endocrine emergencies. adrenal insufficiency adrenal physiology cortisol functions at target...
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Endocrine EmergenciesEndocrine Emergencies
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Adrenal InsufficiencyAdrenal Insufficiency
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Adrenal physiologyAdrenal physiology
Cortisol functions at target tissues to maintain Cortisol functions at target tissues to maintain vascular resistance, cardiac output, hepatic glucose vascular resistance, cardiac output, hepatic glucose production and free water excretionproduction and free water excretion
Cortisol concentration normally demonstrates Cortisol concentration normally demonstrates diurnal variation and increases during times of diurnal variation and increases during times of medical stressmedical stress
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Adrenal physiologyAdrenal physiology
The hypothalamus secretes CRH which in turn The hypothalamus secretes CRH which in turn stimulates ACTH production from the pituitarystimulates ACTH production from the pituitary
ACTH stimlates cortisol production from the adrenal ACTH stimlates cortisol production from the adrenal glandsglands
The hypothalamus and pituitary are influenced by The hypothalamus and pituitary are influenced by negative feedback from cortisolnegative feedback from cortisol
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Adrenal physiologyAdrenal physiology
Aldosterone is controlled primarily by angiotensin II Aldosterone is controlled primarily by angiotensin II and circulating potassium levels; ACTH stimulates and circulating potassium levels; ACTH stimulates aldosterone secretion only transientlyaldosterone secretion only transiently
Aldosterone stimulates sodium exchange for Aldosterone stimulates sodium exchange for potassium in the distal nephronpotassium in the distal nephron
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Autoimmune Adrenal InsufficiencyAutoimmune Adrenal Insufficiency
The most common cause of adrenal insufficiency in The most common cause of adrenal insufficiency in industrialized countriesindustrialized countries
May occur alone or associated with other autoimmune May occur alone or associated with other autoimmune disordersdisorders– Schmidt’s syndrome or type II autoimmune polyglandular Schmidt’s syndrome or type II autoimmune polyglandular
syndrome. Type I diabetes and autoimmune thyroid syndrome. Type I diabetes and autoimmune thyroid diseasedisease
– Type I autoimmune polyglandular syndrome or APECED Type I autoimmune polyglandular syndrome or APECED (autoimmune polyendocrinopathy-candidiasis-ectomdermal (autoimmune polyendocrinopathy-candidiasis-ectomdermal dystrophy) with chronic mucocutaneous candidiasis and dystrophy) with chronic mucocutaneous candidiasis and hypoparathyroidism.hypoparathyroidism.
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Adrenal HemorrhageAdrenal Hemorrhage
Increasingly recognized as a cause of adrenal Increasingly recognized as a cause of adrenal insufficiencyinsufficiency
Meningococcemia (Waterhouse-Friderichsen Meningococcemia (Waterhouse-Friderichsen syndrome) and other forms of sepsissyndrome) and other forms of sepsis
Anticoagulation therapy and coagulation disorders Anticoagulation therapy and coagulation disorders including antiphospholipid antibody syndromeincluding antiphospholipid antibody syndrome
Severe illness and stress; ACTH-induced increases Severe illness and stress; ACTH-induced increases in adrenal blood flow that exceeds the capacity for in adrenal blood flow that exceeds the capacity for venous drainagevenous drainage
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InfectionsInfections
TuberculosisTuberculosis HistoplasmosisHistoplasmosis CryptococcusCryptococcus BlastomycosisBlastomycosis ParacocciciomycosisParacocciciomycosis Cytomegalovirus associated with HIVCytomegalovirus associated with HIV
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Adrenoleukodystrophy and Adrenoleukodystrophy and AdrenomyeloneuropathyAdrenomyeloneuropathy
X-linked peroxisomal disorders of imparied very long chain fatty X-linked peroxisomal disorders of imparied very long chain fatty acid oxidationacid oxidation
In adrenoleukodystropy the neurological features begin in In adrenoleukodystropy the neurological features begin in childhood and progress to coma and deathchildhood and progress to coma and death
Adrenomyeloneuropathy neurological features (central Adrenomyeloneuropathy neurological features (central demyelination, cortical blindness, neuropathies) begin in demyelination, cortical blindness, neuropathies) begin in adolescence or young adulthood, progress more slowly and adolescence or young adulthood, progress more slowly and involve peripheral nervesinvolve peripheral nerves
Diagnosis made by measuring high concentrations of VLCFADiagnosis made by measuring high concentrations of VLCFA Young men with adrenal insufficiency should be screened for Young men with adrenal insufficiency should be screened for
this disorderthis disorder
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Congenital Adrenal HyperplasiaCongenital Adrenal Hyperplasia
A family of autosomal recessive disorders caused by A family of autosomal recessive disorders caused by deficiency of one of the multiple enzymes in the cortisol deficiency of one of the multiple enzymes in the cortisol synthesis pathwaysynthesis pathway
The enzyme deficiency causes inadequate cortisol The enzyme deficiency causes inadequate cortisol production and a compensatory increase in ACTHproduction and a compensatory increase in ACTH
