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Cushing's syndrome > Health and Wellness.

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A syndrome is a set of signs and symptoms. In Cushing's Syndrome they are caused by an excess of chronic cortisol. Cortisol is a hormone type glucocorticoid that is produced naturally in the adrenal capsule, an endocrine organ - i.e. it produces hormones - located above both kidneys. Also known as the "stress hormone" cortisol plays very important actions, such as the maintenance of blood glucose levels and the functioning of the nervous and cardiovascular systems. In addition, the cortisol protects autolesivos mechanisms as, for example, responses inflammatory and immune over which can be harmful for the organism.
Excess cortisol is called hypercortisolism or hipercorticismo and may be endogenous, when the cause is in the body, or exogenous, due to natural or synthetic glucocorticoids above the physiological dose.
High doses of corticoids are part of the treatment of leukemias, lymphomas, transplants, etc., due to their anti-inflammatory properties and its ability to curb some immunological responses. Today, steroids are essential for the treatment of many of these diseases, and why his administration is the most frequent cause of (exogenously) Cushing's syndrome.
In adults, the most frequent endogenous is called disease Cushing, which is due to excessive secretion and chronic way of a hormone that stimulates the production of glucocorticoids for the adrenal; This hormone is the adrenocorticotropic or ACTH, which is released into the blood from the pituitary gland, an organ that produces various hormones which, in turn, act in various hormones (endocrine organs)-producing organs. ACTH induces the glucocorticoid and androgen production by the adrenal.
This disease is approximately eight times more common in women than in men, and usually occurs more between 20 and 40 years of age. In more than 90% of the cases of Cushing's disease is detected a benign tumor in the pituitary gland - usually an adenoma-producer of ACTH.
Endogenous Cushing's syndrome is a rare entity: has an incidence of two to four cases per million inhabitants per year; and the most frequent cause, Cushing's disease, has an incidence of 1.2 to 2.4 cases per million inhabitants per year.

Symptoms of Cushing's syndrome

The symptoms and signs of Cushing's syndrome vary with age, sex, and also with the cause, the intensity and the duration of the hipercorticismo. The disease can cause obesity, variations in skin, arterial hypertension, mental disorders, osteoporosis, disorders of glucose metabolism, etc.
Obesity in this syndrome has very special characteristics, since it affects especially the face and trunk. The sick person's face is round (described in the books of medicine as a "Moon face") and Ruddy, and the neck is very thickened (described as "Buffalo neck"). Skin, in general, is thinned, with streaks of purplish red color in the abdomen, hips, armpits, breasts and thighs. Hematomas appear very easily, spontaneously or with minimal trauma. Wounds heal later than normal.
High blood pressure tends to be moderate. Mental disorders such as depression, irritability, anxiety, insomnia, emotional lability, panic and paranoid pictures occur in approximately half of patients.
Disorders of the metabolism of glucose produce diabetes mellitus. Osteoporosis (bone loss) can be very intense, favouring the emergence of fractures, and occurs most often in young patients. Urinary calculi due to an increased elimination of calcium can also produce in the urine. In children there is a delay of growth and maturation of the bones.
Cases with increased secretion of ACTH in the skin takes a dark color. In addition, women do not have menstruation or it is minimal, and if they also have an increase in androgens of adrenal origin occurs Seborrhea, acne and increase the hair on different parts of the body (HIRSUTISM).

Diagnosis of Cushing's syndrome

Exogenous Cushing Syndrome is easy to detect for physicians that are treating a patient with glucocorticoids. In these situations tries to best balance the effects that they have to exercise on the basic problem being treated and side effects, i.e., the Cushing's syndrome that can cause.
The endogenous hipercorticismo, however, requires a study allowing the diagnosis - elevation of cortisol, ACTH or both - and discover the cause. Not always detect an increase in cortisol or ACTH is easy, since they may not be increased at any time. Thus, endogenous cortisol excess is demonstrated by the detection of free urinary cortisol increased in several determinations, and also if it is detected plasmatic cortisol high blood extracted by the morning after the oral administration of dexamethasone (1 mg) at 23:00 of the day before (called fast inhibition test). There is a test similar to the latter in which a greater amount of dexamethasone is administered during two days (weak inhibition test).
Other tests used for the diagnosis of Cushing's syndrome are the determination of cortisol in the blood and saliva at 12 in the night and during sleep; It is normal that during the night, cortisol levels are low, but in patients with this syndrome are higher.
For the detection of the increase of ACTH, in the most of them cases not is enough it determination direct in the blood, by the contrary, almost always is necessary a test of stimulation with the hormone releasing of ACTH or CRH.

Diagnosis of the origin of the hipercorticismo

For the diagnosis of the origin of the hipercorticismo should consider two types of ACTH-dependent and that it is not. In the first case, there is an increase in the hormone ACTH, which may be of pituitary origin or there is also the possibility of ACTH to be produced by a tumor is in a location different from the pituitary gland. In the second case, when the syndrome is not due to an increase in ACTH, cortisol excess is, in general, a producer of the same tumor located in the adrenal gland.
The CRH stimulation test serves to distinguish if high production of ACTH from the pituitary gland, in which case the response in this test is too large, or does not come from the pituitary gland, with a low response. With this test there is no response if Cushing's syndrome of adrenal origin, i.e. without hiperproducción of ACTH. So what is the cause of Cushing's syndrome there is high-dose dexamethasone suppression test.
Depending on the results obtained in the hormone study will use diagnostic methods that ensure the location of the tumor:
To detect a pituitary adenoma - is a benign tumor, the most common pituitary ACTH producing tumor - cranial magnetic resonance imaging will be used.
If the tumor in the resonance - is not sometimes are tiny, even microscopic-, ACTH in the arterial blood next to the pituitary through an arterial catheterization can be measured.
If you suspect that the origin is adrenal, you can see the tumor by computed tomography, magnetic resonance and ultrasound of the adrenal glands.
If you suspect that the origin is a located outside (ectopic tumor) pituitary ACTH producing tumor, is usually a CT scan of the chest - more than half of ectopic tumors are located here - and abdomen; You can also scan, which can detect the tumor in any part of the body and see your extension.

Treatment of Cushing's syndrome

Endogenous Cushing's syndrome is not and continues to evolve it can cause death, usually caused by cardiovascular alterations. On the other hand, patients have high blood pressure, diabetes mellitus of difficult to control, psychiatric disorders, complications of osteoporosis and serious electrolyte disturbances.
The treatment depends on the cause of the syndrome. If it is a Cushing's disease, should completely remove the pituitary tumor. In the majority of cases this is achieved by an operation to remove the tumor through the base of the skull - specifically bone, sphenoid, which is just below the gland pituitary gland - through the nose.
Cushing's disease can return even after ten years, so that periodic checks are recommended.
There is also a drug treatment, which is often used before surgical treatment, to reduce the symptoms of the syndrome. These drugs are - the most widely used - ketoconazole and the metopirone.
Treatment of ectopic ACTH-producing tumors is, whenever possible, the removal. When this is not possible, the pharmacological treatment mentioned above and also octeótrido or lanreotide is used.
Treatment of adrenal tumors is surgical. Most are adenomas - benign tumors - and his removal involves the cure. Adrenal carcinoma has poor prognosis and the best thing is the total removal of the adrenal, also mitotane (an active principle), is used to treat the symptoms, along with chemotherapy. In case of removal of the two adrenal cortisol and fludrocortisone treatment is necessary in life.
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