Syndrome Itsenko-Cushing (giperkortitsizm)
Itsenko-Cushing's syndrome – the pathological simptomokompleks arising owing to a giperkortitsizm i.e. the increased allocation by bark of adrenal glands of hormone of cortisol or long-term treatment by glucocorticoids. It is necessary to distinguish Itsenko-Cushing's syndrome from Itsenko-Cushing's disease as which understand the secondary giperkortitsizm developing at pathology of gipotalamo-hypophysial system. Diagnostics of a syndrome of Itsenko-Cushing includes a research of level of cortisol and hypophysial hormones, deksametazonovy test, MPT, KT and a stsintigrafiya of adrenal glands. Treatment of a syndrome of Itsenko-Cushing depends on his reason and can will consist in cancellation of glucocorticoid therapy, purpose of inhibitors of a steroidogenez, expeditious removal of a tumor of adrenal glands.
Syndrome Itsenko-Cushing (giperkortitsizm)
Itsenko-Cushing's syndrome – the pathological simptomokompleks arising owing to a giperkortitsizm i.e. the increased allocation by bark of adrenal glands of hormone of cortisol or long-term treatment by glucocorticoids. Glucocorticoid hormones participate in regulation of all types of a metabolism and many physiological functions. Work of adrenal glands is regulated by a hypophysis by secretion of AKTG - the adrenokortikotropny hormone intensifying synthesis of cortisol and kortikosteron. Activity of a hypophysis hypothalamus hormones - statins and liberina operate.
Such multistage regulation is necessary for ensuring coordination of functions of an organism and exchange processes. Violation of one of links of this chain can cause hyper secretion of glucocorticoid hormones bark of adrenal glands and lead to development of a syndrome of Itsenko-Cushing. Itsenko-Cushing's syndrome occurs at women by 10 times more often than at men, developing, mainly, at the age of 25-40 years.
Distinguish a syndrome and Itsenko-Cushing's disease: the last is clinically shown by the same symptomatology, but primary defeat of gipotalamo-hypophysial system is its cornerstone, and hyperfunction of bark of adrenal glands develops again. At the patients having alcoholism or heavy depressive frustration Itsenko-Cushing's pseudo-syndrome sometimes develops.
Reasons and mechanism of development of a syndrome of Itsenko-Cushing
Itsenko-Cushing's syndrome – the wide concept including a complex of various states which are characterized by a giperkortitsizm. More than 80% of cases of development of a syndrome of Itsenko-Cushing agree modern researches in the field of endocrinology it is connected with the hypophysis microadenoma (Itsenko-Cushing's disease) raised by secretion of AKTG. Microadenoma of a hypophysis represents small (no more than 2 cm), is more often the benign, ferruterous tumor producing adrenokortikotropny hormone.
At 14-18% of patients primary defeat of bark of adrenal glands as a result of hyper plastic tumoral formations of bark of adrenal glands – adenomas, adenomatosis, an adenocarcinoma is the reason of a syndrome of Itsenko-Cushing.
1-2% of a disease are caused by AKTG-ektopirovanny or a kortikoliberin-ektopirovanny syndrome – a tumor, sekretiruyushchy kortikotropny hormone (kortikotropinomy). The AKTG-ektopirovanny syndrome can be caused by tumors of various bodies: lungs, testicles, ovaries, timus, okoloshchitovidny, thyroid, pancreas, prostate gland. Frequency of development medicinal Itsenko-Cushing's syndrome depends on correctness of application of glucocorticoids in treatment of patients with system diseases.
Hyper secretion of cortisol at Itsenko-Cushing's syndrome causes catabolic effect - disintegration of proteinaceous structures of bones, muscles (including warm), skin, internals etc., over time leading to dystrophy and an atrophy of fabrics. Strengthening of glucogenesis and absorption in intestines of glucose causes development of a steroid form of diabetes. Violations of fatty exchange at Itsenko-Cushing's syndrome it is characterized by excess adjournment of fat on one parts of the body and an atrophy on others in view of their different sensitivity to glucocorticoids. Influence of excess level of cortisol on kidneys is shown by electrolytic frustration – a gipokaliyemiya and a gipernatriyemiya and, as a result, increase in arterial pressure and aggravation of dystrophic processes in muscular tissue.
