Disorders of Hypothalamus/Pituitary/Adrenal Glands
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- DDx endocrine hypofunction (deficiencies)
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1. damage to gland
2. autoimmune disease
3. defects in hormone production
4. altered hormone metabolism
5. accelerated degradation
6. alteration of act. or inhib. signals
7. Altered target senstivity - DDx endocrine hyperfunction
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1. Tumors
2. Hyperplasia,
3. Hyperfuction
4. Autoimmune Stimulation
5. Exogenous hormone
6. Altered target senstivity - Tests fo Dx and localizing endocrine disorders
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1. History and Physical!! HUGE
2. Hormone Levels-free (sometimes total-complex/bound)
3. Hormone Metabolites--avoids pulsitile problems
4. Urine Assays-steroid, cate, metabolites
5. Assesment of Axis-TSH, Cortisol
6. Stimulation tests
7. Suppression tests
8. Imaging (Tests First) - DDx Hypothalmic Dysfuction
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1. Hypofunction-destructive lesions
2. Craniopharyngioma-tumor, childhood, suprasella area
3. Hyperfuction-tumors, ectopic secretion of hormones from other tumors - Craniopharyngioma
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6-14 years of age
headach, visual loss, growth failure, delayed puberty
vision loss, optic atrophy, hypogonadal, hypopituitarism
surgery, XRT, treatment of hypopituitarism - DDx of Hypopituitarism
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1. insufficient production of pituitary hormones
2. lesions of hypothalmus/pit
3. pititary adenoma (adults)
4. Craniopharyngioma (kids)
5. ischemia, hemorrage,
6. sarcoid, hemochromatosis
7. head trauma, stalk trauma
8. lymphocyte hypophysis - Evaulation of Hypopitutiarism
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tests, labs, signs of focal lesions (head ache, visual loss, CN palsies) symptoms associated with disorders.
first hormones lost are gonadotrophins, GH. ACTH, TRH last to go
Testosterone, T4, Cortisol, ACTH stim test, Estradiol, IGF-1 level, - Pituitary Tumors
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tumors-functional or nonfuctional, compression injury, visual loss, headaches, encase carotid arteries, invade cavernous sinus.
Pituitary Apoplexy, adrenal insufficiency. -
Hormone deficiencies
1. ACTH
2. Growth Hormone
3. FSH LH (gonadotrophins)
4. TSH (thyrotropin)
5. Prolactin -
1. 2 adrenal insuffiency-no cortisol
2. adults, muscle weakness fatigue. Kids- delayed puberty
3. men. impotence, no muscle mass, facial hair, women amenorrhea
4. 2 hypothyropidism (no T3/4)
5. Failure to lactate -
Growth Hormone Excess
Signs, Symptoms, evaulation -
Acromegaly (GH, IGF, glucose), local bone overgrowth, soft tissue expansion, skin thickening, fatigue, galactorrhea. Gigantism in kids,
Stim. production of IGF-1-mediates GH in peripery.
Tx: tranphenoidal hypophysectomy - Presentation and evaluation of Hyperprolactinemia
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Galactorrhea, hypogonadism (with normal FSH/LH) visual field cuts, CN palsies, Anemorrhea, infertility,
Dx: elevated prolactin, microadenoma, macroadenoma in sella, prolactine greated then 100ng/ml
Tx: dompamine, transpheniodal hypophysectomy - DDx Hyperprolactemia
- Pregnancy, nursing, nipple stimulation, exercise, stress, pituitray stalk damage, hypothroidism, injury to breast, chest wall, spine, afferent nerves, uremia, drugs.
- Diabetes Insipidus
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Symptoms: polyuria, nocturia, oncturnal enuresis, thrist, polydipsia
Lab: Elevated osmolality, hypernatremia, decreased urine specific gravity, osmolatity, and Na, impaired urine concentration
Causes: post surgical, tumors, familial, granulomatous disease, infections, idiopathic
DDx: nephrogenic DI, psychogenic polydipsia
Treatment: nasal or subcutaneous vasopression replacement - Define Diabetes Insipidus
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ADH released by posterior pit. binds vasopressin (V2) activates-->a.cyl, cAMP, PKA--> aquaporin channel 2. Into cell membrane, H2O from distal nepron is RA.
DI, No ADH or ADH receptor (in nephron, (X-linking congenital) - When do you suspect Adrenal Insuffiency
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pts with known hypothalmic or pituitary defect.
Hx of TB, disseminated funal infection, HIV, Autoimmune disorder, failure to thrive, fatigue and weight loss, hyperpigmentation, or hypotension, hypoglycemia, hyponatermia, hyperkalemia
glucocorticoid deficiency, weakness, anorexia, amenorrhea,
Mineralcorticoid defiency, acidosis - Dx of Adrenal Insuffiency
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ACTH stimulation test, 1 or 2?
