Abstract

OBJECTIVE

Factor VII (FVII) deficiency has autosomal recessive inheritance and become incre- asingly important in areas where consanguineous marriages are common as is in our country. Clinical heterogeneity is the hallmark of this hemorrhagic disorder. In this study we aimed to assess the relationship between the clinical characteristics and levels of FVII of the patients with FVII deficiencies followed-up in our center.

METHODS

Medical files of 14 patients who were followed in Dr. Behçet Uz Children’s Hospital since 1997 were retrieved. Demographic characteristics, age, initial findings, prothrom- bin time (PT) and FVII levels were obtained from patients’ files. Age at diagnosis, the relationship between FVII levels and PT was evaluated with a nonparametric Pearson correlation test.

RESULTS

Mean age at the time of diagnosis was 8.29±4.86 years. The mean values of PZ and FVII were found as 24 (13.2-82.0) sec and 22.3% (2.32-45.0) respectively. Six patients were presented with different symptoms as intracranial hemorrhage (14.2%), post-traumatic splenic hematoma (7.1%), menorrhagia (7.1%), ecchymosis (7,1%) and epistaxis (7,1%). Eight patients admitted to the pre-operative prolonged PT. The patients’ prolonged PT values were associated with decreased FVII levels (r=-0.62, p=0.02). However no relationship was found between FVII levels and age at diagnosis (r=0.27, p=0.34). PT values were correlated with age of the patients at diag- nosis (r=-0.59, p=0.03).

CONCLUSION

Clinical heterogeneity and its independence to the levels of FVII were also observed in our study A significant correlation was found between PT and age at diagnosis. However, the level of FVII is not associated with age at diagnosis. This finding can be explained by indepen- dence of clinic findings from FVII level and some patients’ asympomatic clinical course.