Endocrine: Adrenal Glands Flashcards
“largest zone of the adrenal cortex”
Zona fasciculata
What organelles are abundant in steroid producing cells?
SER and mitochondria
Why is there Hyperplasia in CAH?
because of the unopposed ACTH effect on the gland
Which zone is the most hyperplastic in CAH?
Zona fasciculata
“female has psuedohermaphroditis and sterile as an adult, male has short stature and azospermia”
Simple virilizing congenital adrenal hyperplasia
21 hydroxylase deficiency
“virilization and hyponatremia, hyperkalemia, dehydration, hypotension, increased renin”
Salt-wasting CAH
21 hydroxylase deficiency
“no abnormalities at birth, virilization at puberty”
Late-onset congenital adrenal hyperplasia
21 hydroxylase deficiency
“high levels of androgen and 11-deoxycortisol, Hypernatremia, hypertension, seen in Jews”
11 beta hydroxylase deficnecy
“autoimmune destruction of the adrenal gland”
Addison’s disease
Aside from autoimmune, what is another common cause of addisons?
TB!
What type of immune problem is addisons?
T cell mediated damage…there are multiple HLA links
What are the symptoms of addisons?
Weight loss, Hypotension, salt wasting, hyperpigmentation, GI symptoms, hymphocytosis and eosinophelia
“adrenal insufficiency, hypoparathyrodism, chronic mucocutaneous candidiasis, type I diabetes, vitiligo, hypothyroidism”
Type I polyglandular autoimmune syndrome
“adrenal insufficiency, women, HLA-DR3, hashimoto’s, graves, T1DM, premature ovarian failure, more common”
Type II polyglandular autimmune syndrome
“acute, bilateral, hemorrhagic infarction of the adrenal cortex, due to meningococcus or psuedomonas”
Waterhous- Friderisch Syndrome
Most common cause of acute adrenal insufficiency?
Abrupt withdrawal of corticosteroids
Most cases of ACTH dependent adrenal hyperfunction (cushing) are cuased by?
Ectopic productino of ACTH
What syndromes are adrenal adenomas associate with? (3)
MEN 1, Carney complex, McCune- Albright Syndrome
Most common cause of cushing syndrome?
chronic corticosteroid use
“pigmented skin lesions, myxomas, testicular tumors, pituitary GH tumors, lipofuscin laden cells in the adrenals”
bilateral adrenal microndular hyperplasia/ Carney complex
“inappropriate secretion of sldosterone due to adrenal adenoma or hyperplasia”
Conn Syndrome (hyperaldosteronism)
“cells derived from primitive pheochromoblasts of the developing SNS”
Chromaffin cells
Blood supply of the adrenal medulla?
portal system blood!
Key symptoms of pheochromocytoma?
HTN
What syndromes can Pheo’s be associated with?
MEN 2A/B, von Hippel- Lindau disease, neurofibromatosis Type I, McCune-Albright Syndrome
“HTN, paroxysmal convulsions/ anxiety/ hyperventilation/ throbbing headache”
Pheo
Classic histologic fundings in pheo?
Zellballen cells…circumscribed nests of neoplastic cells
Pheo’s stain (+) for…
enolase (+)
Chormogranin (+)
Syndaptophysin (+)
“Pheochromocytomas arising in extra-adrenal sites”
Paraganglioma
Who are carotid body tumors more commonly found in?
people living in high altitudes
Mutation in MEN 1 and MEN 2
MEN1= MEN1 tumor suppressor gene
MEN2= RET protoncogene
“constellation of pituitary adenoma, parathyroid hyperplasia/ adenoma, islet cell tumors of the pancreas”
MEN type I
“constellation of medullary thyroid carcinoma, pheochromocytoma, hyperparathyroidism”
MEN type 2A
“constellation of mucosal neuroma syndrome, marfanoid habitus (long fingers and limbs)”
MEN type 2B
Most common solid extracranial neoplasms of children”
neuroblastomas
How are neuroblastomas diagnosed?
Urine levels of norepi, VMA, HVA and DA
Histology seen in neuroblastomas?
Homer-Wright Rosettes, sheets of small, dark, lymphocyte like cells
“mature varient, neural crest tumor, older children/ young adults, benign tumors that arise from sympathetic ganglia”
ganglioneuroma
