Cushings Syndrome

PNT Students - Gertrude's Gardens Children's Hospital (2009) 05/07/09 1

Benkele Rodgers gift – BSc.Nrs., (K.Paed.Nrs.,) Dip. Nrs. Cert. Nrs. PNT Students - Gertrude's Gardens Children's Hospital (2009) 05/07/09 2

Objectives  General objective  To equip the PNT students with knowledge and skill on management of a child with disorders of adrenal gland (Cushing’s Syndrome)  Specific objective  At the end of the presentation PNT students should be able to:-  1. Review the anatomy and physiology of the adrenal gland PNT Students - Gertrude's Gardens Children's Hospital (2009) 05/07/09 3

Objectives  2. Define Cushing's Syndrome  3. Outline the aetiology of Cushing's Syndrome  4. Explain the pathophysiology of Cushing's Syndrome  5. State the clinical manifestations of Cushing's Syndrome  6. Discuss the medical/surgical and nursing management of a child with Cushing's Syndrome  7. State the complications of Cushing's Syndrome PNT Students - Gertrude's Gardens Children's Hospital (2009) 05/07/09 4

Introduction  The term "Cushing's syndrome" is used to describe a condition resulting from long-term exposure to excessive glucocorticoids.  Cushing's syndrome affects about three times more women than men.  It is uncommon in children, when seen it is due to prolonged use of steroids  The condition is reversible once steroids are gradually withdrawn PNT Students - Gertrude's Gardens Children's Hospital (2009) 05/07/09 5

Introduction cont’  Definition  Cushing's syndrome is a characteristic group of manifestations caused by excessive circulating free cortisone (Wong, Hockenberry, Wilson, & Winkelstein, 2005).  The term "Cushing's disease" is reserved for Cushing's syndrome that is caused by excessive secretion of adrenocorticotropin hormone (ACTH) by a pituitary tumor, usually an adenoma (Wong, Hockenberry, Wilson, Winkelstein & Kline, 2003).  END PNT Students - Gertrude's Gardens Children's Hospital (2009) 05/07/09 6

Re vi e w of anat om and y phys i ol ogy of t he adr e nal gl and PNT Students - Gertrude's Gardens Children's Hospital (2009) 05/07/09 7

Review of anatomy and physiology of the adrenal gland  The adrenal glands are located on top of the kidneys;  They are divided into an inner renal medulla and an outer adrenal cortex.  The adrenal cortex is located in the outer portion, while the adrenal medulla is located in the central portion of the adrenal glands PNT Students - Gertrude's Gardens Children's Hospital (2009) 05/07/09 8

The location of adrenal glands The adrenal medulla and cortex PNT Students - Gertrude's Gardens Children's Hospital (2009) 05/07/09 9

Review of anatomy and physiology cont’  a) The adrenal medulla  It produces the catecholamines; epinephrine and norepinephrine.  The hormones function in the sympathetic division of the autonomic nervous system:  They target: the heart (increased heart rate and blood pressure); smooth muscle contraction (blood vessels,); the lungs (increased breathing: rate, rhythm, depth). PNT Students - Gertrude's Gardens Children's Hospital (2009) 05/07/09 10

Review of anatomy and physiology Cont’  Control of secretion of catecholamines in response to physiologic or emotional stress is through the hypothalamus and also stimulation of the sympathetic nervous system  Both systems support each other, hence there is no condition attributable to hypofunction of the adrenal medullar  Catecholamine-secreting tumors are attributable to adrenal medullary hyperfunction e.g. pheochromocytoma PNT Students - Gertrude's Gardens Children's Hospital (2009) 05/07/09 11

Review of anatomy and physiology Cont’  b) The adrenal cortex  It is located in the outer portion of the adrenal glands  It produces three groups of hormones classified according to their biologic function PNT Students - Gertrude's Gardens Children's Hospital (2009) 05/07/09 12

Review of anatomy and physiology Cont’  Glucocorticoids (cortisol – sress hormone and corticosterone which regulates glucose metabolism)  Mineralocorticoids (aldosterone which regulates water and electrolyte levels in the blood there regulating blood pressure PNT Students - Gertrude's Gardens Children's Hospital (2009) 05/07/09 13

