Disorders of the Adrenal Cortex Flashcards by Nicole Quinn

Cortex is split up into 3 zones =

Zona glomerulosa
Zona fasciculata
Zona reticularis

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What hormones are made in the Zona glomerulosa

Mineralocorticoids - Aldosterone and corticosterone

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What hormones are made in the Zona fasciculata

Glucocorticoids - cortisol and cortisone

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What hormones are made in the Zona reticularis

Androgens - testosterone and estrogen

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Adrenal hormones include: (4 S’s)

Aldosterone = SALT
Cortisol = SUGAR
Androgens = SEX
Epi, Norepi = STRESS

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Adrenal Disorders are categorized as:

Hyper / Hypofunction

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Hyperfunctional Adrenal gland results in

hormone excess (autoimmune disorders, neoplasms, exogenous administration)

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Hypofunctional Adrenal gland results in

hormone deficiency (autoimmune, infection, surgery, inflammation, infarction, hemorrhage or tumor)

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Adrenal disorders:
Cushing’s syndrome
Pheochromocytoma
Multiple endocrine neoplasia
Adrenal Adenoma
Adrenal Cancer

Hyperfunctioning

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Adrenal disorders:
Addison’s Disease
Adrenal Deficiency

Hypofunctioning

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Cushing syndrome - Pathophysiology

CUshing Syndrome = Cortisol Unchecked
Excess blood glucose production (excess gluconeogenesis; decreased Glycolysis for brain food)
increase in lipolysis
increase in protein catabolism
decrease insulin production and increased glucagon

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Pathognomonic symptoms:
Central obesity
rounded face (moon facies)
Enlarged fat pad between shoulders (buffalo hump - dorsocervical area)
abdominal striae (purple stretch marks)
thinning skin
easy bruising

Cushing syndrome presentation

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ACTH is released from where in the body

Pituitary

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What stimulates ACTH to be released

CRH from the hypothalamus

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Two types of Cushing’s Syndromes

iatrogenic - exogenous corticosteroid administration
ACTH dependent - problem in the pituitary
ACTH independent - problem in the adrenal

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Cushing’s Syndrome workup (screening):

screening - elevated 24 hour urine free cortisol

elevated midnight plasma cortisol (normally time of lowest cortisol secretion)

Dexamethasone suppression test (should reduce steroid levels - positive is the cortisol levels have not reduced)

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Cushing’s Syndrome workup (if screening is positive):

Plasma ACTH
determines if ACTH dependent or independent etiology
>15 pg/mL = ACTH dependent (problem is in the pituitary)
< 5 pg/mL = ACTH independent (problem is the adrenal gland)

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Localizing the source for ACTH independent

CT of the adrenals
usually benign masses = think malignancy if > 4cm, usually atypically densities or contrast irregularities

Localizing the source for ACTH dependent

MRI of the pituitary if no lesion there –> look for ectopic source =
CT scan of the chest, abdomen, thymus, pancreas and/or adrenals

Treatment for Cushing’s Syndrome

Pituitary source - transsphenoidal resection of mass
Ectopic ACTH-secreting tumors - local surgical resection, if failed or not feasible - bilateral adrenalectomy and replacement hormones
Benign adrenal adenoma - laparoscopic resection (masses < 6cm)
Adrenal Carcinoma - surgical resection
symptomatic treatment if surgical treatment not feasible or declined

What is a pheochromocytoma

sympathetic nervous system arising from the adrenal medulla
catecholamine secreting - epinephrine or norepinephrine

Presentation:
HA
profuse sweating
palpitations and tachycardia
HTN, sustained or paroxysmal (new onset high HTN)
anxiety and panic attacks
(Variable presentation)

Pheochromocytoma

Classic Triad of PHEochromocytoma

Palpitations
HA
Episodic sweating

Diagnosis likely if all three + HTN

Catecholamine pulses are released but with no ________

correlated cause

this is why it can be misdiagnosed as an anxiety attack

Pheochromocytoma can result in

catastrophic HTN crisis fatal arrhythmias pulmonary edema or HF ARDS and multisystem failure

Pheochromocytoma workup (labs)

lab testing - plasma fractionated free metanephrines (most sensitive) specimen obtained after sitting quietly for 15 mins if positive - retest after lying supine in a quiet room 30-90 min confirm with 24 hr urine for fractionated metanephrines and creatine

Pheochromocytoma workup (imaging)

non-contrast abdominal CT MRI with or without gadolinium contrast

Pheochromocytoma treatment

surgical resection (partial or total - laparoscopic preferred) alpha blockers or CCB for HTN beta blockers for arrhythmia caution - treatment resistant hypotension post-op if malignant - add chemo, recheck BP and plasma free metanephrines, follow with serum chromogranin A (CgA)

Four distinct types of Multiple Endocrine Neoplasia (MEN)

MEN 1 MEN 2 (2A) MEN 3 (2B) MEN 4

MEN 1 presentation

classic triad of tumors - parathyroids, pancreatic islets, anterior pituitary hyperparathyroidism is the initial presenting sx

MEN 2 (2A) presentation

classic triad - medullary thyroid carcinoma, pheochromocytomas, parathyroid tumors

MEN 3 (2B) presentation

characterized by mucosal neuromas marphan-like habitus delayed puberty, skeletal abnormalities pheochromocytoma - 60%

MEN 4 presentation

most rare of the MEN subsets often pituitary, parathyroid, pancreatic tumors

MEN workup

primary diagnostic tool is genetic testing screening for patients with known FH screening for neuroendocrine tumors with targeted tests/ imaging (MRI for pituitary and adrenal tumors)

MEN treatment

Parathyroidectomy if sx prophylactic thymectomy if getting parathyroidectomy high dose PPIs and control hypercalcemia if gastrinomas +/- chemo

Classical presentation of Adrenal Insufficiency

fatigue reduced stamina weakness anorexia/ wt loss

Adrenal Insufficiency is

deficiency of the adrenal cortex hormones primary and secondary causes

Primary adrenal disease is aka

addison's disease (adrenal deficiency) Primarily autoimmune in origin

Acute adrenal crisis is

life threatening more common with primary insufficiency N/V, fever, dehydration, profound hypotension progression to shock unresponsive to fluids or vasopressors

Adrenal Insufficiency workup labs

plasma cortisol < 3 mcg/mL is diagnostic often low serum DHEA levels but non-specific conformation with cosyntropin stimulation test (synthetic ACTH) - must hold exogenous steroid meds

Differentiating between primary and secondary adrenal insufficiency

Plasma ACTH differentiates between primary and secondary insufficiency High > 200 pg/mol = primary insufficiency Low or normal = secondary insufficiency

Adrenal insufficiency workup imaging

MRI of hypothalamus and pituitary to look for masses CT of the adrenals for cases of primary insufficiency that are not dx with antibody testing - small noncalcified adrenals in autoimmune, enlarged adrenals in metastatic or granulomatous disease

Adrenal insufficiency treatment

Glucocorticoid replacement therapy 15-30 mg hydrocortisone daily 2-3 doses alternatives prednisone or methylprednisolone increased doses when under stress Mineralocorticoid replacement therapy +DHEA supplementation for women

Treatment for adrenal crisis

loading dose of hydrocortisone 100-300 mg IV Re-hydration with saline solution Hydrocortisone 50-100 mg q6h IV, titrate down and to PO as able broad spectrum abx until infection is ruled out as cause