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41 Cards in this Set
- Front
- Back
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most common cause of cushing syndrome
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corticosteroid therapy
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most common cause of endogenous cushing syndrome
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cushing disease (pituitary adenoma secreting ACTH)
2ndary disease primary hypercortisolism is not as common |
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exogenous cushing will see hyperplasia or atrophy?
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atrophy
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endogenous cushing will see hyperplasia or atrophy?
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hyperplasia
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crooke hyaline change
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excess glucocorticoids -> accumulation of intermediate keratin filaments in basophils
normally basophilic cytoplasm becomes homogenous and pale |
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clinical features of cushings
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moon faces
truncal obesity buffalo hump weakness, fatigue glucose intolerance loss of collagen - osteoprosis, skin stretching hypertension |
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conn syndrome
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hyperaldosteronism caused by adrenal adenoma
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most common cause of primary hyperaldosteronism
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idiopathic
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2ndary hyperaldosteronism
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due to activation of renin-angiotensin system
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clinical features of hyperaldosteronism
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increased Na+, decreased K+
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primary adrenal disorders of sexual differentiation causes
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either due to neoplasia or congenital adrenal hyperplasia
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congenital adrenal hyperplasia is autosomal dom or rec?
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autosomal recessive
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most common form of congential adrenal hyperplasia?
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21 hydroxylase deficiency
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21 hydroxylase deficiency
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see increased androgens
decreased aldosterone decresaed cortisol --> virilization salt-wasting see hyperplasia of adrenals and pituitary corticotroph hyperplasia |
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21 hydroxylase deficiency in boys
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infants will see external genital hypertrophy
children will present with precocious puberty |
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21 hydroxylase deficiency in girls clinical features
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infants will have virilization -> ambigious genetalia
as a child - will show masculization |
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adrenal cortical insufficiency causes
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exogenously - by rapid withdrawl from corticosteroids
endogenously primary: adrenal crisis if acute, addison disease if chronic secondary: pituitary disease, hypothalamic disease |
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adrenal crisis causes
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rapid removal from exogenous corticosteroids
stress superimposed on addison's disease massive adrenal hemmorhage - neonatal hypoxia postoperative DIC waterhouse-friderichsen (Neisseria meningitidis) |
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addison's disease
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primary CHRONIC adrenocortical insufficiency
cause most often autoimmune om the US TB is #1 cause world wide mets to adrenals AIDS -> CMV -> adrenal damage clinical: look for hyperpigmentation of skin and mucous membranes |
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difference between primary and secondary adrenal cortical insufficiency
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2ndary is caused by decreased ACTH so don't see hyperpigmentation or salt wasting
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most common adrenal tumor
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metastasis
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medullary tumors of adrenal gland
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pheochromocytoma
neuroblastoma |
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T/F adrenocortical neoplasms morphology shows no correlation with function
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true
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T/F most adenomas are functional
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false: most are hormonally silent
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T/F most carcinomas are functional
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true
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adernal Adenomas that are functional usually secrete
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aldosterone or cortisol
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adrenal carcinomas usually secrete
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androgens
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adrenocortical adenoma
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yellow, well circumscribed, small (<50grams)
cortisol producing tumors will look atrophic, all others look normal endocrine atypia |
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adrenocortical carcinoma
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larger than adenoma, poorly demarcated, necrosis, hemorrhage, invasion, metastasis
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pheocromocytomas involves which kind of cell
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chromaffin cell
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pheochromocyoma
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tumor of adrenal medulla
secrete catecholamines -> hypertension 10% malignant 10% bilateral (nonfamilial) 10% extra-adrenal yellow to red, well demarcated, with hemorrhage zellballen - clusters of chromaffin cells if spindle cell apperance = malignant look for metanephrins in urine and high catecholamines in plasma |
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paragangliomas
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extra-adrenal pheochromcytomas
occurs anywhere there is paraganglionic tissue - organ of zuckerkandl near inferior mesenteric artery, urinary bladder, carotid body jugulotympanic body |
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MEN 1
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pituitary adenoma
pancreatic adenoma parathyroid tumor 3 Ps MEN1 gene |
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MEN 2a
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aka sipple disease
1 medullary thyroid carcinoma 2 parathyroid hyperplasia 3 pheochromocytoma can see paraganglioma RET gene associated |
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MEN 2a vs 2b
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same as MEN2a except see marfanoid habitus (Connective tissue problem)
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von-reckling hausen syndrome
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associated with NF1
pheochromocytoma neurofibromatosis cafe au lait spots lisch nodules |
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von hippel lindau
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VHL gene mutation
visceral cyst renal cell carcinoma hemangioblastoma pheocrhomocytoma |
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neuroblastoma
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small, round, blue cell tumors
2nd most common malignancy of children (1st are brain tumors) look for VMA and HVA in urine prognostic factors: age (infants do better) stage n-myc (poor) |
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adrenal incidentaloma
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incidental adrenal mass in asymptomatic person
usually a nonfunctioning Adernocortical adenoma |
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sources of mets to the adrenals
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lung
breast melanoma kidney |
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organs most commonly reciving mets
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lung
liver bone adrenal |
