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心血管

13岁少年血压升高 6个科室会诊方确定病因

来源:    时间:2018年11月21日    点击数:    5星

13岁女孩,6年前因视物模糊无意中发现左室增大,血压不详。3年前,偶测血压发现血压增高,最高可达170/110mmHg,检查发现右肾体积缩小、左肾多发结石。当前,因心慌胸闷2周前来就诊。最终6个科室的医生会诊,才确定真正的病因。

病史摘要

患者女,13岁,2015年11月5日入院。

主诉:发现左室肥厚6年余,发现血压升高3年余,心慌2周。

现病史:患者6年余前因视物模糊就诊于当地医院,眼底检查提示眼底血管出血,后于治疗前完善心电图检查时发现左室增大(血压不详),当时无头晕、头痛,未予重视。5年前就诊于××医院完善超声心动图检查提示室间隔及左室肥厚、左室扩大,二尖瓣、主动脉瓣轻度反流,EF51%(血压不详),考虑遗传相关肥厚型心肌病,予营养心肌治疗,此后规律复查,心电图、超声心动图较前无明显变化。3年前偶测血压发现血压增高,最高可达170/110mmHg,于当地医院行腹部CT检查提示:右肾体积缩小、左肾多发结石。就诊于××医院测血压200/110mmHg,肾脏彩超示右肾缩小,肾动脉彩超未见异常。考虑右肾发育不良、高血压、高血压心肌损害,予氨氯地平、奥美沙坦降压治疗。后于××医院完善肾图检查见右肾缩小、血流灌注差、功能严重受损,右肾肾小球滤过率8ml/min;完善动脉彩超示右髂动脉狭窄;免疫相关检查示ANA、ds-DNA升高。因考虑不符合狼疮且大动脉炎诊断证据不充分,嘱其继续口服降压药物控制血压。此后患者长期口服氨氯地平+奥美沙坦+美托洛尔降压,间断更换氨氯地平为硝苯地平控释片,血压可维持于140/80mmHg左右。期间规律复查肌酐波动于57~93mmol/L,尿蛋白(+/-),超声心动图提示左室肥厚亦较前减轻。近2周来间断出现发作性心慌、胸闷,发作时测心率、血压增高。现为进一步诊治收住院。自发病以来,有活动后右下肢疼痛,休息后可减轻;无发作性大汗、面色潮红、多尿、发作性弛缓性瘫痪、血尿、水肿等,精神、睡眠可,大小便正常,体重无明显下降。

既往史、个人史、家族史:4年半前右肱骨内上髁骨折;无肝炎病史及其密切接触史,无结核病史及其密切接触史,无手术史,无血制品输注史,无过敏史,预防接种史按计划进行。足月剖宫产,出生体重2.75kg。父母体健。

体格检查

T37℃,.89次/分,.18次/分,BM.21.45,BP:150/95mmHg(右上肢)、150/90mmHg(左上肢)、110/65mmHg(右下肢)、170/110mmHg(左下肢)。

神清语明,全身皮肤黏膜未见皮疹及出血点。浅表淋巴结未触肿大。双肺呼吸音清,未闻及干湿性啰音。心率89次/分,律齐,各瓣膜听诊区未闻及异常心音及杂音。腹平软,无压痛、反跳痛及肌紧张,肝脾肋下未触及,双下肢无水肿,右侧足背动脉、右股动脉搏动明显减弱。

辅助检查

全血细胞分析:血红蛋白含.119g/L,红细胞平均体.72.4fl↓,平均红细胞血红蛋白含.24.5pg↓,余正常;

生化:丙氨酸氨基转移.15U/L,门冬氨酸氨基转移.15U/L,总蛋.77.8g/L,白蛋白46.0g/L,肌.94μmol/L↑,尿.429μmo./L↑,低密度脂蛋白胆固.1.32mmol/L↓,结合胆红.8.2μmol/L↑,eGF.79.59ml/(min·1.73m2)。

血清同型半胱氨酸:22.70μmol/L↑;贫血检测、贫血组合:铁5.03μmo./.↓,不饱和铁结合力55.28μmol/.↑,铁蛋白11.7ng/m.↓。

自身抗体谱:抗内皮细胞抗体1∶10阳性。

凝血功能、糖化血红蛋白、甲状腺功能5项、BNP、免疫八项、血沉、尿、便常规:无异常。

尿微量白蛋白定量/肌酐:2.4mg/g;24小时尿蛋白定量及定性:24小时尿蛋白:0.05g/d。

肾素-血管紧张素-醛固酮系统:肾素活性:>24.00ng/(ml·h)↑,血管紧张素:>800pg/ ml↑,醛固酮155pg/ml(服药:酒石酸美托洛尔25m.bid,氨氯地平5m.qd,奥美沙坦20m.qd)。

心电图:左室高电压(心前区导联1/4电压)(图44-1);

