20 - Adrenal and Thymus path Flashcards
what is made in adrenal medulla
catecholamines
layers of adrenal cortex from outer to inner
GFR - glomerulosa, fasciculata, reticularis
stress response and adrenal gland path
acute stress in adults - lipid depletion in adrenal cortex
chronic stress - adrenocortical hyperplasia
acute stress in neonates - lipid accumulation in cortex and pseudoglandular change in zona glomerulosa
MC source of ectopic ACTH
small cell lung tumor
MC cause of primary hyperaldosteronism
Conn syndrome (adenoma making aldosterone)
congenital adrenal hyperplasia - inheritance/mutation, pathophys
AR
usually def or absence of 21-hydroxylase (CYP21B gene defect)
> dec cortisol +/- aldosterone, excess androgens (alt means of synthesis) > inc ACTH via feedback > adrenocortical hyperplasia
congenital adrenal hyperplasia - clinical features
variable severity
androgen excess - ambiguous genitalia at birth (esp females), precocious puberty in males
variable aldosterone def - can have salt wasting
Waterhouse-Friderichsen syndrome
bacteremia (most often n. meningitidis but can be pseudomonas, s pneumo, h flu, or staph) causes massive adrenal hemorrhage > adrenal crisis
autoimmune polyendocrinopathy syndrome type 1
chronic mucocutaneous candidiasis, skin/nail/enamel abnl, hypoadrenalism, hypoPTH, hypogonadism, pernicious anemia
AIRE gene
autoimmune polyendocrinopathy syndrome type 2
adrenal insufficiency, autoimmune thyroiditis, T1DM
Beckwith-Wiedemann syndrome
syndrome assoc w/ adrenocortical CA
pheo presentation
paroxysmal BP elevation w/ tachycardia, HA, sweating, tremors, apprehension
pheo rule of 10s
10% familial syndrome (MEN2a,2b, NF1, VHL, SW) 10% extraadrenal 10% bilat 10% malignant behavior 10% childhood
MEN1
parathyroid
pancreas
pituitary
(also gastrinoma in duodenum, carcinoid tumors, thyroid and adrenal adenomas, lipomas)
MEN2a
medullary thyroid CA (100%)
pheo
PTH hyperplasia