Most the cardiac muscle suffers from a giperkortitsizm that is shown in development of a cardiomyopathy, heart failure and arrhythmias. Cortisol has the oppressing effect on immunity, causing in patients with Itsenko-Cushing's syndrome tendency to infections. The current of a syndrome of Itsenko-Cushing can be an easy, average and severe form; progressing (with development of all simptomokompleks in 6-12 months) or gradual (with increase within 2-10 years).
Symptoms of a syndrome of Itsenko-Cushing
The obesity revealed at patients more than in 90% of cases is the most characteristic sign of a syndrome of Itsenko-Cushing. Redistribution of fat has uneven character, on kushingoidny type. Fatty deposits are observed on a face, a neck, a breast, a stomach, a back at rather thin extremities ("a colossus on clay legs"). The person becomes crescent-shaped, red-crimson color with a tsianotichny shade ("matronizm"). Adjournment of fat in the area VII of a cervical vertebra creates, a so-called, "climacteric" or "bizony" hump. At Itsenko-Cushing's syndrome obesity is distinguished by the thinned, almost transparent skin on the backs of palms.
From muscular system the atrophy of muscles, decrease in a tone and force of muscles is observed that is shown by muscular weakness (myopathy). The typical signs accompanying Itsenko-Cushing's syndrome are "sloping buttocks" (reduction of volume femoral and gluteuses), "a frog stomach" (a hypotrophy of muscles of a stomach), hernias of the white line of a stomach.
Skin at patients with Itsenko-Cushing's syndrome has a characteristic "marble" shade with well noticeable vascular drawing, is inclined to a peeling, dryness, alternates with sites of perspiration. On skin of a humeral belt, mammary glands, a stomach, buttocks and hips skin stretching strips – striya of crimson or tsianotichny coloring, from several millimeters to 8 cm long and up to 2 cm wide are formed. Skin rashes (acne), hypodermic hemorrhages, vascular asterisks, a hyperpegmentation of certain sites of skin are observed.
At a giperkortitsizm thinning and injury of a bone tissue - the osteoporosis leading to strong painful feelings, deformation and fractures of the bones, a kifoskolioz and scoliosis which were more expressed in lumbar and chest departments of a backbone quite often develops. At the expense of a compression of vertebras patients become round-shouldered less than growth. At children with Itsenko-Cushing's syndrome the lag in growth caused by delay of development of epifizarny cartilages is observed.
Violations from a cardiac muscle are shown in development of the cardiomyopathy which is followed by arrhythmias (fibrillation of auricles, premature ventricular contraction), arterial hypertension and symptoms of heart failure. These terrible complications are capable to lead of patients to death. At Itsenko-Cushing's syndrome the nervous system suffers that is expressed in its unstable work: block, depressions, euphoria, steroid psychoses, suicide attempts.
The steroid diabetes which is not connected with damages of a pancreas develops in 10-20% of cases during a disease. Such diabetes quite easily, with the long normal level of insulin in blood proceeds, it is quickly compensated by an individual diet and antihyperglycemic medicines. Sometimes also the nikturiya, peripheral hypostases develop poly-.
Giperandrogeniya at women accompanying Itsenko-Cushing's syndrome causes development of a virilization, girsutizm, gipertrikhoz, violations of a menstrual cycle, amenorea, infertility. At male patients feminization signs, an atrophy of testicles, decrease in a potentiality and libido, a ginekomastiya are observed.
Complications of a syndrome of Itsenko-Cushing
The chronic, progressing current of a syndrome of Itsenko-Cushing with the accruing symptomatology can lead to death of patients as a result of complications, incompatible with life: decompensations of warm activity, strokes, sepsis, heavy pyelonephritis, a chronic renal failure, osteoporosis with multiple fractures of a backbone and edges.
Medical emergency at Itsenko-Cushing's syndrome is the adrenalovy (nadpochechnikovy) crisis which is shown consciousness violation, arterial hypotension, vomiting, belly-aches, a hypoglycemia, a giponatriyemiya, a giperkaliyemiya and metabolic acidosis.
