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Increases catecholamine excretion May cause confounding peaks on HPLC chromatograms 3 Decreases catecholamine excretion
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in patients with azotemia or hypergastrinemia, and in those treated with corticosteroids or proton pump inhibitors Serum may also be assayed for neuron-specific enolase; high levels implicate a malignant pheochromocytoma, while normal levels are nonspecific Pharmacologic provocative and suppressive tests that evaluate the rise or fall in blood pressure are usually not required or recommended Genetic testing should ideally be performed on all patients with pheochromocytoma or paraganglioma Testing for VHL, ret protooncogene, and SDHB/SDHD mutations is advisable Family members may then be screened for the specific gene mutation
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Tachycardia, tremor, palpitation, and hypermetabolism may give rise to confusion with thyrotoxicosis Pheochromocytoma may also be misdiagnosed as essential hypertension, myocarditis, glomerulonephritis or other renal lesions, toxemia of pregnancy, eclampsia, and psychoneurosis (anxiety attack) It can sometimes be mistaken for an acute abdomen Other conditions that have manifestations similar to those of pheochromocytoma include acute intermittent porphyria,
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1 CT and MRI scanning Imaging should not usually replace biochemical testing, since incidental adrenal adenomas are common (2 4% of scans) and can be misleading When a pheochromocytoma is suspected because of biochemical testing or a genetic condition predisposing to pheochromocytoma, a CT scan of the abdomen is performed, with
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causes interference in some urinary catecholamine assays, and reduces [123I]mIBG scanning sensitivity After appropriate antihypertensive therapy, propranolol (10 40 mg orally four times daily), can be used to control tachycardia and other arrhythmias Blood pressure control should be maintained for a minimum of 4 7 days or until optimal cardiac status is established The ECG should be monitored until it becomes stable (It may take a week or even months to correct ECG changes in patients with catecholamine myocarditis, and it may be prudent to defer surgery until then in such cases) Patients must be very closely monitored during surgery to promptly detect sudden changes in blood pressure or cardiac arrhythmias Hypertensive crisis can be managed initially with oral nifedipine 10 mg (chewed pierced capsule) Intraoperative severe hypertension is managed with continuous intravenous nicardipine (a short-acting calcium channel blocker), 2 6 mcg/kg/min, or nitroprusside, 05 10 mcg/kg/min Prolonged nitroprusside administration can cause cyanide toxicity Tachyarrhythmia is treated with intravenous atenolol (1 mg boluses), esmolol, or lidocaine Autotransfusion of 1 2 units of blood at 12 hours preoperatively plus generous intraoperative volume replacement reduces the risk of postresection hypotension caused by desensitization of the vascular 1-receptors Shock may therefore occur following removal of the pheochromocytoma It is treated with intravenous saline or colloid and high doses of intravenous norepinephrine Intravenous 5% dextrose is infused postoperatively to prevent hypoglycemia Because there may be multiple or metastatic tumors, it is essential to recheck urinary catecholamine levels postoperatively (at least 2 weeks after surgery) It is also prudent to perform a whole-body [123I]mIBG scan about 3 months postoperatively, since previously undetected metastases may become visible Thereafter, blood pressure and symptoms must be rechecked regularly for life; urinary catecholamines and metanephrines are also rechecked regularly, at least every 6 months for 5 years, and immediately if hypertension or symptoms recur or if metastases are evident For inoperable or metastatic tumors, metyrosine may be added to reduce catecholamine synthesis Metyrosine is a competitive blocker in the synthesis of catecholamines that is also useful; the initial dosage is 250 mg four times daily, increased daily by increments of 250 500 mg to a maximum of 4 g/d Metyrosine causes central nervous system side effects and crystalluria; hydration must be ensured Metastatic pheochromocytomas may be treated with combination chemotherapy (eg, cyclophosphamide, vincristine, and dacarbazine) or with high doses of [131I]mIBG
hypogonadal vascular instability (hot flushes), cocaine or amphetamine use, clonidine withdrawal, hypertensive crisis caused by foods containing tyramine (eg, cheeses) in patients taking monoamine oxidase inhibitor antidepressants, labile hypertension, and unstable angina Patients with erythromelalgia can have hypertensive crises; their episodic painful flushing and leg swelling are relieved by cold, distinguishing this condition from pheochromocytoma Pheochromocytomas can cause chest pain and ECG changes that mimic acute cardiac ischemia Renal artery stenosis can cause severe hypertension and may coexist with pheochromocytoma False-positive testing for catecholamines and metabolites occurs in about 10% of hypertensives, but levels are usually less than 50% above normal and typically normalize with repeat testing
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