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压力限定是预调的呼吸机管道和气道内在吸气相时的最高压力，当压力超过所调定的压力时，气体即通过泄压阀排出，使呼吸机管道和气道内的最高压力等于调定压力。 | [
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时间转换即根据需要直接调定吸气时间和频率，呼气时间和吸、呼比呼吸机自动计算并直接显示。 | [
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该类型呼吸机可供调节的参数为吸气峰压、呼气末正压、呼吸频率、吸气时间、吸入气氧分数和气体流速。 | [
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一、机械通气相关肺力学不论自主呼吸还是辅助机械通气，均需口和肺泡间存在一定的压力差，方能克服肺及胸壁弹性（顺应性）和气道阻力，从而完成吸气和呼气。 | [
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（一）肺顺应性肺顺应性（complianceoflungs，CL）是指肺的弹性阻力，常以施加单位压力时肺容积改变的大小来表示，其公式为：顺应性（L/cmH<sub>2</sub>O）=容量（L）/压力（cmH<sub>2</sub>O）从公式可见，当施给一定压力时，顺应性值越大，容积变化越大。 | [
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呼吸系统的总顺应性是由胸壁顺应性与肺顺应性构成，但由于新生儿胸壁弹性好，其顺应性常忽略不计，故通常肺顺应性即可代表呼吸系统的总顺应性。 | [
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正常新生儿肺顺应性为0.003～0.006L/cmH<sub>2</sub>O；呼吸窘迫综合征（respiratorydistresssyndrome，RDS）时肺顺应性降低，仅为0.0005～0.001L/cmH<sub>2</sub>O，其含义为：在相同的压力下，送入其肺内的潮气量将明显减少，若获得正常的潮气量，则需要更高的压力。 | [
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（二）气道阻力气道阻力（resistance，R）是指气道对气流的阻力。 | [
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常以单位流速流动的气体所需要的压力来表示，其公式为：气道阻力［cmH<sub>2</sub>O/（L•sec）］=压力（cmH<sub>2</sub>O）/流速（L/sec）正常新生儿总气道阻力为20～40cmH<sub>2</sub>O/（L•sec）；气管插管时为50～150cmH<sub>2</sub>O/（L•sec）；胎粪吸入综合征（meconiumaspirationsyndrome，MAS）为100～140cmH<sub>2</sub>O/（L•sec）或更高。 | [
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（三）时间常数时间常数（timeconstant，TC）是指在一定压力下，送入肺内或呼出一定量气体所需要的时间单位，取决于呼吸系统的顺应性及气道阻力，其计算公式为：TC（sec）=CL（L/cmH<sub>2</sub>O）×R［cmH<sub>2</sub>O/（L•sec）］由公式可见：顺应性愈差，气道阻力（包括气管插管和呼吸机管道）愈小，送入肺内气体或呼出气体愈迅速，所需时间愈短，反之亦然。 | [
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正常足月儿：TC=0.005L/cmH<sub>2</sub>O×30cmH<sub>2</sub>O/（L•sec）=0.15sec；RDS患儿：TC=0.001L/cmH<sub>2</sub>O×30cmH<sub>2</sub>O/（L•sec）=0.03sec；MAS患儿：TC=0.003L/cmH<sub>2</sub>O×120cmH<sub>2</sub>O/（L•sec）=0.36sec。 | [
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送入肺内或呼出一定量气体后剩余的潮气量与时间常数有关，其计算公式为：V/Vo=e-TC式中，V：送入肺内或呼出一定量气体后剩余的潮气量；Vo：潮气量；e=2.7134。 | [
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以呼气时间（timeofexpiration，TE）为例，当TE为一个时间常数（TC=1）时，根据公式V/Vo=0.37，V=Vo×0.37即肺内剩余的气量为潮气量的37%，也就是说，当TE为一个时间常数（TC=1）时，可呼出潮气量的63%；当TE分别为2、3、4、5个时间常数时，呼出气量分别为潮气量的86%、95%、98%、99%。 | [
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理论上，吸气时间、呼气时间若为5个时间常数，近乎全部的潮气量能进入肺内或排出体外，但临床实践中吸、呼气时间达3～5个时间常数即可。 | [
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当吸气时间（timeofinspiration，TI）短于3～5个时间常数时，调定压力下的潮气量不能全部送入肺内，使实际的吸气峰压（PIP）低于调定的PIP，称为非调定的PIP下降，此时平均气道压力（meanairwaypressure，MAP）也随之下降，故也称为非调定的MAP下降，其结果导致PaO<sub>2</sub>降低及PaCO<sub>2</sub>升高；当TE短于3～5个时间常数时，即可产生非调定的呼气末正压。 | [
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（四）非调定的呼气末正压当应用高呼吸频率（respiratoryrate，RR）通气时，TE短于3个TC，由于呼气时间不够，肺泡内气体不能完全排出，造成气体潴留，使肺泡内呼气末压力高于调定的呼气末正压（positiveend-expirationpressure，PEEP），其高出的PEEP值称为非调定的呼气末正压（inadvertentpositiveend-expirationpressure，iPEEP）。 | [
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此时功能残气量（functionalresidualcapacity，FRC）增加，肺顺应性和潮气量降低，每分通气量及心搏量减少，PaO2</sub>降低及PaCO2</sub>升高。 | [
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如果调定的PEEP较低，iPEEP则可使萎陷的肺泡在呼气末恢复正常FRC，改善氧合，这可能是对RDS患儿有时增加频率后氧合陡度增加的原因。 | [
