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Most patients with type I congenital methemoglobinemia are asymptomatic except for cyanosis (only of cosmetic significance), but some forms may have serious morbidity (some patients might experience headache, exertional dyspnea, and fatigue)

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At high concentrations, direct parasympathetic cardiac inhibition overrides the baroreceptor-mediated reflex response, resulting in bradycardia with prolonged R–R intervals on ECG