Paediatrics and Child Health
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).
Clinical and Applied Thrombosis/Hemostasis
Alam, M. M.,
Rehman, K. A.,
(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