超小剂量肝素皮下注射治疗新生儿弥散性血管内凝血的临床应用

目的观察超小剂量肝素皮下注射对新生儿弥散性血管内凝血(DIC)的疗效。方法42例确诊DIC的患儿随机分为常规剂量组及超小剂量组:超小剂量组20例,以超小剂量肝素(普通肝素)皮下注射,剂量为6U/(kg·次),每6~8小时1次;常规剂量组22例,剂量为60~125U/(kg·次)静滴,必要时可4~8h给药1次;两组其他治疗原则相同。观察两组治愈率,治疗过程中每12~24小时监测凝血酶时间(TT)、活性部分凝血活酶时间(APTT)、凝血酶原时间(PT)、纤维蛋白原(Fib)、血小板计数等出凝血指标。结果超小剂量肝素组治愈率(85·5%)明显高于常规剂量肝素组(55·6%),P<0·05。超小剂量组17例治愈患儿用药24h后与用药前比较PT缩短(P<0·05)、Fib升高(P<0·05),48h后PT、APTT、TT与用药前比较均缩短(P<0·05),Fib升高(P<0·05),而常规剂量组12例治愈患儿用药24h后PT较用药前延长(P<0·05),APTT、TT、Fib无明显改变(P>0·05),用药48h后PT、TT均延长,与用药前比较差异有显著性(P<0·05)。血小板恢复较慢,两组用药24h及用药48h后与用药前比较差异均无显著性(P>0·05)。用药24h后超小剂量组与常规剂量组PT、Fib差异有显著性(P<0·05),48h后两组PT、APTT、Fib差异有显著性(P<0·05)。结论皮下注射超小剂量肝素治疗新生儿DIC安全、有效,值得推广应用。     

危重患儿凝血功能障碍及弥散性血管内凝血临床分析

目的了解危重患儿凝血功能障碍及弥散性血管内凝血(DIC)患病率及病死率,总结DIC诊断及临床应用肝素预防和治疗的体会。方法对所有PICU住院患儿进行小儿危重评分或美国PICU入出院指南评估及凝血功能监测,确诊DIC者根据临床出血情况应用不同剂量肝素。凝血功能障碍者应用小剂量肝素。结果PICU危重患儿病死率为8.6%。凝血功能异常发生率28.6%,病死率25.7%。其中DIC发生率10.6%,病死率45.2%。凝血功能障碍发生率18.0%,病死率14.1%。原发病为脓毒症者73.8%。在DIC早、中期阶段,使用肝素200～240U/(kg·d),有较好疗效。凝血功能障碍者应用肝素60～120/(kg·d),极少发展为DIC。结论DIC是导致危重患儿死亡的重要原因之一,其主要病因是严重感染性疾病。早期合理应用肝素治疗有效,晚期治疗非常困难。小剂量肝素用于危重患儿可能具有预防DIC的作用。     

弥散性血管内凝血的研究进展

弥散性血管内凝血是一种获得性的临床综合征，发生在许多疾病的病理过程中。由于DIC发病机理比较复杂，诊断和治疗尚有一定困难。本文仅就DIC的发病机理、实验室检查和治疗方面的研究进展，作一扼要综述。     

胎盘早剥早产儿脐血与产妇静脉血血管性血友病因子及其裂解酶水平的变化

目的观察胎盘早剥新生儿脐血与产妇静脉血血管性血友病因子(VWF)及血管性血友病因子裂解酶(ADAMTS13)水平变化,探讨胎盘早剥新生儿凝血功能异常的病理机制。方法北京军区总医院、北京妇产医院、北京市海淀医院产科2010年11月-2011年11月产科住院病人中,纳入发生胎盘早剥的产妇及新生儿作为胎盘早剥观察组(早剥组);非胎盘早剥观察组(非早剥组)为上述单位早剥组每例患者后2名产科入院患者,以办理入院登记时间为准。采集产妇的静脉血及新生儿脐静脉血、脐动脉血,并收集胎盘组织,用ELISA法检测其VWF、ADAMTS13水平。结果早剥组共纳入58例,非早剥组共纳入116例。根据非早剥组检测值计算出单侧95%参考值。产妇静脉血:VWF<857.80 U.L-1,ADAMTS13>119.63 U.L-1;脐静脉血:VWF<852.71 U.L-1,ADAMTS13>28.12 U.L-1;脐动脉血:VWF<1 416.96 U.L-1,ADAMTS13>147.90 U.L-1;胎盘组织VWF<760.66 U.L-1,ADAMTS13>27.53 U.L-1。早剥组各项VWF高于非早剥组(Pa<0.05);早剥组产妇血及脐血ADAMTS13水平均低于非早剥组(Pa<0.05)。结论胎盘早剥孕妇/产妇及胎儿/新生儿体内ADAMTS13水平随VWF水平的升高而下降,VWF是胎盘早剥发生时母儿高凝状态的因素之一,ADAMTS13具有保护作用。     