2AI-more subtile tests to rule out
Plasma ACTH
MRI of hypo/pit/adrenal glands
evaluate for TB, Fungal, HIV, Autoimmune, tumors, - Tx of Adrenal Insuffiency
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glucocorticoid replacement. DHEA. primary-mineralcorticoid.
wear braclet of necklace-ACUTRE ADRENAL CRISIS. minor illness double your dose - Causes of adrenal Insuffiency
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1 AI (addison's): Autoimmune, metastagnant maligancy, hemorrhage, infection, congenital adrenal hyperplais, X-linked adrenoleukodystrophy, drugs
2 AI: tumor, trauma, glucocorticoid therapy, problems with pit or hypo. - Difference between 1 and 2 adrenal insuffiency
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1. high ACTH, hyperpigmentation (binds to melanocortin receptors) mucosal membranes, palmer creases, nail beds
2. decreased ACTH, no hyperpigmentation
1-destruction of adrenal cortex, 2-decrease in ACTH prior to Adrenal cortex - Congental Adrenal Hyperplasia
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inherited defects in adrenal enxymes of steroid hormone metabolism
severe glucocorticoid and minerialcorticoid deficiency to mild hirsutism.
X-linked ad.leuko.dys--men, CNS dysfuction - Acute Adrenal Crisis
- Stress-adrenal cortex increases gluco and mineral corticoids, hypotensive crisis, hit with glucocorticoids, IV saline, antibiotics.
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Primary Hyperaldosteronism
Conn's Syndrome -
weakness, fatigue, paresthesias, headaches, hypertension
Lab Dx: hypokalemia, alkolosis, decreased renin, increased aldosterone, salk loading to determine K and test for suppression of aldosterone, CT or MRO, adrenal vein sampling.
Tx: surgery, spironolactone - DDx Primary Hyperaldosteronism
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adrenal adenoma
adrenal carinoma
nodular adrenal hyperplasia
idopathic
Cushings, renovasuclar hypertension, licorice, Liddle's syndrome - Pheochromocytoma: Signs and Symptoms
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Headache, viscual changes, diaphorisis, palpitations, chest pain, enxiety, termor, abdominal pain, nausea vomiting.
Constipation, weight loss, sweating, cold hands, geet, postural hypotension.
Hypertension, orthostatic hypotension, arrhythmias, sinus tachycardia, low grade fever, weight loss - Pheochromocytoma: Lab Dx
- leukocytosis, erythrocytosis, hyperglycemia, hercalicemia, plasma/urinary catecholiamines, VMA, metanephrine. CT MRI, venous sampling for catecholamines
- DDx Pheochromocytoma
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Adrenal medullary tumor
adrenal medullary carcinoma
Extra-adrenal
Essential Hypertension, Renovascular hyper tesnsion, panic attacks, neurobalstomas in children
complications: left ventricular hypertrophy, dilated cardiomyopathy, MI, sudden death, dissecting AA, diabetes - Pheochromocytoma: Tx
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calcium channel blockers, alpha adrenergic blockers, b-adrengeric blockers, volume replacement
surgery (onyl after stabilization, and volume expansion. - Cushings Syndrome: Causes
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cortisol secreting adenoma
adrenal carcinoma
nodular adrenal hyperplasia
exogenous glucocorticoids
ectopic ACTH
ACTH secreting adenoma (Cushings disease)
ectopic CRH (rare) - Cushings Syndrome: Clinical features
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weight gain, central obesity, moon face, buffulo hump, hypertension, diabeties, hyperlipidemia, kidney stones
hirsutism, plethora, straiae, acne, brusing
osteoporesis, weakness, amenorrhea, oligomenorrhea, decreased libido, impotence
poor concentration, memory
enotional liabitity, insomnia, euphoria, depression, psychosis - Cushings Syndrome: Evaluation
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1. H&P
2. dexamethasone suppression test-supresses cortisol in normal, 24hr cortisol, urine
3. ACTH and CRH stim tests
4. Petrosal sinus draining, (by cavernous sinus, ACTH)
Tx: removal of source of ACTH, drugs that block steroid synthesis. - Evalution of pituitary or adrenal mass
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pit-inpingment of optic chasm, H&P, ACTH stim, free T4, TSH, prolactin, LH FSH
adrenal-urine, androgen measurements, DHEA,
remove any carcinmoa greater the 4-5 - Hirsutism: Causes
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puberty, pregnancy, menopause
drugs
polycystic kidney disease, hyperthecosis, ovarian tumor
adrenal hyperplasia, cushings, adrenal adnemoma/carcinoma
hypothroidsm, acromegaly, - Virilisation
- bitemporal hariline recession, clitorial enlargement, deeping of voice, EXTEREM hirsutism, testosteron leve is gretly elevated, tumor of adrenal or ovary
- Autoimmune polyglandular syndromes-addison's
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APS I-autosomal recessive AIRE gene, mucocutaneous candidiasis, hypoparathyroidism, adrenal insufficiency. autoreactive T-cells
APSII-more common,polygenic; - Multiple endocrine neoplasia syndromes-autosomal dominent
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T1. MEN-1: hyperparathyroidsm, adrenal/pituary adenomas, carcinoid tumors
T2. RET: Pheo, medullary carcinoma Hyperparathyroidism (MEN2A)
MEN2B: mucosal neuromas, mardaniod habitus
Neurofibromatosis-NF1: Pheochromocytoma, pigment changes, optic gliomas, skeletal malformations
von Hippel-VHL: Pheochromocytoma, Islet tumors, renal cell carcinoma, cysts