Ch. 575 - Adrenocortical Insufficiency Flashcards
Acquired primary adrenal insufficiency
Addison disease
Adrenal insufficiency: Deficiency of ACTH
Secondary adrenal insufficiency
MCC of adrenocortical insufficiency
Salt-losing forms of CAH
Conditions that manifest as salt-losing symptoms in newborn period because they are unable to synthesize either cortisol or aldosterone
21-hydroxylase deficiency, lipoid adrenal hyperplasia, 3β -hydroxysteroid dehydrogenase deficiency
Adrenocortical deficiency associated with demyelination in the CNS
ALD
T/F MC form of ALD is X-linked
T
Chronic adrenal insufficiency, isolated deficiency of glucocorticoids, elevated ACTH, generally normal aldosterone
Familial glucocorticoid deficiency
MCC of Addison disease
Autoimmune destruction of adrenal glands
MC infectious etiology for adrenal insufficiency
Meningococcemia
Adrenal crisis from meningococcemia
Waterhouse-Friderichsen syndrome
Most definitive test for primary adrenal insufficiency
Measurement of serum levels of cortisol before and after administration of ACTH
When congenital adrenal hyperplasia is suspected, ___ should be measured along with cortisol in the ACTH stimulation test
Cortisol precursors
Management of acute adrenal insufficiency
IV solution of D5NSS (correct hypogly, hyponat, and hypovolemia), IV hydrocortisone
Used to monitor adequacy of glucocorticoid replacement in primary adrenal insufficiency
ACTH levels
During periods of stress, dose of hydrocortisone for patients with primary adrenal insuff should be increased by
2-3x
Synthetic mineralocorticoid given orally for patients with aldosterone deficiency
Fludrocortisone
High-dose glucocorticoids is defined as
> 10x physiologic
High-dose glucocorticoids can be administered for how long without requiring a subsequent taper of dose
1 week
MCC of corticotropin deficiency
Destructive lesions in the area of the pituitary e.g. craniophayrngioma and germinoma
Up to 60% of children with ___ have secondary adrenal insufficiency
Prader-Willi syndrome
Gold standard to test for secondary adrenal insufficiency
Insulin-induced hypogly BUT too dangerous; second gold std uses metyrapone
MC used test to diagnose secondary adrenal insufficency
Low-dose ACTH stimulation testing
Physiologic dose of hydrocortisone
10mg/BSA/24h