Isolated congenital factor VII deficiency

Authors

  • Anand Suresh Somkuwar
  • Saira Merchant
  • Rajkumar Meshram
  • Mukesh Waghmare
  • Manisha Mahalinge
  • Tushar Somkuwar

DOI:

https://doi.org/10.32677/IJCH.2019.v06.i11.015

Keywords:

Congenital factor VII deficiency, Inherited bleeding disorder, Recombinant factor VIIa

Abstract

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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Published

2019-11-26

Issue

Section

Case Reports

How to Cite

Isolated congenital factor VII deficiency. (2019). Indian Journal of Child Health, 6(11), 629-631. https://doi.org/10.32677/IJCH.2019.v06.i11.015