title: Factor V inhibitor in neonatal intracranial hemorrhage secondary to severe congenital factor V deficiency.
abstract: We report a newborn infant girl, born to consanguineous parents, with recurrent intracranial hemorrhage secondary to congenital factor V deficiency with factor V inhibitor. Repeated transfusions of fresh-frozen plasma (FFP) and platelet concentrates, administrations of immunosuppressive therapy (prednisolone and cyclophosphamide), and intravenous immunoglobulin failed to normalize the coagulation profiles. Exchange transfusion followed-up by administrations of activated prothrombin complex and transfusions of FFP and platelet concentrates caused a temporary normalization of coagulation profile, enabling an insertion of ventriculoperitoneal (VP) shunt for progressive hydrocephalus. The treatment was complicated by thrombosis of left brachial artery and ischemia of left middle finger. The child finally died from another episode of intracranial hemorrhage 10 days after insertion of the VP shunt.
authors.affiliation: Department of Pediatrics, University of Malaya Medical Center, Kuala Lumpur, Malaysia.
authors.forename: W S
authors.initials: WS
authors.lastname: Lee
authors.forename: L A
authors.initials: LA
authors.lastname: Chong
authors.forename: S
authors.initials: S
authors.lastname: Begum
authors.forename: W A
authors.initials: WA
authors.lastname: Abdullah
authors.forename: M T
authors.initials: MT
authors.lastname: Koh
authors.forename: E J
authors.initials: EJ
authors.lastname: Lim
mesh_terms: D001916:Brachial Artery; D002543:Cerebral Hemorrhage / Q000209:etiology*; D003131:Combined Modality Therapy; D003241:Consanguinity; D003520:Cyclophosphamide / Q000627:therapeutic use; D005078:Exchange Transfusion, Whole Blood; D005165:Factor V / Q000276:immunology*; D005166:Factor V Deficiency / Q000150:complications* / Q000276:immunology / Q000628:therapy; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D006849:Hydrocephalus / Q000209:etiology / Q000601:surgery; D007114:Immunization; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007231:Infant, Newborn; D007518:Isoantibodies / Q000096:biosynthesis* / Q000276:immunology; D010949:Plasma; D017713:Platelet Transfusion; D011239:Prednisolone / Q000627:therapeutic use; D012008:Recurrence; D013927:Thrombosis / Q000209:etiology; D017287:Ventriculoperitoneal Shunt
chemical_list: D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents; D007518:Isoantibodies; D003520:Cyclophosphamide; D005165:Factor V; D011239:Prednisolone