图44-1心电图

Holter:共记录心搏12.510次(23:59),窦性心律,平均心率90次/分;最快心率133 次/分,发生于12:53,最慢心率66次/分,发生于04:13;房性期前收缩1次,室性期前收缩1次;ST段J点抬高,不除外早期复极。

动态血压监测:24小时动态血压呈非杓型分布;24小时平均血压132/75mmHg;白天平均收缩压137mmHg,白天平均舒张压80mmHg;夜间平均收缩压124mmHg,夜间平均舒张压64mmHg;全天血压间断轻、中度升高;无晨起高血压现象。

超声心动图:左房前后径、主动脉根部内径、升主动脉内径、E/A等无明显异常,余见表44-1(附既往检查结果)。

表44-2超声心动图结果

肢体动脉功能检测:血压:右上臂143/78mmHg,左上臂140/77mmHg,右脚踝79/56mmHg,左脚踝115/68mmHg;baPWV:右693cm/s,左922cm/s;ABI:右下肢0.55,左下肢0.80。

腹部及肾上腺超声:右肾7.5cm×4.0cm×4.2cm,实质厚1.1cm,左肾11cm×5.2cm× 5.1cm,实质厚1.8cm;肾上腺未见异常。下肢动脉:腹主动脉、右侧髂总动脉,右侧股总动脉、腘动脉缩窄,考虑大动脉炎可能,必要时与动脉肌纤维发育不良鉴别。肾动脉:右肾起始处肾动脉流速增高,轻度狭窄不除外。锁骨下动脉、上肢动脉、颈动脉:未见明显异常。

胸部正位X线片、头部CT:未见明显异常。

散瞳眼底:双眼视网膜动脉细,略迂曲,迂曲程度较5年前好转,眼底未见缺血性改变,双眼视乳头边界清,色略淡,眼底未见其他活动性病变。

既往检查结果:心脏磁共振(2015年10月):左房(31mm)、左室(47mm)内径正常,左室普遍偏厚(室间隔12~15mm,左室游离壁11~12mm),射血分数53.8%,心肌收缩率、舒张期顺应性降低,无流出道狭窄。腹部CT(2013年3月):右肾体积缩小、左肾多发结石,双肾上.(-)。腹部彩超(2013年3月):右肾7.5cm×2.8cm,左肾11.8×5.4cm。腹部彩超(2013年5月):右肾7.7cm×3.3cm×2.4cm,左肾9.9cm×5.6cm×4.8cm。肾动脉彩超(2013年5月):左肾动脉干流速93cm/s,右肾动脉干流速74cm/s。生化(2013年5月):K.4.6mmol/L,CR.57μmo./L。24小时尿蛋白(2013年5月):尿蛋白0.47g/24h,尿量1250ml/24h。肾图(2013年5月):右肾小、血流灌注差、功能严重受损,GFR:左43ml/h,右8ml/h。肢体动脉彩超(2013年5月):右髂动脉狭窄,大动脉炎?颈动脉、锁骨下动脉未见异常。ANCA、CRP、ESR(2013年5月):阴性;ANA:1∶100;ds-DNA:199IU/ml。

入院诊断

血压升高原因待查:多发性大动脉炎?视网膜血管畸形?

诊治经过与诊治思维

1.简要治疗经过

入院后继续原有药物治疗,进一步完善相关检查。入院时服用美托洛尔25m.bid、氨氯地平5m.qd、奥美沙坦20m.qd,血压150/85mmHg,心率89次/分;调整药物为贝尼地平8m.qd、奥美沙坦20m.qd、美托洛尔37.5m.bid,血压:130/60mmHg,心率76次/分左右。

2.多科室病例讨论

本例患者为13岁女患,主因“发现左室肥厚6年余,血压升高3年余,心慌2周”收入院。目前主要存在以下几个问题:①明确血压升高的原因:继发性?原发性?②明确肾脏病变的原因:高血压造成肾损害?肾动脉/肾脏疾病?③明确患者眼部病变的原因:与高血压相关?视网膜血管畸形?大动脉炎引起?④指导下一步诊治。

(1)眼科意见:

大动脉炎时眼部主要表现为眼动脉供血不足,亦称无脉征,可有静脉迂曲扩张及血管瘤的形成等。但该患者的眼部病变不是眼缺血的表现,故患者眼部病变不是由大动脉炎引起。该患者在2010年检查显示生理盲点扩大,考虑是由于高血压导致颅内压升高,进而导致视乳头水肿引起。另外,患者视网膜动脉瘤也可由高血压解释。视网膜动脉瘤不是真性的瘤子,而是高血压引起的囊性扩张。既往患者曾于外院诊断为视网膜炎等,综合患者情况,考虑该诊断不成立。该患右眼自幼视力差,右眼视力模糊5年,当时不合并头痛。查体双上肢脉搏正常搏动,双眼前段无异常。散瞳眼底:双眼视网膜动脉细,略迂曲,迂曲程度较5年前好转,眼底未见缺血性改变,双眼视乳头边界清楚,色略淡,眼底未见其他活动性病变。印象:双眼底表现符合高血压诊断,目前不支持眼底缺血诊断。建议完善眼底相、眼底荧光血管造影、眼动脉血流、OCT、视野。眼动脉血流和眼底血管造影可以鉴别是否是大动脉炎,OCT观察黄斑功能。若患者肾功能不全不能行眼底血管造影,可以行眼底图检查。

