Adrenal

Question 1

Types of hypoadrenalism

Answer 1

Primary eg Addison - Cortisol and aldosterone affected.
- mostly autoimmune eg lymphocytic adrenalitis.
- Infections - TB, AIDS - CMV, Histoplasmosis, Waterhouse friedrichsen syndrome (adrenal haemorrhage in meningococcal septicaemia)
- Tumour - uncommon bilateral.
Secondary eg pituitary - Cortisol only
Iatragenic - Steroid use - Cortisol only.

Question 2

Symptoms of Hypoadrenalism

Answer 2

Non specific - wt loss, lethargy, weakness, nausea and abdo pain
If glucocorticoid and mineralocorticoid deficiency - Hypotension particularly postural.
Pigmentation- nipples, recent scars, face and neck, palmer skin creases and buccal mucosa.
Hypoadrenal crisis - Collapse, vomiting and Electrolyte imbalance of Hypo Na, Hyper K, increase urea, mild acidosis. Common caused by illness or Rifampicin.

Question 3

Ix for hypoadrenalism

Answer 3

Bloods - Hypo Na, Hyper K, raised Urea, Mild acidosis.
Markedly low cortisol with increase ACTH in ill pt is dx.
Synacthen Test - give ACTH and look for cortisol response
CT or MRI - exclude TB calcification and tumours
Ab - against adrenal gland
Renin and aldosterone levels

Question 4

Mx of hypoadrenalism

Answer 4

Adrenal crisis
- Give Na in normal saline
- IV hydrocortisol
- Treat underlying cause.
If mineralocorticoid deficient - oral fludrocortisone
In General non crisis
- Teach pt to carry emergency bracelet and health card. And to insert hydrocortisol when sick or vomiting.
- Hydrocortisol.
If iatrogenic adrenal suppression
- specialist referral
- Need to withdrawal very slowly and look out for crisis
- steroid for more then 3 week carries a risk of adrenal suppression when withdrawal.

Question 5

Congential adrenal hyperplasia

Answer 5

Cause by 21 hydroxylase deficiency which causes cortisol and alderstone deficiency which leads to increase in ACTH which leads to increase in androgens as a byproduct.
Symptoms: PC in first weeks of life with salt losing crises.
Virilisation of female infants
Precocious puberty in males
Ix: Serum 17 hydroxyprogesterone concentration increase after ACTH. Where cortisol only a little.
Tx - Glucocorticoid and mineralocorticoid replacement same as addisons.

Question 6

Cause of Cushing’s syndrome

Answer 6

Pituitary microadenomas - Cushing disease
Adrenal tumours (bengin)
Ectopic ACTH secretion. (Carcinoid tumour)
Prolonged corticosteroid therapy
Alcohol induce pseudo Cushing
Obesity and depression cause the same sign and may be hard to distinguish.

Question 7

Symptoms of cushing

Answer 7

Triad - HTN, proximal myopathy and skin changes
Depression
Weight gain
Weakness
Insomnia, agitated
Sexual dysfunction
Non specific malaise
PmHx: peptic ulcer, renal stones, Osteoporosis, fracture, repeated infections. DM
Menstrual distubances

Question 8

Signs of Cushings

Answer 8

GI: Baffalo bump, moon facies, purple abdominal striae, bruising, skin/muscle atrophy in periphery, Central obesity, thinning hair supraclavicular and suprascaplar fat pads, Hirsutism. Increase skin pigmentation, Acne.
Vital - HTN, BMI high, BSL may be high
Hands: thin skinfolds
Arms: Muscle atrophy, proximal myopathy, bruising
Face: Plethora, moon facies, acne, hirsuitism, Pigmentation, telangiectasia, thinning hair
Eye: visual fields
Neck: buffalo lump
Chest: Central obesity, bony tenderness for crash fractures
Abdo: Striade, adrenal masses, fatty liver or mets.
Legs: Oedema, proximal myopathy, bruising and poor wound healing.

Question 9

Ix for Cushing

Answer 9

Increase plasma or urinary cortisol levels
Loss of circadian rhythm of plasma cortisol levels.
Loss of dexamethasone suppression
Plasma ACTH - if suppressed = adrenal adenoma (confirm with CT/MRI), If raised = Ectopic or pituitary source of ACTH. Scan head and thorax for pituitary adenoma or carcinoid tumour.
To differentiate pituitary ACTH cause vs ectopic do a bilateral simultaneous inferior petrosal sinus sampling.

