Adrenal Path-Usera Flashcards
What are the 3 parts of the adrenal cortex? What does each secrete?
Capsule (GFR)
Zona Glomerulosa-mineralcorticoids, aldosterone
Zona Fasciculata-glucocorticoids-cortisol
Zona Reticularis-sex hormones
What is the adrenal medulla derived from? What does it secrete?
neural crest cells
catecholamines, like NE, Epi
Describe how tyrosine becomes catecholamines?
Tyrosine–>DOPA–>dopamine->NE–>Epi
What are 3 forms of adrenocortical insufficiency?
Acute adrenocortical insufficiency
Chronic adrenocortical insufficiency (Addison’s disease)
Adrenogenital syndrome (congenital adrenal hyperplasia)
What can cause acute adrenocortical insufficiency?
Abrupt withdrawal of corticosteroids
Adrenal Hemorrhage–
1.Anticoagulation therapy
2.Waterhouse-Friderichsen syndrome
What is waterhouse friderichsen syndrome?
bilateral adrenal hemorrhage
associated with neisseria meningitidis sepsis
endotoxic shock with DIC
**can cause acute adrenocortical insufficiency
80% of chronic adrenocortical insufficiency cases are ______.
autoimmune
Other causes of chronic adrenocortical insufficiency?
INfectious–TB, histoplasmosis
metastatic cancer (from lung or renal cell carcinoma)
AIDS
adrenogenital syndrome
What are the clinical findings in chronic adrenal insufficiency?
weakness
hypotension
hyperpigmentation (from too much POMC released w/ ACTH)
What are the lab findings for chronic adrenal insufficiency?
Hyponatremia-no aldosterone Hyperkalemia Metabolic acidosis (normal anion gap)
Other findings in chronic adrenal insufficiency?
Fasting hypoglycemia Lymphocytosis, eosinophilia, neutropenia Increased acth No response to acth stimulation test
Why do you see hypoglycemia and eosinophilia in chronic adrenal insufficiency?
cortisol released from adrenal cortex is gluconeogenic & sequesters eosinophils in lymph nodes.
W/o this–>hypoglycemia & lots of eosinophils in the blood stream.
Once again, why is there hyponatremia, hyperkalemia, and metabolic acidosis with chronic adrenal insufficiency?
Aldosterone causes you too keep Na+, get rid of K+ and get rid of some H+.
W/o it: hyponatremia, hyperkalemia, metabolic acidosis
What is the metyrapone test?
Inhibits 11ß-hydroxylase and prevents cortisol synthesis
Should result in acth secretion and ↑steroid precursors
↑Acth and no increase in 11-deoxycortisol indicates adrenal insufficiency
What causes adrenogenital syndrome/congenital adrenal hyperplasia?
aut rec disorders of enzymes
21-hydroxylase deficiency
11-hydroxylase deficiency
17-hydroxylase deficiency
Which ones of the enzyme deficiencies is the most common cause of adrenogenital syndrome? 90-95% of cases?
21-hydroxylase deficiency
Which enzyme deficiency causes hypogonadism w/ adrenogenital syndrome?
17-hydroxylase deficiency
What happens in adrenogenital syndrome?
Causes ↑acth, and accumulation of precursors proximal to enzyme block
Increased 17-ketosteroids
testosterone
What is the presentation of 21-hydroxylase and 11-hydroxylase deficiency?
Precocious puberty in males, and females
Rapid growth as children, but short stature as adults
What is the presentation of 17-hydroxylase deficiency?
Decrease in Glucocorticoids and androgens
“salt retainers” -b/c still have aldosterone
Females: delayed menarche and 2˚ sexual characteristics
Males: “pseudohermaphroditism”
What are some examples of adrenocortical hyper function?
Cushing Syndrome
Hyperaldosteronism (Conn’s Syndrome)
What are some things that can cause Cushing Syndrome?
