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输血可改善贫血症状,但有时也有一定的危险性,例如给自身免疫性溶血性贫血病儿输血可发生严重的溶血反应。
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大量输血还可抑制病儿的造血机能。
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对某些先天性或遗传性溶血性贫血除给予对症处理外,尚可采取切脾甚至造血干细胞移植等治疗。
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总之,溶血性贫血的治疗应针对某一特定缺陷来选择治疗方案。
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参考文献1.孙锟,陈树宝,周爱卿,等.二维超声技术检测右心室容量.中华医学杂志,1993;7:353-3532.孙锟,陈树宝,江海,等.婴儿心脏三维超声信息采集及重建的方法学研究.中国医学影像技术,1999;15(10):775-7753.周爱卿.心导管术-先天性心脏病的诊断与治疗.济南:山东科技出版社,19974.AllenHD,GutgesellHP,ClarkEB,DriscollDJ.MossandAdam’sheartdiseaseininfants,children,andadolescentsincludingthefetusandyoungadult(Sixthedition).Philadelphia:LippincottWilliams&wilkins,2001.162-1625.BalajiS,LauY,CaseC,etal.VentriculartachycardiaintetralogyofFallot.AmJCardiol,1997.80:1606.BudingerTF,BersonA,McVeighER,etal.CardiacMRimaging:reportofaworkinggroupsponsoredbytheNationalHeart,LungandBloodInstitute.Radiology,1998,208:573-5767.FischC.Evolutionoftheclinicalelectrocardiogram.JAmCollCardiol,1989,14:11278.KuglerJD,DanfordDA,DealBJ,etal.Radiofrequencycatheterablationfortachyarrhythmiasinchildrenandadolescents.NEnglJMed,1994,330:1481-14819.NASPEExpertConsensusConference:RadiofrequencyCatheterAblationinChildrenWithandWithoutCongenitalHeartDisease.ReportoftheWritingCommittee.PACE,2002,25(6):1000-100010.PihkalaJ,NykanenD,FreedomRM,etal.Interventionalcardiaccatheterization.PediatrClinNorthAm,1999,46(2):441-46011.ReichJD,AuldD,HulseE,etal.ThePediatricRadiofrequencyAblationRegistry’sexperiencewithEbstein’sanomaly.PediatricElectrophysiologySociety.JCardiovascElectrophysiol,1998,9:1370-137012.SchelagBJ,LauSH,HelfantRH,etal.CathetertechniquesforrecordingHisbundleactivityinman.Circulation,1969,39:1313.VanHareGF.Indicationsforradiofrequencyablationinthepediatricpopulation.JCardiovascElectrophysiol,1997,8:952-95214.WashingtonRL,BrickerJT,AlperBS,etal.Guidelinesforexercisetestinginthepediatricagegroup:Fromthecommitteeonatherosclerosisandhypertensioninchildrencounciloncardiovasculardiseaseintheyoung,theAmericanHeartAssociation.Circulation,1994,90:216615.ZipesDP,DiMarcoJP,GillettePC,etal.ACC/AHATaskForcereport.Guidelinesforclinicalintracardiacelectrophysiologicalandcatheterablationprocedures.Circulation,1995,92:673
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第九篇心血管系统疾病第一章心脏的胚胎发生学心脏由胚胎的中胚层组织演变而来,在胚胎的头三周内,中胚层细胞分化形成原始心管。
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此后,经过心管的分区、扭转及分隔逐渐形成完整的心脏。
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一、心管管的形成早在胚胎15mm时,胚胎腹面咽喉下部的两侧下部的两侧,由原始基(primordia)形成两个管状结构,其内层为心内膜始基,外层为心外肌膜始基。
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至22天左右,两个内皮管逐渐向正中移动,两个原始基相互融合成一腔,此即原始心管。
