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1.
目的探讨腹内高压(intra-abdominal hypertension,IAH)与急性胰腺炎(acutepancreatitis,AP)疾病严重程度的相关性,为临床诊治提供参考。方法选择AP患者58例,根据APACHEⅡ评分分为轻症急性胰腺炎(minor acute pancreatitis,MAP)组和重症急性胰腺炎(severe acut epancreatitis,SAP)组。监测腹内压(intra-abdominal pressure,IAP),比较两组IAH发生率,分析APACHEⅡ评分与IAP相关性;统计两组患者住院时间、并发症发生率、病死率等临床指标。将SAP组患者分为IAH组和非IAH组,比较两组临床指标情况。结果 MAP组患者IAH发生率为0.00%,SAP组患者IAH发生率为65.22%,差异有统计学意义(P0.01)。两组住院时间、并发症发生率及病死率比较,差异有统计学意义(P0.01)。SAP患者IAP与APACHEⅡ评分明显相关(r=0.769,P=0.043)。结论 IAH显著影响AP患者严重程度,SAP患者APACHEⅡ评分与IAP明显相关,SAP患者应全程监测IAP,做到早期发现与治疗,控制腹腔压力,有利于改善MAP情况、状态及脏器功能。  相似文献   

2.
目的探讨重症监护病房(ICU)中脓毒症相关急性肾损伤(SI-AKI)患者临床特点及连续性肾脏替代治疗(CRRT)时机对28 d预后的影响。方法回顾性分析2017年6月至2018年12月解放军总医院第一医学中心重症医学科SI-AKI患者44例,根据AKI发生48 h内是否行CRRT分为早期CRRT组29例和晚期CRRT组15例,比较2组患者各项生理功能指标及28 d预后情况。应用SPSS 17.0统计软件对数据进行分析。Kaplan-Meier生存分析患者28 d预后。结果根据KDIGO分期标准,AKI 1期13.6%(6/44),2期18.2%(8/44),3期68.2%(30/44)。短暂性AKI占18.2%(8/44),持续性AKI占81.8%(36/44)。AKI发生28 d时15例死亡。早期CRRT组相比晚期CRRT组患者糖尿病比例(31.0%和6.7%,P=0.048)高,CRRT时收缩压[(114±15)和(130±20)mmHg(1 mmHg=0.133 kPa),P=0.005]、平均动脉压[(82±11)和(91±18)mmHg,P=0.040]、血肌酐(197.0和418.9μmol/L,P=0.002)、尿素氮(12.9和35.0 mmol/L,P0.001)、血钙(1.9和2.0 mmol/L;P=0.007)、血镁(0.7和0.8 mmol/L,P=0.013)水平低,血红蛋白[(96±26)和(84±13)g/L,P=0.046]及血乳酸(3.8和1.7 mmol/L,P=0.009)水平高,AKI 3期患者比例(58.6%和86.7%,P=0.041)低。早期CRRT组患者28 d病死率31%(9/29),晚期CRRT组患者28 d病死率40%(6/15),2组比较差异无统计学意义(P=0.575)。结论早期CRRT没有明显改善SI-AKI患者28d病死率。  相似文献   

3.
大鼠重症急性胰腺炎并发腹内压升高导致肺损伤   总被引:1,自引:0,他引:1  
目的:探讨大鼠重型急性胰腺炎(SAP)并发腹内压(IAP)增高(IAH)对肺脏的病理损害.方法:健康Wistar大鼠91只,分为假手术组、SAP模型组及SAP IAH组.在不同时间(1,2,4h)SAP模型的基础上,给大鼠不同IAP(2,4,10cmH2O),观察肺脏的功能及组织结构变化.结果:随着腹内压的升高及作用时间的延长,氧分压(PaO2)明显下降、二氧化碳分压(PaCO2)升高,肺组织含水量增加.光镜下可以观察到肺脏间质及肺泡炎性细胞浸润、水肿、出血,肺泡细胞出现空泡化、脱落及衰变等病理改变.电镜下观察到Ⅱ型肺泡上皮细胞核固缩,板层体呈空泡样,线粒体嵴肿胀,形成凋亡细胞等病理改变.结论:SAP并发IAP增高可导致大鼠肺损伤.  相似文献   

