Isolated congenital factor VII deficiency
DOI:
https://doi.org/10.32677/IJCH.2019.v06.i11.015Keywords:
Congenital factor VII deficiency, Inherited bleeding disorder, Recombinant factor VIIaAbstract
Congenital factor VII (FVII) (proconvertin) is a rare autosomal recessive bleeding disorder. Bleeding manifestations and clinical findings vary widely, ranging from being asymptomatic to life-threatening bleeding. Intracranial bleeding is relatively less common with inherited FVII deficiency than with other coagulation disorders. We report a rare case of congenital FVII deficiency in an 11-year-old male child. The patient had recurrent subdural hemorrhages. The prothrombin time was markedly prolonged with a normal bleeding time, normal partial thromboplastin time and normal platelet count. Treatment consists of replacement therapy with fresh frozen plasma, prothrombin complex concentrates or plasma-derived FVII concentrates, and/or recombinant factor VIIa. Clinical heterogeneity is the hallmark of this disorder.
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Copyright (c) 2020 Anand Suresh Somkuwar, Saira Merchant, Rajkumar Meshram, Mukesh Waghmare, Manisha Mahalinge, Tushar Somkuwar
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