Question 10

Mx of cushing

Answer 10

Adrenal adenomectomy is definitive tx for adrenal cushing
Ectopic tumour require surgical excision
Selective adenomectomy is 1st line for pituitary Cushing’s
Radiotherapy for pituitary tumours not cured by surgery.
Preparation for surgery of any cause
- drugs to block cortisol production - metyrapone, mitotane, aminoglutethimide, ketoconazole.
Prognosis - some changes are irreversible. And surgery not also successful.

Question 11

Hyperaldosteronism Cause and symptoms

Answer 11

Types
- Primary - Conn’s disorder: Hypokalaemia and mild to moderate HTN. Causes include discrete adenoma or bilateral hyperplasia of zona glomerulosa
- Secondary: Due to reduced effective arterial volume/reduced renal perfusion in HF and Hypoalbuminaemic states.
Symptoms
- Hypokalaemia: muscle weakness, cramps, polyuria or asymptomic
- HTN pt who are less then 40, have hypokalaemic and resistance to drug therapy or have a fmHx should be Ix.

Ix - measure renin and aldosterone after overnight lying down and then again after 4hrs of standing. Pt with adrenal adenomas have suppressed renin and a raised serum aldosterone concentration which doesn’t change with posture. Those with bilateral hyperplasia also have hyporeninaemic hyperaldosteronism by plasma aldosterone rises with posture.
CT

Question 12

Ix for hyperaldosterone

Answer 12

Ix - measure renin and aldosterone after overnight lying down and then again after 4hrs of standing. Pt with adrenal adenomas have suppressed renin and a raised serum aldosterone concentration which doesn’t change with posture. Those with bilateral hyperplasia also have hyporeninaemic hyperaldosteronism by plasma aldosterone rises with posture.
CT, MRI and USS - adenoma
Catheterising both renal arteries to determine bilateral hyperplasia

Question 13

Mx of hyperaldosterone

Answer 13

Medical - spironolactone. High doses
Surgery - Adenomas are surgically removed
Prognosis - hypokalaemia resolves by HTN stays in 50%.

Question 14

History and examination of a patient with a incidental finding of adrenal mass

Answer 14

Evidence of hormonal excess
Phaeochromocytoma – paroxysmal tachycardia, palpitations, sweating, anxiety and hypertension, headache*
Conn’s syndrome – Hypertension resistant to drug therapy Cushing’s syndrome – Central obesity, striae, bruising,
cataracts, buffalo hump, muscle wasting, acne, thinning
hair, moon face
Adrenal carcinoma – virilisation/feminisation along with
Cushingoid features
 History of cancer
 Family history of endocrine disease (MEN 1 and 2)
Majority are asymptomatic with no relevant examination findings

Question 15

DDX of incidental finding of adrenal mass

Answer 15

 Adrenal Cortex: Adenoma
Nodular Hyperplasia
Carcinoma
 Adrenal Medulla:
Phaeochromocytoma
Ganglioneuroma  Metastases
 Other:
Myelolipoma, neurofibroma, hamartoma, cyst, pseudocyst, haematoma, schwannoma

Question 16

Ix for adrenal mass

Answer 16

Laboratory studies:
 24 hour urine free cortisol
 24 hour urine catecholamines  Plasma metanephrines
 Serum aldosterone:renin ratio  Serum potassium level
 Adrenal sex steroid panel
Imaging
 CT scan
 MRI
 Nuclear Medicine – MIBG for phaeochromocytoma
 Selected Venous Sampling to lateralise Conn’s tumours
CT-guided biopsy
 RARELY
 May be useful for confirming possible solitary adrenal metastasis prior to considering surgery
 Must exclude phaeochromocytoma 1st!

Question 17

Mx of Adrenal mass

Answer 17

Adrenalectomy:
 Lesions > 3.5cm (malignancy risk)
 Suspected malignant disease or suspicious imaging characteristics (inhomogenous, mixed density, calcification)
 Hormone hypersecretion
 Solitary metastases
Observation:
 Non-functional lesions <3.5cm
CT scan at 6 months, 12 months and repeat biochemistry at 12 months
Laparoscopic adrenalectomy is the standard of care in adrenal surgery
 Open adrenalectomy reserved for suspected primary adrenal carcinoma and very large lesions >12cm

Question 18

Phaeochromocytoma

Answer 18

Secondary HTN: florid and potentially fatal HTN crises.
Known as 10% tumour. 10% bilateral, 10% sweating, 10% palpitations
Symptoms: nervousness, panic attacks and flushing. can be hypotension.
Ix Urinary and plasma catecholamines, CT or MRI scan
Tx - IV then oral Alpha blockers e.g. phentolamine, and phenoxybenzamine. IV fluids. B blockers can be added later. Anti HTN prior to surgery