4.
目的比较吗啡与布托啡诺对胃肠道手术患者腹内压的影响。方法选取2016年11月至2017年9月期间徐州医科大学附属医院收治的胃肠道开腹手术术后入住ICU的患者80例,按照随机数字表法分为吗啡组和布托啡诺组,每组40例。吗啡组镇痛药为盐酸吗啡20 mg+生理盐水至40 ml;布托啡诺组镇痛药为酒石酸布托啡诺4 mg+生理盐水至40 ml。2组均以2 ml/h的速度持续泵入,用药时间24 h。比较2组患者的临床指标及不同时间点的腹内压水平。采用SPSS 21.0软件进行数据处理。依据数据类型,组间比较分别采用t检验或χ2检验。结果布托啡诺组在用药后24 h[(13.38±2.03)vs(14.76±3.05)mm Hg(1 mm Hg=0.133 k Pa)]和48 h[(12.33±1.75)vs(14.04±2.70)mm Hg]的IAP水平均显著低于吗啡组(P0.05)。布托啡诺组患者的胃肠功能恢复时间[(2.65±0.66)vs(3.05±0.67)d]、术后住院时间[(10.08±1.64)vs(12.00±2.56)d]、恶心呕吐发生率(10.00%vs 27.50%)显著低于吗啡组,而嗜睡发生率(25.00%vs 7.50%)显著高于吗啡组,差异均具有统计学意义(P0.05)。结论相较于吗啡,布托啡诺更适合用于胃肠道开放手术患者的镇痛。  相似文献   

5.
目的:观察连续性肾脏替代疗法(CRRT)治疗重症急性胰腺炎(SAP)合并急性肾损伤(AKI)患者的疗效及预后。方法:采用前瞻性临床对照研究方法,将24例SAP合并AKI患者随机分为常规治疗组(12例)和CRRT治疗组(常规治疗+CRRT治疗组,12例)。治疗72h后对2组临床治疗效果进行比较(包括APACHEⅡ评分及其他主要临床指标),同时观察0、6、12、24、48和72h各时间点患者血浆细胞因子TNF-α、IL-1、IL-6及IL-10浓度。结果:2组患者入组基线情况相似,但CRRT组患者存活率明显高于常规治疗组(75.0%vs58.3%,P〈0.05)。治疗72h后,CRRT组患者APACHEⅡ评分(16.7±5.8vs13.1±3.4,P〈0.05)、体温(38.0±1.3vs37.6±0.5,P〈0.05)、血清肌酐(149.9±34.7vs75.6±50.6,P〈0.05)和剩余碱(-4.83±4.06vs0.63±3.78,P〈0.05)较治疗前明显好转,而常规治疗组患者的变化并不显著。同时,CRRT组患者血浆TNF-α,IL-1,IL-10水平均显著下降(均P〈0.05)。结论:CRRT治疗能快速有效改善SAP患者病情,纠正体内酸碱紊乱、清除体内代谢毒素外,还能清除体内生成过多的促炎和抗炎细胞因子,疗效明显优于传统疗法,应在发生AKI之前即进行治疗。  相似文献   