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当然，当产生iPEEP时，呼吸系统也将代偿和限制气体进一步潴留，高FRC使肺顺应性降低，气体潴留则使小气道开放，气道阻力下降，从而缩短相应肺泡的时间常数，在原有TE内，呼出比原来更多的气体，同时高FRC使潮气量减少，故呼出潮气量所需的时间也短，从而缓解气体潴留，达到新的平衡。 | [
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这也可能是调定的PEEP愈高气体潴留愈少和当存在不特别严重气体潴留时肺泡并未破裂的道理所在。 | [
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气管插管较细及气道分泌物增多使气道阻力增加，也是引起气体潴留的重要原因。 | [
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值得注意的是呼吸机经近气道测量的PEEP值不能准确反映肺泡内呼气末压力。 | [
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首先根据疾病的种类或肺功能监测，推断和观察CL、R和TC，结合所调定的TE预测其可能性，肺顺应性高或气道阻力大的患儿易引起iPEEP，可应用长TE。 | [
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气体潴留的表现为：桶状胸，胸动幅度小，呼吸音减弱；PaCO<sub>2</sub>升高；循环障碍，如血压下降、代谢性酸中毒、中心静脉压升高等；胸片示呼气末膈肌低位；肺功能及呼气末闭合气管插管测量其食道或气道压力等方法对发现iPEEP也有一定帮助。 | [
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有的呼吸机可通过呼气保持按钮获得iPEEP。 | [
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（五）TC相关的治疗策略TC是针对不同疾病制定机械通气策略的重要理论依据。 | [
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如上所述，RDS患儿肺顺应性小而气道阻力尚属正常，1个TC仅为0.03秒，3个TC为0.09秒，即使5个TC也只有0.15秒，因此，对RDS极期患儿进行机械通气时，可采用较高频率通气，RDS不至于产生iPEEP；由于RDS以缺氧为主，增加TI可提高MAP即提高PaO<sub>2</sub>，而RDS所需TE很短，故理论上可应用倒置的吸、呼比即2∶1～4∶1，长TI虽可提高PaO<sub>2</sub>，但容易造成肺气压伤，故临床已极少应用。 | [
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MAS患儿气道阻力明显增加，肺顺应性仅略减小，1个TC仅为0.36秒，3个TC则为1.08秒，因此，对MAS应用机械通气，宜选择慢频率和长TE，如果提高频率，则应降低PEEP，以免造成iPEEP；还可根据MAS病理改变（肺不张、肺气肿和正常肺泡同时存在）进行通气，气肿的肺泡TC长为慢肺泡，而正常的肺泡TC相对短为快肺泡，如果以正常肺泡为通气目标，可根据正常肺泡的TC（3～5个TC为0.45～0.75秒）确定TI和TE，采用中等频率，这样既可保证快肺泡有效通气，又可使进出慢肺泡的气体量减少，避免气肿的肺泡破裂，造成气胸；若以气肿肺泡，气肿肺泡TC确定TI和TE，采用慢频率、长TI和长TE，这样虽保证气肿肺泡的有效通气，却使正常肺泡过度... | [
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第八章溶血尿毒综合征【概述】溶血尿毒综合征（hemolyticuremicsyndrome，HUS）是一种累及多系统、以Grasser三联症（即微血管病性溶血、急性肾衰竭和血小板减少）为主要特征的临床综合征，是小儿急性肾衰竭常见的病因之一。 | [
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1/3以上的HUS患儿可有神经系统受累的表现。 | [
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由于HUS与血栓性血小板减少性紫癜（thromboticthrombocytopenicpurpura，TTP）在病因、发病机制、病理改变和临床表现方面难以精确区分，目前越来越多的学者认为两者是同一疾病不同的临床表现，可统称之为HUS/TTP或血栓性微血管病（thromboticmicroangiopathy，TMA）。 | [
{
"id": 0,
"entity": "HUS",
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},
{
"id": 1,
"entity": "血栓性血小板减少性紫癜",
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"label": "dis"
},
{
"id": 2,
"entity": "thromboticthrombocytopenicpurpura",
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"... |
HUS为病变以肾脏累及为主的肾限性TMA，肾衰竭是其主要特征；TTP则为系统性TMA，表现以神经系统症状为主。 | [
{
"id": 0,
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{
"id": 1,
"entity": "肾脏",
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"label": "bod"
},
{
"id": 2,
"entity": "肾限性TMA",
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"end_offset": 20,
"label": "dis"
... |
随着诊疗技术的日趋完善，HUS/TTP的预后已有所改观。 | [
{
"id": 0,
"entity": "HUS/TTP",
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] |
【流行病学】全球不同国家和地区虽均有HUS散发或流行的报道，但本病有一定的地域性，以阿根廷、荷兰、南非、美国加利福尼亚州和加拿大魁北克省居多。 | [
{
"id": 0,
"entity": "HUS",
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"end_offset": 21,
"label": "dis"
}
] |
美国儿童HUS发病率为0.3/10万～10/10万，澳大利亚等国5岁以下儿童为1.35/10万～5.8/10万，日本的HUS病例数则有逐年增多趋势。 | [
{
"id": 0,
"entity": "HUS",
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"end_offset": 62,
"label": "dis"
}
] |
各年龄段均可发生HUS，伴以腹泻的HUS发病高峰年龄为6个月～4岁，无性别差异。 | [
{
"id": 0,
