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:
2B:
|
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 |
|
Pheochromocytoma |
|
Cushing’s syndrome (can be subclinical) |
|
Hyperaldosteronism |
|