Notes in 01a Adrenal Function and Dysfunction

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Published	03/04/2024	Can mineralocorticoids still be produced if the mineralocorticoid pathway is blocked below the level of DOC (i.e. absence of 11β-hydroxylase or aldost…
Published	03/04/2024	Can mineralocorticoids still be produced if the mineralocorticoid pathway is blocked above the level of DOC (i.e. absence of 21β-hydroxylase)?{{c1::No…
Published	03/04/2024	The primary regulation of aldosterone secretion occurs through changes in {{c1::ECF volume}} (via the {{c2::renin-angiotensin II-aldosterone}} system)…
Published	03/04/2024	What classes of steroid hormones are increased in Cushing syndrome/disease?{{c1::Glucocorticoids (cortisol) +/- androgens}}
Published	03/04/2024	What classes of steroid hormones are decreased in 21β-hydroxylase deficiency?{{c1::Glucocorticoids and mineralocorticoids::2}}
Published	03/04/2024	{{c1::21β-hydroxylase}} deficiency is the most common cause of congenital adrenal hyperplasia
Published	03/04/2024	What clinical features are seen in 21β-hydroxylase deficiency that are a result of increased androgen levels in females? {{c1::Masculinization (clitor…
Published	03/04/2024	Which adrenal cortex deficiency results in shunting towards the androgen synthesis pathway?{{c1::21β-hydroxylase deficiency}}
Published	03/04/2024	Cushing syndrome simply refers to an increase in serum {{c1::cortisol}}
Published	03/04/2024	What is the most common cause of Cushing syndrome?{{c1::Exogenous glucocorticoids}}
Published	03/04/2024	What is the most common endogenous cause of Cushing syndrome?{{c1::Cushing disease (i.e. an ACTH-secreting pituitary adenoma)}}
Published	03/04/2024	One clinical feature of Cushing syndrome is muscle weakness with {{c1::thin}} extremities
Published	03/04/2024	Clinical features of {{c2::Cushing syndrome}} include moon facies, buffalo hump, and truncal obesity due to increased {{c1::fat storage}}, secondary t…
Published	03/04/2024	One clinical feature of Cushing syndrome is {{c2::abdominal striae}} which is a result of impaired {{c1::collagen}} synthesis, allowing blood vessels …
Published	03/04/2024	One clinical feature of Cushing syndrome is {{c1::hypertension}}, which is a result of an upregulation of {{c2::alpha1-adrenergic}} receptors, causing…
Published	03/04/2024	One clinical feature of Cushing syndrome is {{c1::osteoporosis}}, which is a result of decreased {{c2::osteoblast}} activity
Published	03/04/2024	One clinical feature of Cushing syndrome is {{c1::immune suppression}}, which is a result of inhibition of phospholipase A2, IL-2, and histamine
Published	03/04/2024	One positive screening test for Cushing syndrome is an increased free {{c1::cortisol}} on 24-hour urinalysis
Published	03/04/2024	One positive screening test for Cushing syndrome is an increased midnight {{c1::salivary}} cortisol
Published	03/04/2024	One positive screening test for Cushing syndrome is no {{c1::cortisol suppression}} with overnight low-dose dexamethasone test (1mg)
Published	03/04/2024	If a patient with ACTH-dependent Cushing syndrome has adequate cortisol suppression following a high-dose dexamethasone suppression test (8mg), the mo…
Published	03/04/2024	If a patient with ACTH-dependent Cushing syndrome has no cortisol suppression following a high-dose dexamethasone suppression test (8mg), the most lik…
Published	03/04/2024	{{c1::Primary}} hyperaldosteronism is characterized by high aldosterone and {{c2::low}} renin
Published	03/04/2024	Which steroid synthesis enzyme deficiency results in salt wasting and hyponatremia due to lack of aldosterone? {{c1::21β-hydroxylase deficiency}}
