胆道手术后继发性硬化性胆管炎诊治体会

目的探讨胆道手术后继发性硬化性胆管炎(SSC)的诊断和治疗。方法回顾性分析2000年1月—2008年6月行胆道手术后出现的7例继发性硬化性胆管炎患者的临床资料。结果患者既往均有反复发作的胆管炎病史,均有胆道手术治疗史。确诊SSC后手术治疗2例,保守治疗5例,其中1例行内窥镜下十二指肠乳头括约肌切开治疗,1例行肝移植治疗。治愈2例(2/7),死亡2例(2/7),3例尚在治疗中。结论胆道手术后继发性硬化性胆管炎的预后较差,预防是关键,早期诊断及合理治疗有一定的疗效。

脾脏硬化性血管瘤样结节性转化2例报告及文献复习

目的探讨脾脏硬化性血管瘤样结节性转化(SANT)的临床、病理、诊断与治疗。方法对2例SANT的临床、病理特征及免疫组化表达及术后随访情况进行分析并复习文献。结果显微镜下见肿物呈多个血管瘤样肉芽肿性结节,结节间为致密或黏液样变的纤维结缔组织,结节中瘤细胞呈卵圆形、短梭形,胞质丰富、可见分枝状及裂隙样不规则毛细血管,间质多量浆细胞、淋巴细胞浸润及红细胞外渗。细胞形态温和,无异型性,核分裂很少见。免疫表型复杂,无特征性表现。2例均行脾切除术治疗。术后随访6个月—1年余均无复发与转移。结论 SANT是一种罕见的具有特征性病理改变的良性增生性病变,临床上易与脾脏恶性肿瘤混淆,病理上易与炎性假瘤、错钩瘤等混淆,脾切除术是有效的治疗方法,预后良好。

急诊冠状动脉内支架置入术全程介入护理60例体会

经皮冠状动脉介入治疗（percutaneouscoronary intervention，PCI）已成为再灌注抢救治疗急性心肌梗死（acutemyocard ialinfarction，AMI）的重要方法。它可以更早、更完全的开通梗死相关血管，使梗死心肌得到再灌注，减少坏死心肌细胞数量，保持心室功能，使患者症状尽快缓解，稳定病情，改善预后。

Pathogenesis of primary biliary cirrhosis: A unifying model

Primary biliary cirrhosis （PBC） is a disease of unknown etiology leading to progressive destruction of small intrahepatic bile ducts and eventually to liver cirrhosis and failure. It is characterised by female predominance and serum auto-antibodies to mitochondrial antigens targeting the E2 components of the 2-oxoacid dehydrogenase complex. Although they are associated with disease pathogenesis, no concrete evidence has been presented so far. Epidemiological data indicate that a geographical clustering of cases and possible environmental factors are implicated in pathogenesis. A number of genetic factors play a role in determining disease susceptibility or progression, although no definitive conclusion has been reached so far. A key factor to immune pathogenesis is considered to be the breakdown of immune tolerance, either through molecular mimicry or through the so called determinant density model. In this review, the available data regarding the pathogenesis of primary biliary cirrhosis are described and discussed. A new unifying hypothesis based on early endothelin overproduction in primary biliary cirrhosis （PBC） is presented and discussed.

Idiopathic sclerosing encapsulating peritonitis (or abdominal cocoon):A report of 5 cases

Sclerosing encapsulating peritonitis (SEP) is a rare cause of intestinal obstruction that is characterized by a thick grayish-white fibrotic membrane encasing the small bowel. SEP can be classified as idiopathic,also known as abdominal cocoon,or secondary. It is difficult to make a definite pre-operative diagnosis. We experienced five cases of abdominal cocoon,and the case files were reviewed retrospectively for the clinical presentation,operative findings and outcome. All the patients presented with acute,subacute and chronic intestinal obstruction. Computed tomography (CT) showed characteristic findings of small bowel loops congregated to the center of the abdomen encased by a soft-tissue density mantle in four cases. Four cases had an uneventful post-operative period,one case received second adhesiolysis due to persistent ileus. The imaging techniques may facilitate pre-operative diagnosis. Surgery is important in the management of SEP.

