questions 5

Cushing's Syndrome / Disease

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Topic updated on 05/14/17 4:21pm

Snapshot
  •  A 42-year-old obese male who does not smoke, presents with diastolic hypertension. Physical exam shows a full, plethoric appearing face, increased facial hair, truncal obesity, and purple stria around the abdomen with scattered echymosis over the entire body. Labs show an HgB of 18 (12-16), a WBC of 18,000 (4,500-11,000) . The leukocyte differential shows and absolute neutrophillic leukocytosis and absolute lymphopenia and eosinopenia. CXR is normal.
Introduction
  • A condition that refers to the manifestations of hypercortisolism
  • Iatragenic and pituitary adenoma are most common causes

    Cushings Syndrome
    Iatrogenic Cushings

    patients taking steroids is the most common cause of Cushings Syndrome.

    low ACTH  
    Pituitary Cushings
    Pituitary Adenoma

    most common pathogenic cause (70%), the majority of adenomas are benign.

    high ACTH
    Cortisol responds to dexamethasone supression test 
    Ectopic Cushings
    Small cell carcinoma of the lung.

    ectopic ACTH secretion

    extremely high ACTH
    Cortisol does not respond to dexamethasone supression test
    Adrenal Cushings
    Adrenal adenoma   low ACTH  
    Bilateral hyperplasia   low ACTH  
    Adrenal Carcinoma   low ACTH  
Presentation
  • Symptoms
    • depression and psychological changes
    • oligomenorrhea
    • growth retardation
    • weakness
    • acne
    • excessive hair growth
    • symptoms of diabetes (polydipsia, polyuria, dysuria)
  • Physical exam
    • hypertension 
    • central obesity
    • muscle wasting
    • thin skin that easily bruises
    • purple striae
    • hirsutism
    • moon facies
    • buffalo hump
Evaluation
  • Labs
    • screen for 24 hour free urinary cortisol as well as a serum cortisol level
    • ACTH to localize lesion
    • high dose dexamethasone supression test (localize ACTH high disease to pituitary)
    • hyperglycemia
    • hypokalemia and hypernatremia
  • CT and MRI to localize lesions
  • Inferior petrosal sinus sampling
Differential
  • Chronic alcoholism, depression, diabetes mellitus, adrenogenital syndrome
Treatment
  • Resection of source
  • For non resectable tumors:
    • ketoconazole (inhibits P450)
    • aminoglutethimide (inhibits P450)
    • metyrapone (blocks adrenal enzyme synthesis)
    • mitotane (adenolytic)


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Qbank (2 Questions)

TAG
(M2.EC.4) A 47-year-old male has been feeling fatigued and has gained 20 pounds during the last two months. On exam, you note central obesity with proximal muscle atrophy and weakness. Vital signs are as follows: T 98.4 F, BP 155/90 mmHg, HR 85 bpm, RR 14 rpm. BMP is as follows: Na 140, K 3.9, Cl 102, CO2 23, BUN 19, Cr 0.9, Glu 115. 24-hour urinary free cortisol level is 165 nmol/day (upper range of normal 110-138 nmol/day) and morning serum ACTH is 83 pg/mL (normal is 9 - 52 pg/mL). Two days later, he receives a high-dose 2 mg dexamethasone suppression test orally every 6 hours for 48 hours. The following morning at 9 am, his serum cortisol level is 300 nmol/L (normal is 50-200 nmol/L). At 48 hours it is 250 nmol/L. Which of the following should be the next step? Topic Review Topic

1. Bilateral inferior petrosal sampling
2. Initiate treatment with metyrapone
3. Initiate treatment with ketoconazole
4. CT of the thorax and abdomen
5. Brain MRI

PREFERRED RESPONSE ▶
TAG
(M2.EC.33) A 69-year-old presents with granulomatosis with polyangiitis (formerly Wegener's) diagnosed about 8 months ago. He was treated with rituximab and prednisone for induction remission and has required prednisone since his diagnosis. Her temperature is 37 deg C (98.6 deg F), pulse is 80/min, blood pressure is 150/90 mmHg, respirations are 14/min, and O2 saturation is 99% on room air. His physical examination is notable for the findings in Figures A and B. What would be the most likely electrolyte abnormality found in this patient? Topic Review Topic
FIGURES: A   B        

1. Hypokalemia and hyponatremia
2. Hyperkalemia and hypernatremia
3. Hypokalemia and hypernatremia
4. Hyperkalemia and hyponatremia
5. Hypercalcemia and hypernatremia

PREFERRED RESPONSE ▶
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