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【治疗】转移性骨肿瘤的治疗在诊断明确之后,应及时采用综合治疗的方法,原发性肿瘤病变的治疗是整个治疗中的主要环节。
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第九章肾血管性高血压儿童高血压中65%~80%为继发性高血压。
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肾血管性高血压(renovascularhypertension,RVH)即为其中之一。
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肾血管性高血压,主要指肾动脉狭窄,系指单侧或双侧动脉及/或其分支病变使肾脏缺血引起的高血压。
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虽然在高血压中的发病率<5%。
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但是可根治的小儿高血压的病因之一,由于诊断方法、治疗技术、血管显微外科和肾移植的进展,本病的早期诊断和治疗后的效果有很大改观。
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【病因】(一)先天性肾动脉纤维肌肉发育不良是国外报道的小儿和青少年RVH的主要病因,病变多发生于肾动脉的中段或远段。
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常累及其分支,常见有几种:①内膜纤维增生:主要是肾动脉主干的狭窄和变形,血管造影显示中段有局灶性狭窄;②中层纤维增生:呈间断性破坏和增厚,多蔓延至肾动脉中远段,血管造影呈念珠状阴影;③纤维肌肉增生:肾动脉壁呈同心性增厚,肾动脉造影示肾动脉及其分支有光滑的狭窄;④外膜下纤维增生:致肾动脉严重狭窄,动脉造影示不规则狭窄及丰富的侧支循环。
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(二)多发性大动脉炎一种非特异性慢性血管炎症性疾病,是我国成人和小儿发生RVH的主要病因。
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基本病变是动脉中层的弹力纤维组织增生变性和不同程度的小圆形细胞浸润,最终导致血管壁增厚,疤痕形成,血管壁弹性消失,管腔狭窄或动脉瘤样膨出。
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病变常位于肾动脉于腹主动脉的起始部狭窄,引起高血压。
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其他血管病变如溶血尿毒综合征、结节性多动脉炎、Ehlers-Danlos综合征及川崎病等。
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(三)其他肾动脉血栓形成或栓塞,见于外伤或新生儿时期有脐静脉插管史者,肾动脉静脉瘘,肾动脉瘤,移植后肾动脉狭窄及先天性肾动脉异常(肾动脉均匀细小扭曲或狭窄),肾发育不良和成神经纤维瘤病的肾动脉受累,以及其他肾肿瘤和肾囊肿使肾动脉受纤维索带及动脉旁淋巴结压迫等。
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也有根据病变所在的部位分为主要侵犯肾门的肾动脉疾病、肾内肾动脉疾病和肾动脉外的病变。
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【发病机制】在肾血管性高血压中肾素-血管紧张素-醛固酮系统(RAAS)起主要作用。
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第一种双肾单夹,类似于单侧RVH。
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系钳夹肾动脉的一侧使钳夹侧肾动脉血流量减少。
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通过刺激压力和化学感受器致密斑,使肾素分泌增多。
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血管紧张素Ⅱ(ATⅡ)形成增多后,通过①直接使全身小动脉收缩;②刺激醛固酮释放,致水、钠潴留;③ATⅡ能刺激交感神经,使其活力增强,还可刺激肾上腺髓质合成并释放去甲肾上腺素增多,引起高血压。
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但此作用机制可通过对侧肾压力性排尿作用部分代偿,最终不出现钠潴留,其结果是:①缺血侧肾素分泌增多;②对侧正常肾由于血压升高及钠潴留的负反馈作用,使肾素分泌抑制;③缺血肾血流减少;④ATⅡ诱发血管收缩导致高血压。
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ATⅡ受体拮抗剂或血管紧张素转移抑制剂(ACEI)可使ATⅡ作用减弱,血压下降,但抑制了对侧肾素的反馈抑制,肾素及ATⅡ反而增加。
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为钳夹一例肾动脉,而对侧肾被切除,孤立肾与此种模型较为一致。
[ { "id": 0, "entity": "钳夹一例肾动脉", "start_offset": 1, "end_offset": 8, "label": "bod" }, { "id": 1, "entity": "肾", "start_offset": 12, "end_offset": 13, "label": "bod" }, { "id": 2, "entity": "肾", "start_offset": 19, "end_offset": 20, "label": "bod" } ]
这样,压力性利尿排钠作用不再发生,导致钠潴留。
[ { "id": 0, "entity": "压力性利尿排钠", "start_offset": 3, "end_offset": 10, "label": "sym" }, { "id": 1, "entity": "钠潴留", "start_offset": 19, "end_offset": 22, "label": "sym" } ]
同时反馈抑制了肾素分泌,外周血浆肾素水平在急性期后正常或降低。
[ { "id": 0, "entity": "肾素", "start_offset": 7, "end_offset": 9, "label": "bod" }, { "id": 1, "entity": "外周血浆肾素", "start_offset": 12, "end_offset": 18, "label": "bod" }, { "id": 2, "entity": "外周血浆肾素水平在急性期后正常或降低", "start_offset": 12, "end_offset": 30, "label": "sym" } ]
单用ACEI不能防止高血压的发生,若同时利尿排钠,可使血压下降。
[ { "id": 0, "entity": "ACEI", "start_offset": 2, "end_offset": 6, "label": "dru" }, { "id": 1, "entity": "高血压", "start_offset": 10, "end_offset": 13, "label": "dis" }, { "id": 2, "entity": "利尿排钠", "start_offset": 20, "end_offset": 24, "label": "pro" }, { "id": 3, "entity": "血压", "start_offset": 27, "end_offset": 29, "label": "ite" }, { "id": 4, "entity": "血压下降", "start_offset": 27, "end_offset": 31, "label": "sym" } ]
此种RVH可以是肾素依赖型,也可以是容量依赖型。
[ { "id": 0, "entity": "RVH", "start_offset": 2, "end_offset": 5, "label": "dis" } ]
双侧肾动脉狭窄是否与此一致尚有争论。
[ { "id": 0, "entity": "双侧肾动脉", "start_offset": 0, "end_offset": 5, "label": "bod" }, { "id": 1, "entity": "双侧肾动脉狭窄", "start_offset": 0, "end_offset": 7, "label": "sym" } ]
此外激肽释放酶-激肽-前列腺素系统在RVH的发病机制中也有一定作用,激肽由激肽释放酶激活后,促进前列腺素合成。
