Table 2.

Preoperative management of PPGL in children (8–11).

		Drug	Mechanism	Dose	Side effects	Comments
Pre operative (start 14 days prior to the surgery)	First-line treatment	Doxazosin	Selective α1-blocker	1-2 mg/day in 1-2 doses; Max 4-16 mg/day	Orthostatic hypotension, dizziness, fatigue, drowsiness	No reflex tachycardia, long action (>24h)
		Prazosin		0.05-0.1 mg/kg/day in 3 doses Max 0.5 mg/kg/day (20 mg/day)		Less used
		Terazosin		1 mg/day; Max 20 mg/day		Less used
	Sequential approach	Phenoxy- benzamine	Non selective α1/α2-blocker	0.2-0.25 mg/kg/day (max 10 mg/dose) Max 2-4 mg/kg/day (60 mg/day)	Orthostatic hypotension, nausea reflex tachycardia, nasal congestion, central sedation, abdominal pain	Associate β blocker 3-4 days prior to the surgery
		Atenolol	Selective β1-blocker	0.5-1 mg/kg/day in 1-2 doses; Max 100 mg/day	Dizziness, Fatigue	Never give before α-blocker
		Metoprolol		1-2 mg/kg/day in 1 dose; Max 200 mg/d
		Propranolol	Non selective β1/β2-blocker	1-2 mg/kg/day in 2-4 doses; Max 640 mg/day	Dizziness, fatigue, bronchoconstriction
		Labetalol	α1/β-blocker	1-3 mg/kg/day in 2 doses; Max 1200 mg/day
	Adjunctive agents	Amlodipine	Calcium channel blocker	0.05-0.1 mg/kg/day; Max 10 mg/day	Peripheral edema, palpitations, gingival hyperplasia, headache	Use as adjunct
		Metyrosine	Tyrosine hydroxylase inhibitor	20 mg/kg/day in 4 doses; Max 2500 mg/day	Lethargy, extrapiramidal symptoms, diarrhea, crystalluria	Never give before α-blocker
	Salt	6-10 g/day
	Fluids	1.5 times maintenance fluids per kg