Comparison of the liver stiffness measurement by transient elastography with the liver biopsy

AIM: To compare the liver stiffness (LS) measurement by transient elastography (TE) to the liver biopsy (LB)-considered the "gold standard" in the evaluation of patients with chronic hepatitis C. METHODS: During a period of 12 mo, we evaluated 199 consecutive patients with chronic hepatitis due to hepatitis C virus (HCV), in which LB and LS assessments (by means of TE) were performed during the same session. RESULTS: Out of 199 patients, a valid measurement of the LS could not be obtained in 8. The mean value of LS in the cohort of 191 valid measurements was 8.45 ± 4.96 kPa, ranging from 2.3 to 38 kPa. The mean value of LS in patients with signifi cant fi brosis at biopsy (161 patients with F ≥ 2 according to Metavir) was 9.02 ± 5.15 kPa, significantly higher than in patients with no or mild fi brosis (30 patients with F < 2 Metavir): 5.39 ± 1.81 kPa (P < 0.0001). For a cut- off value of 6.8 kPa, the LS had a PPV of 98%, a NPV of 30.1%, a sensitivity of 59.6% and a specificity of 93.3% for the presence of signifi cant fi brosis (at least F2 Metavir), with a diagnostic performance of 77.3% (AUROC 0.773). Using this cut-off value, we reached the best discrimination between absence of fibrosis/ mild fibrosis (F < 2 Metavir) and the presence ofmoderate to severe fi brosis (F ≥ 2 Metavir). CONCLUSION: In patients with chronic hepatitis due to HCV, a cut-off value of 6.8 kPa measured by TE can differentiate between significant fibrosis and absent or mild fi brosis, with a PPV of 98%, a NPV of 30.1%, a sensitivity of 59.6%, a specificity of 93.3%, and a diagnostic performance of 77.3%.

Primary sclerosing cholangitis: Updates in diagnosis and therapy

Primary sclerosing cholangitis (PSC) is a chronic cholestatic syndrome of unknown origin mostly found in males, and characterized by diffuse inflammation and fibrosis of both intra- and extra-hepatic bile ducts. So far, PSC is considered as an autoimmune hepatobiliary disease. In most cases the progression of PSC towards liver cirrhosis and liver failure is slow but irreversible, and liver transplantation is currently the only definitive treatment. In recent years,PSC has been an area of active research worldwide with great interest in etiology, pathogenesis, diagnosis, and therapeutic options such as hydrophilic ursodeoxycholic acid and immunosuppressive agent tacrolimus. Recent updates on clinical and therapeutic aspects of PSC are discussed in the present review.

Future prospectives for the management of chronic hepatitis B

Chronic hepatitis B virus infection affects about 400 million people around the globe and causes approximately a million deaths a year. Since the discovery of interferon-α as a therapeutic option the treatment of hepatitis B has evolved fast and management has become increasingly complicated. The amount of viral replication reflected in the viral load （HBV-DNA） plays an important role in the development of cirrhosis and hepatocellular carcinoma. The current treatment modalities for chronic hepatitis B are immunomodulatory （interferons） and antiviral suppressants （nucleoside and nucleotide analogues） all with their own advantages and limitations. An overview of the treatment efficacy for both immunomodulatory as antiviral compounds is provided in order to provide the clinician insight into the factors influencing treatment outcome. With nucleoside or nucleotide analogues suppression of viral replication by 5-7 log10 is feasible, but not all patients respond to therapy. Known factors influencing treatment outcome are viral load, ALT levels and compliance. Many other factors which might influence treatment are scarcely investigated. Identifying the factors associated with response might result in stopping rules, so treatment could be adapted in an early stage to provide adequate treatment and avoid the development of resistance. The efficacy of compounds for the treatment of mutant virus and the cross-resistance is largely unknown. However, genotypic and phenotypic testing as well as small clinical trials provided some data on efficacy in this population. Discontinuation of nucleoside or nucleotide analogues frequently results in viral relapse; however, some patients have a sustained response. Data on the risk factors for relapse are necessary in order to determine when treatment can be discontinued safely. In conclusion： chronic hepatitis B has become a treatable disease; however, much research is needed to tailor therapy to an individual patient, to predict the sustainability of response and determine the best treatment for those failing treatment.