[ { "id": 0, "entity": "激肽释放酶-激肽-前列腺素系统", "start_offset": 2, "end_offset": 17, "label": "bod" }, { "id": 1, "entity": "RVH", "start_offset": 18, "end_offset": 21, "label": "dis" }, { "id": 2, "entity": "激肽", "start_offset": 34, "end_offset": 36, "label": "bod" }, { "id": 3, "entity": "激肽释放酶", "start_offset": 37, "end_offset": 42, "label": "bod" }, { "id": 4, "entity": "前列腺素", "start_offset": 48, "end_offset": 52, "label": "bod" } ]
两者可使全身小动脉扩张,外周血管阻力降低,肾血管扩张,肾血流量增加,促进了水钠排出。
[ { "id": 0, "entity": "全身小动脉", "start_offset": 4, "end_offset": 9, "label": "bod" }, { "id": 1, "entity": "全身小动脉扩张", "start_offset": 4, "end_offset": 11, "label": "sym" }, { "id": 2, "entity": "外周血管", "start_offset": 12, "end_offset": 16, "label": "bod" }, { "id": 3, "entity": "外周血管阻力降低", "start_offset": 12, "end_offset": 20, "label": "sym" }, { "id": 4, "entity": "肾血管", "start_offset": 21, "end_offset": 24, "label": "bod" }, { "id": 5, "entity": "肾血管扩张", "start_offset": 21, "end_offset": 26, "label": "sym" }, { "id": 6, "entity": "肾血流量增加", "start_offset": 27, "end_offset": 33, "label": "sym" } ]
在Glordblatt动物模型中,尿中激肽释放酶活力降低,以上因素又参与了高血压的发生。
[ { "id": 0, "entity": "尿", "start_offset": 17, "end_offset": 18, "label": "bod" }, { "id": 1, "entity": "激肽释放酶", "start_offset": 19, "end_offset": 24, "label": "bod" }, { "id": 2, "entity": "尿中激肽释放酶活力降低", "start_offset": 17, "end_offset": 28, "label": "sym" }, { "id": 3, "entity": "高血压", "start_offset": 37, "end_offset": 40, "label": "dis" } ]
RVH的发生与肾动脉狭窄发生的速度和时期有一定关系,肾动脉发生栓塞的数分钟内可出现高血压,即急性期,此时多为肾素依赖性高血压,如应用ACEI可使血压迅速有效地下降,数日或数周后进入过渡期,此期的血浆肾素和血管紧张素仍维持较高水平,但钠、水潴留已起作用,ACEI的应用仍可使血压下降,但速度减慢。
[ { "id": 0, "entity": "RVH", "start_offset": 0, "end_offset": 3, "label": "dis" }, { "id": 1, "entity": "肾动脉", "start_offset": 7, "end_offset": 10, "label": "bod" }, { "id": 2, "entity": "肾动脉狭窄", "start_offset": 7, "end_offset": 12, "label": "sym" }, { "id": 3, "entity": "肾动脉", "start_offset": 26, "end_offset": 29, "label": "bod" }, { "id": 4, "entity": "肾动脉发生栓塞", "start_offset": 26, "end_offset": 33, "label": "sym" }, { "id": 5, "entity": "高血压", "start_offset": 41, "end_offset": 44, "label": "dis" }, { "id": 6, "entity": "肾素依赖性高血压", "start_offset": 54, "end_offset": 62, "label": "dis" }, { "id": 7, "entity": "ACEI", "start_offset": 66, "end_offset": 70, "label": "dru" }, { "id": 8, "entity": "血压", "start_offset": 72, "end_offset": 74, "label": "ite" }, { "id": 9, "entity": "血压迅速有效地下降", "start_offset": 72, "end_offset": 81, "label": "sym" }, { "id": 10, "entity": "血浆肾素", "start_offset": 97, "end_offset": 101, "label": "bod" }, { "id": 11, "entity": "血管紧张素", "start_offset": 102, "end_offset": 107, "label": "bod" }, { "id": 12, "entity": "血浆肾素和血管紧张素仍维持较高水平", "start_offset": 97, "end_offset": 114, "label": "sym" }, { "id": 13, "entity": "钠、水潴留", "start_offset": 116, "end_offset": 121, "label": "sym" }, { "id": 14, "entity": "ACEI", "start_offset": 126, "end_offset": 130, "label": "dru" }, { "id": 15, "entity": "血压", "start_offset": 136, "end_offset": 138, "label": "ite" }, { "id": 16, "entity": "血压下降", "start_offset": 136, "end_offset": 140, "label": "sym" } ]
最后是慢性期,水、钠潴留和血容量扩张对肾素的分泌起了抑制作用。
[ { "id": 0, "entity": "水、钠潴留", "start_offset": 7, "end_offset": 12, "label": "sym" }, { "id": 1, "entity": "血容量扩张", "start_offset": 13, "end_offset": 18, "label": "sym" }, { "id": 2, "entity": "肾素", "start_offset": 19, "end_offset": 21, "label": "bod" } ]
【临床表现】(一)症状肾血管性高血压可发生在任何年龄,已有许多婴儿病例报告,最小者仅为7~10天。
[ { "id": 0, "entity": "肾血管性高血压", "start_offset": 11, "end_offset": 18, "label": "dis" } ]
小婴儿可有呕吐、发育营养差、充血性心力衰竭及急性肾衰竭等表现。
[ { "id": 0, "entity": "呕吐", "start_offset": 5, "end_offset": 7, "label": "sym" }, { "id": 1, "entity": "发育营养差", "start_offset": 8, "end_offset": 13, "label": "sym" }, { "id": 2, "entity": "充血性心力衰竭", "start_offset": 14, "end_offset": 21, "label": "dis" }, { "id": 3, "entity": "急性肾衰竭", "start_offset": 22, "end_offset": 27, "label": "dis" } ]
大多数患儿是由于严重高血压已存在相当时间,诊断时多已出现心、脑、肾等靶器官受累的症状。
