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ACTH therapy for West syndrome with severe hemophilia A

Hemophilia A is an X-linked recessive disorder caused by factor VIII deficiency, which is an important factor in the coagulation system. Here, we describe a 1-year-old boy with hemophilia A who developed West syndrome (WS). Recombinant factor VIII was administered during adrenocorticotropic hormone...

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Detalles Bibliográficos
Autores principales: Kubota, Kazuo, Kinomura, Yoriko, Yamamoto, Takahiro, Ozeki, Michio, Kawamoto, Minako, Kawamoto, Norio, Fukao, Toshiyuki
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5118558/
https://www.ncbi.nlm.nih.gov/pubmed/27896066
http://dx.doi.org/10.1016/j.ebcr.2016.04.007
Descripción
Sumario:Hemophilia A is an X-linked recessive disorder caused by factor VIII deficiency, which is an important factor in the coagulation system. Here, we describe a 1-year-old boy with hemophilia A who developed West syndrome (WS). Recombinant factor VIII was administered during adrenocorticotropic hormone (ACTH) therapy to prevent intracranial hemorrhage. Infusion of factor VIII at fixed intervals is useful for the safe administration of ACTH therapy for patients with WS with severe hemophilia A. A coagulation screening test should be performed before ACTH therapy.