"entity": "HUS",
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{
"id": 1,
"entity": "腹泻",
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{
"id": 2,
"entity": "HUS",
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... |
【病因及发病机制】（一）病因尚不明确，下列外源或内源性因素可能与HUS的发病有关。 | [
{
"id": 0,
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}
] |
1.感染是诱发儿童HUS的首要因素，根据诱因可以志贺样毒素相关HUS和非志贺样毒素相关HUS。 | [
{
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"entity": "HUS",
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{
"id": 1,
"entity": "志贺样毒素相关HUS",
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"label": "dis"
},
{
"id": 2,
"entity": "非志贺样毒素相关HUS",
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... |
儿童暴发流行的EHECO157：H7感染中，可有高达53%的患者发生HUS。 | [
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"id": 0,
"entity": "EHECO157：H7感染",
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{
"id": 1,
"entity": "HUS",
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"end_offset": 37,
"label": "dis"
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2.药物长春新碱、丝裂霉素、顺铂、氟尿嘧啶、柔红霉素、阿糖胞苷等抗肿瘤药物可引起化疗相关性HUS，环孢霉素等免疫抑制剂也可诱发HUS，偶见奎宁引起HUS的报道。 | [
{
"id": 0,
"entity": "长春新碱",
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"end_offset": 8,
"label": "dru"
},
{
"id": 1,
"entity": "丝裂霉素",
"start_offset": 9,
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"label": "dru"
},
{
"id": 2,
"entity": "顺铂",
"start_offset": 14,
"end_offset": 16,
"label": "dru"
... |
3.器官移植骨髓移植及肾移植后均可发生HUS，发生率分别为3.4%和6%～9%。 | [
{
"id": 0,
"entity": "器官移植",
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"label": "pro"
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{
"id": 1,
"entity": "骨髓移植",
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{
"id": 2,
"entity": "肾移植",
"start_offset": 11,
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"label": "pro"
... |
一旦发生骨髓移植后HUS，预后凶险，可能与大剂量化疗、放疗、排异反应、感染等有关。 | [
{
"id": 0,
"entity": "骨髓移植",
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{
"id": 1,
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"label": "dis"
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{
"id": 2,
"entity": "化疗",
"start_offset": 24,
"end_offset": 26,
"label": "pro"
... |
4.免疫缺陷病如先天性无丙种球蛋白血症和胸腺无淋巴细胞增生症。 | [
{
"id": 0,
"entity": "先天性无丙种球蛋白血症",
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{
"id": 1,
"entity": "胸腺无淋巴细胞增生症",
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] |
5.遗传及基因突变HUS可在同一家族的兄弟姐妹中相继发病。 | [
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"id": 0,
"entity": "HUS",
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"end_offset": 12,
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] |
目前认为其为常染色体隐性遗传，系血管性血友病因子裂解蛋白酶（vonWillebrandfactorcleavingprotease，vWF-CP又称ADAMTS-13，adisintegrinmetalloproteinasewiththrombospondintypeImotif，member13）重度缺乏，导致vWF多聚体增多，损伤内皮细胞。 | [
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"entity": "血管性血友病因子裂解蛋白酶",
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},
{
"id": 1,
"entity": "vonWillebrandfactorcleavingprotease",
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"end_offset": 65,
"label": "bod"
},
{
"id": 2,
"entity": "vWF-CP",
"start_offset":... |
家族性HUS预后不良，病死率达68%。 | [
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"id": 0,