[ { "id": 0, "entity": "高血压", "start_offset": 10, "end_offset": 13, "label": "dis" }, { "id": 1, "entity": "心", "start_offset": 28, "end_offset": 29, "label": "bod" }, { "id": 2, "entity": "脑", "start_offset": 30, "end_offset": 31, "label": "bod" }, { "id": 3, "entity": "肾", "start_offset": 32, "end_offset": 33, "label": "bod" }, { "id": 4, "entity": "靶器官", "start_offset": 34, "end_offset": 37, "label": "bod" }, { "id": 5, "entity": "心、脑、肾等靶器官受累", "start_offset": 28, "end_offset": 39, "label": "sym" } ]
病因为大动脉炎患者,尚可伴有低热、乏力和关节痛等症状。
[ { "id": 0, "entity": "大动脉炎", "start_offset": 3, "end_offset": 7, "label": "dis" }, { "id": 1, "entity": "低热", "start_offset": 14, "end_offset": 16, "label": "sym" }, { "id": 2, "entity": "乏力", "start_offset": 17, "end_offset": 19, "label": "sym" } ]
(二)体征1.高血压几乎一半的患儿在常规体检时发现高血压。
[ { "id": 0, "entity": "高血压", "start_offset": 7, "end_offset": 10, "label": "dis" }, { "id": 1, "entity": "高血压", "start_offset": 25, "end_offset": 28, "label": "dis" } ]
小儿收缩压或舒张压超过该年龄及性别组的第95百分位数值(P95),相当于超过同年龄、同性别组平均值2个标准差为高血压;介于P95~P99者,为有意义的高血压,超过P99者为严重高血压,RVH患者均为严重高血压。
[ { "id": 0, "entity": "收缩压", "start_offset": 2, "end_offset": 5, "label": "ite" }, { "id": 1, "entity": "舒张压", "start_offset": 6, "end_offset": 9, "label": "ite" }, { "id": 2, "entity": "高血压", "start_offset": 55, "end_offset": 58, "label": "dis" }, { "id": 3, "entity": "高血压", "start_offset": 75, "end_offset": 78, "label": "dis" }, { "id": 4, "entity": "高血压", "start_offset": 88, "end_offset": 91, "label": "dis" }, { "id": 5, "entity": "RVH", "start_offset": 92, "end_offset": 95, "label": "dis" }, { "id": 6, "entity": "高血压", "start_offset": 101, "end_offset": 104, "label": "dis" } ]
眼底检查可呈现不同程度高血压眼底改变。
[ { "id": 0, "entity": "眼底检查", "start_offset": 0, "end_offset": 4, "label": "pro" } ]
Ⅰ度正常眼底;Ⅱ度有局灶性小动脉痉挛;Ⅲ度有渗出伴或不伴有出血;Ⅳ度视乳头水肿。
[ { "id": 0, "entity": "正常眼底", "start_offset": 2, "end_offset": 6, "label": "sym" }, { "id": 1, "entity": "小动脉", "start_offset": 13, "end_offset": 16, "label": "bod" }, { "id": 2, "entity": "局灶性小动脉痉挛", "start_offset": 10, "end_offset": 18, "label": "sym" }, { "id": 3, "entity": "乳头", "start_offset": 35, "end_offset": 37, "label": "bod" }, { "id": 4, "entity": "乳头水肿", "start_offset": 35, "end_offset": 39, "label": "sym" } ]
2.血管杂音约有1/3~2/3患儿(多为大动脉炎患者)在中上腹部和/或腰背肋脊角处可闻及血管杂音,空腹时更易听到呈收缩期和舒张期连续性杂音,若听诊器从中上腹向旁平行移动时杂音增强则更有临床意义。
[ { "id": 0, "entity": "血管", "start_offset": 2, "end_offset": 4, "label": "bod" }, { "id": 1, "entity": "血管杂音", "start_offset": 2, "end_offset": 6, "label": "sym" }, { "id": 2, "entity": "大动脉炎", "start_offset": 20, "end_offset": 24, "label": "dis" }, { "id": 3, "entity": "中上腹部", "start_offset": 28, "end_offset": 32, "label": "bod" }, { "id": 4, "entity": "腰背肋脊角处", "start_offset": 35, "end_offset": 41, "label": "bod" }, { "id": 5, "entity": "血管", "start_offset": 44, "end_offset": 46, "label": "bod" }, { "id": 6, "entity": "中上腹部和/或腰背肋脊角处可闻及血管杂音", "start_offset": 28, "end_offset": 48, "label": "sym" }, { "id": 7, "entity": "收缩期和舒张期连续性杂音", "start_offset": 57, "end_offset": 69, "label": "sym" }, { "id": 8, "entity": "听诊器", "start_offset": 71, "end_offset": 74, "label": "equ" }, { "id": 9, "entity": "中上腹向旁平行移动时杂音增强", "start_offset": 75, "end_offset": 89, "label": "sym" } ]
此外尚需注意大动脉炎所致的缺血症状,若累及无名动脉,可出现桡、肱动脉搏动减弱或消失;若累及髂动脉,可致跛行、手足凉,股动脉及足背动脉搏动减弱或消失。
[ { "id": 0, "entity": "大动脉炎", "start_offset": 6, "end_offset": 10, "label": "dis" }, { "id": 1, "entity": "缺血症状", "start_offset": 13, "end_offset": 17, "label": "sym" }, { "id": 2, "entity": "无名动脉", "start_offset": 21, "end_offset": 25, "label": "bod" }, { "id": 3, "entity": "累及无名动脉", "start_offset": 19, "end_offset": 25, "label": "sym" }, { "id": 4, "entity": "桡、肱动脉", "start_offset": 29, "end_offset": 34, "label": "bod" }, { "id": 5, "entity": "桡、肱动脉搏动减弱或消失", "start_offset": 29, "end_offset": 41, "label": "sym" }, { "id": 6, "entity": "髂动脉", "start_offset": 45, "end_offset": 48, "label": "bod" }, { "id": 7, "entity": "累及髂动脉", "start_offset": 43, "end_offset": 48, "label": "sym" }, { "id": 8, "entity": "跛行", "start_offset": 51, "end_offset": 53, "label": "sym" }, { "id": 9, "entity": "手足", "start_offset": 54, "end_offset": 56, "label": "bod" }, { "id": 10, "entity": "手足凉", "start_offset": 54, "end_offset": 57, "label": "sym" }, { "id": 11, "entity": "股动脉", "start_offset": 58, "end_offset": 61, "label": "bod" }, { "id": 12, "entity": "足背动脉", "start_offset": 62, "end_offset": 66, "label": "bod" }, { "id": 13, "entity": "股动脉及足背动脉搏动减弱或消失", "start_offset": 58, "end_offset": 73, "label": "sym" } ]
