Factor V inhibitor in neonatal intracranial hemorrhage secondary to severe congenital factor V deficiency

We report a newborn infant girl, born to consanguine ous 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 (pr...

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Main Authors: Lee, Way Seah, Chong, L.A., Begum, S., Abdullah, W.A., Koh, M.T., Lim, E.J.
Format: Article
Published: Lippincott, Williams & Wilkins 2001
Subjects:
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author Lee, Way Seah
Chong, L.A.
Begum, S.
Abdullah, W.A.
Koh, M.T.
Lim, E.J.
author_facet Lee, Way Seah
Chong, L.A.
Begum, S.
Abdullah, W.A.
Koh, M.T.
Lim, E.J.
author_sort Lee, Way Seah
collection UM
description We report a newborn infant girl, born to consanguine ous 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.
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spelling um.eprints-109242019-03-21T06:51:16Z http://eprints.um.edu.my/10924/ Factor V inhibitor in neonatal intracranial hemorrhage secondary to severe congenital factor V deficiency Lee, Way Seah Chong, L.A. Begum, S. Abdullah, W.A. Koh, M.T. Lim, E.J. R Medicine RJ Pediatrics We report a newborn infant girl, born to consanguine ous 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. Lippincott, Williams & Wilkins 2001 Article PeerReviewed Lee, Way Seah and Chong, L.A. and Begum, S. and Abdullah, W.A. and Koh, M.T. and Lim, E.J. (2001) Factor V inhibitor in neonatal intracranial hemorrhage secondary to severe congenital factor V deficiency. Journal of Pediatric Hematology/Oncology, 23 (4). pp. 244-246. ISSN 1077-4114, DOI https://doi.org/10.1097/00043426-200105000-00013 <https://doi.org/10.1097/00043426-200105000-00013>. http://journals.lww.com/jpho-online/Abstract/2001/05000/Factor_V_Inhibitor_in_Neonatal_Intracranial.13.aspx 10.1097/00043426-200105000-00013
spellingShingle R Medicine
RJ Pediatrics
Lee, Way Seah
Chong, L.A.
Begum, S.
Abdullah, W.A.
Koh, M.T.
Lim, E.J.
Factor V inhibitor in neonatal intracranial hemorrhage secondary to severe congenital factor V deficiency
title Factor V inhibitor in neonatal intracranial hemorrhage secondary to severe congenital factor V deficiency
title_full Factor V inhibitor in neonatal intracranial hemorrhage secondary to severe congenital factor V deficiency
title_fullStr Factor V inhibitor in neonatal intracranial hemorrhage secondary to severe congenital factor V deficiency
title_full_unstemmed Factor V inhibitor in neonatal intracranial hemorrhage secondary to severe congenital factor V deficiency
title_short Factor V inhibitor in neonatal intracranial hemorrhage secondary to severe congenital factor V deficiency
title_sort factor v inhibitor in neonatal intracranial hemorrhage secondary to severe congenital factor v deficiency
topic R Medicine
RJ Pediatrics
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