title: [Diagnosis of mild hemophilia A made by massive intraabdominal bleeding in a 13-year-old boy].
abstract: We report a 13-year-old boy who had massive intra-abdominal bleeding without a history of bleeding episodes or traumatic cause of bleeding. The patient underwent surgical treatment because bleeding was not controlled after treatment with tranexamic acid and transfusions including fresh-frozen plasma. Bleeding was traced to the lower left lobe of the liver. The mother's side of the family had a history of bleeding episodes in the boy's grandfather, great uncle, and son of a great aunt. A low level of plasma factor VIII coagulant activity (22%) led to a diagnosis of mild hemophilia A. Compared with severe hemophilia, mild hemophilia is more difficult to diagnose because bleeding episodes are less frequent. Most cases are found after incidental trauma or uncontrolled surgery-related bleeding, there is rarely a family history of hemophilia and activated partial thromboplastin time is normal or slightly prolonged. However, bleeding episodes in mild hemophilia may result in excessive, sometimes life-threatening hemorrhage and require early diagnosis and replacement treatment with adequate amounts of factor VIII, as in severe hemophilia.
authors.affiliation: Department of Pediatrics, Jikei University School of Medicine, Japan.
authors.forename: Yoko
authors.initials: Y
authors.lastname: Terao
authors.forename: Masaharu
authors.initials: M
authors.lastname: Akiyama
authors.forename: Kentaro
authors.initials: K
authors.lastname: Yokoi
authors.forename: Masayoshi
authors.initials: M
authors.lastname: Yamaoka
authors.forename: Mika
authors.initials: M
authors.lastname: Shimizu
authors.forename: Yoko
authors.initials: Y
authors.lastname: Kato
authors.forename: Keiichiro
authors.initials: K
authors.lastname: Tanaka
authors.forename: Yuji
authors.initials: Y
authors.lastname: Baba
authors.forename: Naruo
authors.initials: N
authors.lastname: Kuwashima
authors.forename: Shuichi
authors.initials: S
authors.lastname: Ashizuka
authors.forename: Jyoji
authors.initials: J
authors.lastname: Yoshizawa
authors.forename: Takanori
authors.initials: T
authors.lastname: Motoki
authors.forename: Yoshihiro
authors.initials: Y
authors.lastname: Saito
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Ida
mesh_terms: D000293:Adolescent; D005169:Factor VIII / Q000008:administration & dosage; D006467:Hemophilia A / Q000145:classification / Q000150:complications* / Q000175:diagnosis*; D006470:Hemorrhage / Q000209:etiology* / Q000601:surgery / Q000628:therapy; D006801:Humans; D008107:Liver Diseases / Q000209:etiology* / Q000601:surgery / Q000628:therapy; D008297:Male; D011182:Postoperative Care; D016896:Treatment Outcome
chemical_list: D005169:Factor VIII