Itsenko-Cushing's disease
Contents:
Description:
Itsenko-Cushing's disease - a disease at which in a hypophysis the excess amount of the adrenocorticotropic hormone (AKTG) regulating work of adrenal glands is developed. At surplus of AKTG adrenal glands increase in a size and strenuously produce the hormones - corticosteroids, as leads to a disease.
Symptoms of this disease were described at different times by two scientists – the American neurosurgeon Harvey Cushing and the Odessa neuropathologist Nikolay Itsenko.
The American specialist connected a disease with a hypophysis tumor, the Soviet scientist assumed that the cause of illness is covered in changes of a hypothalamus - the site of the brain which is responsible for interaction of nervous and endocrine systems. Today scientists came to opinion that Itsenko-Cushing's disease возникаетв result of failures in work of gipotalamo-pituitary system - both researchers were right.
Itsenko-Cushing's disease, or otherwise hypercorticoidism - the serious neuroendocrinal illness arising in case of regulation disturbance гипоталамо - pituitary and adrenal system when the surplus of hormones of adrenal glands - corticosteroids is formed.
Distinguish an Icenco-Cushing syndrome and Itsenko-Cushing's disease. An Icenco-Cushing syndrome call all morbid conditions with one general symptom complex which cornerstone the giperkortizolizm, i.e. the increased allocation by bark of adrenal glands of cortisol is.
The Icenco-Cushing syndrome includes:
Itsenko-Cushing's disease resulting from increase in development by a hypophysis of adrenocorticotropic hormone (pituitary AKTG-zavisimy a syndrome).
AKTG-or kortikoliberin-malrelated syndrome.
iatrogenic or medicamentous sindromrazlichny hyperplastic tumors of bark of adrenal glands – adenomatosis, adenoma, an adenocarcinoma.
Icenco-Cushing syndrome reason:
Various states can be the cause of an Icenco-Cushing syndrome, however most often the hypercorticoidism is caused by the increased production of adrenocorticotropic hormone of a hypophysis (Itsenko-Cushing's disease). Adrenocorticotropic hormone can be produced by hypophysis microadenoma (microadenoma – small volume education in a hypophysis, as a rule, high-quality, the size not exceeding 2 cm, from here and its name) or malrelated kortikotropinomy – a tumor, producing corticotropic hormone in large numbers, but developing slowly. The malrelated kortikotropinoma having oncogenous or other malignant nature can be located in bronchial tubes, testicles, ovaries.
Pathogeny:
The cascade of hormonal changes in an organism is started.
Everything begins with the fact that mysterious nervous impulses which force its cells to produce too many substances activating release of adrenocorticotropic hormone in a hypophysis come to a hypothalamus.
In response to such powerful stimulation the hypophysis throws out a huge amount of this most adrenocorticotropic hormone (AKTG) in blood. It, in turn, influences adrenal glands: forces them to produce many the hormones - corticosteroids. Here also utter chaos begins, corticosteroids influence all exchange processes.
As a rule, at Itsenko-Cushing's disease the hypophysis is increased in sizes (a tumor, or hypophysis adenoma). In process of development of a disease also adrenal glands increase.
Icenco-Cushing syndrome symptoms:
Diagnosis of a disease, as a rule, does not present great difficulty since data of physical inspection of the patient have paramount value. More than at 90% of patients obesity of this or that degree is observed, and special attention is paid on itself by fat distribution – displastichny (on cushingoid type): on a stomach, a breast, a neck, a face (a crescent-shaped face of purple-red color, sometimes with a cyanochroic shade – a so-called matronizm) and spins (so-called "climacteric swelling"). From other forms of obesity the Icenco-Cushing syndrome is distinguished by the become thinner, almost transparent skin on the palm back.
Atrophy of muscles - the reduction of volume of muscle bulk which is followed by decrease in a tone of muscles and force of muscles, especially buttock and femoral ("sloping buttocks") – a typical sign of an Icenco-Cushing syndrome. Also the atrophy of muscles of a front abdominal wall ("a frog stomach") is observed, over time there are hernial protrusions on the white line of a stomach.
Skin of the patient becomes thinner, has a "marble" appearance with the explicit vascular drawing, is inclined to dryness, alternates sites of regional perspiration, is shelled.
Very often on skin striya – strips of stretching of crimson or violet color appear. Generally striya are located on skin of a stomach, the internal and external surface of hips, mammary glands, shoulders, can be from several centimeters to several millimeters. Skin dazzles with various rashes like an acne which are shown owing to an inflammation of sebaceous glands, numerous hypodermic microhemorrhages, vascular "asterisks".
In some cases, at Cushing's disease and a malrelated AKTG-syndrome the hyperpegmentation - the uneven distribution of a pigment which is shown in the form of the dark and lighter spots different from primary color of skin can be observed. The disease connected with injury (thinning) of a bone tissue, leading to fractures and deformation of bones – osteoporosis – one of the hardest complications accompanying an Icenco-Cushing syndrome. As a rule, osteoporosis develops at the vast majority of patients. Changes and deformations of bones, being followed by strong painful feelings, over time lead to scoliosis and a kyphoscoliosis.
At a hypercorticoidism the cardiomyopathy - group of diseases at which first of all the heart muscle suffers that is shown by dysfunction of a cardiac muscle quite often develops.
The reasons capable to cause a cardiomyopathy, a little, main of them - catabolic effects of steroids on a myocardium – a cardiac muscle, electrolytic shifts and arterial hypertension. Manifestations of a cardiomyopathy - disturbance of a heart rhythm and heart failure. The last is in most cases capable to lead the patient to a lethal outcome.
The nervous system can test an overstrain, differs in unstable work: the condition of the patient can vary from block and a depression to euphoria and steroid psychoses. Often patients do not state any complaints concerning the state whereas it can pose a threat for life.
