Adrenocortical Hyperfunction

Question 1

hypercortisolism is called? labs?

Answer 1

Cushing’s Syndrome; too much cortisol, not much ACTH

Question 2

most common etiology of Cushings

Answer 2

iatrogenic

Question 3

etiology of endogenous cushing’s

Answer 3

cushing’s disease; Primary adrenal hyperplasia or neoplasia; non-endocrine tumors that produce ACTH

Question 4

most common endogenous; epidemiology

Answer 4

cushing’s disease; 5:1 F:M 20-40yo, usually no mass effect;

Question 5

what is a no mass hyperplasia?

Answer 5

corticotroph hyperplasia either primary or secondary (pituitary defect, secondary is hypothalamic defect - too much CRH)

Question 6

histologic appearance of cushing’s disease

Answer 6

hyperplasia of ZR, ZF, compression of ZG unaffected medulla; hyperplastic cells are more vacuolated

Question 7

gross appearance of cushing’s disease

Answer 7

brown or yellow (lipid-rich) cortical thickening diffusely - scattered nodules .5 - 2.5 cm ; weigh up to 15g

Question 8

second most common cause of endogenous cushing’s syndrome

Answer 8

primary adrenocortical hyperplasia and neoplasia (15%)
AKA ACTH-independent Cushing syndrome or adrenal Cushing syndrome
low level ACTH (negative feedback)

Question 9

what are the characteristics of primary bilateral hyperplasia?

Answer 9

hyperplasia is nodular, micro/macro nodular 3mm cutoff
micro is familial - pigmented - large cortical cells
other cells atrophied
macro - weigh up to 50g

Question 10

what are teh characteristics of adrenocortical neoplasia

Answer 10

can be benign or malignant; nearly always unilateral; ~3 cm, encapsulated, yellow to black and usually weigh <60g not always functional

Question 11

non-endocrine neoplasms that make ACTH characteristics

Answer 11

small cell lung cancer, medullary thyroid cancer, pancreatic islet tumor, carcinoid tumor, ovarian tumors and thymic carcinomas
can secrete ACTH, CRH, or mimics

Question 12

hypercortisolism manifestations

Answer 12

HTN, weight gain, truncal obesity, fat depo (head and neck), atrophy, gluconeogensis, protein catabolism, cutaneous stria, immunosuppression, hirsuitism, depression, hyperpigmentation, osteoporosis, easy bruising,

Question 13

primary hyperaldosteronism features and etiology

Answer 13

volume overload, HTN, hypoK, hyperNa, low renin and angiotensin
most common etiology adenoma, then carcinoma; adrenal hyperplasia, idiopathic

Question 14

what is an aldo secreting adenoma in the adrenal? epidemiolgy?

Answer 14

Conn’s syndrome; 30-50 yo women

Question 15

usual clinical picture of Conn’s

Answer 15

no suppression of ACTH, no atrophy; new onset HTN

can be treated resection (unilateral adrenalectomy)

Question 16

primary adrenocortical hyperplasia epidemiolgy, histo picture and potential cause

Answer 16

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)

Question 17

what does CYP11B2 do?

Answer 17

deoxycortisone to aldosterone

Question 18

what are the three etiologies of secondary hyperaldosteronism?

Answer 18

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

Question 19

features of adrenogenital syndrome?

Answer 19

can be pure or mixed (mixed are associated with Cushing’s syndrome)
large (up to 500g)

Question 20

etiologies of adrenogenital syndrome and complications

Answer 20

adrenal adenomas, carcinomas, hyperplasias

virilization (carcinomas) rarely feminization

Question 21

what’s another cause of androgen excess? features

Answer 21

CAH; AR, most often affects 21-hydroxylase (CYP21B); results in hypocortisolemia -> increased ACTH release; ACTH promotes androgens instead and results in virilization

Question 22

what is a mixed CAH picture look like?

Answer 22

combined with aldosteronogenic enzyme deficiency, patients are virilized and hypoN and/or hyperN

Question 23

histo features of CAH

Answer 23

bilateral enlargement 10x, nodular, lipid depletion

Question 24

clinical manifestation of CAH

Answer 24

females: pseudohermaphroditism, oligomenorrhea, hirsutism
boys: percocious puberty and big genitals
MC deficiency if 21-hydroxylase (hypoN)
MC production if 11-B hyroxylase (hyperN)

Question 25

other things that can cause virilization

Answer 25

ovarian tumors that produce androgens and drugs