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Cushing’s, Addison’s and Acromegaly

Cushing’s

Syndrome: excessive activation of glucocorticoid receptors.

Disease: excessive ACTH due to pituitary adenoma.

Classification:

 ACTH-dependent  pituitary adenoma, ectopic ACTH, iatrogenic.

 ACTH-independent  iatrogenic, adrenal adenoma/carcinoma.

 Pseudo-Cushing’s  EtOH excess, major depressive disorder, 1  obesity

Clinical features: hair thinning, hirsuitism, psychosis, acne, plethora, cataracts, mild exophthalmos, moon face, peptic ulcers, vertebral #, hypertension, hyperglycaemia, central adiposity, menstrual disturbance, striae, decreased skin thickness, osteoporosis, proximal myopathy,  infections, poor wound healing, bruising, impaired immune response.

Investigations:

 Bedside  cap glucose, BP.

 Bloods  dexamethasone suppression test, CRH test, serum cortisol.

 Imaging  MRI brain, CXR, CT AP/adrenals.

 Others  inferior venous petrosal sampling, urinary free cortisol.

Management:

 Conservative  patient education, reduce oral steroid therapy if possible.

 Medical  ketoconazole/metyrapone (  steroid synthesis).

 Surgical  trans-sphenoidal resection of pituitary.

Addison’s

Definition: a syndrome resulting from inadequate secretion of corticosteroid hormones from progressive destruction of the adrenal cortex.

Causes: “ADDISON” – Autoimmune, Degenerative, Drugs, Infective, Secondary,

Other, Neoplasia.

Clinical features: bronze pigmentation of skin, tiredness, hypoglycaemia, postural hypotension, changes in distribution of body hair, weight loss, GI disturbance. Associated with type 1 diabetes, pernicious anaemia, autoimmune thyroid disease and vitiligo.

Investigations:

 Bedside  lying/standing BP, capillary glucose.

 Bloods  U&Es, synacthen test, plasma renin, aldosterone, HIV test.

 Imaging  AXR, CT/MRI adrenals.

Management:

 Glucocorticoid replacement – hydrocortisone BD.

 Mineralocorticoid replacement – fludrocortisone OD.

 Addisonian crisis (shock, hypotension, abdo pain, N&V,  Na + /  K + ) –

ABCDE, correct volume depletion, replace glucocorticoids, correct metabolic abnormalities, treat underlying cause.

Acromegaly

Definition: a condition caused by excessive growth hormone secretion.

Causes: most commonly, pituitary adenoma.

Clinical features: headache, skull growth (prominent supraorbital ridge with large frontal sinuses), enlargement of lips, nose and tongue, prognathism, cardiomyopathy, increased sweating, thickened skin, hepatomegaly, type 2 diabetes, enlargement of hands, carpal tunnel syndrome, myopathy, enlargement of feet, visual field changes, diplopia.

Investigations:

 Bedside  collateral hx, serial photographs, BP, ECG.

 Bloods  serum GH, OGTT, serum IGF-1.

 Imaging  CT/MRI brain, echo.

 Other  colonoscopy.

Management:

 Conservative  patient education.

 Medical (second line)  somatostatin analogues (octreotide, lanreotide), dopamine agonists, GH receptor anatagonists (pegvisomant).

 Surgery (first line)  trans-sphenoidal debulking of tumour.

 Radiotherapy  if acromegaly persists after surgery.

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