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直流电复律可使房扑即刻转为窦性,在许多场合下是首选的方法。
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先天性心脏病患儿发生慢性房扑时,血栓栓塞与中风的机会增加,因此在电复律前应用抗凝剂。
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洋地黄通过延长房室结传导时间来减慢心室率。
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洋地黄化后通常需给予Ⅰ类抗心律失常药如奎尼丁、普鲁卡因胺来维持疗效。
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第五节乙型肝炎病毒相关肾炎乙型肝炎病毒相关肾炎(hepatitisBvirusassociatedglomerulonephritis,HBV-GN)是指继发于乙型肝炎病毒感染的肾小球肾炎。
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【病因和发病机制】HBV是直径为42~45nm的球形颗粒(Dane颗粒),为DNA病毒,由双层外壳及内核组成,内含双股DNA及DNA多聚酶,其中一条负链为长链,约3.2kb,另一条正链是短链,约2.8kb,长链DNA上有4个阅读框架,分别编码HBsAg、HBcAg、HBeAg、DNA多聚酶和X蛋白。
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乙型肝炎病毒相关性肾炎中沉积于肾小球毛细血管壁的主要是HBsAg和HBeAg。
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Ozawa和Hattor已分别从HBV-GN病人肾组织中洗脱并找到抗HBsAg抗体和抗HBeAg抗体;免疫电镜显示上述HBV抗原与免疫球蛋白是沉积在肾小球同一位点上的,这些结果均支持HBV-GN是由HBV抗原成分引起的一种免疫复合物性肾炎。
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目前有人认为其肾小球基底膜上皮下免疫复合物为原位形成。
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动物实验提示能穿过肾小球基底膜定位于上皮下的抗原多肽分子量一般小于300~500kD。
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HBeAg分子量较小,即使结合上IgG也不超过300kD,且带正电荷(PI4.3~4.8),符合引起膜性肾病的条件。
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HBsAg为3.7MD以上,PI4.0左右,HbcAg8MD以上,PI3.7~4.0,不仅分子量过大,且带阴电荷,因此不太可能穿透基底膜在上皮下形成原位复合物,而有可能沉积在系膜区而致病。
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尽管如此,临床上仍见大多数HBV-MN患儿肾小球上皮下有HBsAg沉积,因此,有人认为此时沉积在上皮下的HBsAg并非完整的分子,而是代谢后产生的含抗原决定簇的多肽亚单位,其分子量小,也能穿过基底膜并原位植入,最终导致膜性肾病的发生。
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此外,还有人认为HBV感染后诱发自身抗体而导致HBV-GN。
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由于HBV可直接感染肾组织,因此,HBV直接感染肾脏致病也存在可能。
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【病理】亚洲小儿肾脏病研究会(Asianstudyofrenaldiseaseinchildren,ASRSC)报告儿童HBV-GN的66.1%为膜性肾病,16.1%为轻微病变,8.1%为膜增殖性肾炎。
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其组织学改变与典型的膜性肾病有所区别:①往往伴有轻中度的系膜细胞增生且增生的系膜有插入,但多限于旁系膜区,很少伸及远端毛细血管内皮下;②基底膜及系膜区沉积的免疫球蛋白更多,使得免疫荧光镜下呈现粗颗粒甚至团块状,而非原发性膜性肾病的细颗粒样外观免疫荧光或酶标检查可发现HBeAg和/或HBsAg在肾小球内沉积,这也是诊断HBV-GN的必备条件。
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多隐匿起病,往往偶然查尿时才发现异常。
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2.水肿多不明显,且无明显尿少,但也有少数患儿呈明显凹陷性水肿并伴有腹水。
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3.血尿几乎均有镜下血尿,并持续存在,往往蛋白尿阴转后镜下血尿仍可持续一段时间。
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部分病人在此基础上出现发作性肉眼血尿。
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4.蛋白尿均有不同程度的蛋白尿,蛋白尿表现出较大的波动性,时轻时重,ASRSC报道约61.3%表现为肾病综合征,但对肾上腺皮质激素治疗一般无反应。
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5.高血压多不明显,主要见于病变为膜增生性肾炎者。
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6.肾功能不全少见。
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7.肝脏症状多不明显。
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约一半患儿有肝大或肝功异常,表现为转氨酶升高,但黄疸者少见。
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【实验室检查】1.尿液可出现血尿及蛋白尿、管型尿,尿蛋白主要为白蛋白。
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2.血生化往往有白蛋白下降,胆固醇增高,谷丙转氨酶及谷草转氨酶可升高或正常,血浆蛋白电泳α2</sub>及β球蛋白升高,γ球蛋白则往往正常。
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3.乙肝血清学标记和HBV-DNA大多数病人为乙肝大三阳(HBsAg、HBeAg及HBcAb阳性),少数病人为小三阳(HBsAg、HBeAb及HBcAb阳性),单纯HBsAg阳性者极少。
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血中HBV-DNA一般阳性。
