CUSHING'S SYNDROME. Bharath University, Chrompet, Chennai, Tamil Nadu, India

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1 TJPRC: International Journal of Nursing and Patient Safety & Care (TJPRC: IJNPSC) Vol. 1, Issue 1, Jun 2016, TJPRC Pvt. Ltd. CUSHING'S SYNDROME R. RAMANI 1 & V. HEMAVATHY 2 1 Associate Professor, Department of Medical Surgical Nursing, Sree Balaji College of Nursing, Bharath University, Chrompet, Chennai, Tamil Nadu, India 2 Principal, Sree Balaji College of Nursing, Bharath University, Chrompet, Chennai, Tamil Nadu, India ABSTRACT Cushing's syndrome is a debilitating endocrine disorder which results from hypercortisolemia. While endogenous Cushing's syndrome can be caused by an adrenocorticotrophic hormone (ACTH)-dependent or ACTHindependent mechanism, it is most often a result of excess secretion of ACTH by a corticotrophin adenoma (Cushing's disease). Untreated hypercortisolemia causes significant morbidity and increased mortality due to its metabolic effects including hypertension, osteoporosis, obesity, dyslipidemia, and glucose intolerance. Although primary therapy is surgical, a substantial portion of patients will go on to require second-line therapies including repeat surgery, radiotherapy or drug therapy. While medical therapy for Cushing's syndrome has been limited, several new agents are being investigated. This aim of this review is to analyze and present the available options for medical management of Cushing's syndrome as well as review potential new therapies and their role in the treatment of this disorder. KEYWORDS: Cushing s Syndrome, Hypercortisolemia, Adrenocorticotrophic Hormone (ACTH) Received: May 03, 2016; Accepted: May 18, 2016; Published: May 21, 2016; Paper Id.: TJPRC:IJNPSCJUN INTRODUCTION Original Article Cushing's syndrome is a rare hormonal disorder that causes sudden weight gain and bloating around the chest and stomach, along with other symptoms. These symptoms are caused by constantly high levels of steroid hormones in the blood. This could be because you are taking high doses of steroid medication for another illness (such as asthma) or due to a disorder that causes your body to produce too much of the steroid hormone cortisol.cortisol is produced by two small organs just above the kidneys called the adrenal glands. Cortisol regulates the level of glucose in the body, suppresses the immune system and helps the body respond to stress (it is also called the 'stress hormone'). Definition Cushing s syndrome, also known as hypercortisolism, is a collection of symptoms that develop due to very high levels of a hormone called cortisol in the body. Causes The use of corticosteroid medication. Excessive secretion of adrenocorticotropic hormone (ATCH). Primary hyperplasia. Ectopic production of ACTH.

2 58 R. Ramani & V. Hemavathy Depression malnutrition alcoholism reducing the immune system s inflammatory response converting carbohydrates, fats, and proteins into energy stress response Pituitary gland tumor. Adrenal gland abnormality or tumor. Pathophysiology The hypothalamus is in the brain and the pituitary gland sits just below it. The paraventricular nucleus (PVN) of the hypothalamus releases corticotropin-releasing hormone (CRH), which stimulates the pituitary gland to release adrenocorticotropin (ACTH). ACTH travels via the blood to the adrenal gland, where it stimulates the release of cortisol. Cortisol is secreted by the cortex of the adrenal gland from a region called the zona fasciculata in response to ACTH. Elevated levels of cortisol exert negative feedback on the pituitary, which decreases the amount of ACTH released from the pituitary gland. Signs & Symptoms Figure 1: Signs & Symptoms of Cushing Syndrome Cardiovascular Hypertension and heart failure. Endocrine and Metabolic Truncal obesity. Moon face. Buffalo hump.

3 Cushing's Syndrome 59 Sodium retention. Metabolic alkalosis. Hyperglycemia. Gastrointestinal Peptic ulcers and pancreatitis. Muscular Myopathy and muscle weakness. Dermatologic Thinning of skin. Ecchymoses. Striae. Psychiatric Mood alterations and psychoses. Immune Functions Impaired wound healing. Increased susceptibility to infections. Decreased inflammatory response. Diagnostic Findings There is no single definitive test for Cushing syndrome. The diagnosis involves a thorough physical examination and a review of your medical history and symptoms. Laboratory tests that help with the diagnosis may include: Urinalysis. Dexamethasone suppression test. Serum cortisol testing. Corticotropin testing. Blood chemistry analysis. Salivary free cortisol analysis.diagnostic Findings cont. Inferior petrosal sinus sampling. Ultrasonography, CT scan, MRI. Treatment for Cushing Syndrome

