CAJ | 학술논문

为提高对蛋白C(PC)/蛋白S(PS)缺乏症相关的肺动脉血栓栓塞症临床表现、诊治方法的认识，本研究分析了湘雅二医院收治的2例PC/PS缺乏相关肺动脉血栓栓塞患者的临床特点，并进行相关文献复习。两位男性患者年龄分别为29，26岁，临床症状主要表现为自发性反复双下肢肿痛，或胸闷气促、胸痛，或视力下将。血管彩超示下肢静脉血栓形成；肺部血管造影(CTA)示双下肺动脉栓塞；血清同型半胱氨酸浓度升高，血浆PC/PS总量明显降低，病例1为PC 57.4%，PS 28.9%；病例2为PS 33.4%。患者行常规抗凝治疗疗效差，予以维生素B6、维生素B12联合抗凝治疗后症状改善。中青年无明显诱因的反复发作性下肢深静脉血栓形成及肺血栓栓塞，应考虑先天性易栓症可能性，血浆PC，PS浓度及活性检测具有重要的诊断治疗意义。
위제고대단백C(PC)/단백S(PS)결핍증상관적폐동맥혈전전새증림상표현、진치방법적인식，본연구분석료상아이의원수치적2례PC/PS결핍상관폐동맥혈전전새환자적림상특점，병진행상관문헌복습。량위남성환자년령분별위29，26세，림상증상주요표현위자발성반복쌍하지종통，혹흉민기촉、흉통，혹시력하장。혈관채초시하지정맥혈전형성；폐부혈관조영(CTA)시쌍하폐동맥전새；혈청동형반광안산농도승고，혈장PC/PS총량명현강저，병례1위PC 57.4%，PS 28.9%；병례2위PS 33.4%。환자행상규항응치료료효차，여이유생소B6、유생소B12연합항응치료후증상개선。중청년무명현유인적반복발작성하지심정맥혈전형성급폐혈전전새，응고필선천성역전증가능성，혈장PC，PS농도급활성검측구유중요적진단치료의의。
To explore the clinical manifestations, diagnosis and treatment of pulmonary thromboembolism associated with protein C (PC)/protein S (PS) deifciency. Two male patients 29 and 26 years old diagnosed with PC deifciency and/or PS deifciency were retrospectively analyzed and related literatures were reviewed. The most common symptoms were pain in the lower limbs with chest pain or decreased vision. Color dopper lfow imaging (CDFI) showed lower deep venous phlebothrombosis. Multislice CT angiography (CTA) revealed pulmonary embolism. The level of serum homocysteine (HCY) increased and the level of plasma PC/PS content decreased to PC 57.4%, and PS 28.9%in patient 1, while PS 33.4%in patient 2. Poor routine anticoagulant response was observed. After the diagnosis of PC/PS deficiency, vitiamin B6 and B12 anticoagulant therapy was added, and the symptoms in the patients improved significantly. Congenital thrombophilia should be taken into consideration for young patients with lower deep venous thrombosis and pulmonary embolism which occur recurrently without obvious predisposing causes before 40. Plasma PC/PS concentrations or activity help a lot in the diagnosis and treatment.