凝血功能障碍患儿临床表现、实验室检查及预后分析

Clinical manifestation,laboratory examination and prognosis analysis of children with coagulation disorders

目的 分析凝血功能障碍(CD)患儿的临床表现、实验室检查、预后等情况。方法 选取自2017年7月至2022年6月安徽省儿童医院收治的209例CD患儿为研究对象,并进行肝肾功能、骨髓细胞学检测等相关检查,寻找病因及原发病。比较遗传性CD患儿与获得性CD患儿出血情况、凝血功能指标、肝功能指标、肾功能指标及预后情况。结果 209例CD患儿中,遗传性CD 38例(纳入遗传性CD组),获得性CD 171例(纳入获得性CD组)。获得性CD组部分活化凝血酶原时间、纤维蛋白原低于遗传性CD组,总胆红素、丙氨酸氨基转移酶、血肌酐水平高于遗传性CD组,差异有统计学意义(P<0.05)。两组患儿均未出现死亡,预后良好。结论 儿童获得性CD多见,出血部位主要为口鼻腔和皮肤黏膜;遗传性CD需要特异性凝血因子终生替代治疗,预后取决于凝血因子缺乏的程度及干预的积极性;获得性CD需要从根本上治疗原发病,血液制品短期替代治疗,预后主要取决于原发病的治疗效果。

Objective To summarize the clinical manifestations,laboratory examination and prognosis of children with coagulation dis-orders(CD).Methods A total of 209 children with CD treated in Children's Hospital of Anhui Province from July 2017 to June 2022 were selected as the study objects.Liver and kidney function,bone marrow cytology and other related tests were performed to find the cause and primary disease.To compare the bleeding,coagulation function,liver function,kidney function and prognosis of inherited CD children and acquired CD children.Results Among the 209 children with CD,38 were hereditary CD(included in the hereditary CD group)and 171 were acquired CD(included in the acquired CD group).The partially activated prothrombin time and fibrinogen in ac-quired CD group were lower than those in inherited CD group,while the levels of total bilirubin,alanine aminotransferase and serum creatinine in acquired CD group were higher than those in inherited CD group,and the differences were statistically significant(P<0.05).There was no death in the two groups,and the prognosis was good.Conclusion Acquired CD was common in children,and the bleeding sites were mainly mouth,nasal cavity and skin mucosa.Hereditary CD requires a lifetime of specific coagulation factor re-placement therapy,and the prognosis depends on the degree of coagulation factor deficiency and the enthusiasm of intervention.Ac-quired CD requires the fundamental treatment of the primary disease,and the short-term replacement treatment of blood products,and the prognosis mainly depends on the therapeutic effect of the primary disease.