6.
目的探讨血清基质金属蛋白酶-9(MMP-9)和胱抑素-C(Cys-C)预测老年急性肾损伤(AKI)的价值。方法纳入山东省泰山疗养院医养结合病房2016年6月至2018年6月发生AKI患者116例为AKI组,同期未发生AKI患者68例为非AKI组,采集空腹外周静脉血,酶联免疫吸附法(ELISA)测定血清MMP-9和Cys-C水平,比较2组患者MMP-9和Cys-C水平。受试者工作特征(ROC)曲线分析MMP-9和Cys-C单独及联合预测AKI的价值。应用SPSS 20.0统计软件对数据进行分析。结果相比非AKI组患者,AKI组MMP-9[(377.61±28.72)vs(96.50±20.42)ng/ml]和Cys-C[(3.22±0.76)vs(0.92±0.16)mg/L]水平明显增高,差异具有统计学意义(P0.05)。MMP-9预测AKI的ROC曲线下面积(AUC)为0.714,截断点190 ng/ml时约登指数最大,灵敏度77%,特异度63%。Cys-C预测AKI的AUC为0.805,截断点2.0 mg/L时约登指数最大,灵敏度80%,特异度75%。MMP-9和Cys-C联合预测AKI的AUC为0.856,灵敏度85%,特异度81%,显著大于各单项标志物,差异具有统计学意义(P0.05)。结论老年AKI患者血MMP-9和Cys-C水平升高,MMP-9和Cys-C联合预测AKI的灵敏度和特异度较单项标志物高。  相似文献   

7.
目的探讨肝硬化并发急性肾损伤(acute kidney injury,AKI)患者的临床预后及影响因素。方法选取2012年1月至2018年11月解放军第928医院收治的55例肝硬化并发AKI患者为研究对象,收集患者的年龄、性别、体重指数(body mass index,BMI)、肝硬化病因、Child-Pugh分级、AKI分期、并发症(高血压、糖尿病、食管静脉曲张、上消化道出血、腹水、感染及肝性脑病)发生率、HBV DNA载量、丙氨酸氨基转移酶(alanine aminotransferase,ALT)、天门冬氨酸氨基转移酶(aspartate aminotransferase,AST)、血氨、血钠、凝血酶原活动度(prothrombin activity,PTA)及门静脉内径等资料,记录是否使用腹水引流及血管活性药物(如特利加压素)等。统计患者的临床预后。住院期间或出院后2个月内病死的患者为病死组(22例),其他患者为存活组(33例),比较两组患者的上述资料,采用多因素Logistic回归分析患者病死的独立影响因素。结果 55例肝硬化并发AKI患者入院后平均住院时间为(19.2±8.9)d,其中27例(49.1%)AKI恢复,2例(3.6%)AKI无变化,26例(47.3%)AKI进展,AKI进展者中22例(40.0%)住院期间或出院后2个月内病死。病死组患者的年龄[(61.93±6.63)岁vs(57.38±8.26)岁]、上消化道出血发生率[31.8%(7/22)vs 9.1%(3/33)]、感染发生率[77.3%(17/22)vs 45.5%(15/33)]、肝性脑病发生率[40.9%(9/22)vs 15.2%(5/33)]、ALT [(105.39±35.59)U/L vs(83.25±28.96)U/L]、AST [(99.52±33.13)U/L vs(82.03±25.58)U/L]、血氨水平[(69.95±20.21)μmol/L vs(58.98±18.26)μmol/L]等均显著高于存活组,PTA显著低于存活组[(55.01±8.58)%vs(65.25±10.63)%],差异均有统计学意义(P 0.05)。两组Child-Pugh分级(A级/B级/C级:6例/5例/11例vs 9例/13例/11例)及AKI分期(1期/2期/3期:6例/5例/11例vs 15例/10例/8例),差异均有统计学意义(P 0.05)。多因素Logistic回归分析表明,Child-Pugh C级(OR=3.568,95%CI:1.082~11.771,P=0.037)、AKI 3期(OR=5.058,95%CI:1.398~18.296,P=0.013)和感染(OR=3.239,95%CI:1.141~9.189,P=0.027)是肝硬化并发AKI患者病死的独立危险因素,PTA升高是独立保护因素(OR=0.813,95%CI:0.670~0.987,P=0.037)。结论肝硬化并发AKI患者的临床预后较差,Child-Pugh C级、AKI 3期及感染是患者病死的独立危险因素,PTA升高是独立保护因素。  相似文献   

