17. Hypocorticism (Adrenal insufficiency) – Addison disease

Question 1

Adrenal insuffeincy are classified according to their etiology such as ?

Answer 1

Disorder of the adrenal cortex - primary

Disorder of anterior pituitary gland - secondary

Disorder of hypothalamus - tertiary

Question 2

What is the etiology of primary adrenal insufficiency ADDISON DISEASE?

Answer 2

Autoimmune adrenalitis
- characterised by destruction of the adrenal cortex

Hemorrhagic infraction
Associated with meningococcemia ( Waterhouse fredrichsen syndrome )
Pseudomonas aeurginosa

Infectious adrenalitis
Tuberculosis , hiv

Drug / adrenal enzyme inhibitors
Etomidate

infiltration of adrenal glands
metastasis
amyloidosis

Genetic disorder
Adrenoleukodystrophy
Congenital adrenal hypoplasia

Question 3

What are the etiology of secondary and tertiary adrenal insufficiency?

Answer 3

Pituitary or hypothalamic :
tumors 
Or surgery 
Or irradiation  
Trauma
Infections
Drug induced - glucocorticoid therapy

Question 4

What are the clinical manifestation of adrenal insufficiency ?

Answer 4

deficiency of cortisol , aldosterone , and androgens = all three

causing hypogonadism : decreased libido and impaired spermatogenesis

hypocortisolism :
hypoglycemia
Skin hyperpigmentation - in only primary
Low blood pressure -pronounced in primary
fatigue , vomiting , diarrhea , weight loss
orthostatic hypotension

hypoaldosternosim :
Salt craving - in only primary 
Hyponatrameia 
Hyperkalemia - in primary only 
Myalgia and joint pain

===========

secondary - decrease in ACTH
= only hypogonadism and hypocortisolilsm
mineral corticoids not affected

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tertiary decrease in CRH - leading to deficiency in ACTH = same manifestations as secondary

Question 5

How do we diagnose adrenal insufficiency

Answer 5

morning serum cortisol measured = they are low

Plasma acth measured

If high = primary adrenal
if low - secondary

if primary screen for :

21 hydroxylase antibodies = positive = autoimmune Addison disease

HYPONATREMIA
HYPERKALEMIA
HYPOGLYCEMIA
metabolic acidosis

confirmatory test

syncathen test
ACTH ANALGOUE

there should be a rise of cortisol in 30 minutes if not :

CT adrenals - infective or infiltrative hemorrhages

Serum of very long chain fatty acids = adrenoleukodystrophy

————-
If ACTH is low or normal

Secondary adrenal insufficiency

MRI of pituatory

Also test for other Anterior pituitary hormones

Question 6

What is the treatment for primary adrenal insufficiency?

Answer 6

Monitor salt craving , postural hypotension

Glucocorticoid regime
Hydrocortisone
Cortisone acetate
Two or three divided oral doses per day , highest dose in the morning next early afternoon

Monitor BMI , postural BP

Mineral corticoid
To maintain sodium fluid balance
Fludrocortisone

decreased libido by DHEA

Question 7

What is causes adrenal crisis ?

Answer 7

Gastroenteritis and fever , stress

Trauma and surgery

sudden discontinuation of glucocorticoid dose

Question 8

What is the symptoms of adrenal crisis ?

Answer 8

Vomiting
Abdominal pain 
Hypotension 
Hypovolemic shock 
Hyperkalemia 
Hyponatremua 
Hypoglycaemia 
Muscle cramps 
Pre renal failure

Question 9

What is the emergency treatment for adrenal crisis

Answer 9

Hydration
1L saline first hour
500ml next hour

Parent real administration of 100mg hydrocortisone followed by 200mg per 24hr

dextrose given for hypoglycemia

Monitor the vital signs and serum electrolytes

Question 10

If hydrocortisone is not available what is an alternative ?

Answer 10

Prednisone

Question 11

How do we prevent adrenal crisis ?

Answer 11

Patient education - tell patient to increase dose during fever or infection
And self parenetral administration of emergency glucocorticoids

All patients equipped with steroid emergency card and medical alert identification card