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4.免疫学检查有人认为血IgG和IgA增高,但也有报道50多例HBV-GN膜性肾病患儿均无IgG和IgA增高,相反,约1/3表现为血IgG降低。
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5.肾活体组织检查肾活体组织检查是确定HBV-GN的最终手段,是诊断HBV-GN的必备条件。
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【诊断】诊断参考1989年10月在北京召开的乙型肝炎(简称乙肝)病毒相关肾炎专题座谈会上对本病诊断的意见。
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诊断条件:1.血清乙肝病毒标志物阳性;2.患肾小球肾炎并可除外狼疮性肾炎等继发性肾小球疾病;3.肾组织切片中找到乙肝病毒(HBV)抗原或HBV-DNA;4.肾组织病理改变为膜性肾炎。
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值得说明的是:①符合第1、2、3条即可确诊,不论其肾组织病理改变如何;②符合诊断条件中的第1、2条且肾组织病理确诊为膜性肾炎时,尽管其肾组织切片中未查到HBV抗原或HBV-DNA,可作为拟诊;③我国为HBV感染高发地区,如肾小球疾病患者同时有HBV抗原血症,尚不足以作为HBV-GN相关肾炎的依据。
[ { "id": 0, "entity": "肾组织", "start_offset": 25, "end_offset": 28, "label": "bod" }, { "id": 1, "entity": "肾组织", "start_offset": 50, "end_offset": 53, "label": "bod" }, { "id": 2, "entity": "膜性肾炎", "start_offset": 58, "end_offset": 62, "label": "dis" }, { "id": 3, "entity": "肾组织切片", "start_offset": 67, "end_offset": 72, "label": "pro" }, { "id": 4, "entity": "HBV", "start_offset": 76, "end_offset": 79, "label": "mic" }, { "id": 5, "entity": "HBV", "start_offset": 100, "end_offset": 103, "label": "mic" }, { "id": 6, "entity": "肾小球疾病", "start_offset": 111, "end_offset": 116, "label": "dis" }, { "id": 7, "entity": "HBV抗原血症", "start_offset": 121, "end_offset": 128, "label": "dis" }, { "id": 8, "entity": "HBV-GN", "start_offset": 135, "end_offset": 141, "label": "dis" }, { "id": 9, "entity": "肾炎", "start_offset": 143, "end_offset": 145, "label": "dis" } ]
【治疗】(一)一般治疗包括低盐饮食、适量优质蛋白饮食;水肿明显时应利尿,可参考本章第三节给予各种口服利尿剂,严重水肿时可静脉应用呋塞米(furosemide,速尿)1~2mg/(kg•次);有高血压时应予硝苯地平(nifedipine,心痛定)每次0.25~0.5mg/kg一日3~4次或ACEI类药物口服治疗,如卡托普利(captopril,巯甲丙脯酸)1~2mg/(kg•d),一日2~3次。
[ { "id": 0, "entity": "一般治疗", "start_offset": 7, "end_offset": 11, "label": "pro" }, { "id": 1, "entity": "蛋白", "start_offset": 22, "end_offset": 24, "label": "bod" }, { "id": 2, "entity": "水肿", "start_offset": 27, "end_offset": 29, "label": "sym" }, { "id": 3, "entity": "利尿", "start_offset": 33, "end_offset": 35, "label": "pro" }, { "id": 4, "entity": "利尿剂", "start_offset": 50, "end_offset": 53, "label": "dru" }, { "id": 5, "entity": "水肿", "start_offset": 56, "end_offset": 58, "label": "sym" }, { "id": 6, "entity": "静脉应用呋塞米", "start_offset": 60, "end_offset": 67, "label": "pro" }, { "id": 7, "entity": "furosemide", "start_offset": 68, "end_offset": 78, "label": "dru" }, { "id": 8, "entity": "速尿", "start_offset": 79, "end_offset": 81, "label": "dru" }, { "id": 9, "entity": "高血压", "start_offset": 96, "end_offset": 99, "label": "sym" }, { "id": 10, "entity": "硝苯地平", "start_offset": 102, "end_offset": 106, "label": "dru" }, { "id": 11, "entity": "nifedipine", "start_offset": 107, "end_offset": 117, "label": "dru" }, { "id": 12, "entity": "心痛定", "start_offset": 118, "end_offset": 121, "label": "dru" }, { "id": 13, "entity": "ACEI类药物", "start_offset": 144, "end_offset": 151, "label": "dru" }, { "id": 14, "entity": "卡托普利", "start_offset": 157, "end_offset": 161, "label": "dru" }, { "id": 15, "entity": "captopril", "start_offset": 162, "end_offset": 171, "label": "dru" }, { "id": 16, "entity": "巯甲丙脯酸", "start_offset": 172, "end_offset": 177, "label": "dru" } ]
(二)肾上腺皮质激素与免疫抑制治疗肾上腺皮质激素治疗一直存在争议。