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参考文献1.王宏伟,杨锡强,朱启镕.小儿HIV感染和艾滋病诊断和处理建议.中华儿科杂志,2003,41(8)∶611-6122.杨锡强等.儿童免疫学.北京:人民卫生出版社,20013.JaneM,PamelaA,DavidW,etal.AntiretroviralAdherenceInterventions:AReviewofCurrentLiteratureandOngoingStudies.InternationalAIDSSocietyUSATopicsinHIVMedicine,2003,11(6):185-1854.OleskeJM.ReviewofrecentguidelinesforantiretroviraltreatmentofHIV-infectedchildren.TopHIVMed,2003,11(6):1805.SaloojeeH,ViolariA.Regularreview:HIVinfectioninchildren.BMJ.2001,323(7314):670
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第四节肾穿刺活体组织检查肾穿刺活体组织检查(简称肾活检)在临床及科研上运用已有60余年的历史。
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近十年来,随着穿刺技术的不断革新,以及光学显微镜、电子显微镜、免疫荧光技术和免疫酶标技术的应用与推广,各种组织化学及分子病理学研究方法的逐步完善,使肾活检的成功率及安全性大大提高,并且成为研究肾脏病变的病因、发病机制、组织分型、病理演变过程、临床与病理形态学的联系,以及指导治疗、评估预后等方面的一种重要的手段,对肾脏病的迅速发展产生了重大影响。
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一、肾活检方法肾活检主要方法有开放性肾活检和经皮肾穿刺活检两种。
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1990年Mal等曾介绍经静脉活检,但临床较少采用。
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(一)开放性肾活检通过手术直接采取肾脏活组织,又称直视下肾活检。
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为肾活检的早期方法,现主要用于经皮肾活检有禁忌证或失败时采用,如孤立肾或对侧肾无功能,有定位好、组织块较大以及止血好等优点。
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还应注意直视下肾活检时,不宜采用锐器切割组织,仍应行穿刺术,减少损伤。
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(二)经皮肾穿刺活检是目前最常用、最安全的方法。
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穿刺部位的定位方法有体表解剖定位、静脉肾盂造影荧光屏直视定位和B型超声波导向定位。
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1.体表定位法儿童一般以背中线与外侧线连线中点内侧0.5~10cm,第一腰椎棘突水平,十二肋下0.5~1.0cm处为穿刺点。
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临床常需参考腹部平片上肾脏的位置,但成功率较低,特别是肾脏位置有变异时,与有效穿刺点的符合率较低。
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2.静脉肾盂造影荧光屏直视定位直接观察电视屏上的肾脏轮廓,确定穿刺部位及进针方向和深度。
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成功率可达90%以上,并能及时发现是否存在肾脏畸形、转位不良以及肾盂积水等,并避免误刺肾门所致的大出血。
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缺点有造影剂的肾毒性、接受X线照射以及需放射科医生协助等。
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3.B型超声波导向定位临床上采用B型超声波定位后,大大提高了定位的正确性,并能精确地测得肾表面与皮肤之间的距离、吸气和呼气时肾脏下缘以及肾脏下极的厚度,使穿刺者能准确掌握位置和针的深度。
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我们体会,实际穿刺的深度常常大于B超探查的深度,最佳比例为1.3∶1~1.4∶1。
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20世纪90年代以后出现了应用穿刺枪作经皮肾穿刺活检的报道,并通过B超附有的特殊穿刺探头,在B超直接引导下进针。
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我院自20世纪80年代中期采用B型超声波定位,1999年开始应用自动穿刺枪在B超穿刺探头的指引下行肾穿刺术,例数已达1100余例,成功率为99%。
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二、补充累积损失量根据脱水程度及性质补充:即轻度脱水约30~50ml/kg(体重);中度为50~100ml/kg;重度为100~150ml/kg。
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通常对低渗性脱水补2/3张含钠液;等渗性脱水补1/2张含钠液;高渗性脱水补1/5~1/3张含钠液。
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补液的速度取决于脱水程度,原则上应先快后慢。