ACTH stimulates adrenal hyperplasia and increased ACTH stimulates adrenal hyperplasia and increased production of precursors proximal to the block in cortisol production of precursors proximal to the block in cortisol synthesissynthesis
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Bilateral Adrenal MetastasesBilateral Adrenal Metastases
Metastases to the adrenal are commonMetastases to the adrenal are common Breast 54%Breast 54% Bronchogenic 44%Bronchogenic 44% Renal 31%Renal 31% Adrenal insufficiency from metastases is very rareAdrenal insufficiency from metastases is very rare
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MedicationsMedications
Accelerate metabolism Accelerate metabolism of cortisolof cortisol
Thyroid hormoneThyroid hormone RifampinRifampin PhenytoinPhenytoin PhenobarbitalPhenobarbital MitotaneMitotane
Inhibit cortisol synthesisInhibit cortisol synthesis Ketoconazole (but not Ketoconazole (but not
fluconazole or itraconazole)fluconazole or itraconazole) EtomidateEtomidate MetyraponeMetyrapone MitotaneMitotane AminoglutethimideAminoglutethimide
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Secondary Adrenal InsufficiencySecondary Adrenal Insufficiency
Pituitary tumors due to mass or treatment of tumorPituitary tumors due to mass or treatment of tumor Metastases to pituitaryMetastases to pituitary CraniopharyngiomaCraniopharyngioma MeningiomaMeningioma Infiltrative disorders (histiocytosis X, lymphocytic Infiltrative disorders (histiocytosis X, lymphocytic
hypophysitis,sarcoidosis, hemochromatosis)hypophysitis,sarcoidosis, hemochromatosis) Postpartum pituitary necrosis (Sheehan’s syndrome)Postpartum pituitary necrosis (Sheehan’s syndrome) Iatrogenic from exogenous steroidsIatrogenic from exogenous steroids High doses of megestrol acetateHigh doses of megestrol acetate
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Clinical PresentationClinical Presentation
FindingFinding PrimaryPrimary SecondarySecondary
Anorexia and weight lossAnorexia and weight loss Yes (100%)Yes (100%) Yes (100%)Yes (100%)
Fatigue and weaknessFatigue and weakness Yes (100%)Yes (100%) Yes (100%)Yes (100%)
Nausea/diarrheaNausea/diarrhea Yes (50%)Yes (50%) Yes (50%)Yes (50%)
Muscle,joint,abdominal painMuscle,joint,abdominal pain Yes (10%)Yes (10%) Yes (10%)Yes (10%)
Orthostatic hypotensionOrthostatic hypotension YesYes YesYes
HyponatremiaHyponatremia Yes (80%)Yes (80%) Yes (60%)Yes (60%)
HyperkalemiaHyperkalemia Yes (60%)Yes (60%) NoNo
HyperpigmentationHyperpigmentation yesyes NoNo
Secondary deficiencies of Secondary deficiencies of testosterone, GH, thyroid, ADHtestosterone, GH, thyroid, ADH
NoNo YesYes
Associated autoimmune diseasesAssociated autoimmune diseases YesYes NoNo
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Adrenal CrisisAdrenal Crisis
Dehydration, hypotenstion, shock out of proportion to severity Dehydration, hypotenstion, shock out of proportion to severity of current illness, nausea, vomiting with anorexia, weight loss, of current illness, nausea, vomiting with anorexia, weight loss, unexplained fever, hyponatremia, hyperkalemia, azotemia, unexplained fever, hyponatremia, hyperkalemia, azotemia, hypercalcemia, eosinophilia, and hypoglycemiahypercalcemia, eosinophilia, and hypoglycemia
Often precipitated by intercurrent illness in patient with Often precipitated by intercurrent illness in patient with unrecognized adrenal insufficiency or in a patient with known unrecognized adrenal insufficiency or in a patient with known disease who did not increase cortisol replacement disease who did not increase cortisol replacement appropriately or patient who recently had glucocorticoid therapy appropriately or patient who recently had glucocorticoid therapy withdrawn, or in patient with bilateral adrenal hemorrhagewithdrawn, or in patient with bilateral adrenal hemorrhage
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Laboratory TestingLaboratory Testing
In acute emergencies “treat first, test later”In acute emergencies “treat first, test later” In the acutely ill patient draw serum cortisol In the acutely ill patient draw serum cortisol
and ACTH then treat with dexamethasone 2-and ACTH then treat with dexamethasone 2-4 mg IV q12 hours or hydrocortisone 100 mg 4 mg IV q12 hours or hydrocortisone 100 mg q6 hours then switch to dexamethasone for q6 hours then switch to dexamethasone for testingtesting
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Laboratory TestingLaboratory Testing
Static testing not very useful Static testing not very useful If cortisol between 8-9 am if less than or If cortisol between 8-9 am if less than or
equal to 3 ug/dl adrenal insufficiency likelyequal to 3 ug/dl adrenal insufficiency likely If cortisol greater than 19 adrenal If cortisol greater than 19 adrenal
insufficiency ruled outinsufficiency ruled out
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Dynamic Testing: CortrosynDynamic Testing: Cortrosyn
A serum cortisol of 20 ug/dl or more 1 hour following 250 ug of A serum cortisol of 20 ug/dl or more 1 hour following 250 ug of cortrosyn IM or IV excludes primary adrenal insufficiencycortrosyn IM or IV excludes primary adrenal insufficiency
Some have suggested a value of 18 is an adequate responeSome have suggested a value of 18 is an adequate respone Difference between baseline and stimulated cortisol no longer Difference between baseline and stimulated cortisol no longer
usedused Does not exclude the presence of secondary adrenal Does not exclude the presence of secondary adrenal
insufficiencyinsufficiency
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Dynamic Testing: CortrosynDynamic Testing: Cortrosyn
Low dose cortrosyn 1 ug IV followed by cortisol Low dose cortrosyn 1 ug IV followed by cortisol measurement in one half hour.measurement in one half hour.