"entity": "家族性HUS",
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] |
近年来，也从一些HUS患者中发现有补体调节因子基因突变现象，如补体因子H（complementfactorH，CFH）、补体因子Ⅰ（complementfactorⅠ，CFI）及补体膜辅助蛋白（membranecofactorprotein，MCP）基因。 | [
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"id": 0,
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{
"id": 1,
"entity": "补体调节因子",
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"end_offset": 23,
"label": "bod"
},
{
"id": 2,
"entity": "补体因子H",
"start_offset": 31,
"end_offset": 36,
"label": "... |
6.其他一些自身免疫相关性疾病如系统性红斑狼疮、类风湿性关节炎、抗磷脂抗体综合征、恶性肿瘤及妊娠，均可引起HUS，成人多见。 | [
{
"id": 0,
"entity": "系统性红斑狼疮",
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"label": "dis"
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{
"id": 1,
"entity": "类风湿性关节炎",
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"label": "dis"
},
{
"id": 2,
"entity": "抗磷脂抗体综合征",
"start_offset": 32,
"end_offset": 40,
"... |
（二）发病机制HUS的发病机制尚不明确。 | [
{
"id": 0,
"entity": "HUS",
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"end_offset": 10,
"label": "dis"
}
] |
不同致病因素引起HUS的发病机制不尽相同，但毛细血管内皮细胞损伤是其共同的致病途径。 | [
{
"id": 0,
"entity": "HUS",
"start_offset": 8,
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{
"id": 1,
"entity": "毛细血管内皮细胞",
"start_offset": 22,
"end_offset": 30,
"label": "bod"
},
{
"id": 2,
"entity": "毛细血管内皮细胞损伤",
"start_offset": 22,
"end_offset": 32,
"la... |
受损的内皮细胞启动凝血系统，致血小板在局部聚集、血栓形成和纤维蛋白沉积，使红细胞和血小板流经时遭受机械损伤而破坏，引起微血管性血栓、溶血性贫血和血小板减少；在肾脏，微血管性血栓致肾内循环障碍，进而发生急性肾衰竭。 | [
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"id": 0,
"entity": "内皮细胞",
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{
"id": 1,
"entity": "血小板",
"start_offset": 15,
"end_offset": 18,
"label": "bod"
},
{
"id": 2,
"entity": "血小板在局部聚集",
"start_offset": 15,
"end_offset": 23,
"label": "... |
近年的研究认为HUS发病机制涉及以下几个方面：1.内毒素及神经氨酸酶致内皮细胞受损EHEC在肠道内产生内毒素，主要有两种：一是志贺样毒素（shiga-liketoxin，SLT），又称维罗毒素（verotoxin，VT），可结合到内皮细胞表面的糖脂质受体（globotriaosylceramide，GB<sub>3</sub>）上，经吞噬进入胞质后分解为A链和B链。 | [
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"id": 0,
"entity": "HUS",
"start_offset": 7,
"end_offset": 10,
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{
"id": 1,
"entity": "内毒素",
"start_offset": 25,
"end_offset": 28,
"label": "mic"
},
{
"id": 2,
"entity": "神经氨酸酶",
"start_offset": 29,
"end_offset": 34,
"label": "mic... |
A链可裂解核糖体转运RNA的腺嘌呤，使蛋白合成障碍致细胞受损或死亡；SLTs尚有诱导肾细胞凋亡作用，细胞凋亡在HUS的发病过程中起一定作用，且凋亡细胞数与疾病严重度相关。 | [
{
"id": 0,
"entity": "核糖体转运RNA的腺嘌呤",
"start_offset": 5,
"end_offset": 17,
"label": "bod"
},
{
"id": 1,
"entity": "细胞",
"start_offset": 26,
"end_offset": 28,
"label": "bod"
},
{
"id": 2,
"entity": "SLTs",
"start_offset": 34,
"end_offset": 38,
"label... |
另一种为细菌脂多糖（lipopolysaccharide，LPS），LPS通过上调纤溶酶原激活抑制剂（plasminogenactivatorinhibitor，PAI）和下调血栓调节素表达而损伤内皮细胞，促进血栓形成。 | [
{
"id": 0,
"entity": "细菌脂多糖",
"start_offset": 4,
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},
{
"id": 1,
"entity": "lipopolysaccharide",
"start_offset": 10,
"end_offset": 28,
"label": "mic"
},
{
"id": 2,
"entity": "LPS",
"start_offset": 29,
"end_offset": 32,
... |
LPS尚可促进白细胞和血小板黏附在内皮细胞上。 | [
{
"id": 0,
"entity": "LPS",
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{
"id": 1,
"entity": "白细胞",
"start_offset": 7,
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"label": "bod"
},
{