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对伴有循环不良和休克的重度脱水患儿,开始应快速输入等渗含钠液(生理盐水或2∶1液)按20ml/kg于30分钟~1小时输入。
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酸碱平衡紊乱及其他电解质异常的纠正见(本章第二、三节)。
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对于高渗性脱水,需缓慢纠正高钠血症(每24小时血钠下降<10mmol/L),也可在数天内纠正。
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有时需用张力较高,甚至等张液体,以防血钠迅速下降出现脑水肿。
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参考文献1.HuW,TongS,OldenburgB,etal.SerumvitaminAconcentrationsandgrowthinchildrenandadolescentsinGansuProvince,china.AsicPacJClinNutr,2001,10(1):63-662.JorgensenFS,FledingP,VintherS,etal.VitaminKtoneonates.Peroralversusintramuscularadministration.ActaPaediatrScand,1991,80(3):304-3043.Arteaga-VizcainoM,Torres-EspinaM,RedondoL,etal.EffectsoftheadministrationofvitaminKontheactivityoffactorⅡ、Ⅶ、Ⅹinhealthynewborns.InvestClin,1995,36(2):83-834.HathawayWE,IsarangkuraPB,MahasandanaC,etal.ComparisonoforalandparenteralvitaminKprophylaxisforpreventionoflatehemorrhagicdiseaseofthenewborn.JPediatr,1991,119(3):461-4645.HuysmanMW,SauerPJ.ThevitaminKcontroversy.CurrOpinPediatr,1994,6(2):129-1296.廖清奎.营养性疾病-小儿营养与营养性疾病.天津:天津科学技术出版社,1990:139-139,157-1577.顾景范,邵继智.临床营养学.上海:上海科学技术出版社,1990:222-2228.史奎雄.蛋白质-热能营养不良//医学营养学.上海:上海交通大学出版社,1998:114-1149.EKHARDEZIEGLER,LJFILER,JR.现代营养学.第7版.北京:人民卫生出版社,1998:116-11610.蒋月英.儿童营养学.北京:科学技术文献出版社,1992:77-7711.林良明,刘玉琳,鲁杰.婴儿维生素K1</sub>10次口服预防效果评价.中国儿童保健杂志,2002,6(10):147-14712.中国营养学会.中国居民膳食营养素参考摄入量.北京:中国轻工业出版社,200013.ThomasGBaumgartner.ClinicalGuidetoParenteralMicronutrition.2nded.1991:193-19314.沈霞,李定国,姚建,等.现代生物化学检验与临床实践.上海:上海科学技术文献出版社,199915.葛可佑.中国营养科学全书.北京:人民卫生出版社,2004
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第六节疟疾【病原】疟疾(malaria)是感染疟原虫所引起的传染病。
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疟原虫可分为四种:间日疟原虫(P.vivax)、三日疟原虫(P.malariae)、恶性疟原虫(P.falciparum)、卵形疟原虫(P.ovale)。
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疟原虫的发育包括两个阶段,在人体内进行无性生殖(裂体生殖),在蚊子体内完成有性生殖及进行孢子增殖。
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疟原虫在人体内的发育包括红细胞外期裂体增殖和红细胞内期裂体增殖。
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按蚊涎腺内的子孢子经血液进入肝细胞,每个子孢子进入一个肝细胞进行裂体增殖,形成潜隐子(裂殖子)。
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被寄生的肝细胞破裂后,一部分潜隐子被吞噬细胞所消灭,一部分进入红细胞内寄生。
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部分红细胞内期的裂殖子不再进行无性分裂,而是发育成雌的或雄的配子体。
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【流行病学】疟疾在我国分布很广,全国大致可分为四个区:①黄河以北为低疟区,多为间日疟,少数三日疟,无恶性疟;②黄河与长江之间为中度疟区,间日疟多于恶性疟;③长江以南至沿海为高疟区,各种疟疾都有;④西北地区是高寒干热地带,为全国疟疾最轻地区。
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按蚊是疟原虫的终末宿主,也是传播疟疾的媒介。
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【病理生理】引起疟疾临床症状主要是红细胞内期原虫。
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裂体增殖过程中,疟原虫的代谢产物——血红蛋白及破坏的红细胞碎片,进入人体血流,刺激体温中枢而引起人的发热及其他有关症状。
