10.2 Adrenal Disorders Flashcards
What hormones are produced in the medulla of the adrenal gland?
Catecholamines
What is the clinical presentation of adrenal hormone deficiency?
Depends on the hormone that is deficient:
• Cortisol = weakness, tiredness, weight loss, hypoglycaemia
• Mineralocorticoid = dizziness, low Na, high K
• Androgen = low libido and loss of body hair in women
What are the 3 different variations in adrenal cortex disease?
- Adrenal hormone deficiency
- Adrenal hormone excess
- ACTH excess form pituitary
What is the clinical presentation of adrenal hormone excess?
Depends of the hormone affected:
• Cortisol = weight gain and cushingoid features
• Mineralocorticoid = high BP and low K
• Androgen = increased male characteristics in women
What is the clinical presentation of ACTH excess from pituitary?
Skin pigmentation due to melanocytes stimulation.
Pigmentation seen in Addisons and ACTH-driven Cushing’s.
What is the presentation of adrenal medulla disease?
Excessive catecholamine secretion leads to: • Acute episodes • Sweating • Anxiety • Palpitations • High or low BP • Collapse Sudden Death
What would biochemical assessments of the adrenal cortex in a patient with adrenal hormone deficiency present?
- Electrolytes = low Na, high K in aldosterone deficiency (low cortisol secretion from Zona glomerulosa)
- 0900 basal cortisol = low – when it should be high
- Stimulation test = inject synthetic ACTH (synacthen)
How would biochemical assessments of the adrenal cortex in a patient with adrenal hormone excess present?
- Electrolytes = high BP, low K
- Midnight cortisol high = should be low
- 24h urine cortisol = high
- Suppression test = failure to suppress
- Androgens and derivatives = high
What biochemical assessments can be conducted to assess the adrenal medulla?
24hour urine catecholamines
24hour urine metanephrines
Plasma metanephrines
Certain foods such as coffee, coke, bananas, chocolate and vanilla should be avoided before collection.
What radiological assessments are conducted with suspected adrenal disease?
CT scan
MRI scan
MIBG scan
PET scan
What is Addison’s disease?
Primary adrenal failure
Commonly caused by TB of the adrenal glands and auto-immune disease.
What are the primary symptoms of Addison’s disease?
Mucous membrane pigmentation Skin pigmentation Freckling Vitiligo Hypotension
What are the clinical signs and symptoms of Addison’s disease?
Symptoms = fatigue, weakness, anorexia, weight loss, nausea, abdominal pain, dizziness, pigmentation
Signs = underweight, signs of weight loss, general malaise, other auto-immune disease, vitiligo, thyroid disorder, postural hypotension, pigmentation.
What are the cause of primary adrenal failure/ Addison’s disease?
Auto-immune disease (isolated or polyglandular)
Infection ( TB, fungal, AIDS)
Infiltration (amyloid, haemochromatosis)
Malignancy (lung, breast, kidney)
Genetic (CAH, adreno-leukodystrophy)
Vascular (haemorrhage or infarction)
Iatrogenic (adrenalectomy, drugs)
What is an adrenal crisis?
Uncontrolled Addison’s disease, leading to dangerously low cortisol levels?
What are the clinical features of an adrenal crisis?
- Collapse
- Hypotension
- Dehydration
- Pigmentation
- Coma
What is the treatment of adrenal crisis?
- Rapid rehydration with fluids
- Intravenous hydrocortisone
- Correction of hypoglycaemia
- Search for precipitating cause
What is the treatment of Addison’s disease?
- lifelong replacement of glucocorticoid (hydrocortisone and prednisolone) and mineralcorticoid (fludrocortisone)
- education to prevent crises
What are the causes of ACTH deficiency?
Hypopituitarism
What are the symptoms of hypopituitarism?
Similar to primary adrenal failure but no pigmentation as ACTH levels are not raised. No hyperkalaemia as no mineralcorticoid (aldosterone) deficiency. Hyponatraemia due to effect of cortisol on free water excretion.
What is steroid-induced hypoadrenalism/
When ACTH secretion from the posterior pituitary gland is suppressed with long-term steroids. Abrupt withdrawal can cause hypo-adrenal crisis.
What is the clinical syndrome of glucocorticoid excess?
- Round pink face with round abdomen
- Skinny and weak arms and legs
- Thin skin and easy bruising
- Red stretch marks (‘striae’) on abdomen
- High blood pressure and diabetes
- Osteoporosis (thin bones)
What causes Cushing’s syndrome?
Pituitary tumour/Adrenal tumour secreting glucocorticoids
Ectopic ACTH
What hormones are affected in adrenal tumour?
Glucocorticoid excess resulting in ACTH secretion being suppressed.
What are the symptoms of an adrenal tumour?
Cushing’s syndrome symptoms Androgenic symptoms Hirsutism Acne Greasy skin Virilising features in large tumours Androgenic slope is, Deep voice, Clitoromegaly
What is the treatment for an adrenal tumour?
Laproscopic adrenalectomy
Large tumours require open surgery
What is primary hyperaldosteronism?
Excess production of aldosterone from adrenal gland
What causes primary hyperaldosteronism?
aldosterone-secreting adrenal adenoma (Conn’s syndrome)
bilateral adrenal hyperplasia
(no discrete adenoma)
What is the commonest form of endocrine hypertension?
Primary hyperaldosteronism
What is the clinical presentation of primary hyperaldosteronism?
Hypertension (especially at a young age)
Hypokalaemia
Elevated aldosterone independent on renin-angiotensin system
How is primary hyperaldosteronism treated?
Conns adenoma amendable to surgery
Bilateral hyperplasia treated with aldosterone antagonists
What is congenital adrenal hyperplasia?
Autosomal recessive inherited disorder. Adrenal crisis and ambiguous genitalia. Caused by a block in adrenal cortex pathway. Presentation depends on enzyme defect. Lack of enzyme leads to low cortisol and aldosterone with high male hormones (androgens)
What is the presentation of congenital adrenal hyperplasia?
Hypotension Hyponatraemia Hyperkalaemia Hypoglycaemia
Virilisation
What is the treatment for congenital adrenal hyperplasia?
Treat adrenal crisis Determine sex of baby Long term GC and MC Corrective surgery
What is phaeochromocytoma?
Tumour of the adrenal medulla
What is paraganglioma?
Extra-adrenal tumour (chromaffin tissue origin)
What sort of adrenal tumours are likely to be due to genetic inheritance?
Extra-adrenal tumours
Malignant
Bilateral
What are the symptoms of phaeochromocytoma and paraganglioma?
- Acute episodes
- Sweating
- Panic attacks
- Palpitations
- High or low BP
- Collapse
What are the clinical presentation of an acute crisis of phaeochromocytoma and paraganglioma?
- Hypertensive crisis
- Encephalopathy
- Hyperglycaemia
- Cardiac arrhythmias
- Sudden Death
What investigations can be conducted on a patient with suspected phaeochromocytoma / paraganglioma?
24h urine metanephrines 2-3 x collections needed Plasma metanephrines Radiological investigations (MRI/PET/MIBG)
What is the management for phaeochromocytoma?
α-blockade (phenoxybenzamine)
β-blockade (bisoprolol)
surgical excision