ISSN 1006-298X      CN 32-1425/R

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肾脏病与透析肾移植杂志 ›› 2014, Vol. 23 ›› Issue (1): 13-17.

• 论文 • 上一篇    下一篇

成人肾病综合征并发脑静脉窦血栓临床特点

  

  • 出版日期:2014-02-28 发布日期:2014-02-25

Cerebral venous sinus thrombosis in adult patients with nephrotic syndrome: Cases report and literature review

  • Online:2014-02-28 Published:2014-02-25

摘要:

摘 要 目的:分析成人肾病综合征(NS)并发脑静脉窦血栓(CVST)的临床特点和实验室检查,旨在提高诊疗水平。方法:回顾性分析2009年至2013年间本研究所收治14例NS并发脑静脉窦血栓的临床特点、实验室检查结果、治疗以及转归。采用头颅磁共振静脉血管成像成像(MRV)方法观察CVST。结果:男10例,女4例,起病平均年龄24.5±7.3岁,年龄19~44岁。CVST好发于6处静脉窦,其中横窦13例,上矢状窦12例,乙状窦10例,下矢状窦3例,直窦4例,窦汇3例,伴左上肢静脉血栓1例和右室附壁血栓1例,肺栓塞1例和左颈内静脉血栓1例。头颅CT检查4例未见异常,5例示静脉窦高密度影,2例蛛网膜下腔出血,3例出血性脑梗塞。肾活检病理诊断:局灶节段性肾小球硬化症(FSGS) 1例,IgAN(微小病变样)1例,IgM肾病1例,膜性肾病1例,微小病变肾病5例,其余5例未行肾活检。3例CVST发生于初治的NS,而11例发生于复发的NS。所有患者起病时均有不同程度头痛、恶心、呕吐,3例癫痫发作,2例视物模糊。4例患者起病前曾使用大量利尿剂并发低钾或低钠血症,双下肢轻度水肿3例,而无水肿11例,且均无高血压。D-二聚体为(1.43±1.68mg/l),D-二聚体阳性8例,血红蛋白为(16.2±2.12g/dl),2例抗心磷脂抗体IgM阳性。治疗上除1例联合巴曲酶治疗,其余均予低分子肝素、普通肝素抗凝过渡至口服华法林抗凝,INR2~3,辅以甘油果糖和利尿剂降低颅内压。所有患者均经泼尼松联合不同免疫抑制剂治疗,至末次随访时,尿检均完全缓解,脑静脉血栓不同程度消失,无1例死亡,仅其中1例肾病复发后血栓加重,再次加用免疫抑制剂和延长抗凝时间后,尿检缓解,血栓减轻。结论:成人NS不论初治或复发,合并高凝倾向者,一旦出现头痛、癫痫发作或偏瘫等症,临床医生需警惕CVST形成,可借助MRV影像学手段证实,以便早期诊断,早期抗凝治疗,改善预后。

关键词: 肾病综合征, 脑静脉窦血栓, 磁共振静脉血管成像

Abstract:

ABSTRACT    Objective: To make analysis of the clinical features of adult nephrotic syndrome complicated with cerebral venous sinus thrombosis (CVST). Methodology:The clinical features, laboratory findings, treatment and outcome of fourteen aldut patients with nephrotic syndrome complicated with CVST from 2009 to 2013 were retrospectively analyzed. The magnetic resonance venography method was used to observe the occurrence of cerebral venous sinus thrombosis. Results:They were 10 males and 4 femals with an average age of 24.5 ± 7.6 years old (ranged from 19 to 44). 9 cases were performed renal biopsy, the histological diagnosis was minimal change in 5 cases, FSGS one, IgAN (minimal change disease-like) one, IgMN one and membranous nephropathy in one case. CVST usually affected 6 sites of sinus, the transverse sinus in 13 cases, sagittal sinus 12 cases, sigmoid sinus 10 cases, sagittal sinus 3 cases, straight sinus 4 cases, and sinus confluences in 3 cases, which accompanied by the left upper extremity venous thrombosis one case, right ventricular mural thrombus one case and pulmonary embolism one case and left internal jugular venous thrombosis one case. 11 cases had CVST when nephrotic syndrome relapsed, and 3 cases complicated with CVST when nephrotic syndrome first happened. The common clinical characteristics were headache, nausea and vomiting, epilepsy was in 2 case and blurred vision in one case. The hypokalemia or hyponatremia due to diuretics was in 4 cases, mild edema of the lower limbs in 3cases and no edema in 11 cases. D-dimer were increased (1.88±1.82mg/l), Hb was high (16.2 ± 2.12g/dl), and anticardiolipin antibody IgM positive was in 2 cases.One case was given Batroxobin, the rest of all recieved low molecular weight heparin, unfractionated heparin anticoagulant and warfarin replaced, INR was maintained 2 to 3. Glycerol and fructose and diuretics was added to reduce intracranial pressure. After immunosuppressive therapy, keeping 20-30mg/d prednisone, all of the patients had got completely remission. During follow up, the thrombus of CVST disappeared in varied degree, none was dead, only one had thrombosis worse because of disesae relapse. Conclusion: CVST in nephrotic syndrome usually presents headache, nausea and vomiting associated with elevated D-dimer, and highly concentrated blood. Once patients have headache, seizure and hemiplegia, clinicians must be aware of CVST.Therefore it is necessary for them perform MRV angiography as soon as possible to make the right diagnosis.

Key words: nephrotic syndrome, cerebral venous sinus thrombosis, magnetic resonance venography