title: [Priapism associated with Fabry's disease].
abstract: We present a rare case of priapism in a child, ten years old, in association with Fabry's disease. The child had a history of disseminated nodular enlargement, crises of fever, intermittent pain in the extremities and ten hours persistent painful erection of the penis. We don't obtain pain or erection relief with sedation, epidural block and irrigation of the corporal bodies. A saphenous-cavernous shunt, in the Grayhack fashion made, being results satisfactory. In the follow-up, the child had sporadic pain in the extremities and no erection of the penis. The cavernosography showed the shunt open. Fabry's disease was confirmed by nodular biopsy and the demonstration of deficient alpha-galactosidase.
authors: Unidad de Urología Pediátrica, Hospital Infantil La Paz, Madrid.
authors: E
authors: E
authors: Jaureguizar Monereo
authors: P
authors: P
authors: López Pereira
authors: J
authors: J
authors: Cabo
authors: J M
authors: JM
authors: Gutiérrez
authors: J
authors: J
authors: García-Consuegra
authors: J
authors: J
authors: Martínez Olivas
authors: M
authors: M
authors: López Santamaría
mesh_terms: D001166:Arteriovenous Shunt, Surgical / Q000379:methods; D002648:Child; D004630:Emergencies; D000795:Fabry Disease / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D006801:Humans; D008297:Male; D008866:Microsurgery; D010413:Penis / Q000098:blood supply / Q000601:surgery; D011317:Priapism / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery; D012501:Saphenous Vein / Q000637:transplantation
vernacular_title: Priapismo asociado a enfermedad de Fabry.