(2)风湿免疫科意见:

高血压一般引起双肾病变,该患者目前考虑肾脏病变引起血压升高。该患者的眼部病变考虑是高血压引起的,因为多发性大动脉炎时一般不会引起小动脉受累。综合考虑患者目前情况,考虑多发性大动脉炎的诊断基本明确,目前为非活动期,确定下一步的治疗方案尤为重要。常规建议保留右肾,因为即使切除右肾,也不一定会使血压下降。建议结合外科意见综合考虑下一步治疗方案。若以内科治疗为主,可以考虑给予甲氨蝶呤15mg每周1次,泼尼松每天10片(50mg/d)。甲氨蝶呤需长期服用;但是泼尼松可引起血压升高等,使用时需密切监测。服用泼尼松1个月后,逐渐减量,每两周减半片。使用过程中监测血压等,3个月时复查肝功能、血常规等。

(3)泌尿外科意见:

患者目前的eGFR为79.59ml/(min·1.73m2),提示右肾仍有一定的肾功能,建议保留右肾。建议行血管造影及分段取血、肾脏磁共振或CT检查,以判断高血压是否由右肾引起,若是单纯的右肾病变引起则具备手术指征,但建议首先考虑血管外科的介入治疗,如果无效再行右肾切除。该患者目前不能单纯归因于右肾病变,自体右肾移植暂时无太大意义。该患者的肾图结果需进一步明确,必要时可复查。

(4)儿科意见:

大动脉炎好发于儿童,6~10岁多见,女性占80%左右。且儿童大动脉炎多与结核有关,占80%。该患者既往曾出现自身抗体的增高,目前多发性大动炎诊断基本明确。建议筛查PPD等,以明确是否由结核引起。同时,可复查肾图,以便进一步明确肾功能情况。

(5)血管外科意见:

该患者年龄小,仍处于生长发育期,因此目前暂不适合行动脉支架介入治疗。正常人腹主动脉直径在20mm左右,该患者腹主动脉最狭窄处直径6mm,严重狭窄,也可以引起血压升高。故切除右肾,血压不一定会下降。该患者右侧足背动脉、右股动脉搏动明显减弱。该患者需进一步明确腹主动脉狭窄部位,是在肾动脉开口处以下还是在其上方。若狭窄段在肾动脉开口处以下,则暂不考虑行介入治疗;患者下肢间歇性跛行可暂以锻炼为主,必要时加用西洛他唑改善下肢动脉供血以及阿司匹林等预防血栓形成。可考虑行大动脉CTA或MRA等检查,评估整体血管病变情况,进一步考虑是否有外科介入指征。目前建议先以内科治疗为主,外科干预主要为补救性措施,且外科治疗后容易复发。

(6)心内科意见:

该患者年龄偏小,病情复杂,综合患者目前情况,考虑诊断为多发性大动脉炎。与患者家属充分沟通后,可考虑先给予免疫抑制治疗,同时完善CTA及肾脏磁共振等,观察血管狭窄程度,判断是否需行外科介入治疗。目前患者肾素水平增高明显,必要时可考虑行分段取血检查,判断肾素分泌来源。目前可予奥美沙坦,美托洛尔缓释片及CCB降压治疗,同时观察肾素水平的变化。若给予抑制肾素的相关药物治疗后,患者肾素水平下降,必要时则可以考虑进一步行外科介入治疗。

专家点评

该患者6年余前因视物模糊就诊于当地医院时,完善心电图检查时发现左室增大,曾于诊室测血压,曾发现血压增高,但未对血压异常情况予以重视。5年前超声心动图检查提示室间隔及左室肥厚、左室扩大,二尖瓣、主动脉瓣轻度反流,此时仍未对血压情况进一步明确,而考虑遗传相关肥厚型心肌病,此后仅规律复查心电图、超声心动图等。该患者3年前偶测血压,明确血压增高,最高可达170/110mmHg;此时完善了有关高血压的部分相关检查,以及部分免疫学的相关检查。但是,仍缺乏进一步完整的系统诊治过程。

该患者早期主要表现为视物模糊、眼底出血等眼科症状等,后期发现血压增高、肾动脉血流异常、髂动脉狭窄、双肾不等大、肾功能异常、免疫学指针异常等,此时即应高度怀疑多发性大动脉炎。

随访情况

该患者于风湿免疫科进行多发性大动脉炎的治疗。

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来源:《中国高血压疑难病例荟萃》
作者:蔡 军 刘力生 孙宁玲 吴海英
页码:202-205
出版:人民卫生出版社
 

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