Cushing Disease–ACTH secreting pituitary adenoma
prolonged corticosteroid use
hyperfunctional adrenal adenoma
ectopic ACTH production
What is involved in Cushing’s syndrome?
moon facies buffalo hump easy bruising abdominal weight gain striae (weakened collagen, rupture bv) hirsutism (increased androgens) diastolic HTN (increased mineral corticoids) weakness
Why the weight gain in Cushing’s syndrome?
Hyperinsulinism 2˚ to hyperglycemia
Why the muscle weakness in Cushing’s?
muscle breakdown b/c of gluconeogenesis
What are the lab findings in Cushing’s Syndrome?
increase in urine free cholesterol
dexamethasone suppression test
hyperglycemia
hypokalemic metabolic alkalosis (too much aldosterone)
What happens in the dexamethasone suppression test for Cushing’s disease?
–in Cushing disease, only high doses can suppress cortisol in cases of ACTH-secreting pituitary adenoma
What is Conn’s Syndrome?
Big yellow benign tumor
ALDOSTERONE SECRETING ADRENAL ADENOMA–in adrenal cortex, zona glomerulosa
What are the clinical findings in Conn’s Syndrome?
diastolic HTN
muscle weakness
What are the lab findings in Conn’s syndrome?
Too much aldosterone
HYPERNATREMIA, HYPOKALEMIA, ALKALOSIS
↓PLASMA RENIN
What are 2 types of adrenal medullary hyper function?
pheochromocytoma
neuroblastoma
What is a pheochromocytoma?
catecholamine secreting tumor in medulla
secretes Norepi & epi
What is the Rule of 10s for pheochromocytoma?
Rule of 10’s
10% bilateral
10% malignant
10% extramedullary (paraganglioma)
What does a paraganglioma produce? Where is it located? What is present in adrenal medulla pheochromocytoma but not in paraganglioma?
paraganglioma–only produces Norepi
located next to sympathetic ganglia in posterior space of thoracic & abdominal cavities
NO phenylethamin-n-methyltransferase
Which genetic things are associated with a pheochromocytoma?
nf-1
men iia and iib
Von hippel lindau syndrome
Pheochromocytoma means increased sympathetic tone. What are the clinical findings?
*Paroxysmal and/or sustained systolic hypertension with palpitations Headache *sweating *Anxiety Chest pain Orthostatic intolerance constipation
Why are palpitations, sweating, and anxiety important symptoms of pheochromocytoma?
b/c these are symptoms that differentiate pheochromocytoma from essential hypertension.
What are the lab findings of pheochromocytoma?
Plasma free metanephrines – best confirmatory test
*24-hour urine metanephrines – best sensitivity
24-hour urine vma
No suppression with clonidine
Hyperglycemia
Neutrophilia
What is involved in the surgery for pheochromocytoma?
can’t touch it easily b/c it can secrete a lot of catecholamines
need to have an alpha inhibitor (like clonidine), & a beta blocker
What are metanephrines?
by-product of catecholamine synthesis
see increase w/ paraganglioma, adrenal medulla pheochromocytoma
What is a neuroblastoma?
Malignant neoplasm of post-ganglionic sympathetic neurons
Common in children, <5 yrs old
After doing cytologic studies…what makes for a better prognosis for neuroblastoma?
Good: <1 yr old, hyper diploid
hyper diploid means more activity, better response to chemotherapy
After doing cytologic studies…what makes for a worse prognosis for neuroblastoma?
N-myc amplification, 20-30% of cases, marker of aggressive behavior
Bad: >1 yr old, 1p deletion
What % of neuroblastoma cases are metastatic when they are first detected?
70% metastatic
When you have to do cytologic studies on a neuroblastoma what fluid must you NOT put it in?
formalin
What is the prognosis of neuroblastoma cases?
overall survival 40%
What is the outside evidence of neuroblastoma?
large palpable abdominal mass
What is the lab finding for neuroblastoma?
increased urine VMA & HVA
What does neuroblastoma look like on histo?
small round blue cell tumor
Homer-wright rosette