Review of anatomy and physiology Cont’  3. Sex steroids (androgens, estrogens and progestins that supplement those of the ovary and testis.  Hypothalamus secretes corticotrophin-releasing factor (CRF) that stimulates the pituitary gland  ACTH targets the adrenal cortex to synthesise glucocorticoids  Aldosterone synthesis is regulated by renin- angiotensin system of the kidney PNT Students - Gertrude's Gardens Children's Hospital (2009) 05/07/09 14

Review of anatomy and physiology Cont’  Increased levels of angiotensin II stimulates adrenal cortex to secrete aldosterone which preserves sodium thereby retaining water  Sex steroids are secreted minimally until adolescence END PNT Students - Gertrude's Gardens Children's Hospital (2009) 05/07/09 15

Cushi ng' s syndr om e PNT Students - Gertrude's Gardens Children's Hospital (2009) 05/07/09 16

Aetiology  The cause of Cushing's syndrome is usually divided into two broad categories, Exogenous or endogenous  Exogenous (outside) causes  Prolonged use of glucocorticoids (e.g. prednisone) for diseases such as asthma and rheumatoid arthritis  Food dependent: - in appropriate sensitivity of adrenal glands to normal postprandial increases in secretion of gastric inhibitory polypeptide PNT Students - Gertrude's Gardens Children's Hospital (2009) 05/07/09 17

Aetiology Cont’  Endogenous (outside) causes  Benign pituitary adenoma secretes ACTH. This is responsible for 65% of endogenous Cushing's syndrome.  Excess cortisol is produced by adrenal gland tumors, hyperplastic adrenal glands, or adrenal glands with nodular adrenal hyperplasia (adrenocortical neoplasms) PNT Students - Gertrude's Gardens Children's Hospital (2009) 05/07/09 18

Pathophysiology  When stimulated by ACTH, the adrenal gland secretes cortisol and other steroid hormones.  The switch that controls the feedback mechanism is cortisol (Wong, Hockenberry, Wilson, Winkelstein & Kline, 2003).  When the levels are low the system turns on and when high the system turns off.  Excessive use of steroids leads to excess free circulation of cortisol in the body. PNT Students - Gertrude's Gardens Children's Hospital (2009) 05/07/09 19

Pathophysiology Cont’  Excess cortisol in the body will cause the liver to release more sugar, increased breakdown of muscle and fat for energy and also lowers the amount of energy used by the cells of the body.  It will also increase the anti-inflammatory effects and lowers the body's ability to protect itself. PNT Students - Gertrude's Gardens Children's Hospital (2009) 05/07/09 20

Clinical manifestations  The clinical manifestations are non-specific and overlap with much more common disorders such as simple obesity, hypertension, type 2 DM and depression.  Typical signs and symptoms are  Weight gain (90%)  An enlarged dorsocervical fat pad (buffalo hump)  Moon facies - thickening of facial fat, which rounds the facial contour PNT Students - Gertrude's Gardens Children's Hospital (2009) 05/07/09 21

Clinical manifestations  Hypertension (85%) - new onset hypertension  Glucose intolerance (80%) - ranging from hyperglycemia to diabetes  Purple striae (65%) Violaceous striae wider than 1 cm on abdomen or proximal extremities  Hirsutism – excessive body hair (65%) - with acne, usually mild.  Menstrual dysfunction - oligomenorrhea or amenorrhea and impotence in males PNT Students - Gertrude's Gardens Children's Hospital (2009) 05/07/09 22

Clinical manifestations striae PNT Students - Gertrude's Gardens Children's Hospital (2009) 05/07/09 23

Clinical manifestations striae PNT Students - Gertrude's Gardens Children's Hospital (2009) 05/07/09 24

Clinical manifestations  Muscle weakness (60%) - with wasting proximal weakness manifested by difficulty in climbing stairs, arising from a low chair or squatting.  Easy bruising (40%) With spontaneous ecchymoses  Osteoporosis (40%) Thinning of the skin Thinning of the skin and osteoporosis, with low back pain and vertebral collapse, are more common in older patients or those with chronic Cushing's Syndrome. PNT Students - Gertrude's Gardens Children's Hospital (2009) 05/07/09 25