弥散性血管内凝血的研究进展

弥散性血管内凝血（DIC）是一种获得必出血综合征，发生在许多疾病的病理过程中，研究发现多种细胞因子释放、生理抗凝固子缺乏及纤维蛋白溶解受抑制等在DIC发病机制中起重要作用。一些新的实验室检查项目以及较敏感或特异指标的测定对DIC的诊断提供了更加可靠的依据，随着对该病发病机制的认识，抗凝血酶Ⅲ、蛋白C浓缩剂、在子抑制剂等新药不断产生，并在临床试用，有些取得了良好的效果。     

弥散性血管内凝血的研究进展

弥散性血管内凝血是一种获得性的临床综合症，发生在许多疾病的病理过程中。由于ＤＩＣ发病机理比较复杂，诊断和治疗尚有一定困难，本文仅就ＤＩＣ的发病机理、实验室检查和治疗方面的研究进展，作一扼要综述。     

弥散性血管内凝血的研究进展

弥散性血管内凝血（ＤＩＣ）是一种获得性出血综合征,发生在许多疾病的病理过程中。研究发现多种细胞团子释放、生理抗凝因子缺乏及纤维蛋白溶解受抑制等在ＤＩＣ发病机制中起重要作用。一些新的实验室检查项目以及较敏感或特异指标的测定对ＤＩＣ的诊断提供了更加可靠的依据。随着对该病发病机制的认识,抗凝血酶Ⅲ蛋白Ｃ浓缩剂、组织因子抑制剂等新药不断产生,并在临床试用,有些取得了良好的效果。     

低分子肝素治疗新生儿脓毒症并弥散性血管内凝血的疗效

目的探讨低分子肝素治疗新生儿脓毒症并DIC的疗效。方法将本院收治的56例脓毒症并DIC的新生儿随机分成对照组和试验组,每组28例。除常规基础治疗外,对照组采用普通肝素治疗,试验组则采用低分子肝素治疗。观察2组临床疗效及其治疗前后DIC指标水平的变化情况。结果试验组肝素使用时间[(3.0±0.5)d]、出血停止时间[(1.3±0.2)d]、DIC指标恢复正常时间[(3.8±0.2)d]均显著短于对照组[(6.4±0.7)d、(4.5±0.3)d、(6.2±0.5)d](t=4.28、5.81、2.76,Pa<0.05),试验组出血加重例数(5例)显著少于对照组(0例)(χ2=2.32,P<0.05);2组治疗前后的DIC指标水平变化比较差异均有统计学意义(Pa<0.05)。结论采用低分子肝素治疗新生儿脓毒症并DIC的效果显著。     

Disseminated Intravascular Coagulation Syndrome in Anorexia Nervosa

A 15 year old boy with anorexia nervosa developed disseminated intravascular coagulation syndrome (DIC). Because of severe cachexia he had been admitted to the Shimane Prefectural Central Hospital. During his hospitalization he developed generalized massive ecchymosis. Laboratory data revealed not only DIC but also multiple organ complications. The patient was treated intravenously with FOY®(gabexate mesilate, a protease inhibitor), heparin, a transfusion of fresh frozen plasma, antithrombin III concentrates and platelets. Intravenous hyperalimentation was also administered. The laboratory data, the general condition and the emotional state of the patient improved remarkably.
We emphasize the importance of keeping in mind coagulopathy as a complication in anorexia nervosa.     

Immunohistochemical Diagnosis of Disseminated Intravascular Coagulation in Newborns

Disseminated intravascular coagulation (DIC) and other clotting abnormalities are common in sick newborn infants who have a variety of conditions. To document evidence of DIC at autopsy, immunoperoxidase staining of fibrin-related antigens (FRA) was used to detect intravascular microthrombi in liver, kidney, and lung from 127 newborns. Patients were selected from seven major disease groups: hyaline membrane disease/bronchopulmonary dysplasia, infection, meconium aspiration, necrotizing enterocolitis, congenital heart disease, other congenital anomalies, and extreme prematurity. Staining for FRA in intravascular microthrombi was seen in 40% of cases studied. The liver showed the highest frequency of intravascular microthombri, located predominantly in the sinusoids. Unlike the adult kidney, the newborn kidney seldom had evidence of intravascular coagulation. Extravascular staining of FRA was observed in the renal distal tubular epithelium in 48 cases, many of which also had evidence of intravascular FRA staining. No significant differences in FRA staining patterns were seen among the disease groups except for cases of extreme prematurity in which all tissues showed minimal staining. Control tissues from SIDS patients also showed minimal FRA staining. Hepatic sinusoidal staining was the only tissue finding that correlated with thrombocytopenia, a clinical indicator of DIC. Despite the use of this immunohistochemical staining method, discrepancies between the clinical and autopsy diagnosis of DIC remain.     