(三)实验室检查血常规、尿常规及尿细菌学检查,血尿素、肌酐、钾、钠、钙、氯化物及血气分析应列为常规检查。
[ { "id": 0, "entity": "血常规", "start_offset": 8, "end_offset": 11, "label": "ite" }, { "id": 1, "entity": "尿常规", "start_offset": 12, "end_offset": 15, "label": "ite" }, { "id": 2, "entity": "尿细菌学检查", "start_offset": 16, "end_offset": 22, "label": "ite" }, { "id": 3, "entity": "血尿素", "start_offset": 23, "end_offset": 26, "label": "ite" }, { "id": 4, "entity": "肌酐", "start_offset": 27, "end_offset": 29, "label": "ite" }, { "id": 5, "entity": "钾", "start_offset": 30, "end_offset": 31, "label": "ite" }, { "id": 6, "entity": "钠", "start_offset": 32, "end_offset": 33, "label": "ite" }, { "id": 7, "entity": "钙", "start_offset": 34, "end_offset": 35, "label": "ite" }, { "id": 8, "entity": "氯化物", "start_offset": 36, "end_offset": 39, "label": "ite" }, { "id": 9, "entity": "血气分析", "start_offset": 40, "end_offset": 44, "label": "ite" } ]
当严重高血压有继发性肾损害时可出现蛋白尿、血尿素和肌酐升高。
[ { "id": 0, "entity": "高血压", "start_offset": 3, "end_offset": 6, "label": "dis" }, { "id": 1, "entity": "继发性肾损害", "start_offset": 7, "end_offset": 13, "label": "dis" }, { "id": 2, "entity": "蛋白尿", "start_offset": 17, "end_offset": 20, "label": "sym" }, { "id": 3, "entity": "血尿素", "start_offset": 21, "end_offset": 24, "label": "sym" }, { "id": 4, "entity": "肌酐升高", "start_offset": 25, "end_offset": 29, "label": "sym" } ]
心电图多呈左心室高电压或左心室肥大。
[ { "id": 0, "entity": "心电图", "start_offset": 0, "end_offset": 3, "label": "ite" }, { "id": 1, "entity": "左心室", "start_offset": 5, "end_offset": 8, "label": "bod" }, { "id": 2, "entity": "左心室高电压", "start_offset": 5, "end_offset": 11, "label": "sym" }, { "id": 3, "entity": "左心室", "start_offset": 12, "end_offset": 15, "label": "bod" }, { "id": 4, "entity": "左心室肥大", "start_offset": 12, "end_offset": 17, "label": "sym" } ]
【诊断】对高血压患儿需进一步检查血清内生肌酐清除率,钾、钠、氯化物及血气分析,血、尿醛固酮、尿儿茶酚胺及其代谢产物和香草苦杏仁酸(VMA)测定,以初步除外肾实质性高血压以及内分泌系、神经系、心血管系等疾病所致者,如:主动脉缩窄、原发性醛固酮增多症和嗜铬细胞瘤等。
[ { "id": 0, "entity": "高血压", "start_offset": 5, "end_offset": 8, "label": "dis" }, { "id": 1, "entity": "血清内生肌酐清除率", "start_offset": 16, "end_offset": 25, "label": "ite" }, { "id": 2, "entity": "钾", "start_offset": 26, "end_offset": 27, "label": "ite" }, { "id": 3, "entity": "钠", "start_offset": 28, "end_offset": 29, "label": "ite" }, { "id": 4, "entity": "氯化物", "start_offset": 30, "end_offset": 33, "label": "ite" }, { "id": 5, "entity": "血气分析", "start_offset": 34, "end_offset": 38, "label": "ite" }, { "id": 6, "entity": "血", "start_offset": 39, "end_offset": 40, "label": "ite" }, { "id": 7, "entity": "尿醛固酮", "start_offset": 41, "end_offset": 45, "label": "ite" }, { "id": 8, "entity": "尿儿茶酚胺", "start_offset": 46, "end_offset": 51, "label": "ite" }, { "id": 9, "entity": "香草苦杏仁酸", "start_offset": 58, "end_offset": 64, "label": "ite" }, { "id": 10, "entity": "VMA", "start_offset": 65, "end_offset": 68, "label": "ite" }, { "id": 11, "entity": "肾实质性高血压", "start_offset": 77, "end_offset": 84, "label": "dis" }, { "id": 12, "entity": "内分泌系、神经系、心血管系等疾病", "start_offset": 86, "end_offset": 102, "label": "dis" }, { "id": 13, "entity": "主动脉缩窄", "start_offset": 108, "end_offset": 113, "label": "dru" }, { "id": 14, "entity": "原发性醛固酮增多症", "start_offset": 114, "end_offset": 123, "label": "dis" }, { "id": 15, "entity": "嗜铬细胞瘤", "start_offset": 124, "end_offset": 129, "label": "dis" } ]
拟诊为RVH者,进行以下检查以明确有无肾动脉狭窄存在,并了解肾动脉狭窄的部位、病变性质和程度。
[ { "id": 0, "entity": "RVH", "start_offset": 3, "end_offset": 6, "label": "dis" }, { "id": 1, "entity": "肾动脉", "start_offset": 19, "end_offset": 22, "label": "bod" }, { "id": 2, "entity": "肾动脉狭窄", "start_offset": 19, "end_offset": 24, "label": "sym" }, { "id": 3, "entity": "肾动脉", "start_offset": 30, "end_offset": 33, "label": "bod" }, { "id": 4, "entity": "肾动脉狭窄", "start_offset": 30, "end_offset": 35, "label": "sym" } ]
