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ACTH therapy for West syndrome with severe hemophilia A
Kazuo Kubota1, Yoriko Kinomura1, Takahiro Yamamoto1
1Department of Pediatrics, Gifu University Graduate School of Medicine, Gifu, Japan.
Abstract:
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.
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