Cushing disease is a hormonal disorder caused by elevated levels of ACTH.
The endocrine system is a group of glands that produces hormones. Hormones are chemical messengers in the body that affect almost all major parts of the body. Cushing disease is a rare, but debilitating endocrine disorder that causes emotional and physical difficulties due to elevated circulatory cortisol levels.Cortisol is a major glucocorticoid belonging to the steroid family secreted by the adrenal gland.
What is Cushing disease and what are its causes?
Any physiological condition which makes the adrenal gland produce too much cortisol results in a disorder known as Cushing’s syndrome.
Causes of Cushing syndrome
- Ectopic Adrenocorticotropic hormone (ACTH) syndrome (tumors usually found in the thyroid, lung, pancreas or thymus gland)
- Pituitary gland tumor
- Tumor in the adrenal gland
- Increased level of cortisol due to steroid medication
Cushing disease is a specific form of Cushing syndrome resulting from the excessive secretion of Adrenocorticotropic hormone (ACTH) from the pituitary gland due to tumor.
What are the signs and symptoms of Cushing syndrome and how it can be diagnosed?
Cushing syndrome is characterized by deposition of fat on a face give appearance like moon so called as “moon like face”.The hump appears behind the shoulder called as “Buffalo hump”. Other signs and symptoms include:
- Rapid weight gain
- Moodiness, depression, irritability
- Bone and Muscle weakness
- Memory and attention dysfunction
- Diabetes mellitus
- Sleep disturbances
- Menstrual disorders like amenorrhea in women
- Decreased fertility in men
- Immune suppression
- Erectile dysfunction
- Loss of sexual interest
- Decreased fertility
The symptoms of Cushing syndrome is similar to other conditions like underactive thyroid gland and high blood pressure so your physician may refer you a number of test for proper diagnosis of Cushing.The amount of cortisol in the body is the basis of diagnosis of Cushing disease that can be diagnosed by
- Urine test
- Blood test
- Saliva test
Saliva test is most accurate test if performed on a midnight saliva sample.Though no test is completely reliable to diagnose Cushing syndrome, but if you have one or more abnormal results, you should contact endocrinologist.
Cushing syndrome treatment and management
Treatment is eventually directed at the cause of the disorder.The chief goal of the treatment of Cushing disorder is to quick normalization of negative metabolic effect and biochemical aberrations like increased level of ACTH that is associated with the disorder. Treatment of Cushing syndrome is essential since significant morbidity and increased mortality due to metabolic effects including hypertension, osteoporosis, obesity, dyslipidemia and glucose intolerance due to high level of cortisol.The following methods can be opted in the treatment of Cushing syndrome.
- Adrenalectomy(surgical removal of the adrenal gland): This method is opted in the case of adrenal adenoma, and it is the final measure when all the recommended treatment get fail. Since adrenal carcinoma is a metastatic disease hence chances of reoccurrence are there so it need second line of treatment after surgery. Adrenalectomy discontinues cortisol production so requires mineralocorticoid and glucocorticoid replacement therapy.
- Transsphenoidal adenoidectomy: It is surgical removal of well defined small pituitary adenoma (carcinoma).It is one of the main treatment strategies of Cushing disease in which the main cause of the elevated level of ACTH is pituitary carcinoma.
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• Radiation therapy: This is used when removal of a tumor in the pituitary is difficult through surgery.
• Medical therapy: Several drugs are used to control the cortisol production when pituitary surgery is unable to restore the normal cortisol production.Following drug therapy is used in the treatment of Cushing disease:
- Cabergoline is the drug that is mostly used to treat prolactin secreting pituitary tumor may normalize the ACTH level. Pasireotide, a newly approved drug in Europe and United state, can normalize ACTH level.
- Cortisol production blockers are Ketoconazole, Metyrapone or Metotane, Aminoglutethimide, Etomidate.
- The agents that lowers the ACTH secretion by pituitary adenomas are the good options to treat the crushing disease. Dopamine Agonists like bromocriptine, Somatostatin analogs like Octreotide, retinoic acid are few names in this category.
- Though surgical methods are there, but the chances of reoccurrence of hypercortisolemia is still with them. The approval of Mifepristone for residual or recurrent hypercortisolemia in the patient with cushing disease represents a hopeful development in the management of cushing syndrome.
- The development of diagnostic, surgical techniques and effective medication has given a new horizon to health care systems. The management of Cushing disease is still challenging, but possible.Timely prognosis and management prevent a disease to be more perilous.Talk to your physician and get the possible ways of effective management of cushing disease .
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