病理资料表明,当肾动脉管腔截面积减少>50%~80%以上才有可能发生RVH。
[ { "id": 0, "entity": "肾动脉管腔", "start_offset": 8, "end_offset": 13, "label": "bod" }, { "id": 1, "entity": "肾动脉管腔截面积减少>50%~80%", "start_offset": 8, "end_offset": 26, "label": "sym" } ]
(一)筛选检查1.快速连续静脉肾盂造影(IVP)在注射造影剂后1、2、3、5、10、15分钟摄片,了解双肾大小、肾脏显影及排泄情况,阳性标准是:①缺血侧肾脏长径缩短1~1.5cm以上(正常小儿左肾稍大于右肾0.8cm);②患肾肾盂肾盏显影延迟,不显影和/或显影浓度降低;③后期造影剂排泄延迟。
[ { "id": 0, "entity": "快速连续静脉肾盂造影", "start_offset": 9, "end_offset": 19, "label": "pro" }, { "id": 1, "entity": "IVP", "start_offset": 20, "end_offset": 23, "label": "pro" }, { "id": 2, "entity": "缺血侧肾脏长径", "start_offset": 73, "end_offset": 80, "label": "ite" }, { "id": 3, "entity": "缺血侧肾脏长径缩短1~1.5cm以上", "start_offset": 73, "end_offset": 91, "label": "sym" }, { "id": 4, "entity": "肾盂肾盏显影延迟", "start_offset": 113, "end_offset": 121, "label": "sym" }, { "id": 5, "entity": "后期造影剂排泄延迟", "start_offset": 136, "end_offset": 145, "label": "sym" } ]
此法在小儿的符合率为42%~65%,假阴性者多为双肾动脉狭窄或肾动脉分支狭窄。
[ { "id": 0, "entity": "双肾动脉", "start_offset": 24, "end_offset": 28, "label": "bod" }, { "id": 1, "entity": "双肾动脉狭窄", "start_offset": 24, "end_offset": 30, "label": "sym" }, { "id": 2, "entity": "肾动脉分支", "start_offset": 31, "end_offset": 36, "label": "bod" }, { "id": 3, "entity": "肾动脉分支狭窄", "start_offset": 31, "end_offset": 38, "label": "sym" } ]
检查时还可静脉注射利尿剂,可使健侧肾盂造影剂迅速“洗脱”,患肾由于肾小球滤过率少,造影剂排出缓慢,从而扩大了两肾排泄造影剂的差别,有利于提高RVH的诊断。
[ { "id": 0, "entity": "静脉注射", "start_offset": 5, "end_offset": 9, "label": "pro" }, { "id": 1, "entity": "利尿剂", "start_offset": 9, "end_offset": 12, "label": "dru" }, { "id": 2, "entity": "肾小球", "start_offset": 33, "end_offset": 36, "label": "bod" }, { "id": 3, "entity": "肾小球滤过率少", "start_offset": 33, "end_offset": 40, "label": "sym" }, { "id": 4, "entity": "造影剂排出缓慢", "start_offset": 41, "end_offset": 48, "label": "sym" }, { "id": 5, "entity": "RVH", "start_offset": 70, "end_offset": 73, "label": "dis" } ]
2.彩色多普勒超声检查可通过二维超声图像了解双肾大小有无差异,如一侧肾动脉狭窄,患肾比健侧明显缩小。
[ { "id": 0, "entity": "彩色多普勒超声检查", "start_offset": 2, "end_offset": 11, "label": "pro" }, { "id": 1, "entity": "一侧肾动脉", "start_offset": 32, "end_offset": 37, "label": "bod" }, { "id": 2, "entity": "一侧肾动脉狭窄", "start_offset": 32, "end_offset": 39, "label": "sym" }, { "id": 3, "entity": "患肾比健侧明显缩小", "start_offset": 40, "end_offset": 49, "label": "sym" } ]
又可通过多普勒超声检查探查双肾动脉直径、血流量及流速,了解有无肾动脉主干狭窄,为一种快速、无创、重复性好的筛选检查。
[ { "id": 0, "entity": "多普勒超声检查探查", "start_offset": 4, "end_offset": 13, "label": "pro" }, { "id": 1, "entity": "双肾动脉直径", "start_offset": 13, "end_offset": 19, "label": "ite" }, { "id": 2, "entity": "血流量", "start_offset": 20, "end_offset": 23, "label": "ite" }, { "id": 3, "entity": "流速", "start_offset": 24, "end_offset": 26, "label": "ite" }, { "id": 4, "entity": "肾动脉主干", "start_offset": 31, "end_offset": 36, "label": "bod" }, { "id": 5, "entity": "肾动脉主干狭窄", "start_offset": 31, "end_offset": 38, "label": "sym" } ]
3.放射性核素检查可初步了解分侧肾的血流灌注、分泌和排泄功能。
[ { "id": 0, "entity": "放射性核素检查", "start_offset": 2, "end_offset": 9, "label": "pro" }, { "id": 1, "entity": "分侧肾", "start_offset": 14, "end_offset": 17, "label": "bod" } ]
近年来应用<sup>99m</sup>锝二巯基丁二酸(<sup>99m</sup>Tc-DTPA)肾动态显像,可更全面地反映两侧肾的大小、肾灌注高峰出现时间、肾功能及两肾间差异程度,还可做ACEI抑制试验以增强健肾和患肾对示踪剂在双肾的灌注、分泌和排泄的差异程度,提高了该检查的敏感性和特异性。
[ { "id": 0, "entity": "<sup>99m</sup>锝二巯基丁二酸(<sup>99m</sup>Tc-DTPA)肾动态显像", "start_offset": 5, "end_offset": 54, "label": "pro" }, { "id": 1, "entity": "两侧肾的大小", "start_offset": 62, "end_offset": 68, "label": "ite" }, { "id": 2, "entity": "肾灌注高峰出现时间", "start_offset": 69, "end_offset": 78, "label": "ite" }, { "id": 3, "entity": "肾功能", "start_offset": 79, "end_offset": 82, "label": "ite" }, { "id": 4, "entity": "两肾间差异程度", "start_offset": 83, "end_offset": 90, "label": "ite" }, { "id": 5, "entity": "ACEI抑制试验", "start_offset": 94, "end_offset": 102, "label": "pro" } ]
4.血浆肾素活性和血管紧张素抑制试验。
[ { "id": 0, "entity": "血浆肾素活性和血管紧张素抑制试验", "start_offset": 2, "end_offset": 18, "label": "pro" } ]
