adrenal disorder: insufficiency Flashcards
adrenal insufficiency
-Primary adrenal insufficiency (Addison disease) is caused by a disorder of the adrenal glands.
-Secondary adrenal insufficiency is the result of decreased production of ACTH (adrenocorticotropic hormone) and
-Tertiary adrenal insufficiency is the result of decreased production of CRH (corticotropin-releasing hormone) by the hypothalamus
addison’s disease causes
-Develops as a result of bilateral adrenal cortex destruction
-Results in decreased production of adrenocortical hormones
-Cortisol - predominantly affected
-Aldosterone may also be affected
-Autoimmune destruction - most common cause
-Signs and symptoms - insidious in onset
-Can be difficult to recognize
addison’s disease: primary or chronic adrenocortical insufficiency- sx,dx,tx
-Usually progressive
-Hypofunctioning
-Symptoms :
-Hypotension
-Hyperpigmentation
-Can lead to adrenal crisis with cardiovascular collapse
-Diagnosis - clinical
-Elevated plasma ACTH with low plasma cortisol
-Treatment d/o cause:
-Hydrocortisone
-Other hormones ±
addison’s disease
-All age groups
-Diagnosis in the second to third decade of life
-Sex distribution
-Hereditary factors are important
-Familial glucocorticoid insufficiency may be associated with a recessive gene pattern
-Clinically apparent during metabolic stress or trauma
-Often present initially with other autoimmune diseases or mucocutaneous Candida infections
etiology
-Idiopathic atrophy of the adrenal cortex -> autoimmune process – 85%
-Destruction – Granuloma (e.g. TB)
-Congenital:
-Congenital adrenal hypoplasia
-Congenital adrenal hyperplasia
-Bilateral adrenal hemorrhage:
-Thrombosis b/o positive lupus anticoagulant or antiphospholipid antibodies (most common causes)
-Anticoagulation medication
-Tumor
-Amyloidosis
-Drugs that block corticosteroid synthesis:
-Antineoplastic meds
-Medications to treat HIV wasting, such as dronabinol
-Ketoconazole
-May coexist with DM or hypothyroidism in polyglandular deficiency syndrome
symptoms
-Weakness
-Chronic fatigue
-Dizziness
-Weight loss
-Anorexia
-Nausea
-Vomiting
-Diarrhea
-Intolerance of temperature extremes
-Abdominal pain
-Amenorrhea in females
-Craving for salty foods
-Depression
-Anxiety, and mental irritability
signs
-Orthostatic hypotension : Most frequent
-Hyperpigmentation:
-Non-exposed as well as exposed parts or where there is pressure (e.g. from a belt)
-Palmar creases, knuckles, elbows, buccal mucosa, pressure points, perianal mucosa, nail beds and nipple areolas; new scars
-Comes from the excessive ACTH secretion
-Occurs mainly on extensor surfaces
-Vitiligo- In cases with autoimmune adrenal insufficiency
-Loss of axillary hair in females - Pituitary tumor and loss of Gonadotropin function
addisonian crisis
-Some presentations – are with Addisonian crisis
-Symptoms of Addisonian crisis include:
-Fever of unknown etiology
-Orthostatic hypotension
-Craving for salt
-Sharp, sudden pain in the abdomen, legs, or lower back
-Severe vomiting and diarrhea
-Hypoglycemia (central adrenal insufficiency b/o pituitary pathology)
-Dehydration
-Loss of consciousness
approach to testing
-After clinical diagnosis of chronic adrenal insufficiency
-Use stepwise endocrine testing in all patients.
-1. Morning cortisol
-2. Morning ACTH
-3. ACTH stimulation test
-1ry adrenal insufficiency: Screen for hypoaldosteronism and hypoandrogenism.
-2ry and 3ry: Differentiating between the two is often not required, as it does not influence management.
-All patients: Investigate for an underlying cause.
tests
-Get baseline cortisol level
-Cortisol level is low:
-< 3mcg/dL @ 8am is diagnostic
-Especially if ACTH > 200 pg/ml
-Or <8mcg/dL at any other time
-Addison’s disease is unlikely with an 8 a.m. cortisol level of >20mcg/dL (>0.55mcmol/L)
-Serum Dehydroepiandrosterone sulfate (DHEA-S) levels are under 1000 ng/mL
-Elevated plasma renin activity
-ACTH - helps distinguish 1ry from 2ry & 3ry adrenal insufficiency -> Useful in borderline cases
diagnosis of addison’s disease
-ACTH stimulation test
-The gold-standard test for 1ry insufficiency
-An accurate diagnosis can be made with this test
-Those with primary adrenal insufficiency have little or no change in cortisol levels after the injection
-Adrenal insufficiency caused by pituitary dysfunction have levels that increase following ACTH injection
clinical suspicion chart
additional tests
-Hyponatremia and hypochloremia
-Hyperkalemia
-↑ blood urea nitrogen (BUN: creatinine ratio)
-Hypercalcemia during adrenal crisis
-Hypocalcemia with associated polyglandular failure and hypoparathyroidism
-Abdominal X-ray
-Abdominal CT:
-Adrenal calcifications (2ry to fungal or TB infection)
-Malignancy
-Chest X-ray
-K can be reduced if there is diarrhea
acute treatment
-Blood pressure, serum glucose, and volume status
-Supplementation of adequate glucocorticoids
-If hypotensive after fluid replacement/has a history of corticosteroid use -> Suspect adrenal insufficiency
-Corticosteroid therapy STAT – dexamethasone -> Until ACTH testing is complete
-Dose needs to be doubled when patient has an episode of minor fever, infection, minor trauma, or minor physical (not emotional) stress
-IV stress dose corticosteroids - needed for surgery
chronic management
-Daily supplementation of glucocorticoids - prednisone
-Aim is to maintain:
-Normal blood pressure, blood glucose, and volume
-Instill a sense of well-being in the patient
-Double their usual dose of glucocorticoid supplementation -> In the summer
-Advise patients on medication for sick days -> E.g. febrile illness or emesis
-Fludrocortisone – for mineralocorticoid replacement
-Monitor regularly to ensure adequate treatment -> E.g. sodium, potassium, glucose, BP, and ACTH levels
-Vigilance in maintaining therapeutic regimen
