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足月新生儿出生时脊髓重2~6g,脊髓功能相对成熟。
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脊髓下端在胎儿时位于第2腰椎下缘,4岁时上移至第1腰椎。
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作腰椎穿刺时应注意,婴幼儿脊髓下端位置较低。
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脊髓的髓鞘由上而下逐渐形成,约于3岁时完成髓鞘化。
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(二)脑脊液的正常值小儿时期脑脊液的正常值为:压力0.69~1.96(新生儿0.29~0.78)kPa,外观清亮透明,潘氏试验阴性,白细胞数0~5(新生儿或小婴儿0~20)×106</sup>/L,蛋白0.2~0.4(新生儿0.2~1.2)g/L,糖2.2~4.4mmol/L。
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(三)神经反射的发育特点正常足月儿出生时即具有觅食、吸吮、吞咽、拥抱及握持等一些先天性(原始)反射和对强光、寒冷及疼痛等的反应。
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3~4个月前小儿肌张力较高,Kernig征可为阳性,2岁以下小儿Babinski征阳性(对称)亦可为生理现象。
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第十节缓慢性心律失常窦性静止与窦房传导阻滞在心电图上可表现为长间隙。
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前者一般认为是因为激动在窦房结内形成异常在窦房传导时发生阻滞心律失常在儿科少见,可见于洋地黄中毒中毒或心房广泛手术后。
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房室传导阻滞(atrialcapture)可分为三种类型,即一度房室传导阻滞、二度房室传导阻滞及三度房室传导阻滞。
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一度房室传导阻滞:PR间期延长,但所有心房激动均能传到心室。
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在莫氏Ⅱ型,由于房室传导组织有效不应期延长,使心房搏动部分不能下传至心室,发生间歇性心室脱落心室脱落前后,下传的P波,其PR间期是恒定的。
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三度房室传导阻滞(完全性房室传导阻滞):心房激动完全不能到达心室。
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先天性完全性房室传导阻滞的发病率一般认为1/2万~1/2.5万活产婴儿,常为自身免疫引起。
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患儿的母亲常患有系统性红斑狼疮(可无症状),其体内的IgG抗体(anti-SSA/Ro、anti-SSB/La)可通过胎盘损害胎儿的传导组织。
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类风湿性关节炎、皮肌炎、或Sjögren综合征偶也可引起胎儿传导组织的自身免疫性损伤。
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自身免疫原因占先天性完全性房室传导阻滞发生率的60%~70%,心脏结构正常患儿的80%。
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有些系统性红斑狼疮母亲的小儿出生时并无传导阻滞,生后3~6个月才发生。
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其他原因包括先天性房室结缺如或房室结纤维化等。
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新生儿或婴幼儿可出现心力衰竭或心源性脑缺氧综合征。
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年长儿往往因心率轻度减慢而无明显症状。
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心电图除显示PP间期与RR间期各有其固定规律外,QRS间期及形态多正常。
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后天获得性完全性房室传导阻滞的病因有:心肌炎、心脏肿瘤、心内膜炎所致的心肌脓疡、药物或电解质紊乱等,也可发生在心脏手术后。
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阻滞部位可在房室结、房室束或其分支以下。
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心电图显示QRS波多宽大畸形,时间>0.1秒,且心室率较慢,多在40次以下,因而症状较明显,轻者可诉疲乏、无力、眩晕,重者可发生心力衰竭或急性心脑缺氧综合征。
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无症状且心率>55次/分者不需给予治疗,也不应限制患儿的活动。
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心率较慢而无晕厥或心力衰竭者可口服阿托品、麻黄碱。
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危重患者可静滴异丙肾上腺素。
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安置人工起搏器的指征为:①有心力衰竭或心源性脑缺氧综合征者;②心室率显著缓慢(新生儿<55次/分,婴儿<50次/分,儿童<40次/分);③有频发室早或室速者;④心脏手术后发生三度房室传导阻滞,观察2~4周未能恢复者(ACC/AHA指征为术后高二度或三度房室传导阻滞无缓解趋势或持续至少术后7天者)。
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参考文献1.V.E.Brown,C.A.Pilkington1,B.M.Feldman,etal.Aninternationalconsensussurveyofthediagnosticcriteriaforjuveniledermatomyositis(JDM).Rheumatology(Oxford),2006,45(8):990-9902.P.Riley,L.J.McCann,S.M.Maillard,etal.Effectivenessofinfliximabinthetreatmentofrefractoryjuveniledermatomyositiswithcalcinosis.Rheumatology(Oxford...
