Congenital factor VII deficiency in children at tertiary health care facility in Pakistan

Muhammad Matloob Alam, Bushra Moiz, Karim Abdur Rehman, Priyanka Jethwani, Zehra Fadoo

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3 Citations (Scopus)


This study presents the demographics, clinical spectrum, and outcome of patients with congenital factor VII (FVII) deficiency at a tertiary care center over a period of 12 years. Of the 49 patients, 27 (55%) patients were males. Consanguinity was found in 92% of the patients. The median age of symptom onset was 2.4 (interquartile range [IQR]: 1.1-6.5) years with a median age of 5.8 (IQR: 3.1-10) years at diagnosis. Life-threatening complications like intracranial bleeding (ICB) and intra-abdominal bleeding (IAB) were observed in 8 (16.4%) patients. We found that 11 (55%) of the 20 patients with FVII coagulant activity (FVIIc) <1% were either asymptomatic or showed mild phenotype. In contrast, 9 (53%) of the 17 patients with FVIIc >5% were affected by severe symptoms. Age <1 year was the only identified risk factor associated with development of life-threatening bleeding episodes (P =.042; odds ratio 6.46). Overall, 4 (8.2%) died as a consequence of ICB (3 patients) and IAB (1 patient).

Original languageEnglish
Pages (from-to)639-644
Number of pages6
JournalClinical and Applied Thrombosis/Hemostasis
Issue number7
Publication statusPublished - 5 Oct 2015


  • bleeding disorder
  • children
  • consanguinity
  • factor VII deficiency
  • intracranial bleeding


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