中华风湿病学杂志
中華風濕病學雜誌
중화풍습병학잡지
CHINESE JOURNAL OF RHEUMATOLOGY
2010年
7期
477-479
,共3页
郑玉灿%钱小青%李娟%张雅媛%俞海国
鄭玉燦%錢小青%李娟%張雅媛%俞海國
정옥찬%전소청%리연%장아원%유해국
组织细胞增多症,非郎格尔汉斯细胞%儿童%巨噬细胞活化综合征
組織細胞增多癥,非郎格爾漢斯細胞%兒童%巨噬細胞活化綜閤徵
조직세포증다증,비랑격이한사세포%인동%거서세포활화종합정
Histiocytosis,non-Langerhans-cell%Child%Macrophage activation syndrome
目的 观察儿童巨噬细胞活化综合征(MAS)与其他噬血细胞综合征(HPS)的临床特点,探究两者在临床特点、诊断、治疗及预后上的不同之处.方法 收集2006年1月至2009年3月所有HPS患儿共36例,分为MAS组13例,其他HPS 23例.运用t检验、χ2检验及Fisher精确概率法统计两者在临床特点、实验室指标、治疗方法及预后情况上的差异.结果 MAS患儿年龄明显大于其他HPS患儿[(7.7±1.3)岁和(2.6±0.5)岁,t=3.899,P＝0.004];而性别分布上差异无统计学意义.临床表现上MAS患儿中枢神经系统受累(69%和13%,P=0.001)、循环系统受累(23%和9%,P=0.047)及泌尿系统受累(38%和9%,P=0.033)更为常见且临床症状更为严重.在实验宅检查上,MAS患儿血清铁蛋白的增高、红细胞沉降率(ESR)的动态变化更为明显,其中铁蛋白增高[(9703±9819) μg/L和(4569μ1396) μg/L,t:2.854,P=0.015]、ESR动态变化[(53±32)mm/1 h和(20±14)mm/1 h,t=2.708,P=0.020].结论 MAS与其他HPS在发病年龄、病因、各系统的累及、临床表现的严重程度、一些实验室检查变化及治疗方法上有所差别.
目的 觀察兒童巨噬細胞活化綜閤徵(MAS)與其他噬血細胞綜閤徵(HPS)的臨床特點,探究兩者在臨床特點、診斷、治療及預後上的不同之處.方法 收集2006年1月至2009年3月所有HPS患兒共36例,分為MAS組13例,其他HPS 23例.運用t檢驗、χ2檢驗及Fisher精確概率法統計兩者在臨床特點、實驗室指標、治療方法及預後情況上的差異.結果 MAS患兒年齡明顯大于其他HPS患兒[(7.7±1.3)歲和(2.6±0.5)歲,t=3.899,P＝0.004];而性彆分佈上差異無統計學意義.臨床錶現上MAS患兒中樞神經繫統受纍(69%和13%,P=0.001)、循環繫統受纍(23%和9%,P=0.047)及泌尿繫統受纍(38%和9%,P=0.033)更為常見且臨床癥狀更為嚴重.在實驗宅檢查上,MAS患兒血清鐵蛋白的增高、紅細胞沉降率(ESR)的動態變化更為明顯,其中鐵蛋白增高[(9703±9819) μg/L和(4569μ1396) μg/L,t:2.854,P=0.015]、ESR動態變化[(53±32)mm/1 h和(20±14)mm/1 h,t=2.708,P=0.020].結論 MAS與其他HPS在髮病年齡、病因、各繫統的纍及、臨床錶現的嚴重程度、一些實驗室檢查變化及治療方法上有所差彆.
목적 관찰인동거서세포활화종합정(MAS)여기타서혈세포종합정(HPS)적림상특점,탐구량자재림상특점、진단、치료급예후상적불동지처.방법 수집2006년1월지2009년3월소유HPS환인공36례,분위MAS조13례,기타HPS 23례.운용t검험、χ2검험급Fisher정학개솔법통계량자재림상특점、실험실지표、치료방법급예후정황상적차이.결과 MAS환인년령명현대우기타HPS환인[(7.7±1.3)세화(2.6±0.5)세,t=3.899,P＝0.004];이성별분포상차이무통계학의의.림상표현상MAS환인중추신경계통수루(69%화13%,P=0.001)、순배계통수루(23%화9%,P=0.047)급비뇨계통수루(38%화9%,P=0.033)경위상견차림상증상경위엄중.재실험택검사상,MAS환인혈청철단백적증고、홍세포침강솔(ESR)적동태변화경위명현,기중철단백증고[(9703±9819) μg/L화(4569μ1396) μg/L,t:2.854,P=0.015]、ESR동태변화[(53±32)mm/1 h화(20±14)mm/1 h,t=2.708,P=0.020].결론 MAS여기타HPS재발병년령、병인、각계통적루급、림상표현적엄중정도、일사실험실검사변화급치료방법상유소차별.
Objective To compare the clinical features, diagnosis, treatment and prognosis between macrophages activation syndrome (MAS) and other hemophagocytic syndrome (HPS). Methods Thirty-six children with HPS were identified at Nanjing Children's Hospital during January 2006 to March 2009. They could be classi-fied into MAS group (13 patients) and other HPS group (23 patients). All relevant clinical features, laboratory data, treatments and outcomes were analyzed with t test,χ2 test and Fisher's exact test.Results Patients with MAS tended to be elder than those with other HPSs [(7.7±1.3) years vs (2.6±0.5)years, t=3.899, P=0.004]. There was no difference in gender distribution. In MAS cases, the central nervous system (69% vs 13%, P=0.001), circulatory system (23% vs 9%, P=0.047) and the urinary system (38% vs 9%, P=0.033) were usually involved. The clinical symptoms of MAS were more sever than other HPS. Serum ferritin [(9703±9819) μg/L vs (4569±1396) μg/L, t=2.854, P=0.015] and erythrocyte sedimentation rate (ESR) [(53±32) mm/1 h vs (20±14) mm/1 h, t=2.708, P=0.020] changed more obviously in MAS cases compared with other HPS. Conclusion, Childhood MAS is different from other HPS in terms of age, etiology, clinical manifestations, laboratory tests and treatments.
