Consist of : Outer cortex . Inner medulla. Overproduction Overproduction of adrenal cortex: 1. Glucocorticoids results in (Cushing`s syndrome). 2. Mineralocorticoids results in ( Conn`s syndrome). Overproduction of adrenal medulla results in (Pheochromocytoma) which secrete epinephrine & norepinephrine. Adrenal insufficiency results in (Addison`s disease)
Avascular necrosis of bone
If the symptoms is due to adenoma or carcinoma , the affected gland should be removed ( unilateral adrenalectomy). For bilateral hyperplasia with ACTH suppression ,bilateral adrenalectomy with permanent steroid replacement therapy. For ACTH secreting pituitary tumors ,hypophysectomy is done. ( transphenoidal resection or an yttrium implant. For ectopic ACTH secretion the source of hormone should be excised if possible ( pneumonectomy for oat cell carcinoma). 1.In inoperable cases , radiotherapy may be useful. 2.Bilateral adrenalectomy may be required to control the symptoms. Metyrapone , which inhibit cortisol secretion may be beneficial in cases not amenable treatment.There is excessive secretion of aldosterone which is independent to renin-angiotensin mechanism. It is more common in females. Usually it is due to unilateral adenoma. Rarely due to bilateral adenoma , carcinoma or bilateral hyperplasia. The primary type must be distinguished from secondary hyperaldosteronism where there is increase plasma renin due to increase activity of renin angiotensin mechanism as in cases of renal artery stenosis ,cirrhosis, congestive cardiac failure and nephrotic syndrome. Symptoms: asymptomatic. Hypertension . Lethargy ,fatigue ,muscle weakness ( hypokalemia). Polyuria and polydipsia. Investigations: Urinary and serum levels of potassium ( hypokalemia, alkalosis). Raised plasma aldosterone level . Low plasma renin level. CT scan to identify adenoma.
Is catecholamine-producing tumor that arises from chromaffin cells of adrenal medulla or other sympathetic nervous tissue e.g. aortic paraganglia. 10% of cases are malignant tumors, are associated with other conditions MEN ,Von Reckling hausen`s disease and Hippel-Lindau disease. Symptoms & signs: Paroxysmal hypertension , head ache, palpitations, sweating , anxiety , chest pain , weakness. Investigations: 24 hours urine for VMA ( vinyl mandelic acid) level is high ( superior results occur after excess of tea,coffee,chocolates,bananas or vanilla extract). CT scan can locate 90%. 131-metaiodobenzylguanidine scanning.
It may be : Primary (Addison's disease): when adrenal destruction occurs due to autoimmune. Sarcoidosis. Tuberculosis. Amyloidosis. secondary deposits. adrenal hemorrhage (anticoagulant therapy or meningococcal septicemia * waterhouse-Friderichsen syndrome. 2. secondary : due to Pituitary disease . Withdrawal of long term steroid therapy, Bilateral adrenalectomy is an iatrogenic cause.
Acute adrenal insufficiency: Shock. Nausea and vomiting. Hyperpyrexia. Rigors. Abdominal pain. Coma. 2.Chronic adrenal insufficiency: Anorexia. Lethargy, malaise. Hypotension. Weight loss. Constipation. Amenorrhea. Hyperpigmentation of skin and mucous membrane of the mouth.
Hyperpigmentation of skin and mucous membranes
Investigations Check steroid therapy. Check blood pressure. Urinary and serum estimation of Na and K show (hyponatremia,hyperkalemia). Blood sugar ( hypoglycemia). Decreased serum cortisol level with no response to synacthen test. ACTH level is high in Addison's disease and low in secondary causes. Adrenal antibody screening test. Chest x ray -----may show TB. Abdominal x-ray --- may show adrenal gland calcifications.in TB.
1. Acute adrenal insufficiency : Resuscitation with IV normal saline + hydrocortisone 100 mg 6 hourly , it will result in dramatic clinical response. 2. Chronic adrenal insufficiency : Oral prednisolone 20 mg at morning and 10 mg at evening + fludrocortisone ( mineralocorticoid) replacement (0.5-1 mg) daily is indicated in most cases. Fatigue ,hypotension and hyperkalemia are signs of under treatment. Hypertension edema and hypokalemia are signs of overtreatment. Patients on replacement therapy should be warrant to increase the dose at time of stress e.g. surgery or acute illness. Care should be taken in reducing the dose or discontinuing the treatment in patients on long term therapy. Gradual withdrawal over weeks will allow endogenous steroid production to resume in some patients but in others signs of insufficiency will appear.
MEN syndromes are patterns of endocrine disease usually inherited as autosomal dominant traits. MEN I ( 3PS) Manifestations are that of hyperparathyroidism, pancreatic tumors( insulinoma,Gastrinoma)& pituitary tumor. Clinically : presents with symptoms of peptic ulceration(Gastrinoma) or pituitary tumor. Hyperparathyroidism is usually asymptomatic and detected incidentally on checking serum calcium level. Treatment : directed towards the tumor that cause symptoms.
MEN IIa (TPP) Manifestation of medullary carcinoma of thyroid ,pheochromocytoma , parathyroid hyperplasia. Symptoms and signs: Check relatives of patient with medullary thyroid carcinoma. Genetic screening ret-proto-oncogene in patient if it is present family screening should be done. If (+ve) family history in children ,brothers , sisters prophylactic thyroidectomy may be considered. Search should be done for all tumors prior to treatment. If pheochromocytoma is diagnosed it should be treated first ,then deal with thyroid and lastly parathyroid.