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Dopa decarboxylase
Dopa decarboxylase
• Dapat terjadi pada semua ras , jarang pada ras kulit hitam
• 25% bilateral
• Myocarditis
• Dilated cardiomyopathy
• Pulmonary edema
Komplikasi Neurologis
Symptoms Signs
Headaches ++++ Hypertension ++++
Palpitations +++ Tachycardia or reflex bradycardia +++
Sweating +++
Anxiety/nervousness ++ Postural hypotension +++
Tremulousness ++ Hypertension, paroxysmal ++
Nausea/emesis ++
Pain in chest/abdomen++ Weight loss ++
Pallor ++
Weakness/fatigue ++ Hypermetabolism ++
Dizziness + Fasting hyperglycemia ++
Heat intolerance + Tremor ++
Paresthesias + Increased respiratory rate ++
Constipation +
Dyspnea + Decreased gastrointestinal motility ++
Visual disturbances + Psychosis (rare) +
Seizures, grand mal + Flushing, paroxysmal (rare) +
Catecholamines Metanephrines
NE E NMN MN
Tricyclic antidepressants
Amitriptyline (Elavil), imipramine (Topfranil), nortriptyline (Aventyl) +++ − +++ −
α-Blockers (nonselective)
Phenoxybenzamine (Dibenzyline) +++ − +++ −
α-Blockers (α1-selective)
Doxazosin (Cardura), terazosin (Hytrin), prazosin (Minipress) + − − −
β-Blockers
Atenolol (Tenormin), metoprolol (Lopressor), propranolol (Inderal), labetalol (Normadyne)*+ + + +
Calcium channel antagonists
Nifedipine (Procardia), amlodipine (Norvasc), diltiazem (Cardizem), verapamil (Calan) + + − −
Vasodilators
Hydralazine (Apresoline), isosorbide (Isordil, Dilatrate), minoxidil (Loniten) + − Unknown
Monoamine oxidase inhibitors
Phenelzine (Nardil), tranylcypromine (Parnate), selegiline (Eldepryl) − − +++ +++
Sympathomimetics
Ephedrine, pseudoephedrine (Sudafed), amphetamines, albuterol (Proventil) ++ ++ ++ ++
Stimulants
Caffeine (coffee*, tea), nicotine (tobacco), theophylline ++ ++ Unknown
Miscellaneous
Levodopa, carbidopa (Sinemet)* ++ − Unknown
Cocaine ++ ++ Unknown
E, Epinephrine; MN, metanephrine; NE, norepinephrine; NMN, normetanephrine; +++, substantial increase; ++, moderate increase;
+, mild increase if any; −, little or no increase.
Clinical Setting Symptoms
Pheochromocytoma multisystem crisis Hypertension and/or hypotension, (PMC)
multiple organ failure, temperature of 40° C,
encephalopathy
Cardiovascular Collapse
Hypertensive crisis
Upon induction of anesthesia
Medication-induced or other
mechanisms
Neurologic Hemiplegia
Limb weakness
Renal Acute renal failure
Acute pyelonephritis
Severe hematuria
Metabolic Diabetic ketoacidosis
Lactic acidosis
• Hypertensi • Weakness
• Headache • Anxiety
• Diaphoresis • Epigastric pain
• Palpitation • Flank pain
• Tremor • Constipation
4 karakteristik terpenuhi
• Nausea • strongly
Weightsuggestive
loss
Pemeriksaan fisik
• Hyperglycemia
• Hypercalcemia
• erythrocytosis
ORALLY ADMINISTERED DRUGS USED TO
TREAT PHEOCHROMOCYTOMA
• MR spectroscopy
Patologi Anatomi
• 10% malignant
Phenoxybenzamine Hydrochloride
• Dosis : 2 X 10 mg po naikkan 10 mg sampai dosis optimum (range : 20-
40 mg bid/tid)
Urapidil
• Dosis : 10-15 mg/jam
Betablocker
Mertyrosyne ( denser )
• Fungsi untuk menghambat sintesa katekolamnin
-blockade
Selective 1-blockers (Prazosin, Terazosin, Doxazosin)
1st line as less side-effects
Phenoxybenzamine: more complete -blockade
-blocker
CCB, ACE-I, etc.
Nuclear Medicine Rx:
Hi dose 131I-MIBG or 111indium-octreotide depending on
MIBG scan or octreoscan pick-up
Sensitize tumor with Carboplatin + 5-FU
Pheo & Pregnancy
Diagnosis with 24h urine collections and MRI
No stimulation tests, no MIBG if pregnant
1st & 2nd trimester (< 24 weeks):
Phenoxybenzamine + blocker prep
Resect tumor ASAP laprascopically
3rd trimester:
Phenoxybenzamine + blocker prep
When fetus large enough: cesarian section followed by
tumor resection
TERIMA KASIH