Actigraphy: A new diagnostic tool for hepatic encephaiopathy

AIM： To assess the actigraphy, an ambulatory and continuous monitoring of wrist motor activity fitted to study sleep/wake patterns in hepatic encephalopathy （HE）. METHODS： Twenty-five cirrhotic patients （17 M, 8 F, mean age 56± 11 years, 24/25 alcoholic, Child-Pugh A, B, C： 2, 6, 17） were included. The patients were classified into 3 groups： stage 0 group （n = 12）, stage 1-2 group （n = 6）, and stage 3-4 group （n = 7） of encephalopathy. Over three consecutive days, patients had clinical evaluation 3 times a day with psychometric test, venous ammoniemia, flash visually evoked potentials （VEP）, electroencephalogram and continuous actigraphic monitoring for 3 d, providing 5 parameters： mesor, amplitude, acrophase, mean duration of activity （MDAI） and inactivity （MDII） intervals. RESULTS： Serum ammonia and VEP did not differ among the 3 groups. Electroencephalography mean dominant frequency （MDF） correlated significantly with clinical stages of HE （r=0.65, P=0.003）. The best correlation with HE stage was provided by actigraphy especially with MDAI （r= 0.7, P〈10^-4） and mesor （r= 0.65, P〈 10^-4）. MDAI correlated significantly with MDF （r= 0.62, 0.004） and was significantly shorter in case of HE compared to patients without HE （stage 0： 5.33± 1.6 min; stage 1-2：3.28±1.4 min; stage 3-4：2.52±1.1 min; P〈0.05）. Using a threshold of MDAI of less than 4.9 min, sensitivity, specificity, positive predictive value, negative predictive value for HE diagnosis were 85%, 67%, 73% and 80%, respectively. CONCLUSION： Actigraphy may be an objective method to identify HE, especially for early HE detection. Motor activity at the wrist correlates well with clinical stages of HE. MDAI and mesor are the most relevant parameters.

A REVIEW ON TRADITIONAL CHINESE MEDICINE IN PREVENTION AND TREATMENT OF MULTIPLE SCLEROSIS

Multiple sclerosis(MS),a diseaseaffecting the central nervous system,ischaracterized by patches of demyelinationand sclerosis of gliosis desseminatedthroughout the white matter of brain andspinal cord.In 1968,Charcot firstdescribed its clinical and pathologicalcharacteristics.Its incidence is very high,especially in Europe and America.Thereis no epidemic data of this

Interferon-γ mRNA attenuates its own translation by activating PKR： A molecular basis for the therapeutic effect of interferon-β in multiple sclerosis

PKR, the interferon （IFN）-inducible protein kinase activated by double-stranded RNA, inhibits translation by phosphorylating the initiation factor eIF2α chain. Uniquely, human IFN-γ mRNA uses local activation of PKR in the cell to control its own translation yield. IFN-γ mRNA activates PKR through a structure in its 5＇- region harboring a pseudoknot which is critical for PKR activation. Mutations that impair pseudoknot stability reduce the ability of IFN-γ mRNA to activate PKR and strongly increase its translation efficiency. The cis-acting RNA element in IFN-γ mRNA functions as a biological sensor of intracellular PKR levels. During an immune response, as IFN-γ and other inflammatory cytokines build up in the cell＇s microenvironment, they act to induce higher levels of PKR in the cell, resulting in a more extensive activation of PKR by IFN-γ mRNA. With the resulting phosphorylation of eIF2α, a negative feedback loop is created and the production of IFN-γ is progressively attenuated. We propose that the therapeutic effect of IFN-β in multiple sclerosis may rest, at least in part, on its exquisite ability to induce high levels of PKR in the cell and thereby to limit IFN-γ mRNA translation through this negative feedback loop, blocking the excessive IFN-γ gene expression that precedes clinical attacks.

Application of drug-coated balloon in coronary artery intervention： challenges and opportunities

In recent decades, the outcomes of coronary heart disease （CHD） have markedly improved, which can be partly attributed to the use of novel drugs （especially statins and antiplatelet drugs） and partly to the evolution ofpercutaneous coronary intervention （PCI）. From percutaneous transluminal coronary angioplasty to bare-metal stent and then to drug-eluting stent, every step of PCI is attractive to interventional cardiologist, great progress has been made for patients with CHD. In the past few years, some successor devices for treating CHD have cmerged. Undoubtedly, drug-coated balloon （DCB）, which was recommended by 2014 ESC Guidelines on myocardial revascularization, is a ＂shining star＂ among them. DCB involves a semi-compliant angioplasty balloon coated with an anti-proliferative agent that can exert antirestenotic efficacy by permeating into the vessel wall during balloon contact. This review discusses the conception and merits, preclinical data, emerging clinical indications, and results from clinical trials of this novel interventional technology. Although DCB has shown authentic efficacy in the treatment ofin-stent restenosis, its use in de novo coronary lesions is still in dispute. Hence, concerns and the future direction of DCB are also covered in this paper.

Lowering vascular calcification burden in chronic kidney disease:Is it possible?