Published	03/04/2024	21β-hydroxylase deficiency presents with {{c1::hyperkalemia::K+}} and {{c2::hypotension::blood pressure}}
Published	03/04/2024	11β-hydroxylase deficiency presents with {{c1::hypokalemia::K+}} and {{c2::hypertension::blood pressure}}
Published	03/04/2024	{{c1::Adrenal insufficiency}} is the inability of the adrenal glands to generate enough glucocorticoids +/- mineralocorticoids
Published	03/04/2024	{{c2::Primary}} adrenal insufficiency presents with {{c1::low}} cortisol and {{c1::high}} ACTH
Published	03/04/2024	{{c2::Pheochromocytoma}} is the most common tumor of the {{c1::adrenal medulla}} in adults
Published	03/04/2024	A pheochromocytoma is a tumor of {{c1::chromaffin}} cells
Published	03/04/2024	The five episodic hyperadrenergic symptoms of a(n) {{c3::pheochromocytoma}} are: 1. {{c1::Pressure (increased BP)}} 2. {{c1::Pain (headache)}} 3.…
Published	03/04/2024	{{c2::Pheochromocytoma}} is diagnosed by increased serum and urine levels of catecholamines, or their breakdown products {{c1::metanephrines}} and {{c…
Published	03/04/2024	Treatment for a pheochromocytoma is {{c1::surgical excision}}
Published	03/04/2024	Up to 25% of cases of pheochromocytoma are associated with germline mutations, including {{c1::NF-1}}, {{c2::VHL}}, and {{c3::RET (MEN2A/2B)}}
Published	03/04/2024	Which three organs are involved in MEN1?{{c1::Pituitary, pancreas, and parathyroids}}
Published	03/04/2024	MEN {{c2::1}} is associated with pituitary tumors that secrete {{c1::prolactin}} or {{c1::GH}}
Published	03/04/2024	MEN 1 is associated with mutation of the {{c1::MEN1}} gene (menin), which is a tumor suppressor gene found on chromosome {{c2::11}}
Published	03/04/2024	Which three organs or diseases are involved in MEN 2A?{{c1::Parathyroid, thyroid (medullary carcinoma) and pheochromocytomas}}
Published	03/04/2024	MEN {{c2::2A}} and {{c2::2B}} are associated with {{c1::medullary thyroid carcinoma::thyroid}}
Published	03/04/2024	MEN {{c2::2A}} and {{c2::2B}} are associated with {{c1::pheochromocytoma::adrenal medulla}}
Published	03/04/2024	Which three organs or diseases are involved in MEN 2B?{{c1::Thyroid (medullary carcinoma), pheochromocytomas, and mucosal neuromas}}
Published	03/04/2024	MEN 2B is associated with mucosal {{c1::neuromas}} (oral/intestinal ganglioneuromatosis)
Published	03/04/2024	Glucocorticoids are useful in treating {{c1::adrenal}} insufficiency, which may progress to circulatory shock, hypoglycemia, and death
Published	03/04/2024	What drug may be used to replace mineralocorticoids in primary adrenal insufficiency?{{c1::Fludrocortisone (aldosterone analog)}}
Published	03/04/2024	What clinical features are seen in 21β-hydroxylase deficiency that are a result of increased androgen levels in males? {{c1::Precocious puberty}}
Published	03/04/2024	In cells containing mineralocorticoid receptors, {{c1::cortisol}} may be converted to {{c2::cortisone}} by the enzyme {{c3::11β-hydroxysteroid dehydro…
Published	03/04/2024	The RET {{c1::proto-oncogene}} encodes for the RET receptor tyrosine kinase, which is involved in embryological developmentThis is a(n) {{c2::growth f…
Published	03/04/2024	How do the following laboratory values change in central adrenal insufficiency?Cortisol: {{c1::decreased}}ACTH: {{c1::decreased}}Aldosterone: {{c1::no…
Published	03/04/2024	How do the following laboratory values change in primary adrenal insufficiency?Cortisol: {{c1::decreased}}ACTH: {{c1::increased}}Aldosterone: {{c1::de…
Published	03/04/2024	Which tumor suppressor gene is associated with multiple endocrine neoplasia type 1?{{c1::MEN1}}
Published	03/04/2024	Ketoconazole can be used to treat patients with {{c1::Cushing}} Syndrome