As a result of decrease in resistance to infections at patients with Itsenko-Cushing's syndrome , phlegmons, nagnoitelny and fungal diseases of skin quite often develop. Development of an urolithic disease is connected with osteoporosis of bones and allocation with urine of excess of calcium and the phosphates leading to formation of oksalatny and phosphatic stones in kidneys. Pregnancy at women with a giperkortitsizm often comes to an end with an abortion or the complicated childbirth.
Diagnostics of a syndrome of Itsenko-Cushing
At suspicion at the patient of a syndrome Itsenko-Cushing on the basis of amnestichesky and fizikalny data and an exception of an exogenous source of glucocorticoids (including inhalation and intra articulate), first of all the giperkortitsizm reason becomes clear. It is for this purpose used screening tests:
- definition of an ekskretion of cortisol in daily urine: increase in cortisol by 3–4 times and more testifies to reliability of the diagnosis of a syndrome or Itsenko-Cushing's disease.
- small deksametazonovy test: normal reception of dexamethasone reduces cortisol level more than in a half, and at Itsenko-Cushing's syndrome of decrease does not happen.
Between a disease and Itsenko-Cushing's syndrome big deksametazonovy test allows to carry out differential diagnostics. At Itsenko-Cushing's disease reception of dexamethasone leads to decrease in concentration of cortisol more than twice from initial; at a syndrome of decrease in cortisol does not occur.
In urine contents 11-OKC is increased (11 oxyketosteroids) and 17-KC is lowered. In blood a gipokaliyemiya, increase in amount of hemoglobin, erythrocytes and cholesterol. For definition of a source of a giperkortitsizm (a bilateral giperplaziya of adrenal glands, adenoma of a hypophysis, a kortikosterom) the stsintigrafiya of adrenal glands is carried out by MPT or KT of adrenal glands and a hypophysis. For the purpose of diagnostics of complications of a syndrome of Itsenko-Cushing (osteoporosis, compression fractures of vertebras, a fracture of edges etc.) the X-ray analysis and KT of a backbone, a thorax is carried out. The biochemical research of indicators of blood diagnoses electrolytic violations, steroid diabetes, etc.
Treatment of a syndrome of Itsenko-Cushing
At the yatrogenny (medicinal) nature of a syndrome of Itsenko-Cushing gradual cancellation of glucocorticoids and their replacement by other immunodepressants is necessary. At the endogenous nature of a giperkortitsizm the medicines suppressing are appointed (, ).
In the presence of tumoral damage of adrenal glands, a hypophysis, lungs surgical removal of new growths is carried out, and at impossibility – one - or a bilateral adrenalektomiya (removal of an adrenal gland) or radiation therapy of gipotalamo-hypophysial area. Radiation therapy is carried often out to combinations with surgical or drug treatment for strengthening and fixing of effect.
Symptomatic treatment at Itsenko-Cushing's syndrome includes use of hypotensive, diuretic, antihyperglycemic medicines, warm glycosides, biostimulators and immunomodulators, antidepressants or sedatives, vitamin therapy, medicinal therapy of osteoporosis. Compensation of proteinaceous, mineral and carbohydrate exchange is carried out. Postoperative treatment of the patients with chronic nadpochechnikovy insufficiency who transferred an adrenalektomiya consists in continuous replacement hormonal therapy.
Forecast of a syndrome of Itsenko-Cushing
When ignoring treatment of a syndrome of Itsenko-Cushing the irreversible changes leading to a lethal outcome at 40-50% of patients develop. If the good-quality kortikosteroma, the forecast satisfactory though functions of a healthy adrenal gland are restored only at 80% of patients was the reason of a syndrome. At diagnostics malignant a kortikoster the forecast of five-year survival – 20-25% (on average 14 months). At chronic nadpochechnikovy insufficiency lifelong replacement therapy mineralo-and glucocorticoids is shown.
In general the forecast of a syndrome of Itsenko-Cushing is defined by timeliness of diagnostics and treatment, the reasons, existence and degree of expressiveness of complications, an opportunity and efficiency of surgery. Patients with Itsenko-Cushing's syndrome are on dynamic observation at the endocrinologist, heavy physical activities, night shifts on production are not recommended to them.