Clinical characteristics of 520 children with hemophilia in a single medical center / 中华血液学杂志

ABSTRACT

<p><b>OBJECTIVE</b>To analyze the clinical characteristics, diagnosis and treatment of pediatric hemophilia in single center over the decade.</p><p><b>METHODS</b>A retrospective study was conducted with 520 hemophilic children hospitalized in our medical center between January 2002 and December 2012.</p><p><b>RESULTS</b>All the patients were male including 438 hemophilia A (HA) and 82 hemophilia B (HB). There were significant differences in APTT between severe and mild- to moderate hemophilia (P<0.05). In pediatric HA and HB, delay time of diagnosis were 1.42 and 1.17 year, respectively. Children of 7-12 years were the largest population of visiting a doctor, and the spontaneous bleeding episode was the main cause. The most common hemorrhage site was soft tissue in early childhood, but joint was increasingly affected with age as children growth. All bleeding sites and frequencies were not associated with plasma factor level of patient (P>0.05). Knee and anKle were mainly involved in early child, while elbow and shoulder were involved increasingly in later childhood. Additionally, in HA and HB, inhibitor occurrence were 8.9%(19/214) and 12.8%(5/39), inducing 78.9%(15/19) and 40.0%(2/5) of high titer inhalator, and antiHCV-positive rate were 2.8%(11/397) and 2.5%(2/79), respectively.</p><p><b>CONCLUSION</b>Our data highlights that delay in diagnosis and blood-borne infections were significantly reduced over the decade, but the development of inhibitor still remains a major challenge with wide-scale usage of factor in replacement therapy.</p>