Published	03/04/2024	Paragangliomas are associated with MEN {{c1::2A/2B}}
Published	03/04/2024	The RET proto-oncogene is expressed by cells of {{c1::neural crest}} lineage, and activating mutations are seen in {{c2::MEN II}} syndromes, specifica…
Published	03/04/2024	What protein is responsible for the rate limiting step in steroidogenesis?{{c1::StAR (Steroidogenic acute regulatory protein)}}
Published	03/04/2024	What is the best screening test for primary hyperaldosteronism?{{c1::Early-morning plasma aldosterone concentration (PAC) to plasma renin activity (PR…
Published	03/04/2024	What is the treatment for primary hyperaldosteronism due to a unilateral adrenoma?{{c1::Surgical resection (adrenalectomy)}}
Published	03/04/2024	What is the recommended treatment for patients with primary hyperaldosteronism that are poor surgical candidates?{{c1::Aldosterone antagonists (e.g. s…
Published	03/04/2024	The initial step in evaluation of Cushing syndrome is confirmation of hypercortisolism via one of the following tests:1. {{c1::24-hour urine free cort…
Published	03/04/2024	Diagnosis of adrenal tumor is supported by an elevated level of {{c1::dehydroepiandrosterone sulfate (DHEAS)}}
Published	03/04/2024	What is the likely diagnosis in a young woman that develops amenorrhea, weight gain, hypertension, hirsutism, muscle weakness, and easy bruising (stri…
Published	03/04/2024	After you diagnose an adrenal tumor via CT/MRI, what would you want to do next to confirm which adrenal gland responsible?{{c1::Adrenal vein sampling}…
Published	03/04/2024	What is the diagnostic test used to confirm suspected primary adrenal insufficiency?{{c1::Cosyntropin or ACTH stimulation tes…
Published	03/04/2024	{{c1::Adrenal}} insufficiency may cause fatigue, muscle pain, and weakness due to decreased cortisol
Published	03/04/2024	Multiple endocrine neoplasia is characterized by tumor formation in multiple endocrine organs that occurs in {{c1::young adults (age <35)::age…
Published	03/04/2024	What is a risk factor for multiple endocrine neoplasia (MEN)?{{c1::Family history of endocrine tumors}}
Published	03/04/2024	What effect does 11- and 21-hydroxylase deficiency have on serum levels of aldosterone?{{c1::Decreased}}
Published	03/04/2024	In females, 11- and 21-hydroxylase deficiency may present with {{c1::irregular menses::symptom}} and {{c1::infertility::symptom}} …
Published	03/04/2024	Primary hyperaldosteronism initially causes severe volume {{c1::overload}} and {{c1::hyper}}tension
Published	03/04/2024	The diagnosis of primary hyperaldosteronism may be confirmed with {{c1::adrenal suppression testing}} after a(n) {{c2::oral saline…
Published	03/04/2024	Administration of Cosyntropin (synthetic ACTH) => high cortisol levels = {{c1::excludes primary adrenal insufficiency::interpretation}}
Published	03/04/2024	Administration of Cosyntropin (synthetic ACTH) => low cortisol levels = {{c1::confirms primary adrenal insufficiency::interpret…
Published	03/04/2024	Adrenal insufficiency may present with weight {{c1::loss}}
Published	03/04/2024	Primary adrenal insufficiency may present with {{c1::hypo}}-tension
Published	03/04/2024	Does adrenal insufficiency present with depression or psychosis?{{c1::Both :)}}
Published	03/04/2024	To test for {{c2::adrenal insufficiency}}, you measure cortisol {{c1::in the morning::when}}, and to test for {{c2::Cushing syndrome}}, you measure co…
Published	03/04/2024	If morning cortisol and plasma ACTH are inconclusive for adrenal insufficiency, you can try {{c1::ACTH stimulation}} test or {{c1::insulin t…
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