中华血液学杂志
中華血液學雜誌
중화혈액학잡지
Chinese Journal of Hematology
2013年
11期
913-917
,共5页
宣旻%付荣凤%薛峰%杨艳辉%张磊%杨仁池
宣旻%付榮鳳%薛峰%楊豔輝%張磊%楊仁池
선민%부영봉%설봉%양염휘%장뢰%양인지
血友病A%血友病B%儿童%抑制物
血友病A%血友病B%兒童%抑製物
혈우병A%혈우병B%인동%억제물
Hemophilia A%Hemophilia B%Child%Inhibitor
目的 了解近10年来非干预条件下单中心儿童血友病的发病和诊疗状况.方法 回顾性分析2002年1月至2012年12月520例血友病患儿的临床资料.结果 520例患儿均为男性,其中血友病A(HA)438例,血友病B(HB)82例;就诊原因均为自发性出血;以7~12岁患儿就诊人数最多;各型患儿之间APTT差异有统计学意义(P< 0.05);HA、HB患儿的诊断延迟时间分别为(1.42±2.81)、(1.17±2.56)年,低龄患儿首次出血部位以皮肤最为多见,随着年龄增长,首发症状为关节出血者逐渐增多,各型之间差异无统计学意义(P>0.05).低龄儿童以膝踝关节受累为主,随年龄增长肘肩关节出血逐渐增多.HA和HB患儿抑制物阳性率分别为8.9%(19/214)和12.8%(5/39),其中高滴度抑制物占78.9%(15/19)和40.0% (2/5);HA和HB患儿HCV感染率分别为2.8% (11/397)和2.5% (2/79).结论 近10年来血友病患儿的诊断延迟时间和血源性传播病毒感染率下降,抑制物成为血友病替代治疗中所面临的重要问题.
目的 瞭解近10年來非榦預條件下單中心兒童血友病的髮病和診療狀況.方法 迴顧性分析2002年1月至2012年12月520例血友病患兒的臨床資料.結果 520例患兒均為男性,其中血友病A(HA)438例,血友病B(HB)82例;就診原因均為自髮性齣血;以7~12歲患兒就診人數最多;各型患兒之間APTT差異有統計學意義(P< 0.05);HA、HB患兒的診斷延遲時間分彆為(1.42±2.81)、(1.17±2.56)年,低齡患兒首次齣血部位以皮膚最為多見,隨著年齡增長,首髮癥狀為關節齣血者逐漸增多,各型之間差異無統計學意義(P>0.05).低齡兒童以膝踝關節受纍為主,隨年齡增長肘肩關節齣血逐漸增多.HA和HB患兒抑製物暘性率分彆為8.9%(19/214)和12.8%(5/39),其中高滴度抑製物佔78.9%(15/19)和40.0% (2/5);HA和HB患兒HCV感染率分彆為2.8% (11/397)和2.5% (2/79).結論 近10年來血友病患兒的診斷延遲時間和血源性傳播病毒感染率下降,抑製物成為血友病替代治療中所麵臨的重要問題.
목적 료해근10년래비간예조건하단중심인동혈우병적발병화진료상황.방법 회고성분석2002년1월지2012년12월520례혈우병환인적림상자료.결과 520례환인균위남성,기중혈우병A(HA)438례,혈우병B(HB)82례;취진원인균위자발성출혈;이7~12세환인취진인수최다;각형환인지간APTT차이유통계학의의(P< 0.05);HA、HB환인적진단연지시간분별위(1.42±2.81)、(1.17±2.56)년,저령환인수차출혈부위이피부최위다견,수착년령증장,수발증상위관절출혈자축점증다,각형지간차이무통계학의의(P>0.05).저령인동이슬과관절수루위주,수년령증장주견관절출혈축점증다.HA화HB환인억제물양성솔분별위8.9%(19/214)화12.8%(5/39),기중고적도억제물점78.9%(15/19)화40.0% (2/5);HA화HB환인HCV감염솔분별위2.8% (11/397)화2.5% (2/79).결론 근10년래혈우병환인적진단연지시간화혈원성전파병독감염솔하강,억제물성위혈우병체대치료중소면림적중요문제.
Objective To analyze the clinical characteristics,diagnosis and treatment ot pediatric hemophilia in single center over the decade.Methods A retrospective study was conducted with 520 hemophilic children hospitalized in our medical center between January 2002 and December 2012.Results All the patients were male including 438 hemophilia A(HA) and 82 hemophila 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),induding 78.9% (15/19) and 40.0% (2/5) of high titer inhilator,and antiHCV-positive rate were 2.8% (11/397)and 2.5% (2/79),respectively.Conclusion 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.