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参考文献1.中华医学会风湿病学分会.风湿热诊治指南草案.中华风湿病学杂志,2004,8(3):504-5042.蒋明等.风湿病学.北京:科学出版社,19983.BehrmanRE,KliegmanRM,JensonHB.NelsonTextbookofPediatrics.16th</sup>ed.Philadelphia:W.B.SaunersCo,20004.杨锡强等.儿童免疫学.北京:人民卫生出版社,2001
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参考文献1.BarkinRM.EmergencyPediatrics-aguidetoambulatorycare.5thed.Mosby.Louis,Missouri,1999:314-3142.BehrmanRE.TextbookofPediatrics.16<sup>th</sup>ed.WBSaundersCompany,Philadelphia,USA,2000:287-2873.BarkinRM.PediatricEmergencyMedicine-conceptsandclinicalpractice.2nded.MosbyYear-bookIncStLouisMissouri,1997:489-4954.Ashcra...
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四、毒蛇咬伤【病因】儿童野外玩耍可被毒蛇咬伤,蛇毒的成分复杂,但主要为神经毒素和血毒素两大类,神经毒素主要作用于神经系统,引起呼吸肌麻痹、肌肉麻痹等症状;血毒素主要引起心力衰竭、溶血、出血、凝血等。
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【临床表现】血毒素主要表现为咬伤局部肿胀剧痛,可出现水疱、血泡、组织坏死、伤口流血不止,并迅即向近心端发展,全身出血或发生溶血、贫血、黄疸、血红蛋白尿及少尿、无尿,心音低钝,心律不齐,血压下降,呼吸急促、休克以致死亡。
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【治疗】(一)现场急救咬伤后立即在伤口近端2~3cm处扎肢体,阻断静脉血液和淋巴液回流,减少吸收。
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紧扎处应每隔10分钟左右放松1~2分钟,以使被扎部分组织不会因血流循环受阻而坏死,并限制患肢活动。
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(二)局部处理伤口局部用水冲洗后,并用5%依地酸二钠或1∶5000高锰酸钾水冲洗至流出的血水变为鲜红色为止。
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局部封闭疗法是在伤口周围或肿胀上方3~4cm处皮下注射0.25%~0.5%普鲁卡因加地塞米松5mg,或用胰蛋白酶4000U溶于25%普鲁卡因5~20ml,以牙痕为中心,在伤口周围作皮下环封。
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(三)全身处理尽快应用蛇药口服(如南通蛇药片、群生蛇药)或注射抗毒血清,注射前应先做皮试,阴性时作静脉注射,阳性者可做脱敏疗法,如无蛇药,可选用中草药解毒。
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常用的中草药如半枝莲、蛇果草、穿心莲、地丁草、蒲公英、马齿苋、拉拉藤、河白草、瓜子金、苦参、青木香、紫花地丁、凤尾草等。
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参考文献1.BarrattTM,AvnerED,HarmonWE.PediatricNephrology,4thedition.Baltimore,LippincottWilliams&Wilkins.1999:565-5822.LashJP,ArrudaJA.Laboratoryevaluationofrenaltubularacidosis.ClinLabMed,1993,13(1):117-1173.FortenberryJD,KenneyRD.Distalrenaltubularacidosisastheinitialmanifestationofsystemiclupuserythematosusinanadolescen...
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附:维生素A过多症维生素A过多症(hypervitaminosisA),即维生素A中毒,根据发病情况,可分为急性中毒及慢性中毒两种。
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当血清维生素A浓度超过5.1μmolL(1500UL)时出现中毒症状。
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【急性中毒】比较多见。
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由于短期内大量摄入维生素A(剂量≥20万U)所致。
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【慢性中毒】由于长期较大剂量摄入维生素A所致,发生中毒所需的累积量及时间因人而异,一般平均摄入量大于1.3万μgd(5万Ud),常发生在因慢性皮肤疾病服药的患儿。
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一般摄入数周或数月后出现症状,主要表现为慢性症状,如食欲减退、体重不增、激怒、脂溢性皮炎、皮肤瘙痒、脱发、口唇皲裂、肝脾大和肝功能损害。
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骨骼症状明显,有骨痛,尤以长骨为主,可有转移性。
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软组织肿胀、压痛而无红热,常误为脓肿。
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骨骼X线显示骨皮质肥厚、骨膜下积液和骨膜分离,尤以长骨中段更为明显。
[ { "id": 0, "entity": "骨骼X线", "start_offset": 0, "end_offset": 4, "label": "pro" }, { "id": 1, "entity": "骨皮质肥厚", "start_offset": 6, "end_offset": 11, "label": "sym" }, { "id": 2, "entity": "骨膜", "start_offset": 12, "end_offset": 14, "label": "bod" ...
