关键词: 儿童, 血友病, 颅内出血

Abstract: Objective Among all the bleeding episodes in hemophilic patients, intracranial hemorrhage(ICH) is associated with the highest mortality and the largest possibility of longterm serious sequelae. Knowing the clinical characteristic, treatment protocol and prognosis of ICH in children with hemophilia in China can help clinician to early diagnosis and treatment in order to reduce ICH sequelae and mortality. Methods 21 hemophilic children with ICH, which were registered in Hemophilia Treatment Center of Beijing Children’s Hospital between December 2006 and May 2009, were reviewed retrospectively. Data was analyzed by SPSS 11.5. Results ① The mean age of ICH was 2.8 years old. 66% of patients were less than 3 years old. 90% of patients were moderate or severe disease, and 76% had head trauma before ICH. ② Hemophilia were diagnosed after ICH in more than 50% of patients. All patients were less than 3 years old and 82% were moderate or severe hemophilia. ③ ICH in 7(33%) patients which were all moderate or severe recurred. 43% of patients were hemophilia B and 57% of patients with recurring ICH had poor prognosis. ④ 3 patients were dead and 5 had sequelae. The dosage of replacement therapy was lower and the period was shorter in team of poor prognosis than in team of good prognosis. Conclusion ① The risk factors of ICH in hemophilic children include moderate or severe disease, age with less than 3 years old and head trauma before ICH. ② ICH may be the first symptom of hemophilia in hemophilic infants, especially in mild and moderate patients. ③ The patients with hemophilia B, moderate or severe disease have more chances of recurring ICH. Recurring ICH has poor outcomes. ④ Delaying treatment, low dosage and insufficient replacement therapy should lead to poor prognosis.

Key words: children, hemophilia, intracranial hemorrhage