There is evidence for and against the utility of this There is evidence for and against the utility of this testtest
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ACTH MeasurementsACTH Measurements
In untreated primary adrenal insufficiency In untreated primary adrenal insufficiency ACTH is greater than 100 pg/mlACTH is greater than 100 pg/ml
Not useful for judging adequacy of therapyNot useful for judging adequacy of therapy
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Insulin Tolerance TestInsulin Tolerance Test
Performed fasting in morningPerformed fasting in morning IV administration of 0.1-0.15 units regular insulin/kgIV administration of 0.1-0.15 units regular insulin/kg Cortisol >18 to 20 during hypoglycemia is normalCortisol >18 to 20 during hypoglycemia is normal Contraindicated in patients with severe illness, coronary artery Contraindicated in patients with severe illness, coronary artery
disease, seizures, psychiatric diseasedisease, seizures, psychiatric disease In patients with pituitary disease growth hormone is measured In patients with pituitary disease growth hormone is measured
simultaneouslysimultaneously
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Metyrapone TestMetyrapone Test
Metyrapone activates the HPA axis by blocking cortiosl Metyrapone activates the HPA axis by blocking cortiosl production at the 11-hydroxylase step, the last step in cortisol production at the 11-hydroxylase step, the last step in cortisol synthesissynthesis
This leads to cortisol deficiency which should activate ACTH This leads to cortisol deficiency which should activate ACTH production and production of precursors proximal to the blockproduction and production of precursors proximal to the block
Metyrapone is given at midnight with a light snackMetyrapone is given at midnight with a light snack Cortisol and 11-deoxycortisol are measured at 8 am. The test Cortisol and 11-deoxycortisol are measured at 8 am. The test
is considered normal if cortisol is less than 5 and 11-is considered normal if cortisol is less than 5 and 11-deoxycortisol is at least 7 ung/dl.deoxycortisol is at least 7 ung/dl.
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Once the diagnosis is made a search for the Once the diagnosis is made a search for the underlying cause is indicated if not immediately underlying cause is indicated if not immediately obviousobvious
For primary adrenal insufficiency adrenal imaging For primary adrenal insufficiency adrenal imaging is indicatedis indicated
For secondary disease MRI imaging of For secondary disease MRI imaging of pituitary/hypothalamus may be needed.pituitary/hypothalamus may be needed.