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人体内残存的疟原虫产生抗原变异,在克服人体免疫后,重新大量繁殖,再次出现临床发作,称为再燃;间日疟因存在“红外期”,当产生的潜隐子重新侵入红细胞内繁殖时,又出现临床症状,称为复发。
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再燃和复发与人体免疫力降低及疟原虫虫株有关。
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【临床表现】(一)潜伏期间日疟的潜伏期为10~12日,三日疟为14~25日或更长,恶性疟为9~16日。
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潜伏期可伴有低热,乏力,全身酸痛等症状。
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(二)寒战期突然发作,畏寒,全身发抖,关节肌肉酸痛面色苍白,发绀,儿童常有抽搐。
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(三)高热期寒战过后,体温迅速升高达39~41℃,患儿面红耳热,结膜充血,头痛,烦躁不安,常发生谵妄,抽搐,昏迷。
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(四)出汗退热期患儿全身大汗淋漓,皮肤变冷1~2小时内体温降至37℃以下。
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(五)周期性发作上述急性发作是周期性的。
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间歇时间因各型疟原虫在红细胞内进行裂体增殖所需时间的长短而不同。
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恶性疟最严重,常出现以下凶险类型:1.脑型疟有高热,谵语,抽搐,昏迷,脑膜刺激征阳性。
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2.胆汁型高热,黄疸,肝脾肿大,昏迷。
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3.黑尿热型高热,葡萄酒样尿,黄疸,贫血,无尿等。
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4.休克虚脱型体温不升,血压下降,脉搏微弱。
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另外,婴幼儿疟疾常不定型,各期不明显,易误诊。
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(六)先天性疟疾指出生后不超过感染子孢子的潜伏期(间日疟为13~15天,三日疟为28~30天),并在疟疾流行区,在没有按蚊感染或输血等情况下发生。
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其母孕期有疟疾史,若婴儿的疟原虫与母体的属同一型,则为先天性疟疾。
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(七)慢性疟疾多为重复感染或未正规治疗的结果,主要症状是精神不振,贫血,肝脾肿大,血液中可找到疟原虫。
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【并发症】(一)支气管炎或肺炎(二)肾炎多见于长期患疟疾的儿童(三)血红蛋白尿又称“黑尿热”,多发生在恶性疟疾。
[ { "id": 0, "entity": "支气管炎", "start_offset": 8, "end_offset": 12, "label": "dis" }, { "id": 1, "entity": "肺炎", "start_offset": 13, "end_offset": 15, "label": "dis" }, { "id": 2, "entity": "肾炎", "start_offset": 18, "end_offset": 20, "label": "dis" }, { "id": 3, "entity": "疟疾", "start_offset": 26, "end_offset": 28, "label": "dis" }, { "id": 4, "entity": "血红蛋白尿", "start_offset": 34, "end_offset": 39, "label": "dis" }, { "id": 5, "entity": "黑尿热", "start_offset": 42, "end_offset": 45, "label": "dis" }, { "id": 6, "entity": "恶性疟疾", "start_offset": 51, "end_offset": 55, "label": "dis" } ]
以前认为是疟原虫的毒素引起的溶血,现多数学者认为是患者的红细胞有遗传缺陷,主要是6-磷葡萄糖脱氢酶的含量减少,使氧化型谷胱甘肽还原不足,在服用抗疟药(如奎宁、伯氨喹)和某些退热药(如阿司匹林)后,红细胞在血管内大量溶解而发生血红蛋白尿。