[ { "id": 0, "entity": "肾上腺皮质激素", "start_offset": 3, "end_offset": 10, "label": "dru" }, { "id": 1, "entity": "免疫抑制治疗", "start_offset": 11, "end_offset": 17, "label": "pro" }, { "id": 2, "entity": "肾上腺皮质激素治疗", "start_offset": 17, "end_offset": 26, "label": "pro" } ]
对轻至中度蛋白尿的患儿应尽量避免使用肾上腺皮质激素及免疫抑制剂,以免加重病毒复制。
[ { "id": 0, "entity": "蛋白尿", "start_offset": 5, "end_offset": 8, "label": "sym" }, { "id": 1, "entity": "肾上腺皮质激素", "start_offset": 18, "end_offset": 25, "label": "dru" }, { "id": 2, "entity": "免疫抑制剂", "start_offset": 26, "end_offset": 31, "label": "dru" } ]
免疫抑制剂更不宜使用。
[ { "id": 0, "entity": "免疫抑制剂", "start_offset": 0, "end_offset": 5, "label": "dru" } ]
(三)抗病毒治疗主要有α-干扰素(α-interferon)、阿糖腺苷(Ara-A)和拉米呋定(lamivudine)等。
[ { "id": 0, "entity": "抗病毒治疗", "start_offset": 3, "end_offset": 8, "label": "pro" }, { "id": 1, "entity": "α-干扰素", "start_offset": 11, "end_offset": 16, "label": "dru" }, { "id": 2, "entity": "α-interferon", "start_offset": 17, "end_offset": 29, "label": "dru" }, { "id": 3, "entity": "阿糖腺苷", "start_offset": 31, "end_offset": 35, "label": "dru" }, { "id": 4, "entity": "Ara-A", "start_offset": 36, "end_offset": 41, "label": "dru" }, { "id": 5, "entity": "拉米呋定", "start_offset": 43, "end_offset": 47, "label": "dru" }, { "id": 6, "entity": "lamivudine", "start_offset": 48, "end_offset": 58, "label": "dru" } ]
目前应用α-干扰素治疗HBV-GN取得明显效果,且远期作用尤佳。
[ { "id": 0, "entity": "α-干扰素治疗", "start_offset": 4, "end_offset": 11, "label": "pro" }, { "id": 1, "entity": "HBV-GN", "start_offset": 11, "end_offset": 17, "label": "dis" } ]
剂量为α-干扰素20万U/(kg•次),隔日肌注或皮下注射一次,疗程不少于半年,几乎均能收到较好疗效。
[ { "id": 0, "entity": "肌注", "start_offset": 22, "end_offset": 24, "label": "pro" }, { "id": 1, "entity": "皮下注射", "start_offset": 25, "end_offset": 29, "label": "pro" } ]
往往在用药4个月左右能促使HBeAg阴转,少数患儿在治疗10个月左右还能使HBsAg阴转,蛋白尿均能明显阴转或明显减轻。
[ { "id": 0, "entity": "蛋白尿", "start_offset": 45, "end_offset": 48, "label": "sym" } ]
注射初期可出现发热及流感样症状,几天后即消失。
[ { "id": 0, "entity": "发热", "start_offset": 7, "end_offset": 9, "label": "sym" }, { "id": 1, "entity": "流感样症状", "start_offset": 10, "end_offset": 15, "label": "sym" } ]
阿糖腺苷(Ara-A)也系抗病毒药物,台湾林氏首先用于治疗HBV-GN,取得一定疗效,剂量为15mg/(kg•d),缓慢静脉滴注,2周为一疗程。
[ { "id": 0, "entity": "阿糖腺苷", "start_offset": 0, "end_offset": 4, "label": "dru" }, { "id": 1, "entity": "Ara-A", "start_offset": 5, "end_offset": 10, "label": "dru" }, { "id": 2, "entity": "HBV-GN", "start_offset": 29, "end_offset": 35, "label": "dis" }, { "id": 3, "entity": "静脉滴注", "start_offset": 60, "end_offset": 64, "label": "pro" } ]
还可并用胸腺肽以增加疗效,剂量为0.2~0.5mg/(kg•d),1次/日,疗程半年。
[ { "id": 0, "entity": "胸腺肽", "start_offset": 4, "end_offset": 7, "label": "dru" } ]
Ara-A不良反应大,疗效不及α-干扰素,因此现已被α-INF取代。
[ { "id": 0, "entity": "Ara-A", "start_offset": 0, "end_offset": 5, "label": "dru" }, { "id": 1, "entity": "α-干扰素", "start_offset": 15, "end_offset": 20, "label": "dru" }, { "id": 2, "entity": "α-INF", "start_offset": 26, "end_offset": 31, "label": "dru" } ]
拉米呋定(lamivudine)为新的核酸类抗病毒药,年长儿童每日100mg口服,疗程半年以上,对乙肝疗效与α-干扰素接近,用于治疗HBV-GN较少,疗效有待更多资料确定。
[ { "id": 0, "entity": "拉米呋定", "start_offset": 0, "end_offset": 4, "label": "dru" }, { "id": 1, "entity": "lamivudine", "start_offset": 5, "end_offset": 15, "label": "dru" }, { "id": 2, "entity": "核酸类抗病毒药", "start_offset": 19, "end_offset": 26, "label": "dru" }, { "id": 3, "entity": "乙肝", "start_offset": 49, "end_offset": 51, "label": "dis" }, { "id": 4, "entity": "α-干扰素", "start_offset": 54, "end_offset": 59, "label": "dru" }, { "id": 5, "entity": "HBV-GN", "start_offset": 66, "end_offset": 72, "label": "dis" } ]
