adrenal gland function and disorders

2. Cushing syndrome
• Cushing's syndrome is a disorder caused by prolonged exposure of the body's tissues
to high levels of corticosteroids (glucocorticoids).
• (hyperfunction of adrenal gland)
• cushing sundrome is a characteristic group of manifestations caused by adrenocortical
hypofunction (excessive circulating free cortisol).
• It is group manifestation related to excess glucocorticoid due to variety of
causes.
• It is uncommon in children, but in young children may be due to adrenal tumor.
• Cushing's syndrome is associated with serious morbidity and increased mortality.

3. Epidemology
• Epidemioly:
• The incidence of Cushing's syndrome is estimated to be equal to 1–3
cases per million inhabitants per year, whereas its prevalence is close to
40 cases per million inhabitants.(Jun 18, 2012)
• An estimated 10 to 15 of every million people are affected a
year and only about ten percent of these new cases occur in
children.

4. Etiology
 Overproduction of corticosteroids . In approximately 70% of patients,
Cushing’s syndrome results from excessive production of corticotropin and
consequent hyperplasia of the adrenal cortex.
 Taking/administration excessive amounts of corticosteroid drugs. The most
common cause of Cushing's syndrome( Exogenous Cushing's syndrome) .The
majority of cases of Cushing's syndrome are caused by administration of
corticosteroids (exogenous) for the treatment of long-term diseases such as
asthma, arthritis, and lupus.

5.  Tumor. In the remaining 30% of the patients, Cushing’s syndrome results from a cortisol-
secreting adrenal tumor, which is usually benign.
 Familial/ Inherted cushing syndrom: tendency to develop tumors of one or more
endocrine gland (Extra pitutary)
 Extrapituitary neoplasm

7. Clinical features
1. Weight gain, especially in the face, neck region, upper back, and torso (buffalo hump)
2. Changes in skin, including purple stretch marks, easy bruising, facial plethora(red cheeks)
and other signs of skin thinning
3. muscle weakness causing difficulty in climbing stairs, getting out of a low chair, and raising
arms, backache
4. Psychological problems such as depression, cognitive dysfunction, and emotional lability
5. New onset or worsening of high blood pressure and diabetes mellitus
6. Polyuria or polydipsia (increased thirst) from diabetes mellitus or diabetes insipidus in 90%.
7. Reduced bone mass and fractures caused by weakened bones (steroid-induced osteoporosis),
short stature
8. Rounded or moon face

8. • Impaired wound healing or predisposition to infections because of impaired
immune function
• Irregular menses, amenorrhea, and hirsutism (overabundance of hair) in women
• Decreased libido, infertility, and impotence in men
• Headaches
• polyuria (increased frequency of urination) and nocturia (increased voiding of urine
at night)
• visual problems
• galactorrhea (breasts produce milk in a woman who is not pregnant or
breastfeeding an infant).
• Hypokalemia due to excess potasium excretion
• Thin skin over forearm and leg.
• renal calculi may develop
• Irritabiity and anxiety and depression are common

9. • Pathophysiogy:

10. Diagnostic evaluation
• Review of medical history
• Physical examination
• Laboratory investigations/tests
 Lab tests-- Increased fasting blood sugar, serum electrolyte for hypokelemia,
hypernatremia.
 Overnight 1-mg dexamethasone suppression test: For this test, 1mg of
dexamethasone (exogenous corticosteroid) is administered at 11 pm, with
measurement of serum cortisol the next morning at 8 am. In healthy
individuals, the serum cortisol should be less than 2-3 mcg/dL. Cushing's
syndrome may be excluded with a cortisol level less than 1.8 mcg/dL

11.  The 48-hour low-dose dexamethasone suppression test: the overnight 1-
mg dexamethasone suppression test may be inconclusive. In such persons,
48-hour low-dose dexamethasone suppression test is performed. In this test,
0.5mg dexamethasone is administered every six hours for eight doses and
serum cortisol levels are measured. (complete absence of adrenocortical
supression suggest tumor)
 Corticotropin-releasing hormone (CRH) stimulation test: This test can
detect mild corticosteroid excess. It combines the 48-hour low-dose
dexamethasone suppression test with CRH stimulation. CRH is administered
intravenously two hours after the eighth dose of 0.5mg dexamethasone.
Corticosteroid levels are measured 15 minutes after CRH administration. A
level of greater than 1.4 mg/dL is suggestive of Cushing's syndrome.

