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.


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gradually withdrawn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).
 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


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 location of
adrenal glands The adrenal medulla and
cortex


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).


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

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’
 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


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


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


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


 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



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.


 Mental changes - major depression (most
common), insomnia, psychosis, mania, euphoria
emotional lability
 Hematologic Leukocytosis, lymphopenia,
eosinopenia
 Hyperpigmentation
 Hypokalemia
 Poor wound healing
 Peripheral edema


 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.

END

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

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.


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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