【处理】一旦发现维生素A过多,应立即停服维生素A制剂及对症处理。
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急性中毒者,待停服维生素A1~2天后症状缓解;慢性中毒者,待停服维生素A,1~2周后症状减轻或消失,但骨骼X线表现需要6个月左右恢复正常。
[ { "id": 0, "entity": "急性中毒", "start_offset": 0, "end_offset": 4, "label": "dis" }, { "id": 1, "entity": "维生素A", "start_offset": 9, "end_offset": 13, "label": "dru" }, { "id": 2, "entity": "慢性中毒", "start_offset": 23, "end_offset": 27, "label": "dis"...
五、戊型肝炎【病原和流行病学】戊型肝炎病毒(hepatitisEvirus,HEV)属嵌杯病毒科,无包膜,核酸为单股正链RNA。
[ { "id": 0, "entity": "戊型肝炎", "start_offset": 2, "end_offset": 6, "label": "dis" }, { "id": 1, "entity": "戊型肝炎病毒", "start_offset": 15, "end_offset": 21, "label": "mic" }, { "id": 2, "entity": "hepatitisEvirus", "start_offset": 22, "end_offset": 37, ...
病毒在体外不稳定,对高盐、氯化铯、氯仿等敏感。
[ { "id": 0, "entity": "病毒", "start_offset": 0, "end_offset": 2, "label": "mic" } ]
细胞培养尚未建立,多种非人灵长类动物可感染HEV。
[ { "id": 0, "entity": "HEV", "start_offset": 21, "end_offset": 24, "label": "mic" } ]
患者于潜伏期末至急性早期从粪便中排出大量病毒,是主要传染源。
[ { "id": 0, "entity": "粪便", "start_offset": 13, "end_offset": 15, "label": "bod" }, { "id": 1, "entity": "病毒", "start_offset": 20, "end_offset": 22, "label": "mic" } ]
病毒经粪-口途径和接触传播。
[ { "id": 0, "entity": "病毒", "start_offset": 0, "end_offset": 2, "label": "mic" }, { "id": 1, "entity": "粪", "start_offset": 3, "end_offset": 4, "label": "bod" }, { "id": 2, "entity": "口", "start_offset": 5, "end_offset": 6, "label": "bod" } ]
我国各地区均有戊型肝炎(viralhepatitisE)发生,吉林、辽宁、河北、山东、内蒙古、新疆和北京曾有暴发流行,流行类型为食物型或水源型。
[ { "id": 0, "entity": "戊型肝炎", "start_offset": 7, "end_offset": 11, "label": "dis" }, { "id": 1, "entity": "viralhepatitisE", "start_offset": 12, "end_offset": 27, "label": "dis" } ]
【发病机制和病理改变】HEV主要侵犯肝脏,通过直接致病作用和(或)免疫性损伤引起肝细胞炎症和坏死。
[ { "id": 0, "entity": "HEV", "start_offset": 11, "end_offset": 14, "label": "mic" }, { "id": 1, "entity": "肝脏", "start_offset": 18, "end_offset": 20, "label": "bod" }, { "id": 2, "entity": "免疫性损伤", "start_offset": 33, "end_offset": 38, "label": "dis...
肝脏病理改变有肝细胞变性,灶性坏死,汇管区淋巴细胞、单核巨噬细胞和NK细胞浸润。
[ { "id": 0, "entity": "肝脏", "start_offset": 0, "end_offset": 2, "label": "bod" }, { "id": 1, "entity": "肝细胞", "start_offset": 7, "end_offset": 10, "label": "bod" }, { "id": 2, "entity": "肝细胞变性", "start_offset": 7, "end_offset": 12, "label": "sym" ...