(四)抗血小板聚集药双嘧达莫(dipyridamole,潘生丁,persantin)5~8mg/(kg•d),分3次口服。
[ { "id": 0, "entity": "抗血小板聚集药", "start_offset": 3, "end_offset": 10, "label": "dru" }, { "id": 1, "entity": "双嘧达莫", "start_offset": 10, "end_offset": 14, "label": "dru" }, { "id": 2, "entity": "dipyridamole", "start_offset": 15, "end_offset": 27, "label": "dru" }, { "id": 3, "entity": "潘生丁", "start_offset": 28, "end_offset": 31, "label": "dru" }, { "id": 4, "entity": "persantin", "start_offset": 32, "end_offset": 41, "label": "dru" } ]
(五)血管紧张素转换酶抑制剂(ACEI)可选用卡托普利(captopril)0.5~1mg/(kg•次),2~3次/日,或依那普利2.5~5mg/次,2~3次/日、西拉普利5~10mg/日,每日1次、福辛普利5~10mg/日,每日1次、贝那普利5mg/d,每日1次等,对降低蛋白尿及保护肾脏有一定效果。
[ { "id": 0, "entity": "血管紧张素转换酶抑制剂", "start_offset": 3, "end_offset": 14, "label": "dru" }, { "id": 1, "entity": "ACEI", "start_offset": 15, "end_offset": 19, "label": "dru" }, { "id": 2, "entity": "卡托普利", "start_offset": 23, "end_offset": 27, "label": "dru" }, { "id": 3, "entity": "captopril", "start_offset": 28, "end_offset": 37, "label": "dru" }, { "id": 4, "entity": "依那普利", "start_offset": 61, "end_offset": 65, "label": "dru" }, { "id": 5, "entity": "西拉普利", "start_offset": 82, "end_offset": 86, "label": "dru" }, { "id": 6, "entity": "福辛普利", "start_offset": 100, "end_offset": 104, "label": "dru" }, { "id": 7, "entity": "贝那普利", "start_offset": 118, "end_offset": 122, "label": "dru" }, { "id": 8, "entity": "蛋白尿", "start_offset": 137, "end_offset": 140, "label": "sym" }, { "id": 9, "entity": "肾脏", "start_offset": 143, "end_offset": 145, "label": "bod" } ]
(六)中医中药中药在护肝治疗及抑制HBV增殖上有一定效果。
[ { "id": 0, "entity": "中药", "start_offset": 5, "end_offset": 7, "label": "dru" }, { "id": 1, "entity": "中药", "start_offset": 7, "end_offset": 9, "label": "dru" }, { "id": 2, "entity": "护肝治疗", "start_offset": 10, "end_offset": 14, "label": "pro" }, { "id": 3, "entity": "HBV", "start_offset": 17, "end_offset": 20, "label": "mic" } ]
作者曾试用三普乙肝健,A、B片各2~3片/次,1日3次,治疗3~6个月,收效尚满意;此外,亦可选用乙肝宁及乙肝解毒胶囊长期口服。
[ { "id": 0, "entity": "三普乙肝健", "start_offset": 5, "end_offset": 10, "label": "dru" }, { "id": 1, "entity": "乙肝宁", "start_offset": 49, "end_offset": 52, "label": "dru" }, { "id": 2, "entity": "乙肝解毒胶囊", "start_offset": 53, "end_offset": 59, "label": "dru" } ]
第七节新生儿肺炎新生儿肺炎(neonatalpneumonia)一般指感染性肺炎,可发生于宫内、出生时或出生后。
[ { "id": 0, "entity": "新生儿肺炎", "start_offset": 3, "end_offset": 8, "label": "dis" }, { "id": 1, "entity": "新生儿肺炎", "start_offset": 8, "end_offset": 13, "label": "dis" }, { "id": 2, "entity": "neonatalpneumonia", "start_offset": 14, "end_offset": 31, "label": "dis" }, { "id": 3, "entity": "感染性肺炎", "start_offset": 35, "end_offset": 40, "label": "dis" } ]
一、宫内与出生时感染性肺炎【临床流行病学】(一)发病率宫内感染性肺炎占活产新生儿的0.5%。
[ { "id": 0, "entity": "感染性肺炎", "start_offset": 8, "end_offset": 13, "label": "dis" }, { "id": 1, "entity": "宫内感染性肺炎", "start_offset": 27, "end_offset": 34, "label": "dis" } ]
(二)病原学在美国,以B族溶血性链球菌(GBS)为主要致病菌,孕妇阴道GBS的带菌率为20%~50%。
[ { "id": 0, "entity": "B族溶血性链球菌", "start_offset": 11, "end_offset": 19, "label": "mic" }, { "id": 1, "entity": "GBS", "start_offset": 20, "end_offset": 23, "label": "mic" }, { "id": 2, "entity": "阴道", "start_offset": 33, "end_offset": 35, "label": "bod" }, { "id": 3, "entity": "GBS", "start_offset": 35, "end_offset": 38, "label": "mic" } ]