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TreatmentTreatment
For primary adrenal crisis: hydrocortisone 100 mg For primary adrenal crisis: hydrocortisone 100 mg q6 hours if diagnosis established or dexamethasone q6 hours if diagnosis established or dexamethasone 2-4 mg q12 hours if diagnostic testing needed 2-4 mg q12 hours if diagnostic testing needed
For secondary adrenal crisis: dexamethasone may For secondary adrenal crisis: dexamethasone may be preferred to avoid fluid retention and be preferred to avoid fluid retention and hypokalemiahypokalemia
Intravenous saline to support volume and treat Intravenous saline to support volume and treat hyperkalemiahyperkalemia
Specific mineralocorticoid is usually not necessary Specific mineralocorticoid is usually not necessary while using high dose hydrocortisonewhile using high dose hydrocortisone
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Maintenance TherapyMaintenance Therapy
Hydrocortisone 10-20 mg in am, 5-10 mg in early pmHydrocortisone 10-20 mg in am, 5-10 mg in early pm Prednisone 5 mg in am, 0-2.5 mg in pmPrednisone 5 mg in am, 0-2.5 mg in pm Florinef 0-0.1 mg per day Florinef 0-0.1 mg per day Adequacy of glucocorticoid judged by patient well-being, Adequacy of glucocorticoid judged by patient well-being,
decrease in pigmentation, electrolytes, blood pressuredecrease in pigmentation, electrolytes, blood pressure Adequacy of mineralocorticoid judged by blood pressure, Adequacy of mineralocorticoid judged by blood pressure,
edema, potassium and plasma renin activityedema, potassium and plasma renin activity All patients with adrenal insufficiency should have MedicAlert All patients with adrenal insufficiency should have MedicAlert
bracelet or carry documentation of this disorderbracelet or carry documentation of this disorder
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Acute Illness CoverageAcute Illness Coverage
Mild to moderate illness: double or triple usual glucocorticoid Mild to moderate illness: double or triple usual glucocorticoid dosagedosage
Severe illness or vomiting: dexamethasone or solucortef IM Severe illness or vomiting: dexamethasone or solucortef IM self-administered by patient then seek prompt medical helpself-administered by patient then seek prompt medical help
Moderately stressful procedures such as endoscopy: Moderately stressful procedures such as endoscopy: hydrocortisone 100 mg one hour before procedurehydrocortisone 100 mg one hour before procedure
Major surgery: hydrocortisone 100 mg IV before induction of Major surgery: hydrocortisone 100 mg IV before induction of anesthesia and repeated q6 hours. Dose then tapered anesthesia and repeated q6 hours. Dose then tapered depending on patient’s rate of recovery, usually 50% decrease depending on patient’s rate of recovery, usually 50% decrease per day until maintenance dose achievedper day until maintenance dose achieved
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Thyroid StormThyroid Storm
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Thyroid StormThyroid Storm
Severe and life-threatening thyrotoxicosisSevere and life-threatening thyrotoxicosis Exaggeration of the typical symptoms of hyperthyroidismExaggeration of the typical symptoms of hyperthyroidism Tachycardia with rate often>140Tachycardia with rate often>140 CHFCHF FeverFever Change in mental status: delirium, psychosis, stupor, comaChange in mental status: delirium, psychosis, stupor, coma Nausea, vomiting, diarrhea, abdominal painNausea, vomiting, diarrhea, abdominal pain Hepatic failure, jaundice, abnormal liver function testsHepatic failure, jaundice, abnormal liver function tests
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PrecipitantsPrecipitants
Usually precipitated by an acute event in a patient with untreated Usually precipitated by an acute event in a patient with untreated hyperthyroidismhyperthyroidism
Thyroid or nonthyroidal surgeryThyroid or nonthyroidal surgery TraumaTrauma InfectionInfection Acute iodine load or radioactive iodineAcute iodine load or radioactive iodine Poor compliance with specific therapyPoor compliance with specific therapy Low socioeconomic statusLow socioeconomic status
Preoperative preparation of patients undergoing thyroidectomy for Preoperative preparation of patients undergoing thyroidectomy for hyperthyroidism has led to dramatic reduction in prevalence of hyperthyroidism has led to dramatic reduction in prevalence of surgically-induced thyroid stormsurgically-induced thyroid storm
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TreatmentTreatment
IV FluidIV Fluid AcetominophenAcetominophen Beta blockade to control adrenergic symptomsBeta blockade to control adrenergic symptoms Thionamide - methimazole or PTUThionamide - methimazole or PTU Iodine solution to block release of thyroid hormoneIodine solution to block release of thyroid hormone Iodinated contrast agent to inhibit the peripheral Iodinated contrast agent to inhibit the peripheral
conversion of T4 to T3conversion of T4 to T3 Glucocorticoids to reduce T4 to T3 conversion and Glucocorticoids to reduce T4 to T3 conversion and
to treat potential coexistent adrenal insufficiencyto treat potential coexistent adrenal insufficiency
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Beta BlockersBeta Blockers
Use with caution in patients with CHF or other Use with caution in patients with CHF or other contraindication contraindication