8.
王伟 《肝脏》2019,24(11)
目的探讨肝硬化并发急性肾损伤(AKI)患者门静脉主干内径(PVD)特征。方法选取湖北省十堰市东风总医院急诊科2015年4月—2018年4月收治的肝硬化并AKI患者62例,作为AKI组,选取同期收治的肝硬化患者62例,作为肝硬化组。两组均于入院当日测定PVD并进行比较,绘制ROC曲线分析PVD对肝硬化并发急性肾损伤的预测价值。于入院当日测定两组血肌酐(Cr)、外周血白细胞计数(WBC)、血小板(PLT)总数、血红蛋白(Hb)水平,分析肝硬化并AKI患者的PVD与各实验室指标的相关性。结果 AKI组PVD为(13.27±2.07)mm,显著高于肝硬化组的(11.19±1.38)mm(P0.05)。PVD预测肝硬化并AKI的曲线下面积为0.683(标准误=0.048,P=0.000,95%CI=0.589~0.776),以PVD12.517mm时,肝硬化患者合并AKI的风险越高。AKI组PLT总数为(55.73±36.42)10~9/L,显著低于肝硬化组的(91.64±35.16)10~9/L(P0.05)。AKI组血Cr为(103.66±47.42)μmol/L,显著高于肝硬化组的(79.41±36.55)μmol/L(P0.05)。Pearson线性相关分析提示肝硬化并AKI患者的PVD与PLT总数呈负相关,其与Cr呈正相关(P0.05)。结论与单纯肝硬化患者相比,肝硬化并AKI患者的PVD明显增高,且其PVD与PLT总数、血Cr有相关性。  相似文献   

9.
目的探讨检测腹内压(IAP)、C反应蛋白(CRP)、降钙素原(PCT)在妊娠晚期急性胰腺炎中的临床价值。方法选取2008年9月-2018年9月于南华大学附属第一医院妇产科和肝胆外科住院的妊娠晚期(28周以上)急性胰腺炎孕妇80例,其中轻症及中重症急性胰腺炎45例(对照组),重症急性胰腺炎35例(观察组)。收集两组孕妇临床资料,包括IAP、CRP、PCT、胎儿窘迫、新生儿Apgar评分等。计量资料两组间比较采用t检验;计数资料两组间比较采用χ2检验。Pearson相关法检验CRP、PCT、IAP与胰腺炎严重程度,以及与胎儿不良结局(胎儿窘迫、出生时1 min Apgar评分)的相关性。根据受试者工作特征曲线(ROC曲线)计算各项指标诊断准确度最高的临界值,以及该值所对应的敏感度和特异度,评价各项指标对妊娠晚期急性胰腺炎的预判价值。结果观察组CRP[(185. 92±23. 59) mg/L vs (120. 92±20. 02) mg/L]、PCT[(12. 93±3. 16) ng/ml vs (5. 67±1. 65) ng/ml]、IAP[(12. 67±1. 40) mm Hg vs (5. 77±1. 10) mm Hg]均显著高于对照组(t值分别为13. 318、12. 298、23. 858,P值均0. 001)。对照组胎儿窘迫发生率显著低于观察组(6/45 vs 15/35,χ2=8. 864,P=0. 003),新生儿1 min Apgar评分显著高于观察组[(8. 22±0. 67)分vs (5. 97±0. 78)分,t=-13. 817,P 0. 001]。相关性分析结果显示,IAP与CRP、PCT水平及妊娠晚期胰腺炎Ranson评分均呈正相关(r值分别为0. 814、0. 712、0. 788,P值均0. 001),与新生儿1 min Apgar评分呈负相关(r=-0. 820,P 0. 001)。CRP、PCT及IAP对应的ROC曲线下面积分别为0. 838、0. 853和0. 903,计算所对应诊断准确度最高的临界值分别为158. 32 mg/L、10. 23 ng/L和10. 09 mm Hg,敏感度分别为77. 1%、71. 4%和82. 9%,特异度分别为93. 3%、97. 8%和95. 6%。结论检测IAP、CRP及PCT对于妊娠期重症急性胰腺炎有较大的早期预判价值,有利于评估妊娠晚期胰腺炎的严重程度及终止妊娠时机。  相似文献   