国内最近北京市妇产医院调查了1037名孕妇,其GBS带菌率为11.07%,新生儿的GBS带菌率为9.95%,然而,新生儿GBS带菌者的肺炎发生率(20%)与非GBS带菌者的肺炎发生率(14.92%)相比无统计学差异。
[ { "id": 0, "entity": "GBS", "start_offset": 23, "end_offset": 26, "label": "mic" }, { "id": 1, "entity": "GBS", "start_offset": 60, "end_offset": 63, "label": "mic" }, { "id": 2, "entity": "肺炎", "start_offset": 67, "end_offset": 69, "label": "dis" }, { "id": 3, "entity": "GBS", "start_offset": 79, "end_offset": 82, "label": "mic" }, { "id": 4, "entity": "肺炎", "start_offset": 86, "end_offset": 88, "label": "dis" } ]
【病因机制和病理】宫内感染的途径有:(一)血行传播途径母孕期受病毒、细菌、原虫、衣原体和支原体等感染,病原体经血行通过胎盘和羊膜侵袭胎儿。
[ { "id": 0, "entity": "血行", "start_offset": 21, "end_offset": 23, "label": "bod" }, { "id": 1, "entity": "病毒", "start_offset": 31, "end_offset": 33, "label": "mic" }, { "id": 2, "entity": "细菌", "start_offset": 34, "end_offset": 36, "label": "mic" }, { "id": 3, "entity": "原虫", "start_offset": 37, "end_offset": 39, "label": "mic" }, { "id": 4, "entity": "衣原体", "start_offset": 40, "end_offset": 43, "label": "mic" }, { "id": 5, "entity": "支原体", "start_offset": 44, "end_offset": 47, "label": "mic" }, { "id": 6, "entity": "血行", "start_offset": 55, "end_offset": 57, "label": "bod" }, { "id": 7, "entity": "胎盘", "start_offset": 59, "end_offset": 61, "label": "bod" }, { "id": 8, "entity": "羊膜", "start_offset": 62, "end_offset": 64, "label": "bod" } ]
(二)通过羊水感染羊膜早破24小时以上或羊膜绒毛膜炎时,产道内细菌上行性感染,或胎儿在宫内、出生时吸入污染羊水而致病。
[ { "id": 0, "entity": "羊水", "start_offset": 5, "end_offset": 7, "label": "bod" }, { "id": 1, "entity": "羊膜", "start_offset": 9, "end_offset": 11, "label": "bod" }, { "id": 2, "entity": "细菌", "start_offset": 31, "end_offset": 33, "label": "bod" }, { "id": 3, "entity": "羊水", "start_offset": 53, "end_offset": 55, "label": "bod" } ]
宫内、出生时感染性肺炎的病理改变广泛,肺泡渗出液中含多核细胞、单核细胞和少量红细胞。
[ { "id": 0, "entity": "感染性肺炎", "start_offset": 6, "end_offset": 11, "label": "dis" }, { "id": 1, "entity": "肺泡渗出液", "start_offset": 19, "end_offset": 24, "label": "bod" }, { "id": 2, "entity": "多核细胞", "start_offset": 26, "end_offset": 30, "label": "bod" }, { "id": 3, "entity": "单核细胞", "start_offset": 31, "end_offset": 35, "label": "bod" }, { "id": 4, "entity": "红细胞", "start_offset": 38, "end_offset": 41, "label": "bod" } ]
镜检可见到羊水沉渣,如角化上皮细胞、胎儿皮脂和病原体等。
[ { "id": 0, "entity": "镜检", "start_offset": 0, "end_offset": 2, "label": "pro" }, { "id": 1, "entity": "羊水", "start_offset": 5, "end_offset": 7, "label": "bod" }, { "id": 2, "entity": "上皮细胞", "start_offset": 13, "end_offset": 17, "label": "bod" }, { "id": 3, "entity": "皮脂", "start_offset": 20, "end_offset": 22, "label": "bod" }, { "id": 4, "entity": "病原体", "start_offset": 23, "end_offset": 26, "label": "mic" } ]
【临床表现】(一)宫内感染多在娩出后24小时内发病,婴儿出生时多有窒息,复苏后可见呼吸快、呻吟、体温不稳定、反应差,逐渐出现啰音等表现。
[ { "id": 0, "entity": "宫内", "start_offset": 9, "end_offset": 11, "label": "bod" }, { "id": 1, "entity": "宫内感染", "start_offset": 9, "end_offset": 13, "label": "sym" }, { "id": 2, "entity": "窒息", "start_offset": 33, "end_offset": 35, "label": "dis" }, { "id": 3, "entity": "呼吸快", "start_offset": 41, "end_offset": 44, "label": "sym" }, { "id": 4, "entity": "呻吟", "start_offset": 45, "end_offset": 47, "label": "sym" }, { "id": 5, "entity": "体温不稳定", "start_offset": 48, "end_offset": 53, "label": "sym" }, { "id": 6, "entity": "反应差", "start_offset": 54, "end_offset": 57, "label": "sym" }, { "id": 7, "entity": "啰音", "start_offset": 62, "end_offset": 64, "label": "sym" } ]
