Adrenocortical Hyperfunction Flashcards
(25 cards)
hypercortisolism is called? labs?
Cushing’s Syndrome; too much cortisol, not much ACTH
most common etiology of Cushings
iatrogenic
etiology of endogenous cushing’s
cushing’s disease; Primary adrenal hyperplasia or neoplasia; non-endocrine tumors that produce ACTH
most common endogenous; epidemiology
cushing’s disease; 5:1 F:M 20-40yo, usually no mass effect;
what is a no mass hyperplasia?
corticotroph hyperplasia either primary or secondary (pituitary defect, secondary is hypothalamic defect - too much CRH)
histologic appearance of cushing’s disease
hyperplasia of ZR, ZF, compression of ZG unaffected medulla; hyperplastic cells are more vacuolated
gross appearance of cushing’s disease
brown or yellow (lipid-rich) cortical thickening diffusely - scattered nodules .5 - 2.5 cm ; weigh up to 15g
second most common cause of endogenous cushing’s syndrome
primary adrenocortical hyperplasia and neoplasia (15%)
AKA ACTH-independent Cushing syndrome or adrenal Cushing syndrome
low level ACTH (negative feedback)
what are the characteristics of primary bilateral hyperplasia?
hyperplasia is nodular, micro/macro nodular 3mm cutoff
micro is familial - pigmented - large cortical cells
other cells atrophied
macro - weigh up to 50g
what are teh characteristics of adrenocortical neoplasia
can be benign or malignant; nearly always unilateral; ~3 cm, encapsulated, yellow to black and usually weigh <60g not always functional
non-endocrine neoplasms that make ACTH characteristics
small cell lung cancer, medullary thyroid cancer, pancreatic islet tumor, carcinoid tumor, ovarian tumors and thymic carcinomas
can secrete ACTH, CRH, or mimics
hypercortisolism manifestations
HTN, weight gain, truncal obesity, fat depo (head and neck), atrophy, gluconeogensis, protein catabolism, cutaneous stria, immunosuppression, hirsuitism, depression, hyperpigmentation, osteoporosis, easy bruising,
primary hyperaldosteronism features and etiology
volume overload, HTN, hypoK, hyperNa, low renin and angiotensin
most common etiology adenoma, then carcinoma; adrenal hyperplasia, idiopathic
what is an aldo secreting adenoma in the adrenal? epidemiolgy?
Conn’s syndrome; 30-50 yo women
usual clinical picture of Conn’s
no suppression of ACTH, no atrophy; new onset HTN
can be treated resection (unilateral adrenalectomy)
primary adrenocortical hyperplasia epidemiolgy, histo picture and potential cause
children and young adults; hyperplasia of the ZG by cells cells that look like ZF cells
AKA idiopathic hyperaldosteronism
may be due to overactive aldo synthase (CYP11B2)
what does CYP11B2 do?
deoxycortisone to aldosterone
what are the three etiologies of secondary hyperaldosteronism?
decreased blood flow to the kidney - renal artery stenosis or nephrosclerosis
hypovolemia and edema due to secondary disease
pregnancy, b/c estrogen increases renin’s potency
features of adrenogenital syndrome?
can be pure or mixed (mixed are associated with Cushing’s syndrome)
large (up to 500g)
etiologies of adrenogenital syndrome and complications
adrenal adenomas, carcinomas, hyperplasias
virilization (carcinomas) rarely feminization
what’s another cause of androgen excess? features
CAH; AR, most often affects 21-hydroxylase (CYP21B); results in hypocortisolemia -> increased ACTH release; ACTH promotes androgens instead and results in virilization
what is a mixed CAH picture look like?
combined with aldosteronogenic enzyme deficiency, patients are virilized and hypoN and/or hyperN
histo features of CAH
bilateral enlargement 10x, nodular, lipid depletion
clinical manifestation of CAH
females: pseudohermaphroditism, oligomenorrhea, hirsutism
boys: percocious puberty and big genitals
MC deficiency if 21-hydroxylase (hypoN)
MC production if 11-B hyroxylase (hyperN)
