Disorders of Hypothalamus/Pituitary/Adrenal Glands

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DDx endocrine hypofunction (deficiencies): 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: 1. Tumors
2. Hyperplasia,
3. Hyperfuction
4. Autoimmune Stimulation
5. Exogenous hormone
6. Altered target senstivity
Tests fo Dx and localizing endocrine disorders: 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: 1. Hypofunction-destructive lesions
2. Craniopharyngioma-tumor, childhood, suprasella area
3. Hyperfuction-tumors, ectopic secretion of hormones from other tumors
Craniopharyngioma: 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: 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: 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: 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: 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: 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: 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: 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: 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: glucocorticoid replacement. DHEA. primary-mineralcorticoid.

wear braclet of necklace-ACUTRE ADRENAL CRISIS. minor illness double your dose
Causes of adrenal Insuffiency: 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: 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: 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.
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: adrenal adenoma
adrenal carinoma
nodular adrenal hyperplasia
idopathic

Cushings, renovasuclar hypertension, licorice, Liddle's syndrome
Pheochromocytoma: Signs and Symptoms: 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: 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: calcium channel blockers, alpha adrenergic blockers, b-adrengeric blockers, volume replacement

surgery (onyl after stabilization, and volume expansion.
Cushings Syndrome: Causes: cortisol secreting adenoma
adrenal carcinoma
nodular adrenal hyperplasia
exogenous glucocorticoids
ectopic ACTH
ACTH secreting adenoma (Cushings disease)
ectopic CRH (rare)
Cushings Syndrome: Clinical features: 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: 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: 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: 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: APS I-autosomal recessive AIRE gene, mucocutaneous candidiasis, hypoparathyroidism, adrenal insufficiency. autoreactive T-cells

APSII-more common,polygenic;
Multiple endocrine neoplasia syndromes-autosomal dominent: 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

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Number of cards 35