12. • Midnight plasma cortisol measurement: cortisol concentration in the blood(normally suppressed at night < 50nmol/l)
• Brain CT scan: In persons with pituitary adenoma, an enlarged pituitary gland can be seen on brain CT scan.
• Abdominal CT scan: The presence of an adrenal mass larger than 4-6 cm in the CT scan raises the possibility that the mass is an
adrenal carcinoma.
• Chest and abdominal CT scans: They should be performed in patients with suspected ACTH production from tumors present
elsewhere in the body (for example, lung).
• Magnetic resonance imaging (MRI) of the brain: If a pituitary source of excess ACTH is suspected, MRI of the brain is performed.
• X- ray skull for evidence of bone oeteoporosis, X - ray of adrenal or pituitary glands to locate any tumors
• Ultrasound of adrenal glands.
• 24 hours urinalysis for 17-hydroxycorticoids and 17 steroids
• Measurement of 24-hour cortisol levels (24 hr urinalysis): The amount of cortisol excreted in the urine during a 24-hour time period
is measured. Values higher/elevated than three to four times the upper limit of normal are suggestive of Cushing's syndrome(> 50-100
micro gm/day in adult suggest cushing’s syndrome).

13. Management
• Management of Cushing's syndrome depends on the primary cause of the
syndrome.
• The treatment may include surgical removal of the tumor/Adrenalectomy
if tumor in adrenal gland
• Radiation,
• The use of corticosteroid-inhibiting drugs.
• Cushing's syndrome is primarily treated surgically. When surgery is
unsuccessful or contraindicated, medical therapy is needed to treat
hypercortisolism.

14. Surgical Management
• Removal of the adrenal or pituitary gland offers a greater success rate for the
recovery of the patient.
• Transsphenoidal hypophysectomy. Surgical removal of the tumor by
transsphenoidal hypophysectomy is the treatment of choice if Cushing’s syndrome
is caused by pituitary tumors and has an 80% success rate.
• Adrenalectomy. Adrenalectomy is the treatment of choice in patients with
primary adrenal hypertrophy.
• When surgery is not successful or cannot be performed, treatment with medication
may be attempted, however, medication failures are common.
• Radiation therapy may be necessary if surgical outcomes are not
optimal or if the patient is not a good surgical candidate.
• post oprative replacement of cortical hormone is necessary

15. • Risk factors associated with TSS for removal of a pituitary
adenoma include temporary or permanent dysfunction of the
pituitary gland
• It is important for the child and adolescent to be monitored on a
routine basis by a pediatric endocrinologist to screen for any
problem with pituitary gland function, including hypothyroidism,
adrenal insufficiency, growth hormone insufficiency, pubertal
delay.
• After TSS for CS, daily cortisol replacement is necessary, typically
for a period of six to eighteen months, until the hypothalamic-
pituitary-adrenal axis (HPA) recovers,

16. • Children and adolescents who undergo bilateral adrenalectomy
for treatment of CS requires daily hormone replacement
(hydrocortisone and fludrocortisone) for the rest of their life.
• In addition, the hydrocortisone dose will require adjustment
during illness episodes and significant physical stress (e.g.
surgery, trauma).
• Children with an adrenal tumor that requires the removal of a
single adrenal need to take daily hydrocortisone replacement
until the HPA axis recovers