"id": 2,
"entity": "血小板",
"start_offset": 11,
"end_offset": 14,
"label": "bod"
... |
肺炎链球菌产生的神经氨酸酶可分解掉N-乙酰神经氨酸，使被其掩盖的T-F抗原（Thomson-Friedenreich抗原）暴露于循环IgM抗体，IgM抗体与血小板和内皮细胞上的T-F抗原结合，导致血小板凝聚和内皮细胞损伤。 | [
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"id": 0,
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{
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},
{
"id": 2,
"entity": "N-乙酰神经氨酸",
"start_offset": 17,
"end_offset": 25,
"label":... |
2.细胞因子作用许多细胞因子参与HUS发病，肿瘤坏死因子（tumornecrosisfactor，TNF）、白细胞介素-6（IL-6）、IL-8、IL-1β等释放增加。 | [
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"id": 0,
"entity": "细胞因子",
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{
"id": 1,
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"label": "bod"
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{
"id": 2,
"entity": "HUS",
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"label": "dis"... |
TNF可诱导上皮细胞促凝血活性及GB<sub>3</sub>受体表达；IL-6是疾病活动性的一个标志物，与疾病严重程度和预后有关；IL-8是一种白细胞激活剂，白细胞激活后释放弹力蛋白酶，使其与内皮细胞黏附性增高，参与发病并加重病损。 | [
{
"id": 0,
"entity": "TNF",
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},
{
"id": 1,
"entity": "上皮细胞",
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"label": "bod"
},
{
"id": 2,
"entity": "GB<sub>3</sub>",
"start_offset": 16,
"end_offset": 30,
"labe... |
3.前列环素（prostacyclin，PGI<sub>2</sub>）和血栓素A<sub>2</sub>（thromboxane，TXA<sub>2</sub>）失衡正常内皮细胞可合成PGI<sub>2</sub>，具扩张血管和抑制血小板聚集作用，与促进血小板凝聚的TXA<sub>2</sub>保持动态平衡。 | [
{
"id": 0,
"entity": "前列环素",
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},
{
"id": 1,
"entity": "prostacyclin",
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"label": "bod"
},
{
"id": 2,
"entity": "PGI<sub>2</sub>",
"start_offset": 20,
"end_offset": 35,... |
本征患者PGI<sub>2</sub>低下，可能与发病有关，推测患儿缺少某种刺激产生PGI<sub>2</sub>的血浆因子或存在PGI<sub>2</sub>合成酶抑制物，尚有可能HUS患者对PGI<sub>2</sub>降解加快有关。 | [
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"id": 0,
"entity": "PGI<sub>2</sub>",
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{
"id": 1,
"entity": "PGI<sub>2</sub>",
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"end_offset": 57,
"label": "bod"
},
{
"id": 2,
"entity": "血浆因子",
"start_offset": 58,
"end_offset"... |
4.凝血与纤溶系统异常促血小板凝聚物质如血小板激活因子（PAF）、体内存在vWF-CP抗体使血管性血友病因子（vWF）多聚体异常增多；血小板释放产物如β-血栓球蛋白（β-TG）等增加；内皮细胞释放组织因子，激活凝血系统，微血栓广泛形成；纤溶破坏，D-二聚体和纤溶酶原激活物抑制因子（plasminogenactivatorinhibitor，PAI）降低。 | [
{
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{
"id": 1,
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},
{
"id": 2,
"entity": "促血小板凝聚物质",
"start_offset": 11,
"end_offset": 19,
"label": "bod... |
5.其他有学者注意到，内皮素-一氧化氮轴和免疫功能紊乱在HUS的发病中也可能起到一定作用。 | [
{
"id": 0,
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{
"id": 1,
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"label": "sym"
},
{
"id": 2,
"entity": "HUS",
"start_offset": 28,
"end_offset": 31,
"labe... |
【病理】肾脏病理改变以血管内血小板聚集伴纤维素沉积、微血栓形成为特点，分三型。 | [
{
"id": 0,
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{
"id": 1,
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},
{
"id": 2,
"entity": "血小板",
"start_offset": 14,
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"label": "bod"
}... |
（一）肾小球型小儿多见，肾小球毛细血管内皮细胞肿胀、脱落，内皮细胞下间歇增宽，可见系膜细胞插入现象。 | [
{
"id": 0,
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{
"id": 1,