Propranolol is frequently selected as it can be given Propranolol is frequently selected as it can be given intravenously and reduces the conversion of T4 to intravenously and reduces the conversion of T4 to T3T3
Esmolol - loading dose of 250-500 ug/kg IV followed Esmolol - loading dose of 250-500 ug/kg IV followed by infusion of 50-100 ug/kg/min. This permits rapid by infusion of 50-100 ug/kg/min. This permits rapid titration of drug and minimizes adverse reactionstitration of drug and minimizes adverse reactions
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ThionamidesThionamides
Block de novo thyroid hormone synthesis within 1-2 hours of Block de novo thyroid hormone synthesis within 1-2 hours of administration but have no effect on preformed thyroid administration but have no effect on preformed thyroid hormone stored in the glandhormone stored in the gland
PTU blocks conversion of T4 to T3 but since other drugs PTU blocks conversion of T4 to T3 but since other drugs given in storm are usually coadministered it is okay to use given in storm are usually coadministered it is okay to use methimazole which has a longer duration of actionmethimazole which has a longer duration of action
High doses needed: Methimazole 30 mg q6 or PTU 200 mg High doses needed: Methimazole 30 mg q6 or PTU 200 mg q4 hoursq4 hours
Both drugs can be suspected in liquid for rectal Both drugs can be suspected in liquid for rectal administrationadministration
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IodineIodine
Iodine blocks release of T4 and T3 from the glandIodine blocks release of T4 and T3 from the gland SSKI 5 drops every 6 hours or Lugol’s solution 10 SSKI 5 drops every 6 hours or Lugol’s solution 10
drops tiddrops tid Delay administration of at least one hour after Delay administration of at least one hour after
thionamide administration to prevent iodine being thionamide administration to prevent iodine being used as a substrate for new hormone synthesisused as a substrate for new hormone synthesis
If iodine allergic, lithium has been used for the same If iodine allergic, lithium has been used for the same purposepurpose
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Iodinated Radiocontrast AgentsIodinated Radiocontrast Agents
Iopanoic acid used for oral cholecystography Iopanoic acid used for oral cholecystography Potent inhibitors of T4 to T3 conversionPotent inhibitors of T4 to T3 conversion Dose 0.5 to 1 gm qdDose 0.5 to 1 gm qd Give at least one hour after thionamide to prevent Give at least one hour after thionamide to prevent
iodine from being used as a substrate for new iodine from being used as a substrate for new hormone synthesishormone synthesis
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GlucocorticoidsGlucocorticoids
Reduce T4 to T3 conversionReduce T4 to T3 conversion May have a direct effect on underlying autoimmune May have a direct effect on underlying autoimmune
process if storm is due to Graves diseaseprocess if storm is due to Graves disease Use of glucocorticoids has improved outcome in Use of glucocorticoids has improved outcome in
one series one series Hydrocortisone 100 mg IV q8 hoursHydrocortisone 100 mg IV q8 hours
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Myxedema ComaMyxedema Coma
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Myxedema ComaMyxedema Coma
Severe hypothyroidism due to severe long-standing Severe hypothyroidism due to severe long-standing untreated hypothyroidismuntreated hypothyroidism
Precipitating acute event almost always present: infection, Precipitating acute event almost always present: infection, myocardial infarction, cold exposure, sedative drugsmyocardial infarction, cold exposure, sedative drugs
Older women affected most frequentlyOlder women affected most frequently May result from any of the usual causes of hypothyroidismMay result from any of the usual causes of hypothyroidism Important clues in a poorly responsive patient include Important clues in a poorly responsive patient include
presence of thyroidectomy scar or history of radioiodine presence of thyroidectomy scar or history of radioiodine treatment or known hypothyroidismtreatment or known hypothyroidism
Mortality rate is high 30-40%Mortality rate is high 30-40%
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Clinical PresentationClinical Presentation
HypothermiaHypothermia Decreased mental statusDecreased mental status HypotensionHypotension BradycardiaBradycardia HyponatremiaHyponatremia HypoglycemiaHypoglycemia HypoventilationHypoventilation
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DiagnosisDiagnosis
History, physical exam, and exclusion of other causes of History, physical exam, and exclusion of other causes of comacoma
Treat before waiting for lab confirmation but draw TSH, free Treat before waiting for lab confirmation but draw TSH, free T4, cortisol before treatmentT4, cortisol before treatment
Most patients will have primary hypothyroidism with high Most patients will have primary hypothyroidism with high TSH and low free T4; rare patients have low free T4 and low TSH and low free T4; rare patients have low free T4 and low TSH consistent with secondary hypothyroidism due to TSH consistent with secondary hypothyroidism due to hypothalamic or pituitary diseasehypothalamic or pituitary disease