17. Medical management
• Adrenal enzyme inhibitors. Metyrapone, aminoglutethimide, mitotane, and
ketoconazole may be used to reduce hyperadrenalism if the syndrome is caused
by ectopic ACTH secretion by a tumor that cannot be eradicated.
• Cortisol therapy. Cortisol therapy is essential during and after surgery, to
help the patient tolerate the physiologic stress imposed by the removal of the
pituitary or adrenals.
• Drugs that inhibit the synthesis of corticosteroid, such as mitotane,
ketoconazole, metyrapone, aminoglutethimide, trilostane, and etomidate,
have been used to treat adrenalectomy.
• These medications are used rarely and often are toxic at the doses required to
reduce corticosteroid secretion.

18. Nursing management
Nursing Assessment
• Assessment focus on the effects on the body of high concentrations of
adrenal cortex to respond to changes in cortisol and aldosterone levels.
• Health history. The history includes information about the patient’s level of
activity and ability to carry out routine and self-care activities.
• Physical exam. The skin is observed and assessed for trauma, infection,
breakdown, bruising, and edema.
• Mental function. The nurse assesses the patient’s mental function including
mood, responses to questions, awareness of environment, and level of
depression.

19. • Monitor vital signs for irregularities, bradycardia and
hypertension
• Monitor for early signs of infection
• Prevention of infection by good hand hygiene practice and
avoiding the exposure of the child to infection
• Reinforce that steroid medicine should not be stopped abruptly
as it can cause adrenal crisis. The symptoms will decrease
gradually after discontinuation of medicine

20. • Many children and adolescents recovering from CS
experience changes in cognitive performance including
difficulty concentrating and problems with memory that
may affect their academic performance for an
indeterminate period that can be stressful for both the child
and the parents.
• It is important to provide appropriate educational and
psychological resources for the child or adolescent during
this period

21. • Help maintain skin integrity with good hygiene and care,
frequently change position, assess for signs of skin
breakdown
• Increased protein and calcium- to prevent loss and
thinning of bones
• low sodium diet with potassium supplements
• Promote adequate rest to prevent fatigue
• Less caffeine- stimulate pituitary gland

22. Thank You!!!!!

23. Addisons Disease

24. • Addison's disease is a rare disorder which is characterized by
adrenocortical insufficiency that causes low levels of aldosterone and
cortisol.
• These hormones are made by child's adrenal glands.
• Cortisol helps child's body handle stress.
• Aldosterone helps child's body balance salt, potassium, fluid. Addison
disease may appear soon after child is born, or when child reaches
adolescence.

25. Causes
• Autoimmune disease like type I diabetes mellitus and Graves disease
• Prolonged administration of glucocorticoids(eg prednisone)
• Infections in the body like TB,HIV, and fungal infection
• Cancer and abnormal growth (tumors)
• Use of anti-coagulating agents
• Pituitary tumors
• Medications
• Genetic defects in pituitary gland
• Traumatic brain injury

26. Pathophysiology
• The pathophysiology of adrenal insufficiency depends on the etiology.
• With most forms of autoimmune (primary) adrenal insufficiency, the patient
has antibodies that attack various enzymes in the adrenal cortex (though
cell-mediated mechanisms also contribute). Various genetic factors have
been identified that play a role in the development of primary adrenal
insufficiency or autoimmune adrenalitis.
• Secondary adrenal insufficiency refers to decreased
adrenocorticotropic hormone (ACTH) stimulation of the adrenal cortex and
therefore does not affect aldosterone levels. Traumatic brain injury (TBI) and
panhypopituitarism are common causes.