[ { "id": 0, "entity": "疟原虫", "start_offset": 5, "end_offset": 8, "label": "mic" }, { "id": 1, "entity": "溶血", "start_offset": 14, "end_offset": 16, "label": "dis" }, { "id": 2, "entity": "红细胞", "start_offset": 28, "end_offset": 31, "label": "bod" }, { "id": 3, "entity": "6-磷葡萄糖脱氢酶的含量减少", "start_offset": 40, "end_offset": 54, "label": "sym" }, { "id": 4, "entity": "氧化型谷胱甘肽", "start_offset": 56, "end_offset": 63, "label": "bod" }, { "id": 5, "entity": "抗疟药", "start_offset": 71, "end_offset": 74, "label": "dru" }, { "id": 6, "entity": "奎宁", "start_offset": 76, "end_offset": 78, "label": "dru" }, { "id": 7, "entity": "伯氨喹", "start_offset": 79, "end_offset": 82, "label": "dru" }, { "id": 8, "entity": "退热药", "start_offset": 86, "end_offset": 89, "label": "dru" }, { "id": 9, "entity": "阿司匹林", "start_offset": 91, "end_offset": 95, "label": "dru" }, { "id": 10, "entity": "红细胞", "start_offset": 98, "end_offset": 101, "label": "bod" }, { "id": 11, "entity": "血管", "start_offset": 102, "end_offset": 104, "label": "bod" }, { "id": 12, "entity": "血红蛋白尿", "start_offset": 112, "end_offset": 117, "label": "sym" } ]
【实验室诊断】(一)末梢血涂片找疟原虫(二)骨髓涂片(三)疟原虫的免疫诊断方法1.抗体的检测间接荧光抗体试验(IFAT)为当前国内外应用最广的一种检测疟原虫抗体的方法,另外还有间接红细胞凝集试验(IHA)、酶联免疫吸附试验(ELISA)。
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2.抗原的检测放射免疫试验和酶联免疫吸附试验。
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3.DNA探针杂交试验。
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(四)用基因工程原理重组的蛋白质或多肽作为抗原诊断疟疾【治疗】(一)一般治疗发作时卧床休息,应进食营养丰富、富含维生素、易消化的食物。
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对贫血者给予铁剂,严重者输血。
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(二)抗疟药物治疗根据抗疟药物的性能和作用,大致分为三类:主要用于控制临床症状,如氯喹、奎宁、青蒿素等,是杀灭滋养体及裂殖体的有效药物;主要用于复发和传播,如伯氨喹、扑疟喹,是杀灭肝内的潜隐子(红细胞外期)及配子体的特效药;主要用于预防疟疾的感染,如乙胺嘧啶是孢子增殖杀灭剂,对红细胞内期裂殖体有抑制作用。
[ { "id": 0, "entity": "抗疟药物", "start_offset": 3, "end_offset": 7, "label": "dru" }, { "id": 1, "entity": "抗疟药物", "start_offset": 11, "end_offset": 15, "label": "dru" }, { "id": 2, "entity": "氯喹", "start_offset": 41, "end_offset": 43, "label": "dru" }, { "id": 3, "entity": "奎宁", "start_offset": 44, "end_offset": 46, "label": "dru" }, { "id": 4, "entity": "青蒿素", "start_offset": 47, "end_offset": 50, "label": "dru" }, { "id": 5, "entity": "伯氨喹", "start_offset": 79, "end_offset": 82, "label": "dru" }, { "id": 6, "entity": "扑疟喹", "start_offset": 83, "end_offset": 86, "label": "dru" }, { "id": 7, "entity": "肝", "start_offset": 90, "end_offset": 91, "label": "bod" }, { "id": 8, "entity": "红细胞", "start_offset": 97, "end_offset": 100, "label": "bod" }, { "id": 9, "entity": "疟疾", "start_offset": 118, "end_offset": 120, "label": "dis" }, { "id": 10, "entity": "乙胺嘧啶", "start_offset": 125, "end_offset": 129, "label": "dru" }, { "id": 11, "entity": "红细胞", "start_offset": 139, "end_offset": 142, "label": "bod" } ]
1.氯喹一般常规为3日疗法,首次量为10mg/kg(最大量不超过600mg),6小时后再服1次,5mg/kg;24小时后再服用5mg/kg;48小时后服最后1次5mg/kg;不良反应有恶心、呕吐、白细胞减少等。
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2.伯氨喹用于氯喹疗程之后,每片含基质7.5mg。
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若发生溶血,白细胞减少,或高铁血红蛋白血症,应立即停药。
