中国医药
中國醫藥
중국의약
CHINA MEDICINE
2012年
4期
461-463
,共3页
龚利民%叶晓芳%楊京华%刘双
龔利民%葉曉芳%楊京華%劉雙
공이민%협효방%양경화%류쌍
马方综合征%肺栓塞%呼吸困难
馬方綜閤徵%肺栓塞%呼吸睏難
마방종합정%폐전새%호흡곤난
Marian's syndrome%Pulmonary embolism%Dyspnea
目的 提高临床医生对马方综合征伴呼吸困难的认识.方法 对1例马方综合征伴呼吸困难患者进行分析,对其发病原因进行探讨,并复习相关文献.结果 患者男,21岁,活动后呼吸困难1个月余入院,既往马方综合征病史15年,此次人院后肺动脉CT血管造影示:多发肺栓塞,累及主肺动脉,左右肺动脉主干及其分支,超声心动图:双房条索状中等回声性质待定-附着于卵圆窝部位的血栓可能性大,右心增大,三尖瓣轻度反流,三尖瓣反流法估测肺动脉收缩压:59 mm Hg(1 mm Hg =0.133 kPa)；双下肢深静脉彩超:左侧股浅浅静脉远段至腘静脉不完全血栓可能,左侧小腿肌间静脉血栓可能,右侧腘静脉陈旧血栓可能,该例患者临床诊断为急性肺血栓栓塞症、马方综合征,经低分子肝素治疗后症状好转.结论 马方综合征的血管病变可能与原纤维蛋白1基因突变有关.马方综合征与肺栓塞是否有必然联系,高同型半胱氨酸血症在马方综合征患者出现肺栓塞的发生发展中的作用等问题均有待于进一步探讨.马方综合征伴呼吸困难应高度重视,警惕急性肺血栓栓塞症的发生.
目的 提高臨床醫生對馬方綜閤徵伴呼吸睏難的認識.方法 對1例馬方綜閤徵伴呼吸睏難患者進行分析,對其髮病原因進行探討,併複習相關文獻.結果 患者男,21歲,活動後呼吸睏難1箇月餘入院,既往馬方綜閤徵病史15年,此次人院後肺動脈CT血管造影示:多髮肺栓塞,纍及主肺動脈,左右肺動脈主榦及其分支,超聲心動圖:雙房條索狀中等迴聲性質待定-附著于卵圓窩部位的血栓可能性大,右心增大,三尖瓣輕度反流,三尖瓣反流法估測肺動脈收縮壓:59 mm Hg(1 mm Hg =0.133 kPa)；雙下肢深靜脈綵超:左側股淺淺靜脈遠段至腘靜脈不完全血栓可能,左側小腿肌間靜脈血栓可能,右側腘靜脈陳舊血栓可能,該例患者臨床診斷為急性肺血栓栓塞癥、馬方綜閤徵,經低分子肝素治療後癥狀好轉.結論 馬方綜閤徵的血管病變可能與原纖維蛋白1基因突變有關.馬方綜閤徵與肺栓塞是否有必然聯繫,高同型半胱氨痠血癥在馬方綜閤徵患者齣現肺栓塞的髮生髮展中的作用等問題均有待于進一步探討.馬方綜閤徵伴呼吸睏難應高度重視,警惕急性肺血栓栓塞癥的髮生.
목적 제고림상의생대마방종합정반호흡곤난적인식.방법 대1례마방종합정반호흡곤난환자진행분석,대기발병원인진행탐토,병복습상관문헌.결과 환자남,21세,활동후호흡곤난1개월여입원,기왕마방종합정병사15년,차차인원후폐동맥CT혈관조영시:다발폐전새,루급주폐동맥,좌우폐동맥주간급기분지,초성심동도:쌍방조색상중등회성성질대정-부착우란원와부위적혈전가능성대,우심증대,삼첨판경도반류,삼첨판반류법고측폐동맥수축압:59 mm Hg(1 mm Hg =0.133 kPa)；쌍하지심정맥채초:좌측고천천정맥원단지객정맥불완전혈전가능,좌측소퇴기간정맥혈전가능,우측객정맥진구혈전가능,해례환자림상진단위급성폐혈전전새증、마방종합정,경저분자간소치료후증상호전.결론 마방종합정적혈관병변가능여원섬유단백1기인돌변유관.마방종합정여폐전새시부유필연련계,고동형반광안산혈증재마방종합정환자출현폐전새적발생발전중적작용등문제균유대우진일보탐토.마방종합정반호흡곤난응고도중시,경척급성폐혈전전새증적발생.
Objective To report one case of Marian's syndrome complicated with dysnea.Methods One case diagnosed of Marfan's syndrome and dyspnea was studied.The related literatures were reviewed.Results One 21 years old young man was diagnosed of Marian's syndrome for 15 years and complained exertional dyspnea on admission.Multiple pulmonary embolism,involving the main pulmonary artery,bilateral main pulmonary artery as well as their branches,were confirmed by computed tomography angiography (CTA).Echocardiography demonstrated medium echo in both left and right atrium with thrombosis attached to round nest eggs.Enlarged right ventricle with mild tricuspid regurgitation and elevated systolic pulmonary artery pressure (59 mm Hg) were detected. Color Doppler showed high possibility of acute thrombosis from left femoral superficial veins to popliteal veins and in left calf muscle venous,while suspicious prior thrombosis was found in the right popliteal veins.The patient was diagnosed of acute pulmonary embolism(APE) and Marian's syndrome.Meanwhile symptoms were improved after treatment with low molecular weight heparin.Conclusions Vascular lesion is associated with gene mutation on fibrillinl with Marian's syndrome.Further study is needed in terms of the relation between Marian's syndrome and APE,as well as the role of hyperhomocysteinemia in the development of APE.Marian's syndrome complicated with dyspnea requires clinical attention,especially for suspicious APE patient.
