Document Type
Article
Department
Paediatrics and Child Health
Abstract
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) 5% were affected by severe symptoms. Age (P = .042; odds ratio 6.46). Overall, 4 (8.2%) died as a consequence of ICB (3 patients) and IAB (1 patient).
Publication (Name of Journal)
Clinical and Applied Thrombosis/Hemostasis
Recommended Citation
Alam, M. M.,
Moiz, B.,
Rehman, K. A.,
Jethwani, P.,
Fadoo, Z.
(2015). Congenital factor VII deficiency in children at tertiary health care facility in Pakistan. Clinical and Applied Thrombosis/Hemostasis, 21(7), 639-644.
Available at:
https://ecommons.aku.edu/pakistan_fhs_mc_women_childhealth_paediatr/448