CAJ | 학술논문

目的了解目前中国学龄前期重型及中间型血友病患儿关节出血和危及生命出血的出血模式及预后,为制定中国儿童血友病治疗策略提供依据.方法收集2008年至2010年首都医科大学附属北京儿童医院6岁以下的重型、中间型血友病患儿首诊临床资料,而后进行跟踪随访,记录临床出血情况.结果可供分析病例119例,入组年龄为(28.40± 18.61)个月,110例(92.4％)接受随访,随访时间(20.38±9.74)个月.(1)首次出血情况:重型患儿首次出血和首次关节出血年龄分别为(8.86±5.41)个月和(20.00±17.00)个月,早于中间型患儿(P=0.034、0.006),2组间首次危及生命出血年龄、关节出血和危及生命出血的发生率比较差异均无统计学意义(P均＞0.05).(2)关节出血模式及预后:随年龄增长患儿关节出血发生率增加明显,半数患儿发生关节出血和频繁关节出血的年龄分别在3岁和6岁左右.(3)危及生命出血模式及预后:危及生命出血发生率为38.7％,病死率10.9％,65.2％发生于2岁前,其中颅内出血占54.4％,病死率8.7％.结论我国学龄前期重型及中间型血友病患儿关节出血发生率高、进展快,危及生命出血以颅内出血多见,其发生率、病死率均较高,中间型患儿情况不容忽视.
목적 료해목전중국학령전기중형급중간형혈우병환인관절출혈화위급생명출혈적출혈모식급예후,위제정중국인동혈우병치료책략제공의거.방법 수집2008년지2010년수도의과대학부속북경인동의원6세이하적중형、중간형혈우병환인수진림상자료,이후진행근종수방,기록림상출혈정황.결과 가공분석병례119례,입조년령위(28.40± 18.61)개월,110례(92.4％)접수수방,수방시간(20.38±9.74)개월.(1)수차출혈정황:중형환인수차출혈화수차관절출혈년령분별위(8.86±5.41)개월화(20.00±17.00)개월,조우중간형환인(P=0.034、0.006),2조간수차위급생명출혈년령、관절출혈화위급생명출혈적발생솔비교차이균무통계학의의(P균＞0.05).(2)관절출혈모식급예후:수년령증장환인관절출혈발생솔증가명현,반수환인발생관절출혈화빈번관절출혈적년령분별재3세화6세좌우.(3)위급생명출혈모식급예후:위급생명출혈발생솔위38.7％,병사솔10.9％,65.2％발생우2세전,기중로내출혈점54.4％,병사솔8.7％.결론 아국학령전기중형급중간형혈우병환인관절출혈발생솔고、진전쾌,위급생명출혈이로내출혈다견,기발생솔、병사솔균교고,중간형환인정황불용홀시.
Objective To investigate the pattern and prognosis of joint and life-threatening bleeding due to moderate and severe preschool hemophilia in China,and to provide a data base for formulating children hemophilia treatment strategies suitable for Chinese children.Methods Data were collected from the records of the first clinic visit of severe and moderate hemophilia children under 6 years old at the Beijing Children's Hospital Affiliated to Capital Medical University during 2008 to 2010,then they were followed up for the clinical bleeding records.Results One hundred and nineteen cases were available for study analysis,and the enrolled age was (28.40 ± 18.61) months,110 cases (92.4％) received follow-up,the duration being (20.38 ± 9.74) months.(1) Status of first bleeding:time of the first bleeding and joint bleeding of severe children were (8.86 ± 5.41) months (P =0.034) and (20.00 ± 17.00) months(P =0.006),respectively,which were earlier than moderate patients.There was no significant difference between 2 groups in first life-threatening bleeding age and the incidence of joint and life-threatening bleeding (P ＞ 0.05).(2) Pattern and prognosis of joint bleeding:morbidity increased rapidly with age,time of half of the children with joint bleeding and frequent joint bleeding was 3 years old and 6 years old,respectively.(3) Pattern and prognosis of life-threatening bleeding:morbidity of life-threatening bleeding was 38.7％,mortality was 10.9％,and 65.2％ bleeding occurred in their first 2 years.Mortality of intracranial hemorrhage was 54.4％,and mortality was 8.7％.Conclusions At present,severe and moderate preschool hemophilia in China has a high mortality and rapid deterioration of joint bleeding.Intracranial hemorrhage is very common in life-threatening bleeding,which has a high incidence and mortality.The situation of moderate patients could not be ignored.