cushings syndrome
TRANSCRIPT
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Benkele Rodgers gift – BSc.Nrs., (K.Paed.Nrs.,) Dip. Nrs. Cert. Nrs.
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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
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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
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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The condition is reversible once steroids are gradually withdrawn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).
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Review of anatomy and physiology of the adrenal gland
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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 adrenal medulla and cortex
The location of adrenal glands
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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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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
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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
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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
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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
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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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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
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Clinical manifestations
striae
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Clinical manifestations
striae
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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.
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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
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Clinical manifestations 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 Cushing’s
syndrome cause by adrenal tumors. Suppressed cortisol level in Cushing’s
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
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.
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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.
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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 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.
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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 Cushing’s 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
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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). 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.
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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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