Congenital factor VII deficiency in Iraqi children (Single Centre Experience)

Congenital factor VII deficiency in Iraqi children

  • Ali Ahmed Khudhair
  • Afrah Abdul-Mahdi Salih
  • Ausama Jamal Kadhum Baghdad university
Keywords: Bleeding disorder, Congenital factor VII deficiency

Abstract

Background and Objective: Factor VII (FVII) deficiency is probably one of the most common of the rare autosomal recessive coagulation disorders, with an estimated prevalence of l: 500000. All age groups can be affected with FVII deficiency. This study aimed to describe the demographic parameters, symptomatology, hemostatic values and the outcome of FVII deficiency.

Methods: This is a retrospective descriptive study of patients with congenital FVII deficiency over a period of seven years from (August 2008 to August 2015). The data were collected by reviewing the files for each patient diagnosed with FVII deficiency. Surgical interventions, complications and follow up visits were recorded.

Results: Twenty-four patients were included in this study, 17 females and seven males, below one year was the most common age at presentation. More than half of patients (58.3%) were diagnosed within six months of symptoms onset. The majority of patients had severe phenotype. The most common symptom was epitaxis (41.7%). Five out of 10 patients with FVII level < 1% have either mild to moderate phenotype of the disease without complications; while six out of 14 patients with FVII > 1% had at least one episode of severe bleeding. Three patients had hepatitis C; all were treated by blood products before the introduction of recombinant FVII in Iraq. The outcome of most patients (75%) was normal without complications at time of study.

Conclusion: Clinical manifestations of FVII deficiency are variable and they are not necessarily correlated to the FVII level.

doi: https://doi.org/10.12669/pjms.36.2.666

How to cite this:
Khudhair AA, Salih AAM, Kadhum AJ. Congenital factor VII deficiency in Iraqi children (Single Centre Experience). Pak J Med Sci. 2020;36(2):177-181. doi: https://doi.org/10.12669/pjms.36.2.666

This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Published
2019-12-15
How to Cite
Khudhair, A. A., Salih, A. A.-M., & Kadhum, A. (2019). Congenital factor VII deficiency in Iraqi children (Single Centre Experience). Pakistan Journal of Medical Sciences, 36(2). https://doi.org/10.12669/pjms.36.2.666
Section
Original Articles