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Adrenal disorder.pptx

  1. 1. ENDOCRINOLOGY Lecture 1 Adrenal Gland Disorders
  2. 2. Cushing's Syndrome A state of hyperfunction of adrenal cortex and it i s caused by: 1. Exogenous cortisol or ACTH. (iatrogenic or exogenous Cushing's Syndrome) 2. ACTH producing pituitary microadenoma (C ushing disease) - 70% 3. Primary adrenal tumors. -20% 4. Ectopic ACTH secretion (bronchial, thymic, pancreatic tumor, medullary thyroid carcino ma, & pheochromocytoma ) -10%
  3. 3. Cushing's Syndrome • Iatrogenic Cushing syndrome is the most com mon cause. • Cushing disease caused by microadenoma of t he pituitary with bilateral adrenal hyperplasia. Both Cushing’s disease and cortisol-secreting a drenal tumours are four times more common in women than men, usually in the young age. • Primary adrenal tumors account for 20% cases, usually unilateral and about 50% are malignan t.
  4. 4. Hypercortisolism due to other causes (als o referred to as pseudo-Cushing’s syndro me) 1- Alcohol excess (biochemical and clinical featu res) 2- Major depressive illness (biochemical features only, some clinical overlap) 3- Primary obesity (mild biochemical features, s ome clinical overlap).
  5. 5. Clinical features 1. Truncal obesity with moon face and buffalo hump i n the interscapular area.. 2. Plethoric face with acne and hirsutism. 3. Easy bruising of skin and violaceous striae in the ab domen and thigh. 4. Proximal myopathy and osteoporosis. 5. Hypertension and DM. 6. Oligmenorrhea. 7. Emotional upset and psychosis.
  6. 6. Cushing's Syndrome
  7. 7. Cushingoid face
  8. 8. Striae
  9. 9. Diagnosis 1. Urine free cortisol(> 2 tests): 24-hr timed collectio n. Normal range depends on assay 2. Overnight dexamethasone suppression test: the pati ent given dexamethasone 1mg at 2300 hrs then mea sure plasma cortisol at 0900 hrs. Plasma cortisol > 5 0 nmol/L (> 1.81 μg/dL) suggest Cushing'syndrome 3. Low dose dex. suppression test: the patient given de x. 0.5 mg qid for two days. Plasma cortisol > 50 nm ol/L (> 1.81 μg/dL) suggest Cushing'syndrome.
  10. 10. Establishing the presence of Cush ing’s syndrome Cushing’s syndrome is confirmed by using two o f three main tests: 1. Failure to suppress serum cortisol with low do ses of oral dexamethasone 2. Loss of the normal circadian rhythm of cortiso l, with inappropriately elevated late-night seru m or salivary cortisol 3. Increased 24-hour urine free cortisol
  11. 11. Determining the cause of confirm ed Cushing’s syndrome. 4. High dose dex. suppression test: used for differentiation between pituitary ad enoma and other causes, the patient given 2 mg 6-hourly for 48 hrs; sample 24-hr urine c ortisol at baseline and during second day. Uri ne cortisol < 50% of basal suggests pituitary- dependent disease; > 50% of basal suggests e ctopic ACTH syndrome. 5. Other tests: ACTH level and imaging study of the pituitary and adrenals.
  12. 12. Treatment • Pituitary microadenoma with adrenal hype rplasia treated by: 1. Surgical removal of the tumor or whole th e pituitary. 2. Bilateral adrenalectomy→ Nelson syndro me with an invasive pituitary macroadeno ma and very high ACTH levels causing pi gmentation. The risk of Nelson’s syndrom e may be reduced by pituitary irradiation.
  13. 13. • Medical blocking of steroidogenesis by keton azole or metyrapone. Typical starting doses a re 500 mg/tid for metyrapone (maximum dos e, 6 g) and 200 mg/tid for ketoconazole (max imum dose, 1200 mg). • Adrenal tumors are treated by: total adrenalectomy ± chemotherapy.
  14. 14. Addison's disease & Pheochromocytoma
  15. 15. Adrenal insufficiency 1. Primary (Addison's disease): it is rare condition, occurs at any age an d caused by: autoimmune atrophy, HIV, CMV, tuberculosis, bilateral hemorrhag e and secondaries. adrenal failure may b e part of PAS I (adrenal and parathyroid failure and mucocutanous candidiasis) o r PAS II. 2. Secondary adrenal failure: due to pituita ry insufficiency or after long use of ster oids that suppress the pituitary.
  16. 16. Clinical features (Addison's disease) Gradual adrenal destruction is cha racterized by an insidious onset of fatigability, malaise, anorexia, nausea and vomiting, weight los s, cutaneous and mucosal pigme ntation, hypotension, and hypogl ycemia.
