Priapism and Fabry disease: a case report.

Abstract:

:A 10-year-old boy presented with priapism of 10 h duration which after unsuccessful conservative measures, was relieved by a saphenocorporeal shunt. A 4-year history of intermittent vague aching of fingers and toes accompanied by low-grade fever was reported. Fabry disease was confirmed by the lack alpha-galactosidase activity 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.

journal_name

Eur J Pediatr

authors

García-Consuegra J,Padrón M,Jaureguizar E,Carrascosa C,Ramos J

doi

10.1007/BF01959404

subject

Has Abstract

pub_date

1990-04-01 00:00:00

pages

500-1

issue

7

eissn

0340-6199

issn

1432-1076

journal_volume

149

pub_type

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