Cushings Syndrome

13/04/23PNT Students - Gertrude's Gardens Children's Hospital (2009) 1

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


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


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The condition is reversible once steroids are gradually withdrawn


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).

END13/04/23PNT Students - Gertrude's Gardens Children's Hospital (2009) 6

Review of anatomy and physiology of the adrenal gland


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



The adrenal medulla and cortex

The location of adrenal glands

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).


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


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


Review of anatomy and physiology Cont’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


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


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

13/04/23PNT Students - Gertrude's Gardens Children's Hospital (2009) 15END

Cushing's syndrome


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


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)


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.


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.


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


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


Clinical manifestations

striae



striae



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.


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


Clinical manifestations Decreased libido Increased susceptibility to infection,

sometimes life-threatening Deepening of voice Clitoral enlargement Tendency of male physique in females


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.


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.


Diagnosis

Skull X-ray detects erosion of the sella turcica by a pituitary tumor;

CT scan and ultrasonography locate tumor.


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.


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.


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.


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.


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



Nursing Management

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.


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


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


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.


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.


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


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.


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.


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


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


Any Questions


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.


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.


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/



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Cushings Syndrome

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