"entity": "肾小球毛细血管内皮细胞肿胀、脱落",
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"label": "sym"
},
{
"id": 2,
"entity": "内皮细胞",
"start_offset": 29,
"end_offset": ... |
肾小球毛细血管腔狭窄、有微血栓形成和节段性纤维素性坏死。 | [
{
"id": 0,
"entity": "肾小球毛细血管腔",
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{
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"entity": "肾小球毛细血管腔狭窄",
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},
{
"id": 2,
"entity": "微血栓",
"start_offset": 12,
"end_offset": 15,
"labe... |
可见新月体形成。 | [
{
"id": 0,
"entity": "可见新月体形成",
"start_offset": 0,
"end_offset": 7,
"label": "sym"
}
] |
（二）血管型以入球小动脉、小叶间和弓状动脉分支为主，可见动脉内膜水肿、纤维素坏死、血管腔内血栓形成、血栓机化、血管内膜葱皮样增生。 | [
{
"id": 0,
"entity": "入球小动脉",
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},
{
"id": 1,
"entity": "小叶间",
"start_offset": 13,
"end_offset": 16,
"label": "bod"
},
{
"id": 2,
"entity": "弓状动脉分支",
"start_offset": 17,
"end_offset": 23,
"label": "... |
（三）皮质坏死型是较大的肾内动脉血栓形成和闭塞的后果。 | [
{
"id": 0,
"entity": "肾内动脉",
"start_offset": 12,
"end_offset": 16,
"label": "bod"
}
] |
免疫荧光检查可见肾小球内纤维蛋白原沉积，有时见IgM及C<sub>3</sub>沉积在肾小球毛细血管壁。 | [
{
"id": 0,
"entity": "免疫荧光检查",
"start_offset": 0,
"end_offset": 6,
"label": "ite"
},
{
"id": 1,
"entity": "肾小球内纤维蛋白原",
"start_offset": 8,
"end_offset": 17,
"label": "bod"
},
{
"id": 2,
"entity": "肾小球内纤维蛋白原沉积",
"start_offset": 8,
"end_offset": 19,
"... |
【临床表现】临床表现典型者常有前驱症状，以胃肠道表现为主，多有腹痛、腹泻和呕吐，可有发热、嗜睡、乏力、食欲缺乏等非特异性表现。 | [
{
"id": 0,
"entity": "胃肠道",
"start_offset": 21,
"end_offset": 24,
"label": "bod"
},
{
"id": 1,
"entity": "腹痛",
"start_offset": 31,
"end_offset": 33,
"label": "sym"
},
{
"id": 2,
"entity": "腹泻",
"start_offset": 34,
"end_offset": 36,
"label": "sym"
... |
患儿明显苍白，临床所见黄疸不显著或仅面部呈柠檬黄色。 | [
{
"id": 0,
"entity": "患儿明显苍白",
"start_offset": 0,
"end_offset": 6,
"label": "sym"
},
{
"id": 1,
"entity": "临床所见黄疸不显著或仅面部呈柠檬黄色",
"start_offset": 7,
"end_offset": 25,
"label": "sym"
}
] |
心血管系统受损表现为高血压、心律失常和心功能不全；胰腺受损者可出现暂时性或永久性胰腺内分泌功能不全；可有短暂的肝损害，偶见胆汁郁积性黄疸；肺、肌肉、皮肤及视网膜损害少见。 | [
{
"id": 0,
"entity": "心血管系统",
"start_offset": 0,
"end_offset": 5,
"label": "bod"
},
{
"id": 1,
"entity": "高血压",
"start_offset": 10,
"end_offset": 13,
"label": "sym"
},
{
"id": 2,
"entity": "心律失常",
"start_offset": 14,
"end_offset": 18,
"label": "sym... |
临床依病情轻重分为轻型和重型，重型标准包括：血红蛋白＜60g/L、BUN≥17.9mmol/L及有少尿或无尿和（或）严重并发症（如高血压脑病、肺水肿等）。 | [
{
"id": 0,
"entity": "血红蛋白",
"start_offset": 22,
"end_offset": 26,
"label": "bod"
},
{
"id": 1,
"entity": "高血压脑病",
"start_offset": 65,
"end_offset": 70,
"label": "dis"
},
{
"id": 2,
"entity": "肺水肿",
"start_offset": 71,
"end_offset": 74,
"label": "d... |
【实验室检查】（一）血液检查血常规示血红蛋白和血细胞比容下降、血小板下降，镜检可见异型红细胞及碎片，网织红细胞计数增高。 | [
{
"id": 0,
"entity": "血液检查",
"start_offset": 10,
"end_offset": 14,
"label": "ite"
},
{
"id": 1,
"entity": "血常规",
"start_offset": 14,
"end_offset": 17,
"label": "ite"
},
{
"id": 2,
"entity": "血红蛋白",
"start_offset": 18,
"end_offset": 22,
"label": "bo... |
生化检查示有代谢性酸中毒、高血钾、高血磷和低血钙、稀释性低血钠、氮质血症、胆红素及转氨酶增高、总蛋白和清蛋白降低。 | [
{
"id": 0,
"entity": "生化检查",
"start_offset": 0,
"end_offset": 4,
"label": "pro"
},
{
"id": 1,
"entity": "代谢性酸中毒",
"start_offset": 6,
"end_offset": 12,
"label": "sym"
},
{
"id": 2,
"entity": "高血钾",
"start_offset": 13,
"end_offset": 16,
"label": "sym... |
血乳酸脱氢酶增高。 | [
{
"id": 0,
"entity": "血乳酸脱氢酶",
"start_offset": 0,
"end_offset": 6,