Cortisol measurement will help exclude coexistent adrenal Cortisol measurement will help exclude coexistent adrenal insufficiencyinsufficiency
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Treatment: Thyroid HormoneTreatment: Thyroid Hormone
Optimal mode of thyroid hormone therapy is controversialOptimal mode of thyroid hormone therapy is controversial Increasing serum thyroid hormones rapidly carries some risk Increasing serum thyroid hormones rapidly carries some risk
of precipitating MI or atrial arrhythmia but this risk must be of precipitating MI or atrial arrhythmia but this risk must be accepted given high mortality rate of myxedema comaaccepted given high mortality rate of myxedema coma
Levothyroxine 0.2-0.4 mg IV initial doseLevothyroxine 0.2-0.4 mg IV initial dose .05 to 0.1 mg IV qd thereafter.05 to 0.1 mg IV qd thereafter Switch to oral when feasibleSwitch to oral when feasible T3 can be given 5-20 ug initially, then 2.5-10 ug q8 hoursT3 can be given 5-20 ug initially, then 2.5-10 ug q8 hours Stop T3 when clinical improvement occursStop T3 when clinical improvement occurs
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Supportive MeasuresSupportive Measures
Avoid dilute fluidsAvoid dilute fluids Severe hypotension that does not respond to fluids Severe hypotension that does not respond to fluids
should be treated with vasopressors until T4 has should be treated with vasopressors until T4 has had time to acthad time to act
Passive rewarming with heating blanket (active Passive rewarming with heating blanket (active rewarming carries risk of vasodilatation)rewarming carries risk of vasodilatation)
Empiric antibiotics until appropriate cultures are Empiric antibiotics until appropriate cultures are proven negativeproven negative
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PheochromocytomaPheochromocytoma
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Catecholamine -Secreting Tumors: Catecholamine -Secreting Tumors: Pheochromocytoma and ParagangliomasPheochromocytoma and Paragangliomas
Arise from chromaffin cells of adrenal medulla and Arise from chromaffin cells of adrenal medulla and sympathetic ganglia sympathetic ganglia
Rare: incidence 2-8 cases per million; prevalence Rare: incidence 2-8 cases per million; prevalence estimates 0.01% to 0.1% of hypertensive population estimates 0.01% to 0.1% of hypertensive population
Occurs equally in men and women, primarily in 3rd Occurs equally in men and women, primarily in 3rd through 5th decadesthrough 5th decades
Curable with surgical removal of tumorCurable with surgical removal of tumor Potential for lethal paroxysmPotential for lethal paroxysm
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SymptomsSymptoms
Usually present and are due to pharmacologic Usually present and are due to pharmacologic effects of excess circulating catecholamineseffects of excess circulating catecholamines
The five P’s:The five P’s:
Pressure- sudden major increase in BPPressure- sudden major increase in BP Pain- abrupt onset of throbbing headache, chest and/or Pain- abrupt onset of throbbing headache, chest and/or
abdominal painabdominal pain Perspiration- profuse generalized diaphoresis Perspiration- profuse generalized diaphoresis PalpitationsPalpitations PallorPallor
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SpellsSpells
Extremely variable in presentationExtremely variable in presentation SpontaneousSpontaneous Precipitated by diagnostic procedures, postural Precipitated by diagnostic procedures, postural
changes, anxiety, exercise, or maneuvers that changes, anxiety, exercise, or maneuvers that increase intra-abdominal pressureincrease intra-abdominal pressure
Duration 10-60 minutes and may occur daily to Duration 10-60 minutes and may occur daily to monthlymonthly
Additional symptoms: constipation, attacks of Additional symptoms: constipation, attacks of hypotension and shock, tremor, anxiety, epigastric hypotension and shock, tremor, anxiety, epigastric and chest painand chest pain
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Clinical SignsClinical Signs
Hypertension - paroxysmal in half, may be severe and resistant Hypertension - paroxysmal in half, may be severe and resistant to conventional therapyto conventional therapy
Orthostatic hypotensionOrthostatic hypotension PallorPallor Grade II-IV retinopathyGrade II-IV retinopathy TremorTremor Weight lossWeight loss FeverFever Café au lait spots in neurofibromatosisCafé au lait spots in neurofibromatosis Painless hematuria and paroxysmal attacks induced by Painless hematuria and paroxysmal attacks induced by
micturition in pheo of bladdermicturition in pheo of bladder HyperglycemiaHyperglycemia HypercalcemiaHypercalcemia ErythrocytosisErythrocytosis
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Rule of 10Rule of 10
10% are extradrenal10% are extradrenal 10% occur in children10% occur in children 10% are multiple or bilateral10% are multiple or bilateral 10% recur after surgical removal10% recur after surgical removal 10% are malignant10% are malignant 10% are familial10% are familial
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Differential DiagnosisDifferential Diagnosis
EndocrineEndocrine ThyrotoxicosisThyrotoxicosis Menopausal syndromeMenopausal syndrome HypoglycemiaHypoglycemia MastocytosisMastocytosis