10.
目的研究血压变异性(blood pressure variability,BPV)与脑血管病患者脑小血管病(cerebral small vessel disease,CSVD)的相关性。方法收集我院神经内科住院的脑血管病患者341例,完成24h动态血压监测及头颅MRI扫描,获得血压变异标准差(SD)等BPV相关参数并根据MRI上CSVD严重程度进行总体评分,根据影像学上总体CSVD评分分为CSVD 0分组55例,CSVD 1分组117例,CSVD 2分组86例,CSVD 3分组49例,CSVD≥4分组34例。收集患者一般临床资料,采用logistic回归分析BPV参数是否为CSVD的独立危险因素。结果不同CSVD评分组性别、糖尿病、高脂血症、吸烟及体质量指数比较,无统计学差异(P0.05),而年龄、高血压比例比较,差异有统计学意义(P0.01);不同CSVD评分组夜间收缩压SD、夜间舒张压SD、夜间动脉压SD比较,无统计学差异(P0.05)。CSVD 0、1、2、3、≥4分组24h收缩压SD[(11.7±3.0)mm Hg(1mm Hg=0.133kPa)vs (12.6±3.1)mm Hg vs(13.6±3.3)mm Hg vs(13.7±3.7)mm Hg vs(13.7±3.2)mm Hg]、昼间收缩压SD[(11.1±3.0)mm Hg vs(12.0±3.2)mm Hg vs(13.0±3.2)mm Hg vs(13.7±3.8)mm Hg vs(12.8±3.1)mm Hg]、24h舒张压SD[(8.4±1.9)mm Hg vs(9.0±2.3)mm Hg vs(9.1±2.2)mm Hg vs(9.7±2.4)mm Hg vs(9.1±2.8)mm Hg]、昼间舒张压SD[(7.9±1.8)mm Hg vs (8.5±2.5)mm Hg vs (8.6±2.3)mm Hg vs (9.4±2.2)mm Hg vs (8.6±3.2)mm Hg]、24h动脉压SD[(9.1±1.9)mm Hg vs (9.9±2.4)mm Hg vs (10.4±2.3)mm Hg vs (10.6±2.4)mm Hg vs(10.1±2.5)mm Hg]、昼间动脉压SD[(8.9±1.9)mm Hg vs (9.5±2.5)mm Hg vs (10.0±2.3)mm Hg vs (10.4±2.4)mm Hg vs (9.7±2.8)mm Hg]比较,差异有统计学意义(P0.05,P0.01)。logistic回归分析显示,24h收缩压SD是CSVD的独立危险因素(P=0.032)。结论 24hBPV及昼间BPV与CSVD严重程度存在一定相关性。  相似文献   

11.
Acute myelofibrosis is a rare and still ill-defined disease. Based on morphological observation, immunophenotyping and ultrastructural analysis, we support the assumption that acute myelofibrosis is a malignant disorder mainly of the megakaryocytic lineage and is closely related to acute megakaryocytic/blastic leukaemia. Consequently, the 11 patients reported here were treated with aggressive polychemotherapy with combinations including daunorubicin and cytosine arabinoside and 6-thioguanin or VP16-213. 4 complete remissions, 2 partial remissions and 1 minor response were observed. Duration of aplasia was not significantly prolonged. These findings indicate that the use of aggressive polychemotherapy is feasible in acute myelofibrosis and results in a significant number of remissions.  相似文献   

