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重庆医科大学附属儿童医院血液肿瘤科儿童发育疾病研究教育部重点实验室儿童发育重大疾病国家国际科技合作基地儿科学重庆市重点实验室
纸质出版日期:2016,
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[1]林莉,徐红珍,陈兰,管贤敏,郭玉霞,肖莉,于洁,苏庸春.儿童纤维蛋白原降低320例临床研究[J].重庆医科大学学报,2016,41(06):645-649.
Lin Li, Xu Hongzhen, Chen Lan, et al. Clinical study of 320 children with hypofibrinogenaemia[J]. Journal of Chongqing Medical University, 2016,41(6):645-649.
[1]林莉,徐红珍,陈兰,管贤敏,郭玉霞,肖莉,于洁,苏庸春.儿童纤维蛋白原降低320例临床研究[J].重庆医科大学学报,2016,41(06):645-649. DOI: 10.13406/j.cnki.cyxb.000926.
Lin Li, Xu Hongzhen, Chen Lan, et al. Clinical study of 320 children with hypofibrinogenaemia[J]. Journal of Chongqing Medical University, 2016,41(6):645-649. DOI: 10.13406/j.cnki.cyxb.000926.
目的:回顾性分析纤维蛋白原(fibrinogen
FIB)降低(FIB≤1.0 g/L)患儿的临床特点
寻找其可能相关继发因素
为FIB降低的病因、早期治疗及防治重要脏器出血提供参考依据。方法:回顾性分析我院320例住院患儿住院期间血浆FIB降低(FIB≤1.0 g/L)的病例资料
对纤维蛋白原降低程度、出血部位、出血发生率、出血程度、临床合并症、治疗反应等进行分析
用SPSS PASW18.0统计软件分析数据
用卡方检验分别对不同纤维蛋白原降低程度及有无合并其他凝血功能障碍或血小板降低时的出血发生率、出血程度进行分析
用t检验对有无输注冷沉淀前后的纤维蛋白原水平进行分析
以P<0.05判定差异有统计学意义
并根据样本量及统计情况进行P值矫正。结果:320例患儿中男性193例
女性127例;以新生儿多见(165例
51.6%)。其中281例(87.8%)患儿合并了已报道的可能导致FIB降低的继发因素
以肝功能损害(179例
55.9%)最常见;259例(80.9%)合并有其他凝血功能障碍。320例FIB降低病例中
162例(50.6%)为轻度降低;149例(46.6%)发生了不同程度的出血。发生出血的患儿中47%为轻度出血(70/149例)
36.9%为重度出血(55/149例)
且重度出血也见于FIB轻度降低者。经分析FIB不同降低程度的出血发生率(P=0.149)及出血严重程度(P=0.088)均无统计学差异。而FIB降低合并其他凝血障碍和/或血小板降低时的出血发生率及出血程度有明显升高(P<0.05)。出血可发生于任何部位
以胃肠道出血(71例
47.7%)最多见。出血组与未出血组分别有40.3%与34.5%的病例给予了FIB制品(新鲜冰冻血浆或冷沉淀)的补充
两组输注FIB制品前后FIB变化均有显著差异(P=0.001)
提示输注FIB制品有利于FIB的升高。结论:纤维蛋白原降低可能发生不同部位不同程度的出血
即使轻微降低仍可发生严重出血
临床应予以重视
当发现纤维蛋白原降低时
应根据患儿出血倾向综合考虑
及时积极采取合理治疗并随访纤维蛋白原水平。
Objective:To probe the clinical and laboratory features of childhood hypofibrinogenaemia
and to provide the clinical evidence for the further administration and prevention of severe hemorrhage. Methods:The clinical data(including the position and seriousness of hemorrhage
the incidence of hemorrhage
clinical complication
and the therapeutic response)of 320 hospitalized children with decreased plasma fibrinogen level(FIB≤1.0 g/L)were retrospectively analyzed by SPSS PASW18.0 software with Kruskal-Wallis Test and T-test(Kruskal-Wallis Test for the seriousness and incidence of hemorrhage
T-test for the therapeutic response). The significance level was set at P<0.05
and P value was corrected according to the sample size and statistics. Results:Totally 320 children(193 males and 127 females) were identified
in which the newborn accounted for 51.6%(165 cases). 281 cases(87.8%)were complicated with reported secondary factors leading by liver dysfunction(55.9 %)
259 cases(80.9%)were complicated with other coagulation disorders. Most cases(162 cases
50.6%)suffered from mild fibrinogen deficiency. There was no hemor-rhage in 171 cases(53.4%)and 149 cases developed hemorrhage(46.6%). Among the hemorrhage cases
47.0% developed mild and36.9% suffered from severe hemorrhage. It’s quite significant that some of the severe hemorrhage cases just got mild or moderate FIB deficiency. There was no significant difference in bleeding morbidity(P=0.149)and severity(P=0.088)among cases under different severities of hypofibrinogenaemia. If FIB deficiency combined with other coagulopathy or thrombocytopenia
both the bleeding morbidity(P <0.05) and severity(P <0.05) were significantly increased. Bleeding occurred in any system
especially in gastrointestinal tract. Fibrinogen product supplementation either FFP or cryoprecipitate were given to 40.3% hemorrhagic patients and 34.5% nonhemorrhagic patients respectively and part of them had FIB follow-up. Fibrinogen level of both groups had significantly elevated
indicating that fibrinogen products could restore plasma fibrinogen level. Conclusion:Hemorrhage possibly occurs when plasma fibrinogen decreased and severe hemorrhage can even occur in patient with mild hypofibrinogenaemia. Children with hypofibrinogenaemia should be carefully and considerably administered with appropriate products and be closely followed up.
纤维蛋白原降低儿童出血
hypofibrinogenaemiachildhoodhemorrhage
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