Endocrinology Week 5 Summary

Question 21

How is the presentation different between patients with primary versus secondary cortisol deficiency?

Primary Secondary
Hyperkalemia,
Hyperpigmented,
May have other autoimmune diseases or another reason for adrenal failure,
High ACTH
May have evidence of sellar space-occupying lesion,
May have other pituitary hormone deficiencies or excesses,
ACTH is not elevated

(Both have hyponatremia)

Question 22

How do you counsel patients with Addison’s disease? List 5 items.

  • It is very important to take your medications (cortef, florinef) every day
  • If you get sick:
    • Double or triple your dose of cortef until you are well
    • See a doctor if you still need a higher dose after 3-5 days
  • If you cannot keep medications down despite Gravol, go to emergency room for intravenous steroids
  • Get a Medic Alert bracelet
    • High dose of steroids will be needed for bad accidents or surgery
  • Fill a prescription for injectable dexamethasone and needles if going to remote areas

Question 23

List 4 genetic syndromes which are associated with pheochromocytoma. What are the other manifestations of these syndromes?

Syndrome Other manifestations
MEN 2A and 2B 2A:

  • hyper PTH
  • medullary thyroid cancer (MTC)
  • pheo

2B:

  • MTC
  • pheo
  • mucosal neuromas
Von Hippel Lindau Renal cell carcinoma,
hemangioblastoma (retina, cerebellar),
endolymphatic middle ear tumours,
pancreatic tumours
Neurofibromatosis-1 Café au lait spots, neurofibromas
SDHA, SDHB, SDHC, SDHD Paragangliomas (extra-adrenal pheos), SDHB: renal cell ca

Question 24

Sarah Hughes is a 45-year old woman with new onset hypertension 180/110. Her blood pressure remains elevated despite the use of three antihypertensive agents.

List 5 lifestyle questions you would ask her.

  • Salt intake
  • Alcohol intake
  • Caffeine
  • Smoking adherence to medications
  • Licorice ingestion
  • Use of NSAIDS
  • Cocaine
  • Amphetamines
  • Cold medications
  • Oral contraception

Her potassium is low at 2.8 mmol/L, and you suspect hyperaldosteronism. How do you screen for hyperaldosteronism? How would you confirm the diagnosis of primary hyperaldosteronism?

Screen:

  • Elevated serum aldosterone (>400 nmol/L);
  • Elevated ratio of aldosterone:renin >140 (100-140 “grey zone”) (using renin measured as mass not activity, beware of units!)

Confirm with saline suppression test: 2 L saline infused IV over 4 hours, plasma aldosterone afterwards elevated >280 nmol/L

CT image (hyperplasia vs adenoma)

Adrenal vein sampling if image is confusing, or patient is >45 years old

Question 25

How do you investigate the patient with an adrenal incidentaloma?

Potential concerning issue Problem if:
Malignancy
  • Size >4cm
  • CT characteristics not consistent with adenoma
  • Known primary malignancy
  • Virilization with DHEAS
Pheochromocytoma
  • Symptoms of triad: pain (headache), palpitations, perspiration
  • Hypertension: 50% will have intermittent
  • 24h urine collection: creatinine, metanephrines, and/or catecholamines
Cushing’s syndrome (can be subclinical)
  • Symptoms and signs
  • 1mg dexamethasone suppression test:
    • Drug is taken between 11PM and midnight
    • Measure cortisol at 8AM following morning
    • >50 nmol/L is abnormal
Hyperaldosteronism
  • Only screen if patient is hypertensive or hypokalemic
  • Screen for aldosterone/renin ratio, aldosterone

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