Abstract
A 10-year-old boy presented with priapism of 10h duration which after unsuccessful conservative measures, was relieved by a saphenocorporeal shunt. A4-year history of intermittent vague aching of fingers and toes accompanied by lowgrade fever was reported. Fabry disease was confirmed by the lack α-galactosidase acitivity in serum and leucocytes. Other characteristic features of Fabry disease were not present. The condition was also diagnosed in his 14-year-old brother and suspected in his maternal grandfather who died at the age of 49 from renal failure of unknown aetiology.
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References
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García-Consuegra, J., Padrón, M., Jaureguizar, E. et al. Priapism and Fabry disease: A case report. Eur J Pediatr 149, 500–501 (1990). https://doi.org/10.1007/BF01959404
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DOI: https://doi.org/10.1007/BF01959404