经颈静脉途径肝内门体分流术（TIPS）治疗门脉高压食管静脉血张出血的临床评估

ＴＩＰＳ术治疗５０例，其中４９例为肝炎后肝硬化，１例为门静脉海绵窦样变，４５例有反复呕血及黑便史，５例无上消化道出血史，均有中－重度食管静脉曲张。１６例有中－大量腹水。结果：（１）４５例门静脉建立有效分流，成功率为９０％。门静脉主干压力从术前的３．９５±０．８５ｋＰａ降至２．３３±０．７２ｋＰａ（Ｐ＜０．００１）。（２）胃镜检查ＴＩＰＳ术后食管静脉曲张消失和基本消失率为４１．４３％，减轻为３７．９％。（３）术后腹水消失或减轻为６２％。（４）并发症与操作有关的为１２％，总发生率为３６％。（５）术后肝功能观察：血清总胆红质升高，血清白蛋白降低。随访１９９３年３月至１９９４年３月置入Ｇｉａｎｔｕｒｃ－ＲｏｓｃｈＺ型、网型支架的２４例患者，随访３个月至２２个月者２０例，支架狭窄、闭塞率半年为２０％，１年为５５％；复发出血８例为４０％。１９９４年４月至１９９５年１月置入ｗａｌｌｓｔｅｎｔ支架的２１例中，尚无支架闭塞及反复出血。全组现共死亡８例，死亡率为１５．６％     

基于PACS医学影像学系列课程网络化教学平台的建设与实践

目的建立基于PACS的医学影像资源库,并以此库作为共享资源,构建一个B/S结构的基于Web的可视化教学与学习平台。方法由专家选取医院PACS系统中典型病案,导入教学专用PACS影像库,运用C#编程语言及SQL Server数据库管理等计算机技术实现设计方案。结果实现了以基于PACS的《断层解剖学》课程为实例的网络化学习平台的建设。结论该教学平台为医学影像学课程的学习、实践、考核提供了新的手段,对于提高医学影像学的总体教学质量具有重要意义。     

Diagnostic value of single‐photon–emission computed tomography in severe central nervous system involvement of systemic lupus erythematosus: A case‐control study

Objective

To evaluate the diagnostic value of single‐photon–emission computed tomography (SPECT) in severe central nervous system (CNS) involvement of systemic lupus erythematosus (SLE).

Methods

Forty‐three patients with SLE, including 22 with CNS‐SLE and 21 with non–CNS‐SLE, underwent SPECT and magnetic resonance imaging (MRI) examinations. SPECT was repeated 1–2 months after treatment in patients with abnormal findings.

Results

SPECT and MRI abnormalities were detected in 20 (90.9%) and 10 (45.5%) of the 22 patients with CNS‐SLE, respectively (P < 0.01). For 4 patients with cerebral infarction or hemorrhage, SPECT was equally as sensitive as MRI (100%). For the patients with CNS‐SLE with diffuse presentations, SPECT was more sensitive than MRI in revealing abnormalities (16 [88.9%] of 18 patients versus 6 [33.3%] of 18 patients; P < 0.01). In 19 (95.0%) patients, the abnormal SPECT finding manifested as moderate to severe perfusion defect (15 in frontal lobe, 11 in parietal lobe, 11 in basal ganglia, 3 in temporal lobe, and 17 in multiple regions). Although mild perfusion defect was also detected in 4 (19.0%) of the patients with non–CNS‐SLE, it only involved a single region and spared the frontal and parietal lobes. Repeated SPECT after treatment showed that perfusion defect had improved significantly or even disappeared in 11 (84.6%) of 13 patients with diffuse CNS‐SLE with abnormal findings before treatment.

Conclusion

Moderate to severe perfusion defect in SPECT involving multiple regions, especially in the frontal and parietal lobes and basal ganglia, in patients with lupus suggests CNS involvement. SPECT is more sensitive than MRI in revealing damage in diffuse CNS‐SLE, and is useful in followup, especially for monitoring disease severity and guiding treatment.

     

基于《支气管哮喘基层诊疗指南（实践版·2018）》构建支气管哮喘个体化用药知识库

支气管哮喘是一种气道慢性炎性、高度异质性、多基因遗传性疾病，病因复杂，合并症多，防治效果常取决于是否进行了个体化的健康管理。为了与国际接轨，更新的《支气管哮喘基层诊疗指南（实践版·2018年版）》提出了哮喘早期干预思路，优化了哮喘用药方案，强化了哮喘规范化管理理念。为了推进基层哮喘个体化用药管理，本研究参照新版指南中的药物治疗路径"初始治疗、长期治疗方案、降级治疗原则"，以"一厂、一品、一规"个性化说明书为基础，通过信息技术数字化采集用药知识，构建了"预判、预警、预知"功能的搜索引擎，为医师提供诊前、诊中、诊后的线上服务，实现为社区患者提供哮喘个体化用药监测与管理。     