  17. 17. Clinical features (Adrenal crisis) • Acute adrenal insufficiency cause d by adrenal hemorrhage or by su dden withdrawal of prolong steroi d therapy. • The patient presents with severe h ypotension and hypoglycemia and may leads to shock.
  18. 18. Hyperpigmentation of mucous membran e in Addison's disease
  19. 19. Hyperpigmentation of skin in Add ison's disease
  20. 20. Diagnosis 1. General: hyponatremia, hyperkalemia. 2. ACTH (Synacthen) stimulation test: it is the best screening test in which plasma cortisol measured 30 min after im inject ion of 250 ug ACTH; normally cortisol l evels should exceed 500 nmol/L (18 ug/ dL). 3. ACTH and aldosterone level help in diff erentiation between primary and second ary adrenal failure.
  21. 21. Treatment 1. Corticosteroid replacement in form of cortisol 15-20 mg/d or prednisolone 5 -7.5 mg/d ( 2/3 on waking and 1/3 at 1500 hrs). The dose should be dou bled in acute stressful conditi ons like infection and surgery.
  22. 22. 2.Mineralcorticoid replacement in f orm of fludrocortisone 0.05-0.15 mg/d with adequate salt intake a nd monitoring the dose by measu ring electrolytes and blood press ure. 3.Acute adrenal failure (addisonian crisis) is treated by parenteral ste roid and normal saline replacem ent
  23. 23. Secondary adrenal insufficiency • Hyperpigmentation is absent, other endoc rine disorders may be present. • Aldosterone is near normal. • Iatrogenic type associated with low cortis ol and ACTH due to prolonged suppressi on of the pituitary. • Pituitary insufficiency is treated with cort isol replacement, iatrogenic type treated b y gradual tapering of steroid therapy and some times with ACTH to stimulate the p ituitary and this may take days to months.
  24. 24. Pheochromocytoma  Are tumors that produce, store, and secre te catecholamines. They are usually deriv ed from the adrenal medulla (80%) but m ay develop from chromaffin cells in or ab out sympathetic ganglia (20%) It has been estimated that phaeochromocyto ma accounts for less than 0.1% of cases o f hypertension , but is, nevertheless, an important correcta ble cause of high blood pressure.
  25. 25. Pathology of Pheochromocytoma • In adults, approximately 80% of pheochr omocytomas are unilateral and solitary, 1 0% are bilateral, 10% are extraadrenal an d about 10% are malignant. • Most of the extraadrenal tumors are locat ed within the abdomen in association wit h the celiac, superior mesenteric, and infe rior mesenteric ganglia. Approximately 1 0% are in the thorax. • Pheochromocytoma may be part of MEN type 2a (Sipple's syndrome).
  26. 26. Biochemistry of pheochromocyto ma • Most pheochromocytomas contain an d secrete both norepinephrine and epi nephrine, and the percentage of norep inephrine is usually greater than in th e normal adrenal. • pheochromocytoma may secrets othe r hormones like PTH hormone and er ythropoietin.
  27. 27. Clinical features 1. Hypertension is the most common manifestation. • In about 60% of cases the hyperten sion is sustained, other 40% have pa roxysmal hypertension. • The hypertension is often severe, an d may be resistant to treatment with standard antihypertensive drugs.
  28. 28. 2. Panic paroxysms (crisis) occur in 50% of patients. • The paroxysms may be frequent or sporadi c occurring at intervals as long as weeks or months. • The paroxysm may be precipitated by any a ctivity that displaces the abdominal contents or occurs spontaneously. • The attack may last from a few minutes to s everal hours. • The attack characterized by headache, profu se sweating, palpitations, and apprehension associated with pallor or flushing. Chest pai n and abdominal pain may occur. The blood pressure is elevated.
  29. 29. 3. Orthostatic hypotension may occur as result of diminished plasma volu me and blunted sympathetic reflex es. 4. Other features are weight loss, glu cose intolerance, polycythemia and Hypercalcemia.
  30. 30. Diagnosis • The diagnosis is confirmed by me asurement of 24 hr urinary excreti on of vanillylmandelic acid (VM A), the metanephrines, or free cat echolamines. • Localization of the tumor is done by CT scan or MRI.
  31. 31. Treatment • Preoperative control of blood pressure by alpha blockers like phenoxybenzamine an d prazocine. Other drugs are calcium cha nnel blockers and ACE inhibitors. • Beta-blockers can be used for control of t achycardia after blocking of alpha recept ors. • After control of blood pressure the tumor is removed by surgery.
  32. 32. THANKS

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