The steroid diabetes mellitus can occur at an insignificant part of patients – 10-20%-. The disease can arise under the influence of various factors which are not connected with damage of a pancreas is characterized by long preservation of normal concentration of insulin in blood. The course of such diabetes quite easy, it is well compensated against the background of an individual dietotherapy and purpose of glucose-lowering drugs.
Excess of a producing adrenal androgens leads to development of a hirsutism - to excess pilosis at women on men's type. In most cases there are disturbances of a menstrual cycle, there is an amenorrhea – absence at the woman of periods within 6 and more months.
Outward of the patient with Itsenko-Cushing's disease
Treatment of an Icenco-Cushing syndrome:
Development and deployment of new diagnostic methods, and also new methods of visualization of a hypophysis and adrenal glands (ultrasonography, computer, magnetic and resonant tomography) allow to establish exact localization of a tumor, its sizes, relationship with surrounding fabrics, the direction of growth now and to choose an optimum method of treatment in each case. Therapy at Itsenko-Cushing's disease is recommended to be carried out in the conditions of a specialized endocrinological hospital.
The selection transsphenoidal adenomectomy remains by the most optimum method of treatment of a disease of Itsenko-Cushing for today. As the indication for carrying out neurosurgical operation serves accurately localized hypophysis adenoma (according to a computer or magnetic and resonant tomography). A contraindication – extremely severe form of a disease of Itsenko-Cushing or existence at the patient of heavy somatopathies. At accurately localized hypophysis tumor irrespective of its sizes the transsphenoidal adenomectomy yields positive takes at most of patients, does not demand long replacement therapy and provides rather bystry recovery of gipotalamo-pituitary relationship. After carrying out an adenomectomy in 70-80% of cases there occurs remission of a disease, in 12-20% there can be a recurrence.
In recent years apply a method of destruction of epinephral glands which assumes destruction of giperplazirovanny substance of an adrenal gland by administration of sclerosing substances under control of ultrasonography or a computer tomography (the selection flebografiya, is more rare – chreskozhno). As sclerosing substance usually use mix of 96% of solution of ethanol and 76% of solution of Urografinum in the ratio 3:1. The amount of the entered liquid is defined by degree of a hyperplasia of an adrenal gland and its volume. As a rule, destruction of adrenal glands is not applied as an independent method of treatment (usually in combination with radiation therapy or an adenomectomy).
In the absence of the data confirming existence of adenoma of a hypophysis carrying out a protonoterapiya on area of a hypophysis which is often combined with a unilateral adrenalectomy is considered expedient.
Medicamentous therapy at Itsenko-Cushing's disease consists in use of the drugs suppressing secretion of AKTG (the central action – substances of dofaminergichesky action, antiserotoninovy GABA-ergic drugs) and blockers of biosynthesis of steroid hormones in adrenal glands. The last subdivide into means which block biosynthesis of corticosteroids and cause destruction of cortical cells (derivatives ortho - couple - difenildikhloretana) and which block only synthesis of steroid hormones (aminoglutethimide derivatives).
It should be noted that inhibitors of a steroidogenesis are appointed at once after confirmation of the diagnosis of a disease of Itsenko-Cushing (both before operation, and after carrying out a protonoterapiya or surgical intervention).
Against the background of treatment of a basic disease symptomatic therapy is surely carried out:
hypotensive medicines (APF inhibitors, Spironolactonum);
potassium drugs;
glucose-lowering drugs (in case of need);
means for treatment of osteoporosis (the drugs reducing a resorption of a bone tissue – calcitonins) and bisfosfonatam (etidronat-ksidifon, памидронат, clodronate); active forms of vitamin D; the drugs stimulating an osteogenesis, anabolic steroids;
antidepressants, sedatives (if necessary);
vitamin therapy.
Carrying out a bilateral adrenalectomy (which is used in recent years less often, however the absolute indication for its performance is the severe disease and impossibility to compensate a state in other available ways) leads to emergence at the patient of chronic insufficiency of adrenal glands which demands lifelong replacement therapy glyuko-and mineralokortikoidam. Patients need dynamic observation of the endocrinologist, they are recommended to avoid a heavy exercise stress, work during a night shift.
The bilateral adrenalectomy can be complicated (in 10-50%) by Nelson syndrome – a combination of primary chronic insufficiency of adrenal glands (after surgical treatment or prolonged use of a hloditan) with progressing kortikotropinomy a hypophysis. All signs of primary hypocorticoidism against the background of the high level of secretion of AKTG are characteristic of Nelson syndrome. The magnetic and resonant research confirms existence of quickly growing hypophysis macroadenoma. Methods of the choice are the transsphenoidal adenomectomy or a protonoterapiya on area of a hypophysis. For replaceable treatment purpose of dexamethasone in low doses (0,125-0,25 mg) in combination with Prednisolonum or a cortisone is reasonable. In all cases it is necessary to use powerful mineralokortikoidny drug 9 - alpha фторкортизол. It should be noted that in clinical practice cases of an individual susceptibility of the patient to this or that drug used for replacement therapy note. Unfortunately, it is not always possible to find medicine which suits the specific patient and at which use the best both clinical, and metabolic compensation of insufficiency of adrenal glands as not all drugs of this group are registered in Ukraine is reached in pharmacy chain.
In the presence of a tumor of an adrenal gland carry out surgical removal of the struck gland with the subsequent temporary replacement therapy. At a malignant tumor if metastasises are not revealed or single metastasises are found, carry out removal of an adrenal gland with the subsequent purpose of a hloditan for a long time (if necessary – in combination with glucocorticoids). After surgical intervention all patients need long observation of the endocrinologist, cardiologist, neurologist, gynecologist.
