CLINICAL ASPECT OF ADRENAL DISEASES (II).pdf

CLINICAL ASPECTS OF ENDOCRINE DISEASES (II) Dr. S. Bowatte Intended Learning Objectives Describe clinical importance of: Hyperaldosteronism Phaeochromocytoma Diabetes Insipidus SIADH Insulinoma Autoimmune Polyglandular Syndromes Multiple En...

CLINICAL ASPECTS OF ENDOCRINE DISEASES (II) Dr. S. Bowatte Intended Learning Objectives Describe clinical importance of: Hyperaldosteronism Phaeochromocytoma Diabetes Insipidus SIADH Insulinoma Autoimmune Polyglandular Syndromes Multiple Endocrine Neoplasia Congenital Adrtenal Hyperplasia (CAH) 1. Hyperaldosteronism Excess secretion of aldosterone Primary Hyperaldosteronism Adrenal Adenoma (Conn’s Syndrome) Bilateral Adrenal Hyperplasia Secondary Hyperaldosteronism (due you to excess Renin production) Renal Artery Stenosis Heart failure Cirrhosis Dr Jerome W Conn (1907 – 1994)  Sodium retention Potassium loss Pathophysiology Severe resistant hypertension (in young individuals) Accelerated hypertension  Plasma Aldosterone level (Diurnal and Postural levels) Plasma Aldosterone : Renin ratio Serum electrolytes Investigations Urinary electrolytes CT/MRI of adrenals Management Surgical removal of adenoma Medical management for hyperplasia Aldosterone antgonist – Spironolactone Aldosterone receptor antagonist – Eplerenone Amiloride - Potassium sparing diuretic 2.Pheochromocytoma  Tumor of adrenal medulla arising from sympathetic nervous tissue Paraganglioma's are similar tumors outside the adrenal medulla Majority are benign, but 10% malignant Pathophysiology Tumor secrete excess adrenaline and noradrenaline Frequently the secretions are intermittent Excess secretion may precipitate during a surgery Sometimes sudden death occur Symptoms and Signs Investigations Urinary metanephrines Urinary vanillylmandelic acid (needs avoidance of certain foods and drugs before testing) CT/MRI of adrenal glands 131I MIBG scan Management Surgical removal of tumor Needs prior preparation of patient with alpha blocker followed by beta blocker Alpha blocker : Phenoxybenzamine Beta blocker : Propranolol In sudden severe hypertension : Phentolamine, Sodium nitroprusside, Magnesium sulfate 3. Diabetes Insipidus “Excess production of tasteless urine” Causes Cranial DI Nephrogenic DI Dipsogenic DI Gestational DI Cranial DI Due to hypothalamic disease leading to deficiency of ADH Tumors, infection, inflammation, infiltrations, brain surgery, autoimmune, familial Poly-nocturia - >3 L/day Dehydration Excess thirst Investigations Hypernatremia Hyperosmolality Failure of urinary concentration on water deprivation Management Desmopressin (synthetic ADH) – intranasal, oral, intramuscular Carbamazepine Chlorpropamide Nephrogenic DI Renal tubular unresponsiveness to ADH Very rare Secondary to different types of renal disease, sickle cell anemia, treatment with lithium ADH levels normal or high Dipsogenic DI Psychiatric or hypothalamic disease Leading to dysfunction of thirst center Difference is hypo-osmolality & hyponatremia 4.Syndrome of inappropriate ADH secretion ( SIADH) Excess ADH cause retention of water Resultant hypervolemia induce natriuresis through ANP Causes Symptoms and Signs Symptoms are vague Confusion, nausea, irritability, fits, coma Investigations Plasma osmolality Serum electrolytes ACTH, TSH Management Fluid restriction Demeclocycline Hypertonic saline Vasopressin receptor antagonist- Tolvaptan 5.Insulinoma Tumor of pancreatic beta cells Cause recurrent hypoglycemia Whipple’s triad : Fasting or exercise induced symptomatic hypoglycemia Biochemical evidence of hypoglycemia Glucose relieving symptoms High fasting insulin or C- peptide level In some cases 72 hour prolonged fasting is necessary to diagnose hyperinsulinemia 6. Autoimmune Polyglandular Syndromes (APS) 7. Multiple Endocrine Neoplasia Congenital adrenal Hyperplasia CAH Autosomal Recessive 6 major types Glucocorticoids/mineralocorticoids are diverted to androgens Unopposed ACTH lead to adrenal hyperplasia CAH Ambiguous genitalia of females Virilization of females Adrenal failure Salt wasting Mostly at birth, but can present in childhood and adulthood. QUESTIONS ?? THANK YOU