医学PPT课件血友病HEMOPHILIA

血友病（HEMOPHILIA）ThispapermainlyintroducesthedesignofanintelligenttemperaturecontrolsystemwhichrealizesthefunctionoftemperaturemeasurementandcontrolbyusingsinglebusdigitaltemperaturesensorDS18B20andsinglechipmicrocomputer.ThecorecomponentsofthesystemareAT89C51microcontrollerandDS18B20temperaturesensor.概述（INTRODUCTION）定义–一组遗传性凝血活酶生成障碍所致的先天性出血性疾病；共同临床表现是轻微外伤出血不止，压迫止血无效。发病情况–93年武汉国际会议（包括成人、儿童）报告5-10/10万人群，国内报告2--3/10万人群，欧美较高。构成比–国内报告血友病甲乙、丙发病率之比9：3：1。我院84例分析结果是8：5：1。除血友病甲乙丙外第4位较常见因子缺乏症是血管性假性血友病。重点介绍血友病甲即因子Ⅷ缺乏症。ThispapermainlyintroducesthedesignofanintelligenttemperaturecontrolsystemwhichrealizesthefunctionoftemperaturemeasurementandcontrolbyusingsinglebusdigitaltemperaturesensorDS18B20andsinglechipmicrocomputer.ThecorecomponentsofthesystemareAT89C51microcontrollerandDS18B20temperaturesensor.病因&发病机理（ETILOLGY）缺陷基因–FⅧ基因的缺陷导致FⅧ的缺乏或结构异常–控制FⅧ基因位于Xq2.8遗传特点–X-连锁遗传性疾病，女性杂合子携带，男性半合子发病，女性纯合子亦可发病但少见。–30%无明显家族史可能是基因突变或跳跃式遗传。ThispapermainlyintroducesthedesignofanintelligenttemperaturecontrolsystemwhichrealizesthefunctionoftemperaturemeasurementandcontrolbyusingsinglebusdigitaltemperaturesensorDS18B20andsinglechipmicrocomputer.ThecorecomponentsofthesystemareAT89C51microcontrollerandDS18B20temperaturesensor.病因&发病机理（ETILOLGY）血友病甲主要是Ⅷ因子凝血活性部分减低而出血，而大分子抗原部分正常。（单独大分子抗原部分缺乏再合并血小板功能障碍临床表现位血管性假性血友病）FⅧThispapermainlyintroducesthedesignofanintelligenttemperaturecontrolsystemwhichrealizesthefunctionoftemperaturemeasurementandcontrolbyusingsinglebusdigitaltemperaturesensorDS18B20andsinglechipmicrocomputer.ThecorecomponentsofthesystemareAT89C51microcontrollerandDS18B20temperaturesensor.临床表现（CLINICALMANIFESTATION）特点–出血-软组织血肿和关节腔出血–终生轻微外伤后出血不止和反复发作–出血的严重程度和发生频率与FⅧ缺乏程度相平行–同一家族FⅧ缺乏程度和临床出血的严重程度相似–新生儿期可发病正常分娩可造成颅内出血，脾破裂，胸腔出血及断脐出血不止。最晚发病可在成人ThispapermainlyintroducesthedesignofanintelligenttemperaturecontrolsystemwhichrealizesthefunctionoftemperaturemeasurementandcontrolbyusingsinglebusdigitaltemperaturesensorDS18B20andsinglechipmicrocomputer.ThecorecomponentsofthesystemareAT89C51microcontrollerandDS18B20temperaturesensor.临床表现（CLINICALMANIFESTATION）关节出血–特征性出血和最常见致残的原因–见于重型患者发生率可达90%–负重关节易受累肌肉软组织血肿–常见和特征性表现发生率75%皮肤黏膜出血手术及注射部位出血血性囊肿（假肿瘤）其他部位出血–消化道–血尿–颅内出血ThispapermainlyintroducesthedesignofanintelligenttemperaturecontrolsystemwhichrealizesthefunctionoftemperaturemeasurementandcontrolbyusingsinglebusdigitaltemperaturesensorDS18B20andsinglechipmicrocomputer.ThecorecomponentsofthesystemareAT89C51microcontrollerandDS18B20temperaturesensor.