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目的:探讨非婴幼儿获得性依赖VitK凝血因子缺乏症患者的临床特征及治疗方法。方法:回顾性分析在我院诊治的获得性依赖VitK凝血因子缺乏症26例患者的病因、临床表现、治疗以及随访情况。结果:18例患者病因不明(A组),6例为杀鼠药中毒(B组),2例为华法令过量(C组)。所有患者均同时或先后出现皮肤、黏膜、皮下及内脏出血,首发最常见出血部位(症状)为血尿;由于失血,15例患者Hb<100g/L,最低仅为36g/L;患者自出血症状始至明确诊断中位时间为10(2～120)d,其中11例患者1～4个月才确诊,各组自出血症状始至确诊的时间差异无统计学意义(P=0.81);全组有15例患者误诊,就诊时所有患者活化部分凝血活酶时间(APTT)、凝血酶原时间(PT)、国际标准化比值(INR)均显著延长或升高,血小板计数、凝血酶时间(TT)纤维蛋白原(Fg)均正常,各组APTT、PT、INR值差异无统计学意义,除2例华法令过量的患者仅停用华法令外,其余24例患者静脉给予VitK1(20～60)mg/d滴注1～2d后出血停止,部分患者同时使用凝血酶原复合物或(和)血浆输注,复查凝血指标恢复正常,治疗后患者APTT、PT及INR值与治疗前相比均差异有统计学意义(均P<0.01),静脉使用VitK1时间为1～10个月。长期静脉给予VitK治疗者中位随访6(1～20)个月,无一例患者复发。结论:非婴幼儿获得性依赖VitK凝血因子缺乏症多数原因不明,容易误诊。首发出血最常见的症状(部位)为血尿,半数以上的患者可因失血并发贫血。不明原因及杀鼠药中毒者临床表现、治疗反应及预后类似,均需接受长疗程静脉VitK治疗,预后良好。
Objective: To investigate the clinical characteristics and treatment of non-infants dependent on VitK deficiency factor. Methods: Retrospective analysis of the etiology, clinical manifestations, treatment and follow-up of 26 acquired and acquired VitK coagulation factor deficiency patients diagnosed and treated in our hospital. Results: The etiology of 18 patients was unknown (group A), 6 were poisonous rats (group B) and 2 were warfarin overdose (group C). All patients had skin, mucous membrane, subcutaneous and visceral hemorrhage at the same time or one after another. The most common bleeding site (symptom) in first episode was hematuria. Because of blood loss, 15 patients had Hb <100g / L, the lowest was only 36g / L; The median time to definite diagnosis was 10 (2 ~ 120) d, of which 11 patients were diagnosed within 1 to 4 months. There was no significant difference in the time from onset of bleeding to diagnosis (P = 0.81) Fifteen patients were misdiagnosed. The APTT, PT and INR were significantly prolonged or elevated in all patients at the time of diagnosis. The platelet count, thrombin time (TT) ) Fibrinogen (Fg) were normal, there was no significant difference in APTT, PT and INR between the two groups. Except for warfarin in 2 patients who were overdosed, only the other 24 patients were given VitK1 (20-60 ) mg / d infusion after 1 ~ 2d stop bleeding, some patients also use prothrombin complex or (and) plasma infusion, review coagulation index returned to normal after treatment, patients with APTT, PT and INR values compared with before treatment All the differences were statistically significant (all P <0.01), intravenous VitK1 for 1 to 10 months. Long-term intravenous VitK treatment of the median follow-up of 6 (1 ~ 20) months, no case of recurrence. CONCLUSIONS: Most of the causes of acquired deficiency of VitK clotting factor deficiency in infants and young children are unknown and easily misdiagnosed. The first most common symptom of hemorrhage (site) is hematuria, more than half of patients with anemia due to blood loss. Unidentified causes and clinical manifestations, response to treatment and similar prognosis of drug poisoning are required to receive a long course of intravenous VitK treatment with good prognosis.