High prevalence of atherosclerosis and arterial calcifca-tion in chronic kidney disease is far beyond the explana-tion by common cardiovascular risk factors such as aging diabetes, hypertension and dyslipidemia. The magnitude of coronary artery calcification is independently and inversely associated with renal function. In addition to cardiovascular risk factors, other chronic kidney disease-related risks such as phosphate retention, excess of calcium and prolonged dialysis vintage also contribute to the development of vascular calcifcation. Strategies to lower vascular calcifcation burden in chronic kidney dis-ease population should include minimizing chronic kidney disease and atherosclerotic risk factors. Current therapies available are non-calcium containing phosphate bind-ers, low dose active vitamin D and calcimimetic agent. The role of bisphosphonates in vascular calcification in chronic kidney disease population remains unclear. Preliminary data on sodium thiosulfate are promising, however, larger studies on effcacy and patient outcomes are necessary. Several large randomized controlled trials have confrmed the lack of beneft of statin in attenuat-ing the progression of vascular calcifcation.

TREATMENT OF NINETY-FIVE CASES OF MULTIPLESCLEROSIS WITH ACUPUNCTURE

From December of 1998 to August of 2002, the author treated 95 cases of multiple sclerosis with acupuncture. The observation has confirmed that acupuncture is a very effective method for treating this disease, especially to sclerosis patients in the early stage; and furthermore it can slow down the development of the pathological conditions of the patients. Thus it is an important therapeutic method worthy of the choice by European and American clinical doctors.

Development of autoimmune hepatitis type 1 after pulsed methylprednisolone therapy for multiple sclerosis: A case report

A 43-year-old woman with multiple sclerosis （MS） was treated with pulsed methylprednisolone and interferon 13 at a hospital. Four weeks after initiating treatment, liver dysfunction occurred and she was referred and admitted to our hospital. Clinical and laboratory findings were consistent with and fulfilled the criteria for drug-induced hepatitis, but not for autoimmune hepatitis （AIH）. She was successfully treated with corticosteroids. As ataxia developed after i year, she was treated with pulsed methylprednisolone for 3 days, then readmitted to our hospital when liver dysfunction occurred. Clinical and laboratory findings led to the diagnosis of AIH. To the best of our knowledge, this is the second case of AIH developed after pulsed methylprednisolone for MS.

Treatment of multiple sclerosis by transplantation of neural stem cells derived from induced pluripotent stem cells

Multiple sclerosis(MS) is an autoimmune disease of the central nervous system(CNS), with focal T lymphocytic infiltration and damage of myelin and axons. The underlying mechanism of pathogenesis remains unclear and there are currently no effective treatments. The development of neural stem cell(NSC) transplantation provides a promising strategy to treat neurodegenerative disease. However, the limited availability of NSCs prevents their application in neural disease therapy. In this study, we generated NSCs from induced pluripotent stem cells(iPSCs) and transplanted these cells into mice with experimental autoimmune encephalomyelitis(EAE), a model of MS. The results showed that transplantation of iPSC-derived NSCs dramatically reduced T cell infiltration and ameliorated white matter damage in the treated EAE mice. Correspondingly, the disease symptom score was greatly decreased, and motor ability was dramatically rescued in the iPSC-NSC-treated EAE mice, indicating the effectiveness of using iPSC-NSCs to treat MS. Our study provides pre-clinical evidence to support the feasibility of treating MS by transplantation of iPSC-derived NSCs.

Variceal bleeding in cirrhotic patients

Variceal bleeding is one of the major causes of death in cirrhotic patients.The management during the acute phase and the secondary prophylaxis is well defined.Recent recommendations(2015 Baveno VI expert consensus)are available and should be followed for an optimal management,which must be performed as an emergency in a liver or general intensive-care unit.It is based on the early administration of a vasoactive drug(before endoscopy),an antibiotic prophylaxis and a restrictive transfusion strategy(hemoglobin target of 7 g/dL).The endoscopic treatment is based on band ligations.Sclerotherapy should be abandoned.In the most severe patients(Child Pugh C or B with active bleeding during initial endoscopy),transjugular intrahepatic portosystemic shunt(TIPS)should be performed within 72 hours after admission to minimize the risk of rebleeding.Secondary prophylaxis is based on the association of non-selective beta-blockers(NSBBs)and repeated band ligations.TIPS should be considered when bleeding reoccurs in spite of a well-conducted secondary prophylaxis or when NSBBs are poorly tolerated.It should also be considered when bleeding is refractory.Liver transplantation should be discussed when bleeding is not controlled after TIPS insertion and in all cases when liver function is deteriorated.