临床表现（CLINICALMANIFESTATION）血友病A的临床分型分型FⅧ：C临床特点重型1%起病早，出血重中型1-5%2岁后出现典型出血表现轻型6-20%无自发出血和关节出血亚临床型21-55%严重外伤或手术后渗血ThispapermainlyintroducesthedesignofanintelligenttemperaturecontrolsystemwhichrealizesthefunctionoftemperaturemeasurementandcontrolbyusingsinglebusdigitaltemperaturesensorDS18B20andsinglechipmicrocomputer.ThecorecomponentsofthesystemareAT89C51microcontrollerandDS18B20temperaturesensor.实验室（LBORTARYSTUDIES）过筛实验–KPTT延长，内源性诸因子均可能有缺陷，–PT正常，外源性Ⅲ，Ⅴ，Ⅶ，Ⅹ因子均正常。–结果：Ⅷ、Ⅸ、Ⅺ、Ⅻ等因子可能有缺陷纠正实验血友病A血友病B血友病C正常血浆纠正纠正纠正正常血清不纠正纠正纠正BaSO4吸附血浆纠正不纠正纠正ThispapermainlyintroducesthedesignofanintelligenttemperaturecontrolsystemwhichrealizesthefunctionoftemperaturemeasurementandcontrolbyusingsinglebusdigitaltemperaturesensorDS18B20andsinglechipmicrocomputer.ThecorecomponentsofthesystemareAT89C51microcontrollerandDS18B20temperaturesensor.诊断及鉴别诊断（DIAGNOSIS&DIFFERENTIALDIAGNOSIS）诊断–性别、家族史–临床出血特点–实验室检查确诊ThispapermainlyintroducesthedesignofanintelligenttemperaturecontrolsystemwhichrealizesthefunctionoftemperaturemeasurementandcontrolbyusingsinglebusdigitaltemperaturesensorDS18B20andsinglechipmicrocomputer.ThecorecomponentsofthesystemareAT89C51microcontrollerandDS18B20temperaturesensor.诊断及鉴别诊断（DIAGNOSIS&DIFFERENTIALDIAGNOSIS）血友病甲与血管性假性血友病鉴别血友病血管性血友病遗传方式伴性遗传常染色体显性/隐性常见出血部位关节肌肉软组织粘膜皮肤胃肠Aspirin耐量试验阴性阳性出血时间正常延长血小板黏附率正常异常ⅧR：C中-重度降低正常或稍降低ⅧR：Ag正常减低血小板聚集率正常明显减低瑞斯托霉素诱导正常降低ThispapermainlyintroducesthedesignofanintelligenttemperaturecontrolsystemwhichrealizesthefunctionoftemperaturemeasurementandcontrolbyusingsinglebusdigitaltemperaturesensorDS18B20andsinglechipmicrocomputer.ThecorecomponentsofthesystemareAT89C51microcontrollerandDS18B20temperaturesensor.预防（PREVENTION）减少本病发生–遗传性疾病婚前检测–杜绝近亲结婚监测出血并及时处理，减少畸形发生ThispapermainlyintroducesthedesignofanintelligenttemperaturecontrolsystemwhichrealizesthefunctionoftemperaturemeasurementandcontrolbyusingsinglebusdigitaltemperaturesensorDS18B20andsinglechipmicrocomputer.ThecorecomponentsofthesystemareAT89C51microcontrollerandDS18B20temperaturesensor.治疗（TREATMENT）原则–尚无根治方法，转基因治疗仍在实验阶段，主要是Ⅸ因子–主要治疗方法仍是终身因子替代疗法。ThispapermainlyintroducesthedesignofanintelligenttemperaturecontrolsystemwhichrealizesthefunctionoftemperaturemeasurementandcontrolbyusingsinglebusdigitaltemperaturesensorDS18B20andsinglechipmicrocomputer.ThecorecomponentsofthesystemareAT89C51microcontrollerandDS18B20temperaturesensor.治疗（TREATMENT）替代疗法–输血、冰冻血浆血友病甲：T1/28--12h，输6h内鲜血及血浆，9-12h重复血友病乙：T1/224h且较稳定，3天内鲜血血友病丙：T1/240--48h但离体不稳定，最好同血友病甲一样的鲜血，但可2-3天重复一次–Ⅷ因子制剂–凝血酶原复合物(PPSB)，内含Ⅰ，Ⅶ，Ⅸ，Ⅹ因子Thispapermainlyintroducesthedesignofanintelligenttemperat