弥散性血管内凝血的诊断与治疗

日本卫生福利部(JMHW)和国际血栓与止血学会(ISTH)制定的弥散性血管内凝血(DIC)评分系统诊断DIC简单易行,实用性强;治疗DIC应基于原发疾病,活化蛋白C和低分子量肝素的使用能明显降低脓毒症DIC的病死率。     

Disseminated Intravascular Coagulation in Post-Dysenteric Haemolytic Uraemic Syndrome

ABSTRACT. Coagulation studies were carried out in 14 children with haemolytic araemic syndrome that followed acute dysentery. Stool cultures showed Shigella dysenteriae in 3 cases and were sterile in the remainder. Prolongation of the prothrombin time, activated partial thromboplastin time and thrombin time and raised levels of fibrinogen degradation products were found in 12 cases, indicating the presence of disseminated intravascular coagulation. Renal histologic examination showed cortical necrosis in 7 cases, which was extensive in S and patchy in 2. Disseminated intravascular coagulation may have a role in the pathogenesis of haemolytic uraemic syndrome associated with acute dysentery.     

Use of Recombinant Thrombomodulin in Disseminated Intravascular Coagulation Complicated Hemophagocytic Lymphohistiocytosis

Hemophagocytic lymphohistiocytosis (HLH) is frequently lethal in its early phase due to complicating disseminated intravascular coagulation (DIC). The authors report a 14-mo-old girl with severe DIC complicating Epstein-Barr virus associated HLH. She was successfully treated with immunochemotherapy consisting mainly of etoposide and additional recombinant thrombomodulin (r-TM), a newly developed anticoagulant. Although the efficacy of r-TM cannot be proven in a single case report, additional anticoagulation therapy with r-TM is safe and may reduce early deaths in patients with DIC-complicated severe HLH. More clinical experience is required, although r-TM is currently licensed only in Japan.     

Screening for Autism Spectrum Disorders in Extremely Preterm Infants

BACKGROUND:: Extremely preterm (EP) infants screen positive for autism spectrum disorders (ASD) at high rates. However, it is not clear whether this is because of high rates of ASD in EPs or to high rates of false-positive screens for ASD in children with a high rate of underlying neurodevelopmental impairments. Combining a parent questionnaire designed to distinguish developmental delay from ASD with direct observation of infant behavior may more accurately screen for ASD in EPs. OBJECTIVES:: To determine rates of positive screen for ASD at 18 to 22 months(m) in EPs using 3 screens; to determine factors associated with a positive screen. METHODS:: Five hundred fifty-four infants born <27 weeks were screened at 18 to 22 m using the Pervasive Developmental Disorders Screening test, second edition Stage 2, and the response to name and response to joint attention items from the Autism Diagnostic Observation Schedule. Infants with severe cerebral palsy, deafness, and blindness were excluded. Associations between positive screen and neonatal/ infant characteristics were determined. RESULTS:: Of 554 infants, 113 (20%) had ≥ 1 positive screen. 10% had a positive Pervasive Developmental Disorders Screening test, second edition, 6% response to name, 9% response to joint attention; in only 1 % all 3 screens were positive. Positive screen was associated with male gender, more hospital days, white race, lower maternal education, abnormal behavioral scores, and cognitive/ language delay. CONCLUSIONS:: The use of 3 screens for ASD in EPs results in higher screen positive rates than use of 1 screen alone. Diagnostic confirmation is needed before true rates of ASD in EPs are known.     

Coagulation Studies in Preterm Infants with Respiratory Distress and Intracranial Haemorrhage

Coagulation studies were performed on 48 preterm infants with respiratory distress or recurrent apnoea who were considered to be at risk from intracranial haemorrhage. One or more coagulation abnormalities were detected in 16 cases though only one infant had a bleeding diathesis in life. Coagulation abnormalities were more frequent in infants subsequently found to have intracranial haemorrhage (intraventricular or subarachnoid haemorrhage) than in those with hyaline membrane disease only.