Adrenocortical Insufficiency (Addison's) and Cushing’s Syndrome — Study Notes

Addison's Disease (Adrenocortical Insufficiency)

• Classification and emergency context

  • Adrenocortical insufficiency may be acute or chronic; an emergency situation is characterised by an exaggeration of the symptoms.

  • Not evident until 90% of adrenal cortex is destroyed.

  • Primary adrenal insufficiency occurs when the adrenal cortex fails.

  • Secondary insufficiency results from absent or low levels of ACTH from the pituitary.

• Signs and symptoms (not evident until 90% destruction)

  • Primary features; insidious onset

  • Progressive weakness

  • Fatigue

  • Weight loss

  • Anorexia

  • Other signs and symptoms

  • Bronze-coloured skin hyperpigmentation if ACTH ↑

  • Orthostatic hypotension

  • Hyponatraemia and salt craving

  • Hyperkalaemia

  • Nausea and vomiting, diarrhoea

  • Irritability, depression

• Addisonian crisis (complications)

  • Acute adrenal insufficiency: insufficient or sudden, sharp decrease in hormones; life-threatening.

  • Various triggers.

  • Manifestations of glucocorticoid and mineralocorticoid deficiencies:

  • Postural hypotension, tachycardia

  • Dehydration

  • Na+ ↓, K+ ↑, glucose ↓

  • Fever, weakness, confusion

  • Severe vomiting, diarrhoea, pain

  • Shock → circulatory collapse

• Diagnosis of Addison’s Disease

  • ACTH stimulation test – If positive response, may indicate pituitary disease.

  • Plasma & urine cortisol levels.

  • Serum electrolyte levels, low sodium.

• Diagnostic studies

  • ↓ serum and urinary cortisol

  • ACTH levels

  • ACTH stimulation test

  • ↓ urinary cortisol and aldosterone

  • K+ ↑, Cl- ↓, Na+, glucose ↓

  • Anaemia

  • ↑ serum urea

  • ECG changes

  • CT scan, MRI

• Interprofessional care

  • Correct underlying cause

  • Hormone therapy

  • Hydrocortisone — Increase during periods of stress

  • Fludrocortisone

  • Addisonian crisis

  • Shock management

  • High-dose hydrocortisone replacement

  • 0.9% saline solution and 5% dextrose

• Nursing implementation — Ambulatory and home care

  • Dosing — Need to increase corticosteroids during times of stress

  • Signs and symptoms of corticosteroid deficiency and excess

  • Wear medical alert bracelet

  • If patient takes mineralocorticoid, check BP, increase salt intake, and know what to report to healthcare provider

  • Emergency kit

  • How to administer IM hydrocortisone

  • Written instructions

Cushing’s Syndrome

• Clinical manifestations (typical signs)

  • Centripetal/generalised obesity

  • Moon face with plethora

  • Purplish red striae

  • Hirsutism (women)

  • Menstrual disorders

  • Hypertension

  • Hypokalaemia

• Signs and symptoms (excess glucocorticoids)

  • Weight gain from accumulation of adipose tissue

  • Hyperglycaemia related to glucose intolerance and ↑ gluconeogenesis

  • Muscle wasting → weakness

  • Loss of bone matrix → osteoporosis and back pain

  • Loss of collagen → thin skin, easily bruised

  • Delay in wound healing

  • Irritability, anxiety, euphoria, psychosis

• Diagnostic studies

  • Plasma cortisol measurement

  • 24-hour urine collection for free cortisol

  • Low-dose dexamethasone suppression test

  • Urine 17-ketosteroids measurement

  • CT scan, MRI

  • Plasma ACTH levels

  • Hypokalaemia and alkalosis

• Treatment

  • Normalise hormone secretion; treatment depends on cause

  • Pituitary adenoma — surgical removal

  • Adrenal tumour or hyperplasia — adrenalectomy

  • Ectopic ACTH-secreting tumour — removal of tumour unless malignant

  • Medication therapy

  • Medical adrenalectomy — suppress synthesis and secretion of cortisol

  • Reduction in corticosteroid therapy

• Nursing care: Cushing’s syndrome

  • Priority care problems: Risk for infection; Imbalanced nutrition; Disturbed body image; Impaired skin integrity

• Nursing care — Acute intervention and Home care

  • Acute intervention

  • Assess and monitor: Vital signs; Daily weight; Glucose; Signs and symptoms of inflammation/infection; Signs and symptoms of thromboembolism; Perioperative care

  • Home care

  • Wear medical alert bracelet at all times

  • Avoid exposure to extremes of temperature, infection and stress

  • Lifetime replacement therapy