PubMed · 8642725
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J M Lusher, S Sarnaik. 1996-06-19. Hematology.. https://pubmed.ncbi.nlm.nih.gov/8642725/
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In patients with hemophilia, prothrombin complex concentrates (PCCs) have been successfully used to bypass inhibitors to factor VIII during bleeding episodes. The use of PCCS, including FEIBA (factor eight inhibitor bypassing activity), has been associated with thromboembolic complications. Myocardial infarction (MI) is a rare but serious complication, reported in 13 previous cases, six in the pediatric age group. In all four patients who died during the acute MI, autopsy revealed extensive myocardial hemorrhage. The hearts of three other patients examined at least 5 months after the acute MI showed no evidence of prior hemorrhage. Magnetic resonance (MR) imaging has been shown to be able to evaluate the sequelae of myocardial infarction in adults with coronary artery disease and in children with Kawasaki syndrome. We report the first case of the use of MR imaging in the evaluation of myocardial damage during the acute stage of a FEIBA-associated MI in a 10-year-old boy.
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BACKGROUND: Congenital factors VII and X deficiency is rare. Association of both deficiencies is exceptional. CASE REPORT: A 3 year-old boy, born to consanguinous Moroccan parents, had a prolonged partial thromboplastin time discovered fortuitously. This finding led to the diagnosis of combined factors VII and X deficiency. His siblings had the same deficiencies. CONCLUSION: Profound deficiencies in factors VII and X are inherited following an autosomal-recessive mode. These deficiencies may be asymptomatic, only discovered by prolonged partial thromboplastin time. They may also be revealed by intracranial bleeding and other severe hemorrhages. Treatment consists of administration of factor VII or PPSB.