CardiacCardiac Essential hypertensionEssential hypertension Cardiovascular deconditioningCardiovascular deconditioning Paroxysmal arrhythmiaParoxysmal arrhythmia Withdrawal of adrenergic inhibiting medications (clonidine)Withdrawal of adrenergic inhibiting medications (clonidine) MAO-inhibitor treatment and ingestion of tyramine or MAO-inhibitor treatment and ingestion of tyramine or
decongestantdecongestant AnginaAngina
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Differential DiagnosisDifferential Diagnosis
PsychoneurologicPsychoneurologic Anxiety and panic attacksAnxiety and panic attacks HyperventilationHyperventilation Migraine headachesMigraine headaches Amphetamine, phenylpropanolamine, or cocaine useAmphetamine, phenylpropanolamine, or cocaine use Diencephalic epilepsyDiencephalic epilepsy
FactitiousFactitious Sympathomimetic ingestionSympathomimetic ingestion
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Familial SyndromesFamilial Syndromes Familial pheochromocytomaFamilial pheochromocytoma
MENII aMENII a PheochromocytomaPheochromocytoma Medullary thyroid carcinomaMedullary thyroid carcinoma HyperparathyroidismHyperparathyroidism
MENII bMENII b Pheochromocytoma (bilateral in >70%)Pheochromocytoma (bilateral in >70%) Medullary thyroid carcinomaMedullary thyroid carcinoma Mucosal neuromasMucosal neuromas Thickened corneal nervesThickened corneal nerves Intestinal ganglioneuromatosisIntestinal ganglioneuromatosis Marfanoid body habitusMarfanoid body habitus
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Familial SyndromesFamilial Syndromes
Neurofibromatosis (von Recklinghausen’s disease)Neurofibromatosis (von Recklinghausen’s disease) 1% develop pheochromocytoma1% develop pheochromocytoma
Von Hippel-Lindau (retinal angiomatosis and cerebellar Von Hippel-Lindau (retinal angiomatosis and cerebellar hemangioblastoma)hemangioblastoma)
Additional pheochromocytoma-related neurocutaneous syndromes:Additional pheochromocytoma-related neurocutaneous syndromes: Ataxia telangiectasiaAtaxia telangiectasia Tuberous sclerosisTuberous sclerosis Sturge-WeberSturge-Weber
Other known associations without familial basisOther known associations without familial basis Carney’s triadCarney’s triad Gastric leiomyosarcomaGastric leiomyosarcoma Pulmonary chondromaPulmonary chondroma Extra-adrenal pheochromocytomaExtra-adrenal pheochromocytoma
CholelithiasisCholelithiasis Renal artery stenosisRenal artery stenosis
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ParagangliomasParagangliomas
Para-aortic sympathetic chainPara-aortic sympathetic chain Organs of Zuckerkandl at origin of inferior Organs of Zuckerkandl at origin of inferior
mesenteric arterymesenteric artery Wall of urinary bladderWall of urinary bladder Sympathetic chain in the neck or mediastinumSympathetic chain in the neck or mediastinum
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Other Endocrine Manifestations of Other Endocrine Manifestations of PheochromocytomaPheochromocytoma
GHRH- acromegalyGHRH- acromegaly ACTH/CRH - Cushing’s syndromeACTH/CRH - Cushing’s syndrome VIP- watery diarrheaVIP- watery diarrhea PTH-RP- hypercalcemiaPTH-RP- hypercalcemia
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Diagnostic EvaluationDiagnostic Evaluation
Biochemical documentation should precede any imaging Biochemical documentation should precede any imaging studiesstudies
24 hour urine collection for catecholamines, metanephrine and 24 hour urine collection for catecholamines, metanephrine and VMA VMA
24 hour urine collection should start with the onset of a spell in 24 hour urine collection should start with the onset of a spell in pateints with episodic hypertensionpateints with episodic hypertension
Usually more than 2 fold increase above the upper normal limitUsually more than 2 fold increase above the upper normal limit No role for provocative testing with histamine or glucagonNo role for provocative testing with histamine or glucagon
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Medications Interfering with AssessmentMedications Interfering with Assessment
Increase valuesIncrease values Tricyclic antidepressantsTricyclic antidepressants LabetololLabetolol LevodopaLevodopa DecongestantsDecongestants Amphetamines, busipirone and most psychoactive medicationsAmphetamines, busipirone and most psychoactive medications SotalolSotalol MethyldopaMethyldopa EthanolEthanol BenzodiazepinesBenzodiazepines
Decrease valuesDecrease values MetyrosineMetyrosine MethylglucamineMethylglucamine
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Plasma CatecholaminesPlasma CatecholaminesPlasma catecholamines Plasma catecholamines must be obtained from fasting supine patient with indwelling must be obtained from fasting supine patient with indwelling
catheter in place for 20 minutescatheter in place for 20 minutes affected by diuretics, smoking, renal insufficiencyaffected by diuretics, smoking, renal insufficiencyPlasma metanephrinesPlasma metanephrines Recent report shows accuracy for diagnosisRecent report shows accuracy for diagnosisChromogranin AChromogranin A Costored and secreted with catecholamines and increased in Costored and secreted with catecholamines and increased in
80-90% of patients with catecholamine secreting tumors80-90% of patients with catecholamine secreting tumorsNeuropeptide Y increased in 87%Neuropeptide Y increased in 87%Measurements of urinary catecholamines and metabolites, Measurements of urinary catecholamines and metabolites,
