny of a disease of Itsenko — Cushing (see. Itsenko — Cushing a disease ).
the Main place in a pathoanatomical picture K. the page is occupied by the tumor of cortical substance of adrenal glands producing corticosteroid hormones (see. Kortikosteroma , Kortikoestroma ), and also cancer of a lung (see. Lungs, tumors ), ectopia of cortical substance of adrenal glands. If To. the page develops in connection with a syndrome of ectopic AKTG (see. Adrenocorticotropic hormone ), the hyperplasia of cortical substance is found adrenal glands (see); at a medicamentous origin To. the page — the changes of internals corresponding to a basic disease apropos to-rogo is carried out corticosteroid therapy.
The clinical picture
the Clinical picture is similar to displays of a disease of Itsenko — Cushing. Usually appearance changes, the ABP raises, the menstrual cycle is broken, the libido and a potentiality at men decreases. Outward of patients — a so-called crescent-shaped face of purple-red color, at some patients with an earthy shade is characteristic. The adiposity in hypodermic cellulose in a neck, a shoulder girdle along with an atrophy of muscles of extremities and muscles of a front abdominal wall is observed. Skin dry with bent to a hyperkeratosis; at minor injuries there are petechias, bruises. On skin of a stomach, a breast, hips, in axillary poles striya are formed. The hyperpegmentation of skin of elbows, skin folds and places of friction of skin is characteristic of patients with AKTG-produtsiruyushchimi tumors. Arterial hypertension (see. arterial hypertension ) it is sharply expressed and usually badly gives in to therapy by antihypertensives. Osteoporosis most often develops in a backbone (chest and lumbar departments), a skull, edges, a breast and often defines weight of a condition of patients. Disturbances of water and electrolytic balance lead to development of muscular weakness, paresthesias, spasms in gastrocnemius muscles, to changes on an ECG. At nevrol, inspection reveal proximal paresis of extremities, disorders of skin sensitivity in the form of a segmented hyperalgesia. Various mental disturbances can take place. In blood the hypopotassemia, a hypernatremia, a hyperglobulia, a leukocytosis, an eosinopenia, a lymphocytopenia, disturbances of coagulant system of blood is noted.
Disturbances of carbohydrate metabolism are shown by a hyperglycemia, a glucosuria.
Degree of manifestation of symptoms depends on hormonal activity of a tumor. Over time cardiovascular insufficiency, disturbances of cerebral circulation, a diabetes mellitus can develop, spinal fractures, edges are possible, innidiation of a malignant tumor, etc.
the Diagnosis is made on the basis a wedge., rentgenol, and lab. data. The main method of topical diagnosis is the gas retroperitoneografiya (see. Pneumoretroperitoneum ) in combination with tomography (see); the infusional urography, an angiography, a radio isotope research of adrenal glands help to carry out topical diagnosis. In cases when a wedge, data allow to assume a tumor of an adrenal gland, and it does not come to light, carried out by above-mentioned methods a pnevmosuprarenorentgenografiya (see. Adrenal glands ); in hard cases diagnostic operation with audit of places of a possible arrangement of a tumor is shown. Lab. inspection reveals in a blood plasma and in daily urine the increased maintenance of cortisol and 17 oxycorticosteroids, and also substantial increase of speed of secretion of cortisol at normal concentration of AKTG in plasma; at AKTG-produtsiruyushchikh tumors the maintenance of AKTG considerably exceeds norm. Excretion with urine of the general 17 ketosteroids at benign tumors of adrenal glands does not differ from norm, and at malignant increase in all fractions 17 of ketosteroids with dominance in certain cases of a degidroepiandrosteron is observed.
Differential diagnosis it is carried out with Itsenko's disease — Cushing, for a cut primary dysfunction of gipotalamo-pituitary system is characteristic; load tests with dexamethasone, metopirony have great diagnostic value (see. Itsenko — Cushing a disease ). At medicamentous To. pages make the diagnosis on the basis of the anamnesis.
Treatment at tumors of adrenal glands and AKTG-produtsiruyushchikh tumors consists in their operational removal with the subsequent compensation of the caused disturbances; after removal of tumors of an adrenal gland replacement therapy has special value (see. Kortikosteroma ). For treatment To. pages, developed as a result of prolonged use of corticosteroid drugs, appoint anabolic steroids, drugs of potassium, diuretics.
The forecast and Prevention
the Forecast at high-quality new growths and a timely operative measure is favorable, at malignant new growths is defined by operability of a tumor.
Prevention is possible concerning medicamentous To. page; major importance is attached to respect for the principles of corticosteroid therapy (see. Corticosteroids, corticosteroid drugs ).
Bibliography: Disease and Itsenko's syndrome — Cushing (a pathogeny, clinic, diagnosis and treatment), in book: Sovr. vopr, endokrinol. * under the editorship of N. A. Yudayev, century 5, M., 1975; Egorova E. I. Treatment by glucocorticoids and AKTG, M., 1972, bibliogr.; Krakow V. A. Sindr Itsenko — Cushing, M., 1963, bibliogr.; Nikolaev O. V. and E. I Cockroaches. Hormonal and active tumors of bark of an adrenal gland, M., 1963, bibliogr.
S. A. Butrova.