title: An infant with prolonged circumcision bleeding and unexplained coagulopathy.
abstract: Tyrosinemia type I is a rare autosomal recessive disorder. Fulminant onset of liver failure can occur in the first few months of life. Because all of the clotting factors are produced exclusively in the liver except factor VIII, coagulation abnormalities are very common in patients with severe liver disease. Rarely a significant coagulopathy in the absence of overt signs of liver disease may be seen in hereditary tyrosinemia. We present a 4 weeks-old tyrosinemic infant who presented with severe bleeding after circumcision and no other signs of liver failure. The diagnosis of tyrosinemia should be kept in mind in differential diagnosis of bleeding disorders especially a severe coagulopathy unresponsive to vitamin K, and fresh frozen plasma, even when other signs of liver failure are absent.
authors.affiliation: Division of Pediatric Hematology, Department of Pediatrics, Gaziantep Universitesi Tip Fakultesi Cocuk Hastaliklari Klinigi, Gaziantep University, Gaziantep, Turkey.
authors.forename: Ali
authors.initials: A
authors.lastname: Bay
authors.forename: Ozlem
authors.initials: O
authors.lastname: Karaoglu
authors.forename: Ercan
authors.initials: E
authors.lastname: Sivasli
authors.forename: Göksel
authors.initials: G
authors.lastname: Leblebisatan
authors.forename: Mehmet
authors.initials: M
authors.lastname: Keskin
keywords: Bleeding; Liver failure; Tyrosinemia