[ { "id": 0, "entity": "溶血", "start_offset": 3, "end_offset": 5, "label": "dis" }, { "id": 1, "entity": "白细胞减少", "start_offset": 6, "end_offset": 11, "label": "sym" }, { "id": 2, "entity": "高铁血红蛋白血症", "start_offset": 13, "end_offset": 21, "label": "dis" } ]
3.青蒿素及其衍生物青蒿素成人剂量为:口服每次0.6g,每天3次,连用3天为一疗程;肌内注射剂量为200~300mg/次,每天1~2次,连用3天。
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青蒿琥酯片(每片20mg),成人5天疗程总剂量440mg,首剂80mg,6小时后服40mg,以后每天上,下午各40mg,共5天。
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复方蒿甲醚是由抗疟新药蒿甲醚和苯芴醇组成的新抗疟复方,在抗氯喹恶性疟流行区,具有广泛的应用前景。
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4.乙胺嘧啶主要用于预防,一般与氯喹同用。
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每隔10~14天口服乙胺嘧啶1次,年长儿为25mg,学龄前儿童为12.5mg,同时加氯喹0.25~0.5g。
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(三)对脑型疟疾的救治脑型疟疾大都是恶性疟原虫引起,因此,对恶性疟要早诊早治,药物以青蒿素类药为首选。
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【预防】措施有:①治疗带疟原虫的患者,消灭传染源;②消灭疟疾的传播媒介按蚊;③个人预防,避免蚊虫叮咬;④疟疾疫苗;⑤旅行疫区应用预防剂,服药应自进入流行区前1周开始,并持续到离开流行区后6~8周。
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如乙胺嘧啶。
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第十四章常见小儿外科消化系统疾病第一节幽门肥厚性狭窄幽门肥厚性狭窄(hypertrophicpyloricstenosis,HPS)是因幽门环状括约肌增厚幽门管延长正常结构消失,导致胃出口部梗阻胃代偿性扩张、肥厚和蠕动加快幽门平滑肌细胞肥大,而非增生幽门肥厚性狭窄的临床及病理表现,认为该病是一种先天性疾病。
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胃空肠吻合术是当时经典的治疗方法,死亡率高达60%。
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黏膜外幽门成形术是另一种术式,但因为缝合易撕裂水肿的肌肉,导致大出血,疗效也不理想。
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自从1911年Ramstedt首次放弃缝合肌肉后,幽门环肌切开术成为标准术式。
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【发病率】普通人群中幽门肥厚发病率为0.1%~1%,且有上升趋势,40年前的报告是1/900~1/300;而英国最近调查表明过去几十年以来,发病率已从0.1%~0.2%上升到0.3%~0.8%。
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MayoClinic的大样本人群调查显示:1950~1984年间美国Minnesota州Olmsted郡人群HPS总体发病率为0.26%,但至调查后期,发病率已接近0.5%。
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另一项前瞻性调查研究发现:随机抽取1400名足月新生儿接受B超检查,9名(0.65%)出现肥厚性幽门狭窄的患儿出生时幽门B超检查均无异常表现。
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此外,有这样一种观点:头胎男孩最易患HPS,但未获证实。
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幽门狭窄的遗传特征多变,母亲遗传的较父亲多见。
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【病因】HPS的病因尚不清楚,目前有几种假说。
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其中之一是胃蠕动和幽门松弛之间不协调胃内压升高时,幽门反而不恰当收缩幽门肌肉代偿性肥厚胃收宫内胃扩张患儿生后发生HPS的报道外,其他均未发现HPS患儿在生后数周内有胃排空异常。
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有人试图将血胃泌素升高、高胃酸与HPS联系在一起,但未获成功。
[ { "id": 0, "entity": "HPS", "start_offset": 16, "end_offset": 19, "label": "dis" } ]
事实上,任何原因所致胃排出口梗阻均可引起胃扩张,通过胃泌素使胃酸分泌增加。
[ { "id": 0, "entity": "胃排出口梗阻", "start_offset": 10, "end_offset": 16, "label": "dis" }, { "id": 1, "entity": "胃泌素", "start_offset": 26, "end_offset": 29, "label": "bod" }, { "id": 2, "entity": "胃酸", "start_offset": 30, "end_offset": 32, "label": "bod" } ]
有报道指出某些患儿的前列腺素(如PGE2</sub>和PGF2</sub>-α)水平升高可促使幽门收缩HPS。