27. • Tertiary adrenal insufficiency refers to decreased hypothalamic stimulation
of the pituitary to secrete ACTH. Exogenous steroid administration is the
most common cause of tertiary adrenal insufficiency. Surgery to correct
Cushing disease can also lead to tertiary adrenal insufficiency.
• The other forms of adrenal insufficiency usually relate to destruction by
infectious agents or infiltration by metastatic malignant cells. Hemorrhagic
infarction occurs due to sepsis with certain organisms
(Neisseria species, tuberculosis, fungal infections, Streptococcus species,
Staphylococcus species) or due to adrenal vein thrombosis. Death
associated with adrenal insufficiency is usually of septic shock,
hypotension, or cardiac arrhythmias

28. Sign and symptoms
Signs and symptoms may be present at birth or show up slowly over months or years:
• Dizziness, weakness, and tiredness
• Decreased appetite or weight loss
• Nausea, vomiting, or diarrhea
• Muscle, joint, stomach, or back pain
• A craving for salty foods
• An increased sensitivity to cold temperatures or sweating more than usual
• Darkening of the skin on child's lips, palms, nipples, genital area, and inside child's mouth
• Depression or trouble thinking clearly
• Low blood pressure
• Adolescent female children may stop having a monthly period
• High potassium (hyperkalemia) and low sodium (hyponatremia)

29. Diagnosis
• ACTH stimulation test: in this test synthetic derivative of ACTH 1 mg is given
IM and then blood sample are taken 1,4,8, and 24 hours of injection. Normally
the plasma cortisol level should reach 1000n mol/L by 4 hours. But in primary
Addisons disease, the cortisol level is reduced at all stages.
• Blood and urine tests may be collected to measure child's hormone levels. child's
blood may be collected after he or she is injected with a hormone. The hormone
causes the adrenal glands to make cortisol and aldosterone. Blood tests also check
child's blood sugar levels, electrolyte levels, blood cell levels, and kidney function.
child's pituitary and thyroid gland hormone levels may also be measured.
• An x-ray, CT, or MRI are done to check the size of child's adrenal gland. These tests
also check for problems that may cause Addison disease. child may be given
contrast liquid to help the pictures show up better.
• A biopsy takes a sample of child's adrenal gland. The sample can be checked for
signs of Addison disease.

30. Treatment
• Steroid medicine is given to increase child's cortisol level. This
medicine helps child's body handle stress and prevent an adrenal
crisis. Steroid medicine will also help decrease child's symptoms such
as weakness and fatigue. Always carry extra steroid medicine for
child. Do not skip a dose of child's medicine. Do not stop giving child
this medicine without talking to healthcare provider. child may have
an adrenal crisis if he or she misses a dose of medicine or stops taking
medicine.

31. • Aldosterone supplements may be given to help child's body balance
salt and fluid. This can help prevent dehydration and low sodium
(salt) levels.
• Sodium supplements help increase the amount of salt in child's
blood. child may need to take salt supplements every day. Instead, he
or she may need to take salt supplements before exercise, in hot
weather, or when he or she has diarrhea or vomiting.

32. Complication
Addisonian crisis
Untreated Addison's disease may develop an addisonian crisis as a result of physical stress,
such as an injury, infection or illness. Normally, the adrenal glands produce two to three
times the usual amount of cortisol in response to physical stress. With adrenal insufficiency,
the inability to increase cortisol production with stress can lead to an addisonian crisis.
Sometimes the signs and symptoms of Addison's disease may appear suddenly. Acute
adrenal failure (addisonian crisis) can lead to life-threatening shock. Where patient
experience the following signs and symptoms:
• Severe weakness
• Confusion
• Pain in your lower back or legs
• Severe abdominal pain, vomiting and diarrhea, leading to dehydration
• Reduced consciousness or delirium
• Low blood pressure
• High potassium (hyperkalemia) and low sodium (hyponatremia)

33. Prevention
Addison's disease can't be prevented, but there are steps can be taken to
avoid an addisonian crisis:
• Consult doctor if symptoms arises like feel tired, weak, or losing weight. Ask
about having an adrenal shortage.
• Child may need to adjust or increase dose of corticosteroids when feel sick.

34. • Thank you ……….