Clinical manifestations  Mental changes - major depression (most common), insomnia, psychosis, mania, euphoria emotional lability  Hematologic Leukocytosis, lymphopenia, eosinopenia  Hyperpigmentation  Hypokalemia  Poor wound healing  Peripheral edema PNT Students - Gertrude's Gardens Children's Hospital (2009) 05/07/09 26

Clinical manifestations  Decreased libido  Increased susceptibility to infection, sometimes life-threatening  Deepening of voice  Clitoral enlargement  Tendency of male physique in females PNT Students - Gertrude's Gardens Children's Hospital (2009) 05/07/09 27

Diagnosis  History and physical examination  Excessive plasma cortisol levels  Increased blood glucose levels, decreased serum potassium level.  Plasma ACTH elevated in patients with pituitary tumors, very low in patients with adrenal tumor.  Eosinophils decreased on complete blood count. PNT Students - Gertrude's Gardens Children's Hospital (2009) 05/07/09 28

Diagnosis  Elevated urinary 17-hydroxycorticoids and 17- ketogenic steroids.  Overnight dexamethasone suppression test, possibly with cortisol urinary excretion measurement, to check for:  Unsuppressed cortisol level in Cushing’s syndrome cause by adrenal tumors.  Suppressed cortisol level in Cushing’s disease caused by pituitary tumor. PNT Students - Gertrude's Gardens Children's Hospital (2009) 05/07/09 29

Diagnosis  Skull X-ray detects erosion of the sella turcica by a pituitary tumor;  CT scan and ultrasonography locate tumor. PNT Students - Gertrude's Gardens Children's Hospital (2009) 05/07/09 30

Management  Treatment depends on the cause  Pituitary surgery to treat pituitary Cushing’s syndrome.  Transsphenoidal adenomectomy or hypophysectomy.  Transfrontal craniotomy may be necessary when a pituitary tumor has enlarged beyond the sella turcica.  Bilateral adrenalectomy is used to treat adrenal causes. PNT Students - Gertrude's Gardens Children's Hospital (2009) 05/07/09 31

Management  Most patients are rendered hypoadrenal for months to years after the procedure.  During this period, they require glucocorticoid replacement therapy.  Radiation therapy may also be used to treat pituitary or adrenal tumors.  Patients who have been surgically treated for Cushing's disease require careful long-term follow-up and monitoring for signs and symptoms of tumor recurrence. PNT Students - Gertrude's Gardens Children's Hospital (2009) 05/07/09 32

Management  The pituitary adrenal axis must be evaluated six to 12 months after surgery to determine the potential need for lifetime exogenous steroid replacement therapy.  Patients with panhypopituitarism subsequent to surgery require lifetime monitoring and titration of hormone therapy. PNT Students - Gertrude's Gardens Children's Hospital (2009) 05/07/09 33

Management  All patients who need glucocorticoid replacement therapy should be given careful instructions about the effects of stress and illness on glucocorticoid dosages.  In addition, these patients should wear appropriate medical alert labels. PNT Students - Gertrude's Gardens Children's Hospital (2009) 05/07/09 34

Algorithm for the suggested work-up of patients with suspected Cushing's syndrome. PNT Students - Gertrude's Gardens Children's Hospital (2009) 05/07/09 35 END

Nursing Management PNT Students - Gertrude's Gardens Children's Hospital (2009) 05/07/09 36

Nursing Management  Case Study: X is a 15-year-old girl living in West Lands . She stays with her parents. Her physician recently diagnosed X as having Cushing’s syndrome and admits her to the hospital for treatment. She has been having increased muscle weakness, so much so that she has difficulty climbing the one flight of stairs to her apartment. She has also had difficulty sleeping, irregular menstrual periods, and hypertension. PNT Students - Gertrude's Gardens Children's Hospital (2009) 05/07/09 37

Nursing Management  She is concerned about her protruding abdomen, round face, development of facial hair, and the numerous bruises that have appeared on her skin.  Assessment  Enlarged abdomen  Striae over the abdomen and buttocks, a round face, and obvious facial hair.  Her blood pressure is 160/96.  Low self-esteem PNT Students - Gertrude's Gardens Children's Hospital (2009) 05/07/09 38

