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Disorders of the Adrenal Gland
Omar DHAIMAT MD 11/22/2018
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Introduction Understand the physiology of adrenal hormone production.
Memorize the major adrenal hormones and their effect on tissues. Discuss adrenal insufficiency and Cushing syndrome. 11/22/2018
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Adrenal Insufficiency
Classification:1.Primary adrenal insufficiency (loss of adrenocortical hormones due to destruction or impairment of the adrenal cortex).2.Secondary A.I(reduced secretion by the pituitary gland of ACTH).3.Tertiary(failure of the hypothalamus to produce CRH). 11/22/2018
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Adrenal Insufficiency
Causes: most common cause of primary AI is autoimmune adrenalitis.Infectious adrenalitis due to TB or disseminated fungal infection.Bilateral adrenal hemorrhage.Adrenomyeloneuropathy.Bilateral metastasis disease.Medications:ketoconazole,aminoglutethimide,rifampin. 11/22/2018
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Adrenal Insufficiency
Secondary causes:usually associated with panhypopituitarism(space occupying lesions,primary tumors,mets from breast,prostate orlung,aneurysms,TB,Histoplasmosis,Sheehan syndrome,pituitary apoplexy,surgery or radiation). 11/22/2018
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Adrenal Insufficiency
Tertiary:long term use of suppressive doses of glucocorticoids.Rarely,tumors,infiltrative diseases and cranial radiotherapy. Presentation: non specific. 11/22/2018
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Adrenal Insufficiency
Hyper pigmentation ,caused by high ACTH >90% of primary AI,which can be generalized or localized to elbows,knees,knuckles and buccal mucus.Vitiligo,may coexist in 10-20% of patients with autoimmune adrenalitis.hypo tension.Loss of axillary and pubic hair.Auricular calcification in long standing adrenal insufficiency.Psychiatric disorders. 11/22/2018
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Adrenal Insufficiency
Lab abnormalities: NA,K are the most common,mild NN anemia,eosinophilia and lymphocytes.Prerenal azotemia.Moderate Hypercalcemia in <10%.Moderate elevation of TSH. DX:low cortisol<5microgram/dl in the face of stress.ACTH stimulation test .Low dose ACTH test.Insulin induced hypoglycemia(gold standard),metyrapone test. Treatment: 11/22/2018
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Adrenal Insufficiency
Acute crisis.5 S’s(salt,sugar,steroids,support and search for a precipitating illness). Chronic:replacement:HC,Prednisone,Dexamethasone. During stress:dose changes,iv forms.Bracelet . Role of Fludrocortisone and DHEA. 11/22/2018
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Cushing”s Syndrome Cortisol increases glucose production,inhibit protein synthesis,increase protein breakdown,stimulate lipolysis and affect immunologic and inflammatory responses. 11/22/2018
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Cushing”s Syndrome Clinical symptoms:obesity(truncal,wasting of extremities,moon facies,supraclavicular fat pads and buffalo hump).Thinning of the skin with facial plethora,easy bruising&violaceous striae.Muscular weakness.Hypertension,atherosclerosis,CHF,edema.Gonadal dysfunction,menstrual irregularities.Psychological disturbances.Osteoporosis &fractures.Increased rate of infections and poor wound healing. 11/22/2018
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Cushing”s Syndrome Causes: 1.Exogenous steroids.
2.Pituitary Cushing’s syndrome:pituitary adenoma with ACTH. 11/22/2018
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Cushing”s Syndrome 3.Ectopic production of ACTH.Malignant: lung cancer,slowly growing :carcinoids. 4.Adrenal causes:adenoma,carcinoma,micronodular hyperplasia,macronodular hyperplasia. Most common:is exogenous.Endogenous causes:mc is pituitary adenoma(70%). 11/22/2018
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Diagnosis and Treatment:
Single best screening test is 24 hr collection for urinary free cortisol and creatinine. Overnight I mg Dexamethasone test. Loss of diurnal variation and DEX-CRH test. ACTH level. 11/22/2018
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Diagnosis and Treatment:
High dose Dexamethasone test. Imaging study,Petrosal sinus sampling. Treatment:1.ACTH pituitary producing adenomaTSS.Otherwise,medications:Ketoconazole.Radiation.Last resort:bilateral adrenalectomy(30% to develop NELSON syndrome). 2.ACTH-independent cushing’s syndrome.Adenomaresection.PPNADbilateral adrenalectomy.MacroNAHvariable. 11/22/2018
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