[ { "id": 0, "entity": "前列腺素", "start_offset": 10, "end_offset": 14, "label": "bod" }, { "id": 1, "entity": "PGE2", "start_offset": 16, "end_offset": 20, "label": "bod" }, { "id": 2, "entity": "幽门收缩", "start_offset": 47, "end_offset": 51, "label": "sym" }, { "id": 3, "entity": "HPS", "start_offset": 51, "end_offset": 54, "label": "dis" } ]
但是,临床上接受前列腺素治疗的新生儿,即使出现幽门痉挛性梗阻,亦未继发肌肉肥厚原发性肠神经系统发育异常学说来解释HPS的形成或许更能令人信服。
[ { "id": 0, "entity": "前列腺素治疗", "start_offset": 8, "end_offset": 14, "label": "pro" }, { "id": 1, "entity": "幽门痉挛性梗阻", "start_offset": 23, "end_offset": 30, "label": "dis" }, { "id": 2, "entity": "肌肉肥厚", "start_offset": 35, "end_offset": 39, "label": "dis" }, { "id": 3, "entity": "原发性肠神经系统发育异常", "start_offset": 39, "end_offset": 51, "label": "dis" }, { "id": 4, "entity": "HPS", "start_offset": 56, "end_offset": 59, "label": "dis" } ]
这是因为一方面免疫组化研究发现,HPS患儿体内神经肽,如生长激素释放因子(GRP)、血管活性多肽(VIP)、胃泌素及P物质等较正常儿童明显减少;另一方面,有研究表明幽门区平滑肌松弛介质NO浓度降低是肥厚性幽门狭窄形成的重要因素。
[ { "id": 0, "entity": "HPS", "start_offset": 16, "end_offset": 19, "label": "dis" }, { "id": 1, "entity": "神经肽", "start_offset": 23, "end_offset": 26, "label": "bod" }, { "id": 2, "entity": "生长激素释放因子", "start_offset": 28, "end_offset": 36, "label": "bod" }, { "id": 3, "entity": "血管活性多肽", "start_offset": 42, "end_offset": 48, "label": "bod" }, { "id": 4, "entity": "胃泌素及P物质等较正常儿童明显减少", "start_offset": 54, "end_offset": 71, "label": "sym" }, { "id": 5, "entity": "幽门区平滑肌", "start_offset": 82, "end_offset": 88, "label": "bod" }, { "id": 6, "entity": "松弛介质", "start_offset": 88, "end_offset": 92, "label": "bod" }, { "id": 7, "entity": "肥厚性幽门狭窄", "start_offset": 99, "end_offset": 106, "label": "dis" } ]
正常情况下,幽门环肌、纵肌及肌间神经丛中都存在一定浓度的NO合成酶,而HPS患儿环肌层则缺乏该物质,且伴有ENS轴突形态的异常HPS患儿幽门肌间神经丛及肌肉内神经轴突退化HPS的病理原因是什么,其作用都是暂时的,因为术后罕见复发。
[ { "id": 0, "entity": "幽门环肌", "start_offset": 6, "end_offset": 10, "label": "bod" }, { "id": 1, "entity": "纵肌", "start_offset": 11, "end_offset": 13, "label": "bod" }, { "id": 2, "entity": "肌间神经丛", "start_offset": 14, "end_offset": 19, "label": "bod" }, { "id": 3, "entity": "NO合成酶", "start_offset": 28, "end_offset": 33, "label": "bod" }, { "id": 4, "entity": "HPS", "start_offset": 35, "end_offset": 38, "label": "dis" }, { "id": 5, "entity": "ENS轴突形态的异常", "start_offset": 53, "end_offset": 63, "label": "sym" }, { "id": 6, "entity": "HPS", "start_offset": 63, "end_offset": 66, "label": "dis" }, { "id": 7, "entity": "幽门肌间神经丛", "start_offset": 68, "end_offset": 75, "label": "bod" }, { "id": 8, "entity": "肌肉", "start_offset": 76, "end_offset": 78, "label": "bod" }, { "id": 9, "entity": "神经轴突退化", "start_offset": 79, "end_offset": 85, "label": "sym" }, { "id": 10, "entity": "HPS", "start_offset": 85, "end_offset": 88, "label": "dis" } ]