Nursing Management  Nursing Diagnosis  Fluid volume excess, related to sodium retention causing edema and hypertension  Risk for injury, related to generalized fatigue and weakness  Risk for infection, related to impaired immune response and oedema  Body image disturbance, related to physical changes secondary to Cushing’s syndrome PNT Students - Gertrude's Gardens Children's Hospital (2009) 05/07/09 39

Nursing Management  Patient expected outcome  Will regain a normal body fluid balance.  Will remain free of injury.  Will remain free of infection.  Will verbalize understanding of the physical effects of the disease process and realistic expectations of desired changes in appearance. PNT Students - Gertrude's Gardens Children's Hospital (2009) 05/07/09 40

Nursing Management  Planning and implementation  Weigh patient each morning, using the same scale.  Maintain an accurate record of intake and output.  Develop a written schedule of rest and activity periods.  Monitor intake and output, daily weights, and serum glucose and electrolytes. PNT Students - Gertrude's Gardens Children's Hospital (2009) 05/07/09 41

Nursing Management  Provide time for discussion of the disease and treatment; encourage verbalization of feelings and identify successful coping mechanisms used in the past.  Encourage turning, coughing, and deep breathing  Monitor for signs of infection because risk is high with excess glucocorticoids.  Advise the patient how to recognize signs and symptoms PNT Students - Gertrude's Gardens Children's Hospital (2009) 05/07/09 42

Nursing Management  Assess the skin frequently to detect reddened areas, skin breakdown or tearing, excoriation, infection or edema.  Handle skin and extremity gently to prevent trauma; prevent falls by using side rails.  Avoid using adhesive tape on the skin to reduce trauma on its removal.  Encourage the patient to turn in bed frequently or ambulate to reduce pressure on bony prominences and areas of edema. PNT Students - Gertrude's Gardens Children's Hospital (2009) 05/07/09 43

Nursing Management  Assist the patient with ambulation and hygiene when weak and fatigued.  Use assistive devices during ambulation to prevent falls and fractures.  Help the patient to schedule exercise and rest. PNT Students - Gertrude's Gardens Children's Hospital (2009) 05/07/09 44

Complications  Infection – due to decreased production and circulating levels of antibodies by lysis of plasma cells and lymphocytes  Hypokalaemia – due to increased excretion of potassium and hydrogen ions  Hypertension – due to increased salt and water retention  Peptic ulcer disease – due to increased production of hydrochloric and pepsin and decreased gastric mucus production PNT Students - Gertrude's Gardens Children's Hospital (2009) 05/07/09 45

Complications  Osteoporosis – due to increased glomerular filtration rate and excretion of calcium and decreased absorption of calcium from intestinal tract  Retarded linear growth – due to increased levels of cortisol interfering with growth hormone  Vilirisation – due to excess production of androgens PNT Students - Gertrude's Gardens Children's Hospital (2009) 05/07/09 46

Any Questions END PNT Students - Gertrude's Gardens Children's Hospital (2009) 05/07/09 47

Summary  Cushing's syndrome can result from several different conditions that affect the control of cortisol synthesis.  Most commonly caused by the therapeutic administration of exogenous glucocorticoids.  Because the condition is potentially fatal if untreated, patients should have regular medical care and follow their treatment plan closely. PNT Students - Gertrude's Gardens Children's Hospital (2009) 05/07/09 48

References  Wong D. L., Hockenberry, M. J., Wilson, D., Winklstein, M. L. and Kline, N. E. (2003). Wong’s nursing care of infants and children, (7th ed.), St Louis: Mosby.  Hockenberry M. J., Wilson D., & Winkelstein M. L. (2005). Wong’s essentials of pediatric nursing, (7th ed.), St Louis: Mosby. PNT Students - Gertrude's Gardens Children's Hospital (2009) 05/07/09 49

References  Nursing Crib (2008). Cushing’s Syndrome, The Student Nurses Comment, On line [Accessed on 12.06.2009: 16:25Hrs], http://nursingcrib.com/category/nursing-notes- reviewer/medical-surgical-nursing/ PNT Students - Gertrude's Gardens Children's Hospital (2009) 05/07/09 50

PNT Students - Gertrude's Gardens Children's Hospital (2009) 05/07/09 51

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Cushings Syndrome

by Rodgers Benkele on Jul 05, 2009

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Cushings Syndrome — Presentation Transcript