4 60 R. Ramani & V. Hemavathy Treatment will depend on what is causing the problem. Your doctor may prescribe a medication to help, control cortisol production and ease symptoms.if you use corticosteroids, a change in medication or dosage may be required. Do not attempt to change the dosage yourself. Close medical supervision is required. Tumors can be cancerous (malignant) or noncancerous (benign). Surgical removal may be required. Radiation therapy or chemotherapy may also be recommended. Treatments for Cushing syndrome are designed to lower the high level of cortisol in your body. The best treatment for you depends on the cause of the syndrome. Treatment options include: Reducing Corticosteroid Use If the cause of Cushing syndrome is long-term use of corticosteroid medications, your doctor may be able to keep your Cushing signs and symptoms under control by reducing the dosage of the drug over a period of time, while still adequately managing your asthma, arthritis or other condition. For many of these medical problems, your doctor can prescribe noncorticosteroid drugs, which will allow him or her to reduce the dosage or eliminate the use of corticosteroids altogether. Don't reduce the dose of corticosteroid drugs or stop taking them on your own. Do so only under your doctor's supervision. Abruptly discontinuing these medications could lead to deficient cortisol levels. Slowly tapering off corticosteroid drugs allows your body to resume normal cortisol production. Surgery If the cause of Cushing syndrome is a tumor, your doctor may recommend complete surgical removal. Pituitary tumors are typically removed by a neurosurgeon, who may perform the procedure through your nose. If a tumor is present in the adrenal glands, lungs or pancreas, the surgeon can remove it through a standard operation or in some cases by using minimally invasive surgical techniques, with smaller incisions. After the operation, you'll need to take cortisol replacement medications to provide your body with the correct amount of cortisol. In most cases, you'll eventually experience a return of normal adrenal hormone production, and your doctor can taper off the replacement drugs. However, this process can take up to a year or longer. In some instances, people with Cushing syndrome never experience a resumption of normal adrenal function; they then need lifelong replacement therapy. Radiation therapy If the surgeon can't totally remove a pituitary tumor, he or she will usually prescribe radiation therapy to be used in conjunction with the operation. Additionally, radiation may be used for people who aren't suitable candidates for surgery. Radiation can be given in small doses over a six-week period or by a technique called stereotactic radiosurgery (Gamma Knife surgery). In the latter procedure, administered as a single treatment, a large dose of radiation is delivered to the tumor, and the radiation exposure to surrounding tissues is minimized. Medications Medications can be used to control cortisol production when surgery and radiation don't work. Medications may also be used before surgery in people who have become very sick with Cushing syndrome. Doctors recommend drug therapy before surgery to improve signs and symptoms and minimize surgical risk. Medications to control excessive production of cortisol include ketoconazole (Nizoral), mitotane (Lysodren) and metyrapone (Metopirone). The Food and

5 Cushing's Syndrome 61 Drug Administration has also approved the use of mifepristone (Korlym) for people with Cushing syndrome who have type 2 diabetes or glucose intolerance. Mifepristone does not decrease cortisol production, but it blocks the effect of cortisol on your tissues. In some cases, the tumor or its treatment will cause other hormones produced by the pituitary or adrenal gland to become deficient and your doctor will recommend hormone replacement medications. Nursing Management for Cushing s Syndrome Consult a dietician to plan a high in protein and potassium but low in calories, carbohydrates, and sodium. Use protective measures to reduce the risk of infection. Schedule activites around the patient s rest periods to avoid fatigue. Institute safety precaustions to minimize the risk of injury from the falls. Help the client to walk, to avoid bumps and bruises. Help the bedridden patient to turn and reposition herself every 2 hours. Use extreme caution when moving the patient to minimize trauma and bone stress. Provide frequent skin care, especially over bony prominences. Provide support with pillows and a convoluted foam mattress. Encourage the patient to verbalize her feelings about the body image changes and sexual dysfunction. Prepare the patient for surgery if indicated. Complications of Cushing Syndrome If left untreated, Cushing syndrome can lead to bone loss, bone fractures muscle loss and weakness high blood pressure diabetes infections enlargement of a pituitary tumor kidney stones REFERENCES 1. Nieman, L. K. & Ilias, I. Evaluation and treatment of Cushing's syndrome. Am. J. Med.118, (2005). 2. Raff, H. & Findling, J. W. A physiologic approach to diagnosis of the Cushing syndrome. Ann. Intern. Med. 138, (2003).

6 62 R. Ramani & V. Hemavathy 3. Arnaldi, G. et al. Diagnosis and complications of Cushing's syndrome: a consensus statement. J. Clin. Endocrinol. Metab. 88, (2003). 4. Biller, B. M. et al. Treatment of adrenocorticotropin-dependent Cushing's syndrome: a consensus statement. J. Clin. Endocrinol. Metab. 93, (2008). 5. Nieman, L. K. et al. The diagnosis of Cushing's syndrome: an Endocrine Society Clinical Practice Guideline. J. Clin. Endocrinol. Metab. 93, (2008). 6. Bonneville, J. F., Cattin, F., Bonneville, F., Schillo, F. & Jacquet, G. Pituitary gland imaging in Cushing's disease [French]. Neurochirurgie48, (2002). 7. Dwyer, A. J. et al. Pituitary adenomas in patients with Cushing disease: initial experience with Gd-DTPA-enhanced MR imaging. Radiology 163, (1987). 8. Hardy, J. Excision of pituitary adenomas by transsphenoidal approach [French]. Union Med. Can.91, (1962). 9. Oldfield, E. H. et al. Preoperative lateralization of ACTH-secreting pituitary microadenomas by bilateral and simultaneous inferior petrosal venous sinus sampling.n. Engl. J. Med. 312, (1985). 10. Oldfield, E. H. et al. Petrosal sinus sampling with and without corticotropin-releasing hormone for the differential diagnosis of Cushing's syndrome. N. Engl. J. Med. 325, (1991). 11. Barker, F. G. 2 nd, Klibanski, A. & Swearingen, B. Transsphenoidal surgery for pituitary tumors in the United States, : mortality, morbidity, and the effects of hospital and surgeon volume. J. Clin. Endocrinol. Metab. 88, (2003). 12. Semple, P. L. & Laws, E. R. Jr. Complications in a contemporary series of patients who underwent transsphenoidal surgery for Cushing's disease. J. Neurosurg. 91, (1999).