(1)外周血浆肾素活性(PRA)测定:肾素分泌有昼夜节律性,PRA活性上午8时许最低,中午至晚上8时分泌量最高,PRA与高血压不呈简单的平行关系。
[ { "id": 0, "entity": "外周血浆肾素活性", "start_offset": 3, "end_offset": 11, "label": "ite" }, { "id": 1, "entity": "PRA", "start_offset": 12, "end_offset": 15, "label": "ite" }, { "id": 2, "entity": "肾素", "start_offset": 19, "end_offset": 21, "label": "bod" }, { "id": 3, "entity": "PRA活性", "start_offset": 30, "end_offset": 35, "label": "ite" }, { "id": 4, "entity": "PRA", "start_offset": 56, "end_offset": 59, "label": "ite" }, { "id": 5, "entity": "高血", "start_offset": 60, "end_offset": 62, "label": "dis" }, { "id": 6, "entity": "高血", "start_offset": 60, "end_offset": 62, "label": "pro" } ]
由于RVH存在的时间、单侧或双侧及严重程度的不同,PRA值的变化很大。
[ { "id": 0, "entity": "RVH", "start_offset": 2, "end_offset": 5, "label": "dis" }, { "id": 1, "entity": "PRA值", "start_offset": 25, "end_offset": 29, "label": "ite" } ]
患者的PRA可显著增高,少数正常或降低。
[ { "id": 0, "entity": "PRA", "start_offset": 3, "end_offset": 6, "label": "ite" }, { "id": 1, "entity": "PRA可显著增高", "start_offset": 3, "end_offset": 11, "label": "sym" } ]
需在停用降压和利尿药2周后检测,而停用降压利尿药有发生高血压严重并发症的危险,又因小儿原发性高血压、肾实质性高血压,PRA亦可升高,故对RVH的诊断的敏感性和特异性均很差。
[ { "id": 0, "entity": "降压和利尿药", "start_offset": 4, "end_offset": 10, "label": "dru" }, { "id": 1, "entity": "降压利尿药", "start_offset": 19, "end_offset": 24, "label": "dru" }, { "id": 2, "entity": "高血压", "start_offset": 27, "end_offset": 30, "label": "dis" }, { "id": 3, "entity": "高血压严重并发症", "start_offset": 27, "end_offset": 35, "label": "sym" }, { "id": 4, "entity": "小儿原发性高血压", "start_offset": 41, "end_offset": 49, "label": "dis" }, { "id": 5, "entity": "肾实质性高血压", "start_offset": 50, "end_offset": 57, "label": "dis" }, { "id": 6, "entity": "PRA", "start_offset": 58, "end_offset": 61, "label": "ite" }, { "id": 7, "entity": "PRA亦可升高", "start_offset": 58, "end_offset": 65, "label": "sym" }, { "id": 8, "entity": "RVH", "start_offset": 68, "end_offset": 71, "label": "dis" } ]
(2)血管紧张素抑制试验:比较方便的是采用口服卡托普利(captopril),通过口服卡托普利,可阻断ATⅡ生成,通过负反馈效应使肾素分泌显著增多,以提高检查的敏感性和特异性,观察试验前后PRA的变化。
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方法:试前2周停服利尿及降压药,给普食,患儿取平卧位,卡托普利(开博通)按0.7mg/kg加水20ml口服(服药后盛药容器需以温开水冲洗,再次服下),在服药前30分钟及服药后1小时采血测PRA及血压,阳性结果是:①舒张压下降≥15%;②血PRA用药前>5ngAI/(ml•h),用药后>10ngAI/(ml•h),用药后比用药前PRA之差>4ngAI/(ml•h)。
[ { "id": 0, "entity": "利尿及降压药", "start_offset": 9, "end_offset": 15, "label": "dru" }, { "id": 1, "entity": "卡托普利", "start_offset": 27, "end_offset": 31, "label": "dru" }, { "id": 2, "entity": "开博通", "start_offset": 32, "end_offset": 35, "label": "dru" }, { "id": 3, "entity": "PRA", "start_offset": 93, "end_offset": 96, "label": "ite" }, { "id": 4, "entity": "血压", "start_offset": 97, "end_offset": 99, "label": "ite" }, { "id": 5, "entity": "舒张压", "start_offset": 107, "end_offset": 110, "label": "ite" }, { "id": 6, "entity": "舒张压下降≥15%", "start_offset": 107, "end_offset": 116, "label": "sym" }, { "id": 7, "entity": "血PRA", "start_offset": 118, "end_offset": 122, "label": "ite" }, { "id": 8, "entity": "血PRA用药前>5ngAI/(ml•h),用药后>10ngAI/(ml•h),用药后比用药前PRA之差>4ngAI/(ml•h)", "start_offset": 118, "end_offset": 182, "label": "sym" } ]
用卡托普利后,ATⅡ生成减少,钠潴留反馈抑制肾素分泌解除,肾素分泌增加。
[ { "id": 0, "entity": "卡托普利", "start_offset": 1, "end_offset": 5, "label": "dru" }, { "id": 1, "entity": "ATⅡ", "start_offset": 7, "end_offset": 10, "label": "bod" }, { "id": 2, "entity": "钠潴留", "start_offset": 15, "end_offset": 18, "label": "sym" }, { "id": 3, "entity": "肾素", "start_offset": 22, "end_offset": 24, "label": "bod" }, { "id": 4, "entity": "抑制肾素分泌", "start_offset": 20, "end_offset": 26, "label": "sym" }, { "id": 5, "entity": "肾素", "start_offset": 29, "end_offset": 31, "label": "bod" }, { "id": 6, "entity": "肾素分泌增加", "start_offset": 29, "end_offset": 35, "label": "sym" } ]
此试验观察用药后PRA上升幅度比血压下降更有诊断意义。
[ { "id": 0, "entity": "PRA", "start_offset": 8, "end_offset": 11, "label": "ite" }, { "id": 1, "entity": "血压", "start_offset": 16, "end_offset": 18, "label": "ite" }, { "id": 2, "entity": "血压下降", "start_offset": 16, "end_offset": 20, "label": "sym" } ]