chromogranin A, plasma norepi and dopamine are invalid In chromogranin A, plasma norepi and dopamine are invalid In advanced renal insufficiency. Plasma epi levels more reliableadvanced renal insufficiency. Plasma epi levels more reliable
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Localization StudiesLocalization Studies
90% of tumors are found in the adrenal and 98% are in the 90% of tumors are found in the adrenal and 98% are in the abdomenabdomen
Pheo’s have a characteristic T2-weighted appearance on MRIPheo’s have a characteristic T2-weighted appearance on MRI Common locations of extradrenal paragangliomas are superior Common locations of extradrenal paragangliomas are superior
para-aortic region in 46%, inferior para-aortic in 29%, urinary para-aortic region in 46%, inferior para-aortic in 29%, urinary bladder in 10%, thorax in 10%, head and neck 3%, pelvis 2%bladder in 10%, thorax in 10%, head and neck 3%, pelvis 2%
If results of imaging studies are negative an MIBG scan can be If results of imaging studies are negative an MIBG scan can be performed. Sensitivity 88%, specificity 99%performed. Sensitivity 88%, specificity 99%
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Treatment of PheochromocytomaTreatment of Pheochromocytoma
Surgical resection after careful pre-op alpha and Surgical resection after careful pre-op alpha and beta adrenergic blockadebeta adrenergic blockade
Controls blood pressure and prevents intraoperative Controls blood pressure and prevents intraoperative hypertensive crisishypertensive crisis
Alpha blockade started at least 10 days preop to Alpha blockade started at least 10 days preop to allow for contracted blood volumeallow for contracted blood volume
Encourage high salt intake during this timeEncourage high salt intake during this time
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Alpha blockadeAlpha blockade
Phenoxybenzamine 10 mg bid and increased 10-20 Phenoxybenzamine 10 mg bid and increased 10-20 mg every 2 days until BP and spells controlledmg every 2 days until BP and spells controlled
Average dosage 0.5-1.0 mg/kg dailyAverage dosage 0.5-1.0 mg/kg daily Orthostatic hypotension increased, tachycardia, Orthostatic hypotension increased, tachycardia,
miosis, nasal congestion, diarrhea, fatiguemiosis, nasal congestion, diarrhea, fatigue
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Beta blockadeBeta blockade
Administer only after alpha inhibition is effective because beta Administer only after alpha inhibition is effective because beta blockade alone may result in more severe hypertension due to blockade alone may result in more severe hypertension due to unopposed alpha adrenergic stimulationunopposed alpha adrenergic stimulation
Indicated to control tachycardia associated with high circulating Indicated to control tachycardia associated with high circulating catecholamines and alpha blockadecatecholamines and alpha blockade
Use cautiously and at low dose as chronic circulating Use cautiously and at low dose as chronic circulating catecholamines may cause a cardiomyopathy and beta catecholamines may cause a cardiomyopathy and beta blockers can result in pulmonary edemablockers can result in pulmonary edema
Labetolol is a combined beta blocker and alpha blocker but Labetolol is a combined beta blocker and alpha blocker but instances of paradoxic hypertensive crisis (due to incomplete instances of paradoxic hypertensive crisis (due to incomplete alpha blockade) have been reported; safety as primary agent is alpha blockade) have been reported; safety as primary agent is controversialcontroversial
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Catecholamine Synthesis Inhibitor: Catecholamine Synthesis Inhibitor: Metyrosine Metyrosine
Useful in patients with persistent catecholamine Useful in patients with persistent catecholamine producing tumors that cannot be treated with producing tumors that cannot be treated with combined alpha and beta blockadecombined alpha and beta blockade
Inhibits tyrosine hydroxylaseInhibits tyrosine hydroxylase Side effects: diarrhea, sedation, anxiety, Side effects: diarrhea, sedation, anxiety,
nightmares, urolithiasis, galactorrhea, nightmares, urolithiasis, galactorrhea, extrapyramidal manifestationsextrapyramidal manifestations
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Acute Hypertensive Crises Acute Hypertensive Crises
Phentolamine test dose of 1 mg followed by repeat Phentolamine test dose of 1 mg followed by repeat 5 mg IV boluses5 mg IV boluses
Response maximal in 2-3 minutes and lasts 10-15 Response maximal in 2-3 minutes and lasts 10-15 minutesminutes
100mg/500 cc 5% dextrose can be infused IV and 100mg/500 cc 5% dextrose can be infused IV and titrated to BP controltitrated to BP control
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Postoperative CoursePostoperative Course
Hypotension may occur after surgery: treat with fluids and Hypotension may occur after surgery: treat with fluids and colloidcolloid
Less frequent in patients who have had adequate alpha Less frequent in patients who have had adequate alpha blockade preoperativelyblockade preoperatively
HypoglycemiaHypoglycemia BP usually normal prior to dischargeBP usually normal prior to discharge Some patients remain hypertensive for up to 4-8 weeksSome patients remain hypertensive for up to 4-8 weeks 2 weeks after surgery 24 hour urine obtained to insure cure 2 weeks after surgery 24 hour urine obtained to insure cure
then every 5 yearsthen every 5 years