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Adrenal Disorders Associate Professor Geoff Braatvedt. Cholesterol. Pregnenolone. DHEAS. Physiology. Cortex (steroids). Cholesterol. Pregnenolone. DHEAS. Progesterone. 170H PROG. Testosterone. Physiology. Cortex (steroids). Cholesterol. Pregnenolone. DHEAS. Progesterone.
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Cholesterol Pregnenolone DHEAS Physiology Cortex (steroids)
Cholesterol Pregnenolone DHEAS Progesterone 170HPROG Testosterone Physiology Cortex (steroids)
Cholesterol Pregnenolone DHEAS Progesterone 170HPROG Testosterone Aldosterone (Mineralocorticoid) Physiology Cortex (steroids)
Cholesterol Pregnenolone DHEAS Progesterone 170HPROG Testosterone *21 Hydroxylase Aldosterone (Mineralocorticoid) Cortisol (Glucocorticoid) Physiology Cortex (steroids)
Circadian Stress + + HigherCentres + Hypothalamus CRF + - Pituitary ACTH + - Adrenal Glucocorticoid Control
Exogenous steroids Glucocorticoid Mineralo Corticoid Daily Replacement Potency Potency Dose Hydrocortisone 1 1 25 mg
Exogenous steroids Glucocorticoid Mineralo Corticoid Daily Replacement Potency Potency Dose Hydrocortisone 1 1 25 mg Cortisone Acetate 0.9 0.8 25 - 30 mg
Exogenous steroids Glucocorticoid Mineralo Corticoid Daily Replacement Potency Potency Dose Hydrocortisone 1 1 25 mg Cortisone Acetate 0.9 0.8 25 - 30 mg Prednisone 5 0.8 5 - 7 mg
Exogenous steroids Glucocorticoid Mineralo Corticoid Daily Replacement Potency Potency Dose Hydrocortisone 1 1 25 mg Cortisone Acetate 0.9 0.8 25 - 30 mg Prednisone 5 0.8 5 - 7 mg Dexamethasone 50 0 0.5 mg
Exogenous steroids Glucocorticoid Mineralo Corticoid Daily Replacement Potency Potency Dose Hydrocortisone 1 1 25 mg Cortisone Acetate 0.9 0.8 25 - 30 mg Prednisone 5 0.8 5 - 7 mg Dexamethasone 50 0 0.5 mg 9 Flurocortisone 0 125 0.1 - 0.2 mg
Side Effects • CVS • GIT
Side Effects • CVS • GIT • CNS • Bone
Side Effects • CVS • GIT • CNS • Bone • Endo • Renal • Immune • Skin
Advice • Life long
Advice • Life long • Steroid card / bracelet
Advice • Life long • Steroid card / bracelet • IMI hydrocortisone
Advice • Life long • Steroid card / bracelet • IMI hydrocortisone • Illness • Withdrawal: • acute dosing • chronic dosing
Presentation may be • Psychiatric - depression, hypo mania etc • Endocrine - ‘spot’ diagnosis, diabetes, hirsuitism, amenorrhoea etc • Orthopaedic - osteoporosis, fractures • Cardiac - BP etc
Diagnosis • 24 hour urinary free cortisol high • 1 mg overnight dexamethasone suppression test –fails to suppress = the syndrome of Cushings Now is it pituitary or adrenal or ectopic??
Pituitary dependent Cushing’s • ACTH measurably normal to high • 8 mg dex test suppresses • MRI positive • Consider petrosal sinus sampling
Inferior petrosal sinus sampling for adrenocorticotropic hormone after corticotropin-releasing hormone
Hypothalamic-pituitary-adrenal axis in ACTH-dependent Cushing's syndrome Cortisol ACTH /
Adrenal Cushing’s • ACTH unmeasurably low • MRI / CAT scan of adrenals
Hypothalamic-pituitary-adrenal axis in ACTH-independent Cushing's syndrome Cortisol ACTH
Ectopic Cushing’s • ACTH may be very high • Fails to suppress to high dose dexamehtasone • Normal pituitary MRI • Negative petrosal sinus sampling • Possibly CXR / CT chest
Hypothalamic-pituitary-adrenal axis in ACTH-dependent Cushing's syndrome Cortisol ACTH //
Hypoadrenalism • Exogenous steroids
Hypoadrenalism • Exogenous steroids • 1° Addison’s
Hypoadrenalism • Exogenous steroids • 1° Addison’s • 2° to pituitary disease
Hypoadrenalism • Exogenous steroids • 1° Addison’s • 2° to pituitary disease • Adrenal enzyme deficiencies = CAH
Cholesterol Pregnenolone DHEAS Progesterone 170HPROG Testosterone *21 Hydroxylase Aldosterone (Mineralocorticoid) Cortisol (Glucocorticoid) Physiology Cortex (steroids)
Hypoadrenalism • Exogenous steroids • 1° Addison’s • 2° to pituitary disease • Adrenal enzyme deficiencies = CAH • (21 OH progesterone) • Addison’s: • autoimmune (glucocorticoid and mineralocorticoid) • adrenal destruction - TB
Symptoms • Weight loss • Pigmentation • Anorexia • Abdominal pain
Symptoms • Weight loss • Pigmentation • Anorexia • Abdominal pain Signs • Pigmentation