(二)确诊检查1.数字减影血管造影(digitalsubstractionangiography,DSA)为一项以电子计算机为辅助的X线成像技术,其原理是应用数字式视频影像处理系统,在一张血管造影片中,减去一张尿路平片的骨骼及软组织等阴影。
[ { "id": 0, "entity": "数字减影血管造影", "start_offset": 9, "end_offset": 17, "label": "pro" }, { "id": 1, "entity": "digitalsubstractionangiography", "start_offset": 18, "end_offset": 48, "label": "pro" }, { "id": 2, "entity": "DSA", "start_offset": 49, "end_offset": 52, "label": "pro" }, { "id": 3, "entity": "骨骼", "start_offset": 110, "end_offset": 112, "label": "bod" }, { "id": 4, "entity": "软组织", "start_offset": 113, "end_offset": 116, "label": "bod" } ]
由于消除了其他组织阴影,只剩下唯一的肾动脉图像,使肾动脉显影的清晰度明显提高,可辨认到肾实质内直径<1mm大小的血管。
[ { "id": 0, "entity": "肾实质内直径", "start_offset": 43, "end_offset": 49, "label": "ite" }, { "id": 1, "entity": "肾实质内直径<1mm", "start_offset": 43, "end_offset": 53, "label": "sym" }, { "id": 2, "entity": "血管", "start_offset": 56, "end_offset": 58, "label": "bod" } ]
2.肾动脉造影对筛选试验阳性或筛选试验阴性而仍高度怀疑RVH者可作此项检查。
[ { "id": 0, "entity": "肾动脉造影", "start_offset": 2, "end_offset": 7, "label": "pro" }, { "id": 1, "entity": "RVH", "start_offset": 27, "end_offset": 30, "label": "dis" } ]
采取经皮穿刺插管做血管造影可较好显示包括了弓状动脉在内的肾动脉及其分支的病变、部位、范围、狭窄程度及侧支循环情况,为确诊RVH的可靠方法。
[ { "id": 0, "entity": "经皮穿刺插管做血管造影", "start_offset": 2, "end_offset": 13, "label": "pro" }, { "id": 1, "entity": "弓状动脉", "start_offset": 21, "end_offset": 25, "label": "bod" }, { "id": 2, "entity": "肾动脉及其分支", "start_offset": 28, "end_offset": 35, "label": "bod" }, { "id": 3, "entity": "弓状动脉在内的肾动脉及其分支的病变", "start_offset": 21, "end_offset": 38, "label": "sym" }, { "id": 4, "entity": "RVH", "start_offset": 60, "end_offset": 63, "label": "dis" } ]
必要时于造影同时还可施行腔内血管扩张术(PTA)治疗,幼年儿童因血管细小,且又不合作,有时需在静脉麻醉辅助下施行,有一定的危险性。
[ { "id": 0, "entity": "腔内血管扩张术", "start_offset": 12, "end_offset": 19, "label": "pro" }, { "id": 1, "entity": "PTA", "start_offset": 20, "end_offset": 23, "label": "pro" }, { "id": 2, "entity": "血管", "start_offset": 32, "end_offset": 34, "label": "bod" }, { "id": 3, "entity": "静脉麻醉", "start_offset": 47, "end_offset": 51, "label": "pro" } ]
此项检查前有如伤口出血、血管栓塞和急性肾衰竭等并发症,故应慎重选择病例,术前需作好充分准备。
[ { "id": 0, "entity": "伤口出血", "start_offset": 7, "end_offset": 11, "label": "sym" }, { "id": 1, "entity": "血管栓塞", "start_offset": 12, "end_offset": 16, "label": "dis" }, { "id": 2, "entity": "急性肾衰竭", "start_offset": 17, "end_offset": 22, "label": "dis" } ]
如造影前,应控制高血压,以防伤口出血,造影后立即静注20%甘露醇20~40ml,继予补液,以减少急性肾衰竭和血管栓塞的并发症。
[ { "id": 0, "entity": "高血压", "start_offset": 8, "end_offset": 11, "label": "dis" }, { "id": 1, "entity": "静注", "start_offset": 24, "end_offset": 26, "label": "pro" }, { "id": 2, "entity": "甘露醇", "start_offset": 29, "end_offset": 32, "label": "dru" }, { "id": 3, "entity": "急性肾衰竭", "start_offset": 48, "end_offset": 53, "label": "dis" }, { "id": 4, "entity": "血管栓塞", "start_offset": 54, "end_offset": 58, "label": "dis" } ]
3.磁共振血管成像(magneticresonanceangiography,MRA)是一种可靠的非创伤性检查方法,对RVH诊断的准确性可与DSA相同或更完美,因为它是三维空间肾动脉的血管像,可清晰显示肾动脉在主动脉开口处的情况。
[ { "id": 0, "entity": "磁共振血管成像", "start_offset": 2, "end_offset": 9, "label": "pro" }, { "id": 1, "entity": "magneticresonanceangiography", "start_offset": 10, "end_offset": 38, "label": "pro" }, { "id": 2, "entity": "MRA", "start_offset": 39, "end_offset": 42, "label": "pro" }, { "id": 3, "entity": "非创伤性检查方法", "start_offset": 49, "end_offset": 57, "label": "pro" }, { "id": 4, "entity": "RVH", "start_offset": 59, "end_offset": 62, "label": "dis" }, { "id": 5, "entity": "DSA", "start_offset": 70, "end_offset": 73, "label": "pro" }, { "id": 6, "entity": "肾动脉", "start_offset": 88, "end_offset": 91, "label": "bod" }, { "id": 7, "entity": "肾动脉", "start_offset": 101, "end_offset": 104, "label": "bod" }, { "id": 8, "entity": "主动脉", "start_offset": 105, "end_offset": 108, "label": "bod" } ]
同时它可以避免由肾动脉造影可能引起的碘过敏,出血、血栓形成等危险和并发症。
[ { "id": 0, "entity": "肾动脉造影", "start_offset": 8, "end_offset": 13, "label": "pro" }, { "id": 1, "entity": "碘过敏", "start_offset": 18, "end_offset": 21, "label": "sym" }, { "id": 2, "entity": "出血", "start_offset": 22, "end_offset": 24, "label": "sym" }, { "id": 3, "entity": "血栓形成", "start_offset": 25, "end_offset": 29, "label": "sym" } ]