"label": "bod"
},
{
"id": 1,
"entity": "血乳酸脱氢酶增高",
"start_offset": 0,
"end_offset": 8,
"label": "sym"
}
] |
可见补体C<sub>3</sub>水平降低。 | [
{
"id": 0,
"entity": "补体C<sub>3</sub>",
"start_offset": 2,
"end_offset": 17,
"label": "bod"
},
{
"id": 1,
"entity": "可见补体C<sub>3</sub>水平降低",
"start_offset": 0,
"end_offset": 21,
"label": "sym"
}
] |
累及胰腺者有高血糖。 | [
{
"id": 0,
"entity": "胰腺",
"start_offset": 2,
"end_offset": 4,
"label": "bod"
},
{
"id": 1,
"entity": "高血糖",
"start_offset": 6,
"end_offset": 9,
"label": "sym"
}
] |
凝血因子检查结果与病程有关，早期可有凝血酶原时间延长、纤维蛋白原降低、纤维蛋白降解产物增高及凝血Ⅱ、Ⅷ、Ⅸ及Ⅹ因子减少，但数天后即恢复正常。 | [
{
"id": 0,
"entity": "凝血因子检查",
"start_offset": 0,
"end_offset": 6,
"label": "ite"
},
{
"id": 1,
"entity": "凝血酶原",
"start_offset": 18,
"end_offset": 22,
"label": "bod"
},
{
"id": 2,
"entity": "凝血酶原时间延长",
"start_offset": 18,
"end_offset": 26,
"label"... |
（二）尿检查血尿、蛋白尿和血红蛋白尿，尿沉渣镜检有红细胞碎片、白细胞及管型。 | [
{
"id": 0,
"entity": "尿检查",
"start_offset": 3,
"end_offset": 6,
"label": "ite"
},
{
"id": 1,
"entity": "血尿",
"start_offset": 6,
"end_offset": 8,
"label": "ite"
},
{
"id": 2,
"entity": "蛋白尿",
"start_offset": 9,
"end_offset": 12,
"label": "ite"
},
... |
（三）粪便检查典型的腹泻后HUS有赖于粪便细菌培养和血清学分型。 | [
{
"id": 0,
"entity": "粪便检查",
"start_offset": 3,
"end_offset": 7,
"label": "ite"
},
{
"id": 1,
"entity": "腹泻",
"start_offset": 10,
"end_offset": 12,
"label": "sym"
},
{
"id": 2,
"entity": "HUS",
"start_offset": 13,
"end_offset": 16,
"label": "dis"
... |
用免疫磁分离技术（immunomagneticseparation）分离EHECO157：H7，较培养方便快速。 | [
{
"id": 0,
"entity": "EHECO157：H7",
"start_offset": 36,
"end_offset": 47,
"label": "bod"
}
] |
【诊断】患儿有前驱胃肠症状史，临床见急性溶血性贫血、血小板减少和肾功能急性减退，表现为苍白、尿量减少，尿检红细胞、蛋白及管型，血象呈贫血状，血小板下降，涂片见异型红细胞和碎片，血生化示急性肾衰竭改变，即可诊断本症。 | [
{
"id": 0,
"entity": "胃肠",
"start_offset": 9,
"end_offset": 11,
"label": "bod"
},
{
"id": 1,
"entity": "急性溶血性贫血",
"start_offset": 18,
"end_offset": 25,
"label": "sym"
},
{
"id": 2,
"entity": "血小板",
"start_offset": 26,
"end_offset": 29,
"label": "bo... |
婴儿期应注意与中毒性或缺血性肾小管坏死区别，年长儿则应与结缔组织病所致肾脏病变鉴别。 | [
{
"id": 0,
"entity": "中毒性或缺血性肾小管坏死",
"start_offset": 7,
"end_offset": 19,
"label": "dis"
},
{
"id": 1,
"entity": "结缔组织病",
"start_offset": 28,
"end_offset": 33,
"label": "dis"
},
{
"id": 2,
"entity": "肾脏",
"start_offset": 35,
"end_offset": 37,
"labe... |
【治疗】对HUS的治疗强调加强支持、早期透析和积极对症处理的原则。 | [
{
"id": 0,
"entity": "HUS",
"start_offset": 5,
"end_offset": 8,
"label": "dis"
},
{
"id": 1,
"entity": "早期透析",
"start_offset": 18,
"end_offset": 22,
"label": "pro"
}
] |
（一）支持疗法及早加强营养支持、维持水和电解质平衡及控制高血压。 | [
{
"id": 0,
"entity": "高血压",
"start_offset": 28,
"end_offset": 31,
"label": "sym"
}
] |
（二）透析疗法早期透析可明显改善急性期症状，降低病死率。 | [
{
"id": 0,
"entity": "透析",
"start_offset": 3,
"end_offset": 5,
"label": "pro"
},
{
"id": 1,
"entity": "早期透析",
"start_offset": 7,
"end_offset": 11,
"label": "pro"
}
] |
适应证为无尿＞12小时、氮质血症伴脑病或BUN＞53.55mmol/L、血钾＞6mmol/L和（或）心功能衰竭、顽固性高血压者。 | [
{
"id": 0,
"entity": "无尿＞12小时",
"start_offset": 4,
"end_offset": 11,
"label": "sym"
},
{
"id": 1,
"entity": "氮质血症",
"start_offset": 12,
"end_offset": 16,
"label": "dis"
},
{
"id": 2,
"entity": "脑病",
"start_offset": 17,
"end_offset": 19,
"label": "d... |
目前，在儿科较为广泛使用的是腹膜透析，也可采用血液透析。 | [
{
"id": 0,
"entity": "腹膜透析",
"start_offset": 14,
"end_offset": 18,
"label": "pro"
},
{
"id": 1,
"entity": "血液透析",
"start_offset": 23,
"end_offset": 27,