严重病例可出现呼吸衰竭。
[ { "id": 0, "entity": "呼吸衰竭", "start_offset": 7, "end_offset": 11, "label": "sym" } ]
血行感染者常缺乏肺部体征,而以黄疸、肝脾大、脑膜炎等多系统受累为主。
[ { "id": 0, "entity": "肺部", "start_offset": 8, "end_offset": 10, "label": "bod" }, { "id": 1, "entity": "黄疸", "start_offset": 15, "end_offset": 17, "label": "sym" }, { "id": 2, "entity": "肝脾大", "start_offset": 18, "end_offset": 21, "label": "sym" }, { "id": 3, "entity": "脑膜炎", "start_offset": 22, "end_offset": 25, "label": "dis" } ]
通过羊水感染者,常有明显的呼吸困难和肺部啰音(二)出生时感染出生时获得的感染需经过数天至数周潜伏期后始发病,如细菌性肺炎常在出生后3~5小时发病,疱疹病毒感染多在分娩后5~10天出现症状,而衣原体感染潜伏期则长达3~12周。
[ { "id": 0, "entity": "羊水", "start_offset": 2, "end_offset": 4, "label": "bod" }, { "id": 1, "entity": "呼吸困难", "start_offset": 13, "end_offset": 17, "label": "sym" }, { "id": 2, "entity": "肺部", "start_offset": 18, "end_offset": 20, "label": "bod" }, { "id": 3, "entity": "肺部啰音", "start_offset": 18, "end_offset": 22, "label": "sym" }, { "id": 4, "entity": "细菌性肺炎", "start_offset": 55, "end_offset": 60, "label": "dis" }, { "id": 5, "entity": "疱疹病毒感染", "start_offset": 73, "end_offset": 79, "label": "dis" }, { "id": 6, "entity": "衣原体感染", "start_offset": 95, "end_offset": 100, "label": "dis" } ]
出生时感染的肺炎,患儿因病原不同而临床表现差别较大,且容易发生全身感染。
[ { "id": 0, "entity": "肺炎", "start_offset": 6, "end_offset": 8, "label": "dis" } ]
宫内感染者,X线胸片常显示间质性肺炎改变;通过羊水感染者,X线胸片多显示支气管肺炎改变。
[ { "id": 0, "entity": "宫内", "start_offset": 0, "end_offset": 2, "label": "bod" }, { "id": 1, "entity": "X线胸片", "start_offset": 6, "end_offset": 10, "label": "pro" }, { "id": 2, "entity": "间质性肺炎", "start_offset": 13, "end_offset": 18, "label": "dis" }, { "id": 3, "entity": "羊水", "start_offset": 23, "end_offset": 25, "label": "bod" }, { "id": 4, "entity": "X线胸片", "start_offset": 29, "end_offset": 33, "label": "pro" }, { "id": 5, "entity": "支气管肺炎", "start_offset": 36, "end_offset": 41, "label": "dis" } ]
【治疗】对羊膜早破的孕妇在分娩期可用抗生素预防胎儿感染。
[ { "id": 0, "entity": "羊膜", "start_offset": 5, "end_offset": 7, "label": "bod" }, { "id": 1, "entity": "抗生素", "start_offset": 18, "end_offset": 21, "label": "dru" } ]
携带GBS的孕妇在分娩期可用青霉素或氨苄西林预防用药。
[ { "id": 0, "entity": "GBS", "start_offset": 2, "end_offset": 5, "label": "mic" }, { "id": 1, "entity": "青霉素", "start_offset": 14, "end_offset": 17, "label": "dru" }, { "id": 2, "entity": "氨苄西林", "start_offset": 18, "end_offset": 22, "label": "dru" } ]
呼吸困难者给氧或采用机械呼吸,加强营养,维持水、电解质和酸碱平衡。
[ { "id": 0, "entity": "呼吸困难", "start_offset": 0, "end_offset": 4, "label": "sym" }, { "id": 1, "entity": "给氧", "start_offset": 5, "end_offset": 7, "label": "pro" }, { "id": 2, "entity": "机械呼吸", "start_offset": 10, "end_offset": 14, "label": "pro" }, { "id": 3, "entity": "加强营养", "start_offset": 15, "end_offset": 19, "label": "pro" }, { "id": 4, "entity": "维持水、电解质和酸碱平衡", "start_offset": 20, "end_offset": 32, "label": "pro" } ]
第四节先天性肾盂输尿管连接部梗阻先天性肾盂输尿管连接部梗阻(congenitalureteropelvicjunctionobstruction,UPJO)是最为常见的小儿泌尿系畸形之一,可导致肾盂积水。
[ { "id": 0, "entity": "先天性肾盂输尿管连接部梗阻", "start_offset": 3, "end_offset": 16, "label": "dis" }, { "id": 1, "entity": "先天性肾盂输尿管连接部梗阻", "start_offset": 16, "end_offset": 29, "label": "dis" }, { "id": 2, "entity": "congenitalureteropelvicjunctionobstruction", "start_offset": 30, "end_offset": 72, "label": "dis" }, { "id": 3, "entity": "UPJO", "start_offset": 73, "end_offset": 77, "label": "dis" }, { "id": 4, "entity": "小儿泌尿系畸形", "start_offset": 84, "end_offset": 91, "label": "dis" }, { "id": 5, "entity": "肾盂积水", "start_offset": 97, "end_offset": 101, "label": "dis" } ]