适用于对血管造影剂过敏,心、肾功能不全或有出血素质者。
[ { "id": 0, "entity": "血管造影剂过敏", "start_offset": 4, "end_offset": 11, "label": "sym" }, { "id": 1, "entity": "心、肾功能不全", "start_offset": 12, "end_offset": 19, "label": "sym" }, { "id": 2, "entity": "有出血素质", "start_offset": 20, "end_offset": 25, "label": "sym" } ]
药物治疗原则为控制高血压,以防止发生高血压严重并发症的危险,避免肾功能损害,或使已受损的肾功能得到改善,减少心、眼、脑等靶器官的损伤。
[ { "id": 0, "entity": "高血压", "start_offset": 18, "end_offset": 21, "label": "dis" }, { "id": 1, "entity": "肾功能损害", "start_offset": 32, "end_offset": 37, "label": "dis" }, { "id": 2, "entity": "肾功能", "start_offset": 44, "end_offset": 47, "label": "ite" }, { "id": 3, "entity": "已受损的肾功能得到改善", "start_offset": 40, "end_offset": 51, "label": "sym" }, { "id": 4, "entity": "心", "start_offset": 54, "end_offset": 55, "label": "bod" }, { "id": 5, "entity": "眼", "start_offset": 56, "end_offset": 57, "label": "bod" }, { "id": 6, "entity": "脑", "start_offset": 58, "end_offset": 59, "label": "bod" }, { "id": 7, "entity": "减少心、眼、脑等靶器官的损伤", "start_offset": 52, "end_offset": 66, "label": "sym" } ]
作为手术前准备或其他原因不能或不愿进行手术者,常联合应用以下药物以达到控制血压的目的。
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1.β受体阻滞剂可通过抑制肾素而减少醛固酮分泌和水钠潴留而起降压作用,可用盐酸普萘洛尔(心得安)1~3mg/(kg•d),分3次服,阿替洛尔(氨酰心安)或美托洛尔(倍他洛克)学龄前小儿按成人剂量1/4~1/2给药,每日1~2次。
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2.血管紧张素抑制剂(ACEI)可抑制ATⅡ的血管收缩和醛固酮分泌作用,对RVH有良好的效应,尤其对合并心力衰竭者更为合适。
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但对一侧肾已有严重肾实质病变,一侧为肾动脉狭窄(RAS)或双侧RAS患者,可能诱发急性暂时性肾功能不全,不宜应用,因ACEI可减少ATⅡ生成使肾小球出球动脉舒张致肾小球滤过率下降,以致用药后肾功能恶化。
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用药1周后要随访血尿素氮和肌酐,并定期以B超声检查随访肾脏大小变化。
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新一代高效低毒长效的ACEI如依那普利(enalapril)和福辛普利(fosinpril,monopril)已应用于临床,后者从肾和胆汁两条途径排泄,对肾功能已有不全者(Cr30~60ml/min)亦可应用。
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3.利尿剂常与其他降压药联合使用,单独使用效果欠佳,对低肾素性高血压最有效,常用有氢氯噻嗪,按1~2mg/(kg•d),分2~3次口服,注意低血钾副作用。
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4.钙离子通透阻滞剂如硝苯地平,可减低血管阻力,保持肾脏血流灌注,通过扩张血管而起降压作用,安全可靠,用于治疗单侧或双侧肾动脉狭窄性高血压。
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舌下含片3~5分钟可起降压作用,亦用于高血压危象的紧急处理。
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5.硝普钠严重高血压,伴有心、脑器官损害,心功能不全及肾功能不全者,应紧急处理,但降压不能过快或降至正常,以控制血压不发生高血压脑病水平为宜。
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静脉滴注硝普钠效果可靠,剂量为1~8μg/(kg•min)持续静滴,以后每分钟增加0.1~0.2μg/kg,直至生效或出现不良反应,停止输注后药效只维持2~5分钟,因其作用时间短暂,应同时与其他降压药联合应用。
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(二)外科治疗1.腔内血管扩张术(PTA)在行动脉造影确立诊断时即可行该手术,如PTA未成功或扩张后发生再狭窄可重复再扩张,对PTA无效者可作自体肾移植术或血管重建术。
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若一侧肾已失去功能或旁路手术失败,对侧肾功能良好者可根据病情施行部分或全肾切除术。
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近年来由于肾移植和显微外科的发展,肾脏冷却保存可达24小时,有充分时间修复肾动脉,又有肾动脉体外整形术以治疗RAH。
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2.肾动脉腔内支架术以膨胀性支架放置于经球囊导管扩张的狭窄肾动脉获得成功,为本病治疗开创了新途径。
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第七节主动脉弓异常主动脉弓异常(abnormalitiesoftheaorticarch)指主动脉弓及其分支发育异常。
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主要有血管环血管环(vascularring)及主动脉分支悬带悬带(vascularsling),可致气管及(或)食管受压迫。
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主动脉双弓、右位主动脉弓伴迷走左锁骨下动脉及左侧动脉导管或动脉韧带、左肺动脉异常或肺动脉悬带为其中较多见的3种类型。
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在先天性心脏血管畸形中,主动脉弓及其分支畸形仅占1%~2%。
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在胚胎发育的第4周,从主动脉囊发出6对腮动脉弓并与背主动脉相连接。
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此后,第Ⅰ、Ⅱ对鳃动脉弓退化消失。
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第Ⅲ对鳃动脉弓形成颈总动脉和一部分颈内动脉,第Ⅳ对鳃动脉弓左侧形成主动脉弓,右侧形成无名动脉和右锁骨下动脉。
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第Ⅴ对鳃动脉弓不久亦退化。
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第6对鳃动脉弓形成肺动脉,其右侧远端与背主动脉分离,左侧在胎儿期持续存在称为动脉导管(图9-9)。
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图9-21正常大动脉发育一、血管环血管环(vascularring)最常见类型是主动脉双弓及右位主动脉弓伴迷走左锁骨下动脉及左侧动脉导管或动脉韧带,多为单独畸形,占先天性心脏病1%以下。
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