3.2.2 Adrenal Cortex and Medulla Clinical Cases II Flashcards
Where to look for pigmentation in Addison dz
This is a great summary chart. Fill it out
What are two treatment options for the treatment of hyperaldesteronism?
Surgical ressection
Aldosterone receptor antagonists (spironolactone/eplerenone)
What are some treatment methods for the various presentations of CAH 21 OH deficiency?
GC for classic
No GC for asymptomatic
Mineralcorticoids for salt wasting
GC can help hirsutism in non-classic CAH adults
What are some symptoms of chronic adrenal insuffiency?
Weakness, fatigue, anorexia, nausea, abdominal pain, diarrhea, occasional orthostatic dizzineiness/hypotension, weight loss
Primary: salt cravings, pigmentation, and hyperkalemia
Primary adrenal insufficiency due to destruction of adrenal glands
Addison’s Dz
When should you consider adrenalectomy?
Size greater than 4 cm
Evidence of growth
Suspicious findings from CT
Evidence of hormonal secretion
What are the three common features of polyglandular autoimmune syndrome Type I (most common cause of primary AI)?
Hypoparathyroidism, onset childhood AI, Mucocutaneous candidiasis
(HAM)
What are the aldosterone and renin levels in secondary hyperaldosteronism?
Aldosterone: High
Renin: High
Most commonly are clinically non-hypersecreting benign tumors, an adrenal mass >1 cm discovered serendipitously
Adrenal incidentaloma
What precursor is elevated due to a 21-hydroxylase deficiency?
17-OH pregesterone
Describe the ACTH levels in primary/secondary adrenal insuffiency?
Primary: Elevated ACTH
Secondary: Normal or low ACTH
If you positively screen a patient, what are the next steps?
Adrenal imaging and adrenal vein sampling
What are some of the primary AI causes worldwide?
TB, fungi, metastatic cancer, AIDS, waterhouse-friederichson syndrome
Most common cause of secondary AI?
adrenal suppression after exogenous GC
What are some common features of polyglandular autoimmune syndrome type II?
Onset young adults, autoimmune thyroid dz, type 1 diabetes
What are the clinical features in hyperaldosteronism? Who is most commonly effected?
HTN and hypokalemia
Middle-aged women
What some of the common clinical features in 21 OH deficiency?
Females: ambiguous genitalia (large clit, fused labia, comon urogenital sinus)
Boys: No overt signs - hyperpigmentation and penile enlargement
Untreated - rapid somatic growth, advanced bone age, premature epiphyseal fusion and short stature
Have you ever seen the human version of Lady and The Tramp?
Now you have
What is used to diagnose CAH prenatally?
Molecular analysis of CYP21A2 gene
What are the aldosterone and renin levels in primary aldosterone excess?
Aldosterone: High, duh!!!!
Renin: Low
Tests to diagnose adrenal sufficiency
Acute setting: serum cortisol
Check at 8 am cortisol if borderline
ACTH stimulation test
If you incidentally find an adrenal mass, what additional tests would you order?
Size matters: if greater than 4 cm, could be malignant
What might be going on in the hypothalmamic-pituitary system in secondary adrenal insufficiency?
Relative or complete ACTH deficiency
What are some of the clinical features of Addison’s dz?
What conditions is a group of AR disorders resulting from a deficiency of an enzyme (90% are 21 hydroxylase def) required for the synthesis of cortisol in the adrenal cortex), which leads to excessive sex hormone?
Congenital adrenal hyperplasia
Who should be screened for hyperaldosteronism?
HTN and hypokalemia
HTN resistant to Rx
Incidental adrenal nodule on imaging
What is the biochemical testing for Cushing’s Syndrome?
1 mg dexamathasome suppression test
How do we screen for hyperaldosteronism?
Plasma aldosterone: plasma renin ratio in AM
Ratio >20 ng/dl and plasma aldo > 15 ng/dl w/ suppressed renin
Confirmatory test: salt loading and saline infusion test
What are three conditions that can lead to primary aldosterone excess?
Adrenocortical adenoma (Conn’s syndrome), Bilateral idiopathic hyperaldosteronism (IHA), Adrenal carcinoma
These are in order of most to least common.
D. Hyperkalemia
Treatment of AI?
Replace GC w/ hydrocortisone
Replace MC w/ Fludrocortisone (PRIMARY ONLY)
