Clinical Analysis Of65Pediatric Haemophilic Patients

Objective:This research is carried out to analyze the clinical data of pediatric haemophilic paitents and to study the cause, the symptoms clinical presentation, the diagnosis, and the treatment of haemophilia.Method:Retrospective studies and analysis are carried out about the clinical datas of65pediatric haemophilic patients hospitalized in NO.l Affiliated Hospital of Guangxi Medicine University from January,2005to November,2011.Result:Among the65children haemophilic paitents,54children suffered from haemophilia A, comprising83.08%of cases, with13(24.07%) severe cases,22(40.74%) moderate cases,17(31.48%) mild cases and2(3.70%) subclinical cases;11children suffered from haemophilia B, comprising16.92%of cases, with3(27.27%) severe cases,4(36.36%) moderate cases,3(27.27%) mild cases and1(9.09%) subclinical case. The ratio of haemophilia A cases and haemophilia B cases was4.9to1. The median age of initial bleeding was1.0year old, and the age of initial diagnosis was2.2years old. Among the65patients,33(50.77%) children suffered from joint bleeding, there were13 severe,15moderate and5mild cases respective;17(26.15%) children suffered from joint deformity, among them7(53.85%) were cases of severe haemophilia A,6(27.27%) were cases of moderate haemophilia A,2(66.67%) were cases of severe haemophilia B,2(50.00%) were cases of moderate haemophilia B. Severe bleeding occurred in10children, all being severe and moderate cases.27children,24of whom were severe or moderate cases, suffered from bleeding in more than2positions. All the subclinical cases were all iatrogenic.18(27.69%) among65patients were linked to explicit family history of haemophilia.62children received replacement therapy,29(39.73%) children received clotting factor infusions only when acute bleeding occurred;35(47.95%) children received infusions of Cryoprecipitate and/or fresh frozen plasma besides clotting factor infusions;9(12.33%) children received infusions of Cryoprecipitate and/or fresh frozen plasma only. None of the patients received any prophylactic replacement therapy, nor did they receive any systematic test of infectious diseases related to blood products. There was a14-year old child with positive HCV antibody.Conclusion:(1) There are gaps between the outbreak age of the disease and the age of their initial diagnosis, which indicates delayed diagnosis;(2) Bleeding position and severity of bleeding are closely related to clinical form and classification of haemophilia;(3) The incidence of joint bleeding and joint deformity is high;(4) Many patients can not receive timely treatment, and clotting factor products can not be widely used in replacement therapy;(5) None of the children has received any systematic test of infectious diseases related to blood products.