Priapism after Phenoxybenzamine in a Patient with Fabry's Disease

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Abstract
To the Editor: Fabry's disease has recently been described as another cause of priapism.1 We wish to report the unfortunate occurrence of priapism after the third dose of phenoxybenzamine in a 12-year-old boy known to have Fabry's disease.Distal leg and ankle swelling with intermittent vague aching had been present in our patient from eight years of age. One episode of fever occurring with the onset of leg swelling led to the misdiagnosis of rheumatic fever and one year of aspirin administration. Physical examination at 11 years of age at the NPI-UCLA revealed pitting edema of the ankles and lower . . .