Adrenal Disordes Flashcards
What are the symptoms of adrenal hormone deficiency
Cortisol - weakness, tiredness, weight loss, hypoglycaemia
Mineralocorticoid - dissiness, low Na, high K
Androgen - low libido and loss of body hair in women
What are the symptoms of adrenal hormone excess
Cortisol - weight gain and cushings I’d fetures
Mineralocorticoid- high be and low k
Androgen - increased male characteristics in woman
What is seen in acth excess from pituitary
• Skin pigmentation pigmentation seen in Addison’s and ACTH driven cushings melanocyte stimulation
What is the presentation of adrenal medulla disease
- Acute episodes
- Sweating
- Anxiety
- Palpitations
- High or low BP
- Collapse
Sudden death
What tests should be performed for suspected adrenal hormone deficiency
Electrolytes - low Na, High K in aldosterone def
9:00 basal cortisol - it should be high here
Stimulation test - inject synthetic acth (synacthen)
What tests should be reformedd for suspected adrenal hormone excess
- Electrolytes - high BP, low K
- Midnight cortisol - high -when it should be low
- 24h urine cortisol - high
- Suppression test - failure to suppress
- Androgens and derivatives - high
Describe the biochemical assessment of the adrenal medulla
24h urine catecholamines adrenaline, noradrenaline, dopamine, 3-Methoxy-Tyramine
24h urine metanephrines metabolites of adrenaline and noradrenaline metadrenaline, normetadrenaline
plasma metanephrines more sensitive than 24h urine
avoid certain foods before collection coffee, coke, bananas, chocolate, vanilla
What results from adrenal insufficiency
Primary adrenal failure destruction of adrenal cortex Addison’s ACTH deficiency from hypopituitarism Secondary adrenal failure Steroid-induced hypoadrenalism ACTH suppression
What are the symptoms of Addison’s disease
- Fatigue • Weakness • Anorexia • Weight loss • Nausea • Abdominal pain
- Dizziness • Pigmentation
Signs
• Underweight • Signs of weight loss • General malaise • Other auto-immune disease • Vitiligo • Thyroid
• Postural hypotension • Pigmentation
What are the causes of primary adrenal failure
Auto-immune 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 ae teh clinical features and treatment of adrenal crisis
- Collapse • Hypotension • Dehydration • Pigmentation • Coma
- Rapid rehydration with fluids • Intravenous hydrocortisone • Correction of hypoglycaemia • Search for precipitating cause
What is the maintenance treatment of assistants disease?
Lifelong replacement glucocorticoid - hydrocortisone, prednisolone what is the maintenance treatment of Addison’s disease?mineralocorticoid - fludrocortisone
Education to prevent crises double dose glucocorticoid at times of illness emergency HC injection if vomiting steroid card and bracelet
What is acth deficiency?
ACTH deficiency
Occurs in any cause of hypopituitarism
Similar symptoms to primary adrenal failure
No pigmentation as ACTH not raised
No hyperkalaemia as no mineralocorticoid deficiency Hyponatraemia due to effect of cortisol on free water excretion
What is steroid induced hypoadrenalism
Steroid-induced hypoadrenalism
ACTH suppressed with long-term steroids
Abrupt withdrawal can cause hypo-adrenal crisis
Important to consider in any unwell patient on steroids
What are the symptoms of Cushing’s syndrome
- 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 are the causes of Cushing’s syndrome
Pituitary tumour
Adrenal tumour
Ectopic ACTH
Describe the presentation of adrenal Cushing’s syndrome
ACTH is suppressed ‘ACTH-independent’ cushing’s unlike pituitary and ectopic ACTH ‘ACTH-dependent’
androgenic symptoms may be present hirsutism, acne, greasy skin
virilising features in large tumours androgenic alopecia, deep voice, clitoromegaly
What is primary hyperaldosteronism
excess production of aldosterone from adrenal gland aldosterone-secreting adrenal adenoma - Conn’s syndrome
bilateral adrenal hyperplasia - no discrete adenoma
What is primary hyperaldosteroism
Commonest form of endocrine hypertension Hypertension at young age or refractory to treatment
Combination of hypertension and hypokalaemia (low potassium)
Elevated aldosterone independent of renin-angiotensin system
Deserves ethe diagnosis of primary hyperaldosteronism
clinical suspicion – hypertension and hypokalaemia confirmation of high aldosterone and suppressed renin scan shows adrenal adenoma or bilateral hyperplasia
if lesion too small may need sampling or functional scan
Conn’s adenoma amenable to surgery bilateral hyperplasia treated with aldosterone antagonists spironolactone / eplerinone
What is CAH?
Congenital Adrenal Hyperplasia (CAH)
Rare but important inherited disorder
Autosomal recessive
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 High male hormone (androgens)
Describe the presentation and treatment of CAH
Presentation
Hypotension Hyponatraemia Hyperkalaemia Hypoglycaemia Virilisation
Treatment Treat adrenal crisis Determine sex of baby Long term GC and MC Corrective surgery
What are phaeochromocytoma and paraganglioma
Phaeochromocytoma Tumour of adrenal medulla Paraganglioma Extra-adrenal tumour chromaffin tissue origin 90% from adrenal medulla 10% extra-adrenal origin Genetic inheritance common More likely if: • Extra-adrenal • Malignant • Bilateral
What are the symptoms of phaeochromocytoma and paraganglioma
Symptoms • Acute episodes • Sweating • Panic attacks • Palpitations • High or low BP • Collapse
Acute Crisis • Hypertensive crisis • Encephalopathy • Hyperglycaemia • Cardiac arrhythmias • Sudden Death
What are investigations for phaeochromocytoma and paraganglioma
Investigations
24h urine metanephrines
2-3 x collections needed
Plasma metanephrines
Neuro-endocrine marker Chromogranin A
Describe the management of phaeochromocytoma and paraganglioma
Management α-blockade β-blockade surgical excision
Important to α-block before β-blockade Unopposed α-stimulation can cause a hypertensive crisis
phenoxybenzamine bisoprolol
Peri-operative management Specialist anaesthetic team Risk of crisis during operation Maximal vasodilation and filling with IV fluid
What are the familial causes of phaeochromocytoma and paraganglioma
Familial causes
Multiple Endocrine Neoplasia type 2
Von Hippel-Lindau
Neurofibromatosis type 1
Succinate dehydrogenase subunit B and D
MEN-2- medullary thyroid cancer, hyperparathyroidism
VHL - renal cancer, cerebellar hamangioblastoma, angiomas
NF1 - café au-lait patches, neurofibromas
SDHB/Dphaeos, paragangliomas in neck, thorax, abdomen