急性胆源性胰腺炎的内镜下介入治疗

目的 :综合评价早期内镜下介入治疗急性胆源性胰腺炎的方法、疗效及并发症等。方法 :10 3例胆源性胰腺炎患者中 ,5 7例在 72h内行早期内镜下介入治疗 ,4 3例保守治疗 ,保守治疗中的 19例择期内镜检查及治疗 ,3例外科手术治疗。结果 :早期内镜治疗组 80 .70 %患者临床症状得到迅速缓解 ;保守治疗组 6 2 .79%也可迅速缓解。全部内镜治疗后 3例出现胰腺炎加重。结论 :早期内镜介入治疗对于急性胆源性胰腺炎是一种有效而安全的方法 ,对于有明显黄疸、感染、结石嵌顿的病例应急诊内镜下治疗 ;对于病情较轻、黄疸不重的患者可先保守治疗后再行择期内镜治疗     

Cerebellar involvement in patients withprimary Sjögren’s syndrome: diagnosis and treatment

The aim of this study is to describe the clinical features of cerebellar involvement in patients with primary Sjögren’s syndrome (pSS). We retrospectively analyzed the manifestations, treatments, and outcomes in patients with pSS-cerebellar complication in Peking Union Medical College Hospital and cases reported in literature. Altogether 13 patients were identified. They were 2 males and 11 females with a mean age at disease onset of 45.2?±?14.6 years. Nine (69.2%) patients went to the clinic because of ataxia, and pSS was not suspected until accidental screening for autoantibodies. Dysarthria (7, 59.8%), limb tremor (4, 30.8%), and nystagmus (2, 15.4%) were the rest symptoms related to cerebellum. Of the patients, 81.8% (9/11) had abnormal cerebrospinal fluid findings, and 11 patients (84.6%) had cerebellar atrophy in the brain MRI. Dry eyes and dry mouth were detected in 9 (69.2%) and 7 (59.8%) patients, while positive objective xerostomia and ocular test in 82.5% (7/8) and 100% (10/10) of the patients, respectively. Anti-Ro/SSA antibody was positive in 12 (92.3%) and anti-La/SSB in 6 (46.2%) patients. Glucocorticoids were applied in 12 patients (92.3%). Cyclophosphamide (3, 20.1%), mycophenolatemofetil (1, 7.7%), and hydroxychloroquine (4, 30.8%) were chosen as immunosuppressants or anti-inflammatory drug. During a median follow-up of 9 months (range, 1–18 months), 8 (61.5%) patients remained stable, 3 (20.1%) patients were in remission, and 2 (15.4%) patients were in progression. Clinical cerebellar complication secondary to pSS was rare, and sometimes pSS was not suspected until accidental screening for autoantibodies. Because the onset of cerebellar manifestation is often insidious and rapid deteriorates, early diagnosis and empirical aggressive glucocorticoid treatment is warranted.     

The Clinical Characteristics of Primary Sjogren's Syndrome With Neuromyelitis Optica Spectrum Disorder in China: A STROBE-Compliant Article

The aim of the present study was to analyze the clinical characteristics of primary Sjogren''s syndrome (pSS) with neuromyelitis optica spectrum disorder (NMOSD). We retrospectively reviewed the medical records of 616 patients who were admitted to the Peking Union Medical College Hospital from 1985 to 2013. Of these patients, 43 developed NMOSD. The median duration of symptoms was 60 months and 72% of the patients experienced neurological complications onset in the pSS with NMOSD group. Twenty-one out of 43 patients had neuromyelitis optica (NMO), and 22 exhibited a limited form of NMO. Serum anti-aquaporin-4 (AQP4) antibody positivity was detected in 89.3% of the patients. A total of 60.5% of the patients (26 patients) complained of dry mouth, 72.1% were positive for objective xerostomia, 53.5% complained of dry eyes, and 74.4% had a positive ocular test. Biopsy of the minor salivary glands was performed in 33 patients, 28 of whom (84.8%) had a lymphocytic focus score of ≥1. Anti-Ro/SSA or anti-La/SSB antibodies were detected in 41 patients (95.3%). Compared with the pSS patients without NMOSD, the incidences of xerophthalmia, xerostomia, arthritis, interstitial lung disease, and renal tubular acidosis were significantly lower in the patients with NMOSD.NMOSD is a neurologic complication of pSS. The presence of anti-AQP4 antibody may be a predictor for pSS patients with NMOSD. Neurological manifestations are prominent in these patients. In clinical scenarios involving pSS or NMOSD, rheumatologists and neurologists should be aware of this association and perform the appropriate tests.