12.
Acute gastrointestinal injury (AGI) is commonly present in patients with acute pancreatitis (AP). It is often difficult to predict gastrointestinal function in the early stage due to lack of reliable markers. We aimed to assess whether early plasma trefoil factor 2 (TFF-2) is a potential predictor for AGI.Fifty one patients were included for the onset of AP (from developing abdominal pain) within 72 hours in this prospective observational single-center study from January 2013 to July 2015. Among them 23 patients were classified as mild, 17 as moderately severe, and 11 as severe according to 2012 Atlanta classification. Plasma samples were collected only once at admission to the ICU. Twenty samples of healthy adults were also collected as control. The TFF-2 levels were determined by using a human TFF-2 enzyme-linked immunoassay. AGI grades from 1st to 7th day after admission were observed.The plasma TFF-2 levels among AP patients in early stage were significantly higher than healthy controls (766.41 ng/mL vs 94.37 ng/mL, P < .0001). The correlations between TFF-2 levels and AGI grades from 1st to 4th day after admission were positive (r = 0.47, 0.43, 0.42, 0.40 respectively, P < .05). As a predictor of acute gastrointestinal failure, plasma TFF-2 was superior to others: Acute Physiology and Chronic Health Evaluation II, sequential organ failure assessment, procalcitonin, C-reactive protein, serum calcium. In addition, TFF-2 increased along with the severity of AP (r = 0.554, P < .0001) and associated with Acute Physiology and Chronic Health Evaluation II, sequential organ failure assessment, C-reactive protein, serum calcium.The plasma TFF-2 levels were increased in patients in early stage of AP and correlated with AGI grades and disease severity in our study. TFF-2 might be a potential predictor for acute gastrointestinal failure in patients with AP.  相似文献   

13.
重症胰腺炎合并ARDS的诊断治疗   总被引:1,自引:1,他引:0  
目的探讨重症胰腺炎(SAP)并发成人呼吸窘迫综合症(ARDS)的诊断和治疗。方法对我院近10年收治的72例重症胰腺炎(SAP)其中并发成人呼吸窘迫综合症38例进行回顾性临床总结,分析其血气PaO2/FiO2、Qs/Qt等与诊断和治疗的关系。结果重症胰腺炎(SAP)并发ARDS的发生率52.77%(38/72).在及时合理治疗原发病的基础上,均给予机械通气,平均6.5天,机械通气24h后血气分析显示PaO2为(94.85±12.25)mmHg,PaO2为(34.4±7.05)mmHg,Qs/Qt值为5.25%±6.85%,全组死亡10例,放弃治疗3例,死亡率26.31%。结论重症胰腺炎(SAP)在及时处理原发病的基础上,对并发的ARDS进行早期诊断和治疗,正确使用呼吸机,防治并及时处理其它并发症,是降低病死率的有效措施。  相似文献   

14.
Background and aimAcute pancreatitis (AP) is associated with organ failures and systemic complications, most commonly acute respiratory failure (ARF) and acute kidney injury. So far, no studies have analysed the predictors and hospitalisation outcomes, of patients with AP who developed ARF. The aim of this study was to measure the prevalence of ARF in AP and to determine the clinical predictors for ARF and mortality in AP.MethodsThis is a retrospective cohort study using the Nationwide Inpatient Sample database from the year 2005–2014. The study population consisted of all hospitalisations with a primary or secondary discharge diagnosis of AP, which is further stratified based on the presence of ARF. The outcome measures include in-hospital mortality, hospital length of stay and hospitalisation cost.ResultsIn our study, about 5.4% of patients with AP had a codiagnosis of ARF, with a mortality rate of 26.5%. The significant predictors for ARF include sepsis, pleural effusion, pneumonia and cardiogenic shock. Key variables that were associated with a higher risk of mortality include mechanical ventilation, age more than 65 years, sepsis and cancer (excluding pancreatic cancer). The presence of ARF increased hospital stay by 8.3 days and hospitalisation charges by US$103 460.ConclusionIn this study, we demonstrate that ARF is a significant risk factor for increased hospital mortality, greater length of stay and higher hospitalisation charges in patients with AP. This underlines significantly higher resource utilisation in patients with a dual diagnosis of AP-ARF.  相似文献   

