Adrenal Disorders Flashcards
What symptoms do adrenal hormones deficiency cause?
- Cortisol: weakness, tiredness, weightloss, hypoglycaemia
- Mineralcorticoids: dizziness, low Na, high K
- Anderogen: Low libido and loss of body hair in women
What are the symptoms of adrenal hormone excess?
Cortisol: weight gain and cushingoid features
Mineralocorticoid: High BP and low K
Androgen: Increased male characteristics in women.
ACTH excess fron pituitary:
Skin pigmentation: Melanocyte stimulation - pigmentation seen in Addison’s and ACTH driven cushings
How do patients with adrenal medulla disease present?
- Acute episodes
- Sweating
- Anxiety
- Palpitations
- High or low BP
- Collapse
SUDDEN DEATH
Biochemical assessment of adreal cortex (cortisol deficiency)
Suspected adrenal hormone deficiency:
Electrolytes - low Na, high K in aldosterone deficiency. (In ACTH deficiency only Na is low but K is normal)
9:00 basal cortisol - low when should be high
stimulation test - inject synthetic ACTH (syncthen)
Biochemical assessment of adrenal cortext (suspected excess)
Suspected adrenal hormone excess:
- Electrolytes - hihg BP, low K
- Midnight cortisol -High when should be low
- 24hr urine cortisol - High
- Suppression test - Failure to suppress
- Androgens and derivatives - High
Biochemical assessment of adrenal medulla
24hr urine catecholamines
24hr urine metanepherines
Plasma metanephrines (more sensitve than 24hr urine)
avoid certain foods before collection: coffee, coke, bananas, chocolate, vanilla.
Addisons disease
- Symptoms
- Fatigue,
- Weakness
- Anorexia
- Weight loss
- ausea
- Abdomina pain
- Dizziness
- Pigmentation
- Signs
- Underweight
- Signs of weight loss
- General malaise
- Other auto-immune disease
- Vitiligo
- Thyroid
- Postural hypotension
- Pigmentation
What are some causes of primary adrenal failure?
- Autoimmune
- Infection
- Infiltration
- Malignancy
- Genetic
- Vscular
- Iatrogenic (relating to medical examination or treatment)
What are the clinical features and treatment for addisonian crisis?
Clinical Features:
- Collapse
- Hypotension
- Dehydration
- Pigmentation
- Coma
Treatment:
- Rapid rehydration with fluids
- IV hydrocortisone
- Correction of hypoglycaemia
- Search for precipitating cause
Maintenance treatment of Addison’s disease
Lifelong replacement - Glucocorticoid (hydrocortisone, prednisolone) and mineralcorticoid (fludrocortisone)
Education to prevent crises - Double dose glucocorticoid at times of illness, emergency HC injection if vomiting, Steroid card and bracelet.
ACTH deficiency and steroid-suppression
ACTH deficiency
Occurs in any cause of hypopituitarism
Similar symptoms to primary adrenal failure
No pigmentation as ACTH not raised
No hyperkalaemia as no mineralcorticoid deficiency
Hyponatraemia due to effect of cortisol on free water excretion
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 clinical symptoms an causes of Cushing’s syndrome?
Clinical syndrome due to glucocorticoid excess
- Round pink face with round abdomen
- Skinny and weak arms and legs
- Think skin and easy bruising
- Red stretch maerks ‘striae’ on abdomen
- High BP and diabetes
- Osteoporosis (thin bones)
Causes:
- Pituitary tumour
- Adrenal tumour
- Ectopic ACTH
How do patients with Cushings present?
ACTH is suppressed ‘ACTH independant’ Cushing’s
Unlike pituitary and extopic ACTH ‘ACTH-dependant)
Androgeic symptoms may not be present - Hirusutism, acne, greasy skin
Virilising features in large tumours - androgenic alopecia, deep voice, clitoromegaly
Treatment of Cushing’s
Adrenalectomy (Laproscopic)
Large tumours need open surgery
Post-op hypodrenalism a risk due to contra-lateral adrenal suppression
Primary hyperaldosteronism
Excess production of aldosterone from adrenal gland.
Aldosterone-secreting adrenal adenoma - Conn’s syndrome
Bilateral adrenal hyperplasia - No discrete adenoma
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.
Diagnosis of primary hyperaldosteronism
Hypertension and hypokalaemia = clinical suspision
Confirmation of high aldosterone and suppressed renin
Scan shows adrenal adenoma or bilateral hyperplasia.
If the lesion is too small, it may need sampling or a functional scan.
Congenital Adrenal Hyperplasia (CAH)
Rare but important inherited disorder
- Autosomal recessive
- Adrenal crisis and ambiguous genitalia
- Causes by a block on adrenal cortex pathway
- Presentation depends on enzyme defect
Lack of enzyme leads to:
- Low cortisol and aldosterole
- High male hormone (androgens)
Presentation and treatment of CAH
Presentation
- Hypotension
- Hyponatraemia
- Hyperkalaemia
- Hypoglycaemia
- Virilisation
Treatment:
- Treat adrenal crisis
- Determine sex of baby
- Long term glucocorticoids and mineralocorticoids
- Corrective surgery
What are Phaeochromocytoma and paraganglioma?
Phaeochromocytoma - Tumour of adrenal medulla
Paraganglioma - Extra-adrenal tumour (chromaffin tissue origin)
Symtoms of phaeochromocytoma and paraganglioma
- Acute episodes
- Sweating
- Panic attacks
- Palpitations
- High or low BP
- Collapse
Acute crisis of phaeochromocytoma
- Hypertensive crisis
- Encephalopathy
- Hyperglycaemia
- Cardiac arrhythmias
- Sudden death
Investigations for phaeochromocytoma and paraganglioma
- 24hr urine metanephrines
- 2-3 x collections needed
- Plasma metanephrines
- Neuroendrocrine marker: Chromogranin A
Causes of phaeochromocytoma and paraganglioma
Familial causes
- Multiple endocrine neoplasia type 2
- Von Hippel-Lindau
- Neurofibromatosis type 1
- Succinate dehydroginase subunit B and D
Many diseases associated with these four familial causes can also cause this.