肾盂输尿管连接部梗阻包括三种类型:①内源性梗阻;②外源性梗阻;③继发性梗阻。
[ { "id": 0, "entity": "肾盂输尿管连接部梗阻", "start_offset": 0, "end_offset": 10, "label": "dis" }, { "id": 1, "entity": "内源性梗阻", "start_offset": 18, "end_offset": 23, "label": "dis" }, { "id": 2, "entity": "外源性梗阻", "start_offset": 25, "end_offset": 30, "label": "dis" }, { "id": 3, "entity": "继发性梗阻", "start_offset": 32, "end_offset": 37, "label": "dis" } ]
由于连接部的梗阻,使得肾盂内的尿液无法通畅、及时地流入输尿管,导致肾脏集合系统的持续和进行性扩张,其结果进一步破坏肾盂的排空能力。
[ { "id": 0, "entity": "肾盂", "start_offset": 11, "end_offset": 13, "label": "bod" }, { "id": 1, "entity": "尿液", "start_offset": 15, "end_offset": 17, "label": "bod" }, { "id": 2, "entity": "输尿管", "start_offset": 27, "end_offset": 30, "label": "bod" }, { "id": 3, "entity": "肾脏", "start_offset": 33, "end_offset": 35, "label": "bod" }, { "id": 4, "entity": "肾盂", "start_offset": 57, "end_offset": 59, "label": "bod" } ]
【发病情况】肾盂输尿管连接部梗阻可以发生在任何年龄儿童,根据美国的统计资料大概有1/4的病例是发生于1岁以内。
[ { "id": 0, "entity": "肾盂输尿管连接部梗阻", "start_offset": 6, "end_offset": 16, "label": "dis" } ]
从最新的材料来看,随着产前超声的广泛应用,几乎所有的病例都可在围生期得以发现并做出诊断。
[ { "id": 0, "entity": "超声", "start_offset": 13, "end_offset": 15, "label": "pro" } ]
实际上UPJO是胎儿肾脏集合系统扩张最常见的原因,约占其80%,远远超过多囊性肾发育不良的发生率。
[ { "id": 0, "entity": "UPJO", "start_offset": 3, "end_offset": 7, "label": "dis" }, { "id": 1, "entity": "肾脏集合系统扩张", "start_offset": 10, "end_offset": 18, "label": "bod" }, { "id": 2, "entity": "多囊性肾发育不良", "start_offset": 36, "end_offset": 44, "label": "dis" } ]
进入青春期和成人期,其发现的可能性变小,如果发现肾积水,多系供应肾下极皮质的迷走血管下极皮质的迷走血管跨于UPJO之上造成。
[ { "id": 0, "entity": "肾积水", "start_offset": 24, "end_offset": 27, "label": "dis" }, { "id": 1, "entity": "肾下极皮质的迷走血管", "start_offset": 32, "end_offset": 42, "label": "bod" }, { "id": 2, "entity": "UPJO", "start_offset": 53, "end_offset": 57, "label": "dis" } ]
UPJO多发生于男孩,在新生儿期,男女比例可达2∶1;左侧稍多于右侧,新生儿中2/3发生于左侧。
[ { "id": 0, "entity": "UPJO", "start_offset": 0, "end_offset": 4, "label": "dis" } ]
双侧UPJO所占比例约为10%~40%,其多发生于新生儿期或小于6个月的婴幼儿。
[ { "id": 0, "entity": "UPJO", "start_offset": 2, "end_offset": 6, "label": "dis" } ]
已有一家中几代发生UPJO的报道,但遗传倾向尚待进一步证实。
[ { "id": 0, "entity": "UPJO", "start_offset": 9, "end_offset": 13, "label": "dis" } ]
【病因】尽管从胚胎学、组织学和解剖学进行了多方面的研究,UPJO准确的发生原因仍不清楚。
[ { "id": 0, "entity": "UPJO", "start_offset": 28, "end_offset": 32, "label": "dis" } ]
目前多认为系肾盂输尿管连接部处发育停滞或输尿管在胎儿期有一实质化和再腔化的过程,如再腔化不完全,则造成肾盂输尿管连接部的内源性梗阻。
[ { "id": 0, "entity": "肾盂输尿管", "start_offset": 6, "end_offset": 11, "label": "bod" }, { "id": 1, "entity": "输尿管", "start_offset": 20, "end_offset": 23, "label": "bod" }, { "id": 2, "entity": "肾盂输尿管连接部的内源性梗阻", "start_offset": 51, "end_offset": 65, "label": "dis" } ]
(一)内源性梗阻1958年,Murnaghan发现肾盂输尿管连接部处环行肌肉发育停顿现象,其破坏了连接部的漏斗样结构,造成尿液引流不畅,而肾积水可加重漏斗样结构的破坏。
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1968年Nutley,1976年Hanna等在电镜中发现,UPJO处的肌肉细胞排列方向是正常的,但胶原纤维的含量大大超标,由此导致肌纤维间的间距加大,许多肌细胞甚至发生萎缩,使得肾盂输尿管连接部肌肉收缩功能破坏,肾盂内尿液不能排空。
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息肉一般不大,多位于肾盂输尿管连接部或输尿管的上段,可形成不全梗阻。
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输尿管先天性黏膜皱襞尿管先天性黏膜皱襞是输尿管瓣膜尿管瓣膜的一种,在4个月以后胎儿的上段输尿管中是一种非常常见的现象,这种黏膜皱襞甚至可延续至新生儿期。