15.
Abstract: Background/Aims: Haematological malignancies seldom cause clinically significant liver disease. Acute liver failure as the initial manifestation of acute leukaemia is very rare and carries a very poor prognosis. Methods/Results: Three cases of acute liver failure secondary to acute leukaemia are described. Each case presented initially as acute liver failure of uncertain cause. Specific treatment for the leukaemia was instituted; however, all three patients died as a consequence of the liver failure. We describe the clinical course and relevant investigations of these patients and discuss possible mechanisms of acute liver failure in this setting. Conclusion: Acute leukaemia presenting as acute liver failure has a very poor prognosis. Although a rare cause of acute liver failure, it should be considered in any patient presenting with acute liver failure with prodromal symptoms and a raised peripheral white cell count, lactate dehydrogenase and uric acid.  相似文献   

16.
Two patients with haemophilia and acute leukaemia   总被引:1,自引:0,他引:1  
Acute leukaemia is the commonest form of malignancy in childhood. The coincidental development of leukaemia in children or adults with haemophilia is extremely rare, although cases of leukaemia and other malignancies have been reported previously in HIV-positive subjects. Of a total of 440 people with haemophilia registered with our society, two were diagnosed with acute leukaemia last year. The development of leukaemia in a subject with haemophilia has previously been reported from our country in 1985, but the negative HIV status of these recent cases is very interesting. The first case involved a 14-year-old boy with moderate haemophilia A, who developed acute lymphoblastic leukaemia (ALL) [French-American-British (FAB) classification L2]. The second subject was a 16-year-old boy who had moderately severe haemophilia A with no previous family history, and developed acute nonlymphocytic (myelomonocytic) leukaemia (FAB-M4). Both patients received conventional chemotherapy and this report discusses the potential problems in management of such cases, including diagnosis and administration of chemotherapy in subjects with a pre-existing haemorrhagic disorder. Extensive cutaneous and mucosal bleeding, as well as bleeds in joints previously affected by haemarthrosis and alterations of haematological values were all initially suggestive of the development of inhibitors against factor VIII, but the appearance of blasts in the peripheral blood and bone marrow led to the definitive diagnosis. The risk of bleeding, due to the combination of both leukaemia and the consequences of the chemotherapy, was overcome by the administration of coagulation factor concentrates (daily initially followed by prophylactic doses after successful induction of remission in both patients). The young patient with ALL is now receiving the maintenance phase of the Children's Cancer Study Group 1961 protocol and is in the 15th month of follow-up, without any complications. The other case relapsed in the seventh month, developing enterobacter sepsis, and died. An important lesson to be learnt from these cases is that the possible diagnosis of leukaemia should not be overlooked in a patient with haemophilia and severe haemorrhagic problems, if the first-line differential diagnosis of inhibitor development against factor VIII (or IX) has been excluded.  相似文献   

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A small but important proportion of patients with myelodysplasia (MDS) and acute leukaemia (AL) have underlying germline mutations in leukaemia susceptibility genes. The majority of these variants predispose to myeloid neoplasms with a smaller number associated with acute lymphoblastic leukaemia (ALL). The 2016 revision of the WHO classification of tumours of haematopoietic and lymphoid tissues has defined a number of myeloid neoplasms with germline predisposition (Blood, 127 , 2016, 2391) alerting clinicians to the importance of this underlying diagnosis. Advances in genetic technology and access to testing will undoubtably result in increased numbers of patients and families with leukaemia predisposition syndromes being identified. Here we summarize the salient biology and genetic and clinical features of a number of these conditions including some more recently described genetic variants.  相似文献   

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