CUSHINGS SYNDROME

Prof.T.P.K

• A 45-year- old woman is evaluated for a 20 lb weight gain over the preceding 2 years. She was also recently found to have a fasting blood glucose of 135 mg/dl and 140 mg/dl on two separate days. She is on no prescribed medications. On physical exam, her BMI is 36 and blood pressure is 165/96 mm Hg. The patient has mild facial acne, and purple abdominal striae, approximately 1 cm wide. Her face is full, abdomen is prominent, and her arms and legs are disproportionately thin.

Which of the following is the most appropriate next step in the evaluation of this patient?

a. 0800 serum cortisol preceded by 8 mg of Dex the prior eveningb. 24-hour urinary free cortisolc. Cortrosyn stimulation testd. 2300 ACTH levele. MRI of the head

• A 51-year-old man is evaluated for recent onset of frontal headaches, along with diffuse abdominal discomfort. Review of systems showed a 12-lb weight gain, fatigue, muscle weakness, and depression. On physical examination, he is 75 inches tall and weighs 205 lbs; his pulse rate is 88/ min, and blood pressure is 170/95 mm Hg. He has a round plethoric face and supraclavicular and posterior cervical fullness, benign abdominal exam, no petechiae, and a few ecchymotic spots on the lower extremities. Neurologic exam is normal, except for 3/5 strength in the proximal leg muscles. Abdominal CT scan revealed a 2 cm adrenal mass. A 0800 plasma cortisol was 26 mcg/dl and ACTH level was 2 pg/mL.

Which of the following is the most likely diagnosis?

a. Subclinical Cushing's Diseaseb. Cushing's Syndromec. Hemochromatosisd. Cushing's Diseasee. Small vessel vasculitis

• The incidence of endogenous CS is:

a. 2 to 3 in 100b. 2 to 3 in 1,000c. 2 to 3 in 10,000d. 2 to 3 in 100,000e. 2 to 3 in 1,000,000

• A 54-year-old woman was admitted with polyphagia, 50 lb weight gain, and a four month history of generalized edema, weakness, fatigue, dyspnea, and facial hirsutism. She also had a nine-month history of type 2 DM treated with sulfonylurea. Physical exam revealed a blood pressure of 160/98 mm Hg. She had moon facies, truncal obesity, and lanugo type hair on her face. She had a trace of pretibial edema. There were no striae, acne, or skin atrophy. Laboratory results: 8 am serum cortisol= 32.6 µg/dl; 4 pm serum cortisol = 34.2 µg/dl; 24-hour UFC = 399 µg; 8 am plasma ACTH, undetectable. CT scan of the abdomen revealed a 12-cm right suprarenal mass.

What is the most appropriate next step?

a. Right adrenalectomyb. Mitotanec. Pituitary irradiationd. Ketoconazolee. Obtain an MRI

• A 45 yrs old man is being evaluated for Cushing's syndrome.He has central obesity,hypertension,diabetes mellitus,acne and proximal muscle weakness. He has a protuberant abdomen and some purplish stretch marks on the abdomen. He says that he drinks about 1alcoholic drink daily, although his wife says that he drinks much more than that. A 24 hours UFC was increased to X 3 normal. 8 AM Plasma cortisol after 1 mg Dexamethasone at 11 PM the night before was 5 µg /dl.

You suspect that he may have "pseudo cushing's".Which of the following tests would be most useful to differentiate Pseudocushing's from true hypercortisolism.

a. Repeat overnight dexamethasone testb. MRI of the pituitary glandc. CT scan of the adrenal to exclude an adrenal adenomad. 2mg Dexamethasone -CRH stimulation teste. Plasma free cortisol at midnight

• A 34-year-old man is convinced he has Cushing's syndrome. Over the past five years, he has become increasingly withdrawn associated with a 45- lb weight gain despite dieting and exercise and the development of red striae on the abdomen and flanks. His physical exam reveals a blood pressure of 150/96 mm Hg, a dorsocervical fat pad, acne, and thin purplish striae. His 24-hour urinary free cortisol measures 200 µg and 220 µg (Normal up to 45mcg). Plasma ACTH is 23 pg/ml, and 0800 serum cortisol =18 µg/dl.

Which of the following tests should be obtained next?

a. BIPSSb. Bilateral adrenal vein samplingc. 8 mg Dex suppression testd. 1 mg overnight Dexamethasone suppression teste. Pituitary MRI

• A 70-year old woman is referred for the evaluation of signs and symptoms consistent with Cushing's syndrome. She has noted easy bruising and lower extremity edema. She has had rheumatoid arthritis for several years and osteoporosis for which she takes alenodronate, 70 mg week, and calcium tablets, 600 mg twice daily. She takes no other medication. On exam, she is not overweight but has a full round face. Her arms and legs show muscular atrophy with proximal muscle weakness and ecchymoses. Laboratory results: Serum electrolytes, normal; 0800 serum cortisol = 2µg/dl; serum ACTH = < 10 pg/ml; and 24-hour UFC, undectectable.

Which of the following is the best procedure to do next?

a. Measure salivary cortisol X 2 b. Perform an overnight 1 mg Dexamethasone suppression testc. Assay urine for synthetic glucocorticoidsd. Perform a Dex suppressed CRH stimulation teste. Perform an 8 mg Dex suppression test

• A 55-year-old man reports fatigue and weakness for the past 6 months associated with occasional blood-tinged sputum. He has a 50 pack-year history of smoking. On physical exam, blood pressure is 150/90 mm Hg. He appears thin and weak His skin appears dark, but no other stigmata of CS. However, The 24-hour UFC is 300 µg. Serum cortisol level is 30 µg/dl at baseline, 28 µg/dl at 0800 after 1 mg Dexamethasone the night before . Plasma ACTH is 280 pg/ml.

What is the most likely source of hypercotisolism in this patient?

a. Adrenal glandsb. Hypothalamusc. Lungsd. Laboratory errore. Pituitary gland

• Which of the following would be the most appropriate patient to evaluate for Cushing's syndrome?

a. A 50-year-old woman who has gained 10 lbs in 9 months after becoming menopausalb. A 25-year-old male who was recently diagnosed with Diabetes Mellitus Type 2 associated with complaints of a 30 lb weight gain, purplish abdominal striae, and acnec. A 45-year-old woman with amenorrhea, hirsutism, BMI= 30, and a childhood history of irregular mensesd. A 74-year-old woman on calcium, vitamin D, and alendronate, who has recently suffered a hip fracturee. A 80-year-old man with complain of headaches and pituitary microadenoma finding on MRI of the brain

• A 34-year-old man with a four-year history of Cushing's disease with a macroadenoma that has been refractory to therapy, despite transphenoidal surgeries. guided by IPSS data that showed a central to peripheral ACTH gradient of 30:1. Despite medical therapy with Ketoconazole he continued to have hypercortisolemia, HTN, DM, and osteoporosis. He underwent bilateral adrenalectomy seven months ago and now complains of visual disturbances and headaches. Physical examination was normal except for a blood pressure of 160/100 mm Hg, and tanned skin. Visual field testing revealed bitemporal visual defect.

Which of the following is the best procedure to do next?

a. Measure a 24-hour UFCb. Refer to a neuro-ophthalmology for formal visual field testingc. Refer to neurosurgeon for repeat transphenoidal pituitary resectiond. Control blood pressuree. Pituitary irradiation