Diagnosis and treatment of the syndrome Cushing
Endocrinology / / May 11, 2016
diagnosis of Cushing's syndrome - Cushing in severe forms can be delivered based on the nature of fat distribution with excessive deposition in the body, a person with view of the full moon, purple-cyanotic skin color with marble its pattern in the limbs and trunk, atrophicprocesses in the skin with the development of stretch marks is red-violet color, atrophy of muscles, menstrual disorders in women, atrophic processes in the testicles in men, hypertrichosis, high blood pressure, disorders of the metabolism of proteins, fats and carbohydrates and a number of other changes.
auxiliary diagnostic value is often observed increase in blood glucocorticoids 17 (above 25%) or increased release of urine (greater than 5.6 mg / 24 h), determined by the method of Porter - Zilber.
Definition of neutral 17-keto steroids in the urine has no diagnostic value due to the fact that increased their allocation occurs only in a small proportion of patients.
High blood levels of glucocorticoids 17 and high urinary excre
Significant diagnostic value has a test with dexamethasone, which also makes it possible to distinguish between the form caused by hyperplasia and adrenal tumors.This trial is also important in diagnostically unclear forms of the disease.Suppression of adrenocortical function and a decrease in the content of 17 glucocorticoids in the blood, as well as the decrease in allocation of urine is less than 50% from baseline at a daily dose of dexamethasone 8 mg.no suppression at a daily dose of 2 mg.He speaks in favor of adrenal hyperplasia.Lack of suppression at a dose of 8 mg dexamethasone.per day suggesting the presence of benign or malignant tumor of the adrenal cortex.
When using the ACTH stimulation test in patients with Cushing's syndrome - Cushing, caused by adrenal hyperplasia, often there is a significant increase in the blood and urine excretion 17 glucocorticoids.The syndrome caused by a tumor of the cortex, the response is lowered.However, the diagnostic value of this test is significantly lower than the test with dexamethasone.
To identify hyperplasia and adrenal tumors especially used oksisuprarenorentgenografiya - kidneys and adrenal glands after preliminary introduction to perinephric tissue oxygen.
indirect indicator of the presence of a tumor of the adrenal cortex may be kidney displacement and deformation of the renal pelvis revealed by pyelography.
Difficulties in the differential diagnosis can often occur in patients suffering from both obesity, high blood pressure, menstrual disorder, mild hypertrichosis.The absence of these patients skin color changes localized fat deposits on the face and trunk, no atrophic processes in the skin and muscles and a lack of characteristic external form often offers an opportunity to differential diagnosis even without special laboratory tests.In case of doubt, a decisive diagnostic importance is the determination of blood and urinary 17-glucocorticoids dexamethasone and samples without giving deviations from normal values in these patients.
Some diagnostic difficulties may arise in the differential diagnosis of Cushing's syndrome - Cushing and obesity with the presence of multiple pink stretch marks in young people.But unlike Cushing syndrome - Cushing these striae pink, tight, without atrophy of the skin and subcutaneous tissue, often numerous.They are usually located on the upper parts of the inner arms, thighs, buttocks, in the breast.The secretion of hydrocortisone increased slightly, but the release of urine 17-ketogenic steroids and 17-keto steroids, levels in urine and plasma hydrocortisone, response to ACTH and dexamethasone test allow to distinguish this disease from syndrome Cushing - Cushing.Average precipitation tests with dexamethasone and ACTH in these patients shows that the pituitary-adrenal system is functioning properly.
in various forms virilism (eg, androgen-producing tumors of the adrenal cortex, arenoblastoma) differential diagnostic value are pronounced in these diseases hypertrichosis, often severe muscle development, deepening of voice, clitoral hypertrophy and the appearance of other signs virilism and other irregularities in theno symptoms of Cushing's syndrome - Cushing.
Figure: Cushing's syndrome - Cushing: A - the patient before the operation, B - the same patient after surgery .
flow syndrome Cushing - Cushing largely determined by the nature of the underlying disease process - hyperplasia, adenoma or cancer of the adrenal cortex or cancer vnenadpochechnoy localization.
Cushing's syndrome - Cushing's caused by hyperplasia or adenoma of the adrenal cortex, the progression of the disease occurs in different patients differently, but life expectancy in untreated patients from the time of diagnosis to death in an average of five years.The cause of death is the most common infection (pneumonia, erysipelas, cellulitis) and vascular disorders (cerebral, renal and myocardial infarction less frequently).When conducted in a timely manner total or subtotal removal of the adrenal glands and properly pursued in the future replacement therapy with hydrocortisone, cortisone or their analogues clinical manifestations of the syndrome are eliminated, greatly lengthens the life of patients and partially restored ability to work.Developing these patients, chronic adrenal insufficiency causes to be more cautious with regard to the forecast.
- Syndrome Cushing - Cushing
- Physiology of the adrenal cortex
- Chronic adrenal insufficiency.Addison's disease
- androgen-producing tumors of the adrenal cortex
- Primary aldosteronism (Conn's syndrome)
- Clinical evaluation of laboratory studies of the functional state of the adrenal cortex
- feminizing tumor of the adrenal cortex
- Diagnosis and differential diagnosis of congenital adrenal hyperplasia
- Treatment of chronic diseaseadrenal
- congenital (inborn) hyperaldosteronism
- congenital virilizing adrenal hyperplasia
- Treatment of congenital adrenal hyperplasia
- Acute failure of the adrenal cortex
The syndrome Cushing - Cushing,caused by adenoma of the adrenal cortex, in the absence of irreversible complications, removal of adenomas leading to recovery.
In cancer of the adrenal cortex, due to the early appearance of metastases, the prognosis is usually unfavorable.