Cushings Syndrome

PNT Students - Gertrude's Gardens Children's Hospital (2009)
04/08/2013
MANAGEMENT OF A CHILD WITH DISORDERS OF ADRENAL GLAND
Benkele Rodgers gift BSc.Nrs., (K.Paed.Nrs.,) Dip. Nrs. Cert. Nrs.

PNT Students - Gertrude's Gardens Children's Hospital (2009) 04/08/2013
Objectives
General objective To equip the PNT students with knowledge and skill on management of a child with disorders of adrenal gland (Cushings 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
04/08/2013
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

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gradually withdrawn
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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
Review of anatomy and physiology of the adrenal gland
04/08/2013
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
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The location of adrenal glands
The adrenal medulla and cortex
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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).
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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
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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
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1. Glucocorticoids
(cortisol sress hormone and corticosterone which regulates glucose metabolism)

2. Mineralocorticoids
(aldosterone which regulates water and electrolyte levels in the blood there regulating blood pressure
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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 reninangiotensin system of the kidney

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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
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Cushing's syndrome
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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
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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)
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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.
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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.
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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
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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
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striae
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striae
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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.
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Mental changes - major depression (most
common), insomnia, psychosis, mania, euphoria emotional lability Hematologic Leukocytosis, lymphopenia, eosinopenia Hyperpigmentation Hypokalemia Poor wound healing Peripheral edema
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Decreased libido
Increased susceptibility to infection, sometimes life-threatening

Deepening of voice
Clitoral enlargement
Tendency of male physique in females
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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.
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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 Cushings syndrome
cause by adrenal tumors. Suppressed cortisol level in Cushings disease caused by pituitary tumor.
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Diagnosis
Skull X-ray detects erosion of the sella turcica
by a pituitary tumor; CT scan and ultrasonography locate tumor.
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Management
Treatment depends on the cause
Pituitary surgery to treat pituitary Cushings
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.

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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.
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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.
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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.
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Algorithm for the suggested work-up of patients with suspected Cushing's syndrome.
END
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Nursing Management
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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 Cushings 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.
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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
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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 Cushings syndrome
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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.
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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.
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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

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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.
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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.
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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
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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
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Any Questions
END
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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.
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References
Wong D. L., Hockenberry, M. J., Wilson, D., Winklstein, M. L. and Kline, N. E. (2003). Wongs nursing care of infants and children, (7th ed.), St Louis: Mosby. Hockenberry M. J., Wilson D., & Winkelstein M. L. (2005). Wongs essentials of pediatric nursing, (7th ed.), St Louis: Mosby.
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References
Nursing Crib (2008). Cushings Syndrome, The Student Nurses Comment, On line [Accessed on 12.06.2009: 16:25Hrs], http://nursingcrib.com/category/nursing-notesreviewer/medical-surgical-nursing/
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PNT Students - Gertrude's Gardens Children's Hospital (2009)

MANAGEMENT OF A CHILD WITH DISORDERS OF ADRENAL GLAND

Benkele Rodgers gift BSc.Nrs., (K.Paed.Nrs.,) Dip. Nrs. Cert. Nrs.

PNT Students - Gertrude's Gardens Children's Hospital (2009)

7. State the complications of Cushing's Syndrome

It is uncommon in children, when seen it is due to prolonged use of steroids

PNT Students - Gertrude's Gardens Children's Hospital (2009)

Review of anatomy and physiology of the adrenal gland

PNT Students - Gertrude's Gardens Children's Hospital (2009)

Review of anatomy and physiology of the adrenal gland

PNT Students - Gertrude's Gardens Children's Hospital (2009)

The location of adrenal glands

The adrenal medulla and cortex

PNT Students - Gertrude's Gardens Children's Hospital (2009)

Review of anatomy and physiology cont

Review of anatomy and physiology Cont

Review of anatomy and physiology Cont

PNT Students - Gertrude's Gardens Children's Hospital (2009)

Review of anatomy and physiology Cont

(cortisol sress hormone and corticosterone which regulates glucose metabolism)

PNT Students - Gertrude's Gardens Children's Hospital (2009)

Review of anatomy and physiology Cont

Aldosterone synthesis is regulated by reninangiotensin system of the kidney

Review of anatomy and physiology Cont

PNT Students - Gertrude's Gardens Children's Hospital (2009)

PNT Students - Gertrude's Gardens Children's Hospital (2009)

PNT Students - Gertrude's Gardens Children's Hospital (2009)

PNT Students - Gertrude's Gardens Children's Hospital (2009)

Weight gain (90%)

PNT Students - Gertrude's Gardens Children's Hospital (2009)

Glucose intolerance (80%) - ranging from hyperglycemia to diabetes

PNT Students - Gertrude's Gardens Children's Hospital (2009)

PNT Students - Gertrude's Gardens Children's Hospital (2009)

PNT Students - Gertrude's Gardens Children's Hospital (2009)

Increased susceptibility to infection, sometimes life-threatening

PNT Students - Gertrude's Gardens Children's Hospital (2009)

Excessive plasma cortisol levels

PNT Students - Gertrude's Gardens Children's Hospital (2009)

by a pituitary tumor; CT scan and ultrasonography locate tumor.

PNT Students - Gertrude's Gardens Children's Hospital (2009)

Pituitary surgery to treat pituitary Cushings

Bilateral adrenalectomy is used to treat adrenal causes.

PNT Students - Gertrude's Gardens Children's Hospital (2009)

PNT Students - Gertrude's Gardens Children's Hospital (2009)

PNT Students - Gertrude's Gardens Children's Hospital (2009)

PNT Students - Gertrude's Gardens Children's Hospital (2009)

PNT Students - Gertrude's Gardens Children's Hospital (2009)

Her blood pressure is 160/96.

PNT Students - Gertrude's Gardens Children's Hospital (2009)

PNT Students - Gertrude's Gardens Children's Hospital (2009)

PNT Students - Gertrude's Gardens Children's Hospital (2009)

Advise the patient how to recognize signs and symptoms

Use assistive devices during ambulation to prevent falls and fractures.

PNT Students - Gertrude's Gardens Children's Hospital (2009)

PNT Students - Gertrude's Gardens Children's Hospital (2009)

PNT Students - Gertrude's Gardens Children's Hospital (2009)

PNT Students - Gertrude's Gardens Children's Hospital (2009)

PNT Students - Gertrude's Gardens Children's Hospital (2009)

PNT Students - Gertrude's Gardens Children's Hospital (2009)

PNT Students - Gertrude's Gardens Children's Hospital (2009)

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