"label": "pro"
}
] |
（三）血浆置换疗法传统采用血浆输入技术，近年来血浆置换技术被广泛采用。 | [
{
"id": 0,
"entity": "血浆置换疗法",
"start_offset": 3,
"end_offset": 9,
"label": "pro"
},
{
"id": 1,
"entity": "血浆",
"start_offset": 13,
"end_offset": 15,
"label": "bod"
},
{
"id": 2,
"entity": "血浆置换技术",
"start_offset": 23,
"end_offset": 29,
"label": "p... |
由于除了补充血浆成分，血浆置换可以清除血液中的有害毒素和炎症因子，已经证实，血浆置换比血浆输入效果显著。 | [
{
"id": 0,
"entity": "血浆",
"start_offset": 6,
"end_offset": 8,
"label": "bod"
},
{
"id": 1,
"entity": "血浆置换",
"start_offset": 11,
"end_offset": 15,
"label": "pro"
},
{
"id": 2,
"entity": "血液",
"start_offset": 19,
"end_offset": 21,
"label": "bod"
... |
血浆补充或置换能补充刺激PGI<sub>2</sub>生成的血浆因子，去除PGI<sub>2</sub>合成的抑制物。 | [
{
"id": 0,
"entity": "血浆补充或置换",
"start_offset": 0,
"end_offset": 7,
"label": "pro"
},
{
"id": 1,
"entity": "PGI<sub>2</sub>",
"start_offset": 12,
"end_offset": 27,
"label": "bod"
},
{
"id": 2,
"entity": "血浆因子",
"start_offset": 30,
"end_offset": 34,
... |
当出现肾功能不全或者心力衰竭时，血浆交换更是第一选择，或合用血液透析技术。 | [
{
"id": 0,
"entity": "肾功能不全",
"start_offset": 3,
"end_offset": 8,
"label": "sym"
},
{
"id": 1,
"entity": "心力衰竭",
"start_offset": 10,
"end_offset": 14,
"label": "sym"
},
{
"id": 2,
"entity": "血浆交换",
"start_offset": 16,
"end_offset": 20,
"label": "pr... |
血浆处理应在征兆出现的24小时内，通常血浆交换量每次40ml/kg，每天或隔天置换1次，3～4次后逐渐减少，增加血浆置换量能提高治疗效果；不耐受患儿，可以每天分2次进行置换，以减少输入的循环血浆，血浆的置换量第一天30～40ml/kg，此后10～20ml/kg，每天或隔天置换1次，3～4次后逐渐减少，直至完全缓解。 | [
{
"id": 0,
"entity": "血浆",
"start_offset": 0,
"end_offset": 2,
"label": "bod"
},
{
"id": 1,
"entity": "血浆",
"start_offset": 19,
"end_offset": 21,
"label": "bod"
},
{
"id": 2,
"entity": "血浆置换",
"start_offset": 56,
"end_offset": 60,
"label": "pro"
... |
（四）甲基泼尼松龙冲击治疗能控制溶血的发展，促进肾损伤的恢复。 | [
{
"id": 0,
"entity": "甲基泼尼松龙冲击治疗",
"start_offset": 3,
"end_offset": 13,
"label": "pro"
},
{
"id": 1,
"entity": "溶血",
"start_offset": 16,
"end_offset": 18,
"label": "sym"
},
{
"id": 2,
"entity": "肾",
"start_offset": 24,
"end_offset": 25,
"label": "b... |
（五）其他疗法抗生素、肝素及链激酶、抗血栓制剂（阿司匹林、双嘧达莫）、纤溶药物和维生素E等疗效不确切，一般并不提倡。 | [
{
"id": 0,
"entity": "抗生素",
"start_offset": 7,
"end_offset": 10,
"label": "dru"
},
{
"id": 1,
"entity": "肝素",
"start_offset": 11,
"end_offset": 13,
"label": "dru"
},
{
"id": 2,
"entity": "链激酶",
"start_offset": 14,
"end_offset": 17,
"label": "dru"
... |
对有血小板聚集者，可用PGI<sub>2</sub>静滴，其机制可能为抑制肾小球内血栓形成，利于肾功能恢复。 | [
{
"id": 0,
"entity": "血小板",
"start_offset": 2,
"end_offset": 5,
"label": "bod"
},
{
"id": 1,
"entity": "PGI<sub>2</sub>",
"start_offset": 11,
"end_offset": 26,
"label": "dru"
},
{
"id": 2,
"entity": "静滴",
"start_offset": 26,
"end_offset": 28,
"labe... |
初始剂量为每分钟2.5ng/kg，1周内逐渐加量，疗程8～12天；也可用前列腺素E<sub>1</sub>10μg/次，1～2次/天，用7～10天。 | [
{
"id": 0,
"entity": "前列腺素E<sub>1</sub>",
"start_offset": 36,
"end_offset": 53,
"label": "dru"
}
] |
剂量大时可致低血压及心律改变。 | [
{
"id": 0,
"entity": "低血压",
"start_offset": 6,
"end_offset": 9,
"label": "sym"
},
{
"id": 1,
"entity": "心律改变",
"start_offset": 10,
"end_offset": 14,
"label": "sym"
}
] |
关于急性期后治疗：急性期后指患儿溶血停止，以乳酸脱氢酶下降、血红蛋白和血小板开始回升为标志。 | [
{
"id": 0,
"entity": "溶血停止",
"start_offset": 16,
"end_offset": 20,
"label": "sym"
},
{
"id": 1,
"entity": "乳酸脱氢酶",
"start_offset": 22,
"end_offset": 27,
"label": "bod"
},
{
"id": 2,
"entity": "乳酸脱氢酶下降",
"start_offset": 22,
"end_offset": 29,
"label"... |
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