We described a case of obstructive uropathy due to retroperitoneal fibrosis associated with congenital factor VII deficiency. Nephrectomy and ileoureter reconstruction was performed uneventfully using recombinant factor Vlla perioperatively. There was no excessive bleeding or thrombosis. Key Words: Recombinant factor Vlla-rFVlla-Retroperitoneal fibrosis-Factor VII deficiency.
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