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一般来说,黏膜皱襞并不形成梗阻,随着生长发育可以消失,在年长的儿童或成人中是很少见的。
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如这种胎儿黏膜皱襞持续存在并不断增厚,再有肌肉组织进入,或在肾盂输尿管连接部形成瓣膜样的结构,则可形成梗阻。
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手术时,包含瓣膜的输尿管部分必须切除。
[ { "id": 0, "entity": "瓣膜的输尿管", "start_offset": 6, "end_offset": 12, "label": "bod" } ]
(二)外源性梗阻外源性梗阻最常见的是支配肾下极皮质的迷走或副支血管压迫肾盂输尿管连接部,这支血管常在肾盂输尿管连接部或输尿管上端的前面穿过。
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迷走血管使输尿管折叠成角,当肾盂充盈时可在肾盂输尿管连接部和血管通过输尿管处两处形成梗阻而被迷走血管牵拉折叠向上的输尿管可以和肾盂之间形成筋膜粘连输尿管长期受压可以导致缺血、纤维化以及狭窄,故尽管有人认为松解粘连及游离血管即可解除UPJO,但还是将病变梗阻的输尿管切除为好。
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(三)继发性梗阻UPJO可由于严重的膀胱输尿管反流引起,占其总数的10%左右。
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反流可引起输尿管的扭曲、增粗及拉长粗及拉长,而因肾盂输尿管连接部的位置相对固定,产生折叠,导致梗阻。
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同样原理UPJO也由于膀胱输尿管连接部梗阻引起。
[ { "id": 0, "entity": "UPJO", "start_offset": 4, "end_offset": 8, "label": "sym" }, { "id": 1, "entity": "膀胱输尿管连接部梗阻", "start_offset": 11, "end_offset": 21, "label": "dis" } ]
【伴发畸形】UPJO患儿伴发对侧肾脏畸形的几率相当高,也包括其他一些先天性畸形。
[ { "id": 0, "entity": "UPJO", "start_offset": 6, "end_offset": 10, "label": "dis" }, { "id": 1, "entity": "对侧肾脏畸形", "start_offset": 14, "end_offset": 20, "label": "dis" }, { "id": 2, "entity": "先天性畸形", "start_offset": 34, "end_offset": 39, "label": "dis" } ]
对侧UPJO的发生率报告为10%~40%,其他可以为肾发育不良及多囊性肾病等。
[ { "id": 0, "entity": "UPJO", "start_offset": 2, "end_offset": 6, "label": "dis" }, { "id": 1, "entity": "肾发育不良及多囊性肾病", "start_offset": 26, "end_offset": 37, "label": "dis" } ]
UPJO也可发生在重复肾的上半肾或下半肾,也可发生于马蹄肾或异位肾。
[ { "id": 0, "entity": "UPJO", "start_offset": 0, "end_offset": 4, "label": "dis" }, { "id": 1, "entity": "重复肾", "start_offset": 9, "end_offset": 12, "label": "dis" }, { "id": 2, "entity": "上半肾", "start_offset": 13, "end_offset": 16, "label": "bod" }, { "id": 3, "entity": "下半肾", "start_offset": 17, "end_offset": 20, "label": "bod" }, { "id": 4, "entity": "马蹄肾", "start_offset": 26, "end_offset": 29, "label": "dis" }, { "id": 5, "entity": "异位肾", "start_offset": 30, "end_offset": 33, "label": "dis" } ]
UPJO患者轻度膀胱输尿管反流的发生率可达40%左右,其更多可能是尿路感染造成,可以自行消失。
[ { "id": 0, "entity": "UPJO", "start_offset": 0, "end_offset": 4, "label": "dis" }, { "id": 1, "entity": "轻度膀胱输尿管反流", "start_offset": 6, "end_offset": 15, "label": "dis" }, { "id": 2, "entity": "尿路感染", "start_offset": 33, "end_offset": 37, "label": "dis" } ]
UPJO在Vater综合征中的发生率为21%。
[ { "id": 0, "entity": "UPJO", "start_offset": 0, "end_offset": 4, "label": "dis" }, { "id": 1, "entity": "Vater综合征", "start_offset": 5, "end_offset": 13, "label": "dis" } ]
【症状】在新生儿或婴幼儿中UPJO导致肾积水可表现为无症状的腹块,在体检中被偶然发现。
[ { "id": 0, "entity": "UPJO", "start_offset": 13, "end_offset": 17, "label": "dis" }, { "id": 1, "entity": "肾积水", "start_offset": 19, "end_offset": 22, "label": "dis" }, { "id": 2, "entity": "无症状的腹块", "start_offset": 26, "end_offset": 32, "label": "sym" }, { "id": 3, "entity": "在体检中被偶然发现", "start_offset": 33, "end_offset": 42, "label": "sym" } ]
随着孕期超声的常规使用,近年来许多UPJO导致肾积水的病例在胎儿期就诊断出来。
[ { "id": 0, "entity": "超声", "start_offset": 4, "end_offset": 6, "label": "pro" }, { "id": 1, "entity": "UPJO", "start_offset": 17, "end_offset": 21, "label": "dis" }, { "id": 2, "entity": "肾积水", "start_offset": 23, "end_offset": 26, "label": "dis" } ]