急性黄疸型患者半数以上可见淤胆和胆栓形成。
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临床表现与甲肝类似,不发展成慢性。
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儿童多为亚临床型感染。
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常见临床类型有:(一)急性黄疸型占显性感染的86.5%。
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临床三期经历同甲肝,前驱期症状可持续到黄疸出现后第4~5天。
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淤胆较为常见。
[ { "id": 0, "entity": "淤胆", "start_offset": 0, "end_offset": 2, "label": "sym" } ]
(二)急性无黄疸型表现与甲肝类似。
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(三)淤胆型较为常见,病程可长达2个月以上。
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高危因素包括:①妊娠妇女;②年老体弱者;③合并HBV感染。
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(五)与其他病毒混合感染1.HEV与HAV同时或先后感染者并不加重病情。
[ { "id": 0, "entity": "病毒混合感染", "start_offset": 6, "end_offset": 12, "label": "dis" }, { "id": 1, "entity": "HEV与HAV同时或先后感染", "start_offset": 14, "end_offset": 28, "label": "dis" } ]
2.HEV与HBV重叠感染者患者HBV常有活动性复制,HEV不易被清除。
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病情易迁延或反复发作。
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病情重,发生重型者多,印度报告80.7%的急性重型和75.5%的亚急性重型为HBsAg携带者重叠HEV感染引起。
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【病原学诊断】(一)病毒颗粒和抗原检查在潜伏期末至急性早期取粪便用免疫电镜检出病毒颗粒或用酶免疫法检测病毒抗原。
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(二)血清学检查急性期特异性IgM阳性有临床诊断价值。
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发病后2~3周,特异性IgG检出率为72.7%,4~8周时为84.9%。
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采用完整的ORF2蛋白作抗原建立的EIA法具有较高的灵敏度和特异性。
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(三)病毒基因检查用RT-PCR法可在血清和粪便中检测HEVRNA。
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【预防和治疗】(一)预防主要是保护水源、加强食品卫生管理、注意个人卫生和改善环境卫生。
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人丙种球蛋白对本病无明显预防作用。
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基因重组疫苗和核酸疫苗正在研究之中。
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(二)治疗尚无特异性抗病毒药物。
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综合对症措施同甲型肝炎。
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二、病理生理(一)低氧血症及其对机体的影响1.氧摄取困难当通气不足或通气中氧含量太低时,会出现机体氧摄取不足。
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氧分压降低时,刺激颈动脉体和主动脉弓的化学感受器,通过兴奋呼吸中枢,增强呼吸活动。
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慢性缺氧对刺激呼吸的影响,则主要通过促红细胞生成素调节机制,使红细胞生成增加,提高携带氧功能,以保证组织脏器供氧。
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全肺各部分通气-灌流比例实际上并不一致,只是理论上每一部分肺泡保持此比例,才能保持和发挥肺脏的最大换气效率。
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如果肺泡通气量显著大于灌流,或肺灌流量显著减少,此部分通气-灌流比例显著大于1.0,则该部分肺泡不能保证血液氧和二氧化碳的交换,通气无效,无效腔通气量增加。
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如果通气量显著减少,此时肺内通气-灌流比例低于0.8,没有获得气体交换的血液经肺泡毛细血管流入肺静脉,出现静动脉分流。
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3.对脏器功能的影响小儿体内氧储存量较少,以10kg体重小儿为例,肺泡功能残气中氧含量50~60ml,血液中氧与血红蛋白的结合量约180ml,总计约240ml。
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按动静脉氧含量差为33%(相当于SaO<sub>2</sub>由90%下降到60%),可以提供基础代谢所需耗氧60~80ml/min。
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体内储氧量仅够维持数分钟,且PaO<sub>2</sub><4kPa时,大脑皮层出现不可复原的损伤。
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从有氧代谢转化为无氧代谢,能量转化效率显著降低,产生大量乳酸,可以引起代谢性酸中毒等代谢紊乱和脏器系统功能失调。
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(1)肺:持续处于低氧状态可以使肺小动脉痉挛,产生肺动脉高压和肺水肿,可以导致严重的肺通气-灌流失调。
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(2)心血管:缺氧通过交感神经兴奋使心率加快、血压升高、心输出量增加。
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(3)中枢神经:随缺氧程度逐渐加重,可以出现脑细胞水肿,血-脑屏障通透性增加,脑血管扩张,脑血流增加,最终导致脑水肿和颅内高压,出现中枢性呼吸衰竭的症状。
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(4)肾脏:缺氧非常容易导致肾脏血管痉挛,肾血流显著下降,滤过减少,出现少尿和无尿。
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肾素-血管紧张素-醛固酮系统对血管张力、水、盐、电解质代谢的调节作用,亦随全身性低氧状况而丧失,进一步加重临床症状。
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(5)胃肠道和肝脏:缺氧导致的循环障碍使胃肠道淤血,引起出血、坏死性小肠结肠炎,肝脏出现小叶中心坏死,功能受损失去对体内代谢产物的加工处理。
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(6)造血系统:低氧可以增加促红细胞生成素(erythropoietin,EPO),刺激骨髓红细胞生成增加。
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