A case of idiopathic portal hypertension associated with rheumatoid arthritis

Abstract

A 53-year-old woman who had been diagnosed with rheumatoid arthritis was found to have thrombocytopenia, splenomegaly, and gastric varices. She was diagnosed as having idiopathic portal hypertension on the basis of liver biopsy and angiography. Treatment with prednisolone was not sufficiently effective for thrombocytopenia. After transabdominal devascularization with splenectomy, thrombocytopenia subsided and gastric varices disappeared. In this case, the autoimmune mechanism as well as hypersplenism was suspected of being involved in the mechanism of thrombocytopenia.     

Immunohistochemical and morphometric evaluation of COX-1 and COX-2 in the remodeled lung in idiopathic pulmonary fibrosis and systemic sclerosis ,

OBJECTIVE:

To study the expression of COX-1 and COX-2 in the remodeled lung in systemic sclerosis (SSc) and idiopathic pulmonary fibrosis (IPF) patients, correlating that expression with patient survival.

METHODS:

We examined open lung biopsy specimens from 24 SSc patients and 30 IPF patients, using normal lung tissue as a control. The histological patterns included fibrotic nonspecific interstitial pneumonia (NSIP) in SSc patients and usual interstitial pneumonia (UIP) in IPF patients. We used immunohistochemistry and histomorphometry to evaluate the expression of COX-1 and COX-2 in alveolar septa, vessels, and bronchioles. We then correlated that expression with pulmonary function test results and evaluated its impact on patient survival.

RESULTS:

The expression of COX-1 and COX-2 in alveolar septa was significantly higher in IPF-UIP and SSc-NSIP lung tissue than in the control tissue. No difference was found between IPF-UIP and SSc-NSIP tissue regarding COX-1 and COX-2 expression. Multivariate analysis based on the Cox regression model showed that the factors associated with a low risk of death were younger age, high DLCO/alveolar volume, IPF, and high COX-1 expression in alveolar septa, whereas those associated with a high risk of death were advanced age, low DLCO/alveolar volume, SSc (with NSIP), and low COX-1 expression in alveolar septa.

CONCLUSIONS:

Our findings suggest that strategies aimed at preventing low COX-1 synthesis will have a greater impact on SSc, whereas those aimed at preventing high COX-2 synthesis will have a greater impact on IPF. However, prospective randomized clinical trials are needed in order to confirm that.     

Shingles vaccine

Importance of the field: Herpes zoster or shingles is a condition with the potential to result in severe debilitation. It affects approximately 10 – 30% of the population. Until recently there were only treatments to shorten the duration and lessen the symptoms of herpes zoster, but no practical or approved method of prevention for susceptible immunocompetent adults. The live attenuated zoster vaccine (Zostavax^®, Merck & Co., Inc.) is effective in preventing shingles in individuals 60 years of age and older and recommended by the Center for Disease Control's (CDC) Advisory Committee for Immunization Practices (ACIP).

Areas covered in this review: Literature related to the live attenuated zoster vaccine is reviewed from its beginnings in the early 1970s through to the present.

What the reader will gain: Background information on herpes zoster and up to date information on the live attenuated zoster vaccine including pharmacology, efficacy and safety are covered. New areas of research in zoster vaccination are also discussed.

Take home message: The live attenuated zoster vaccine is an effective and well-tolerated method of preventing zoster and the potentially debilitating sequelae and is recommended for immunocompetent patients 60 years of age and older. Ongoing clinical trials are investigating new means of effective prevention.     

Chronic Administration of Ketamine for Analgesia

Use of the dissociative anesthetic ketamine in subanesthetic doses has demonstrated efficacy in neuropathic pain. This article reviews the scientific and clinical literature on ketamine. Mechanisms of both central and peripheral neuropathic pain are described. Studies of ketamine analgesia in postherpetic neuralgia, phantom pain, complex regional pain syndrome and cancer pain are reviewed. A range of administration methods for ketamine including neuroaxial administration are described.     

A Randomized,Prospective Study of Efficacy and Safety of Oral Tramadol in the Management of Post‐Herpetic Neuralgia in Patients from North India

Objective: To evaluate the safety and efficacy of oral tramadol therapy (50 to 200 mg/day) in the treatment for post‐herpetic neuralgia (PHN). Methods: The study was a prospective, single‐blind, non‐responder vs. responder, randomized trial conducted in 100 outpatients of PHN after oral administration of tramadol for 4 weeks. Those patients who had achieved 50% or greater pain relief after 14 days of oral tramadol treatment were categorized as responders and those reporting < 50% pain relief were categorized as non‐responders. Rescue analgesia was provided by the topical application of a cream consisting of the combination of 3.33% doxepin and 0.05% capsaicin to the affected areas of PHN patients of both groups for at least 14 days, along with tramadol therapy. The rescue analgesia was extended to 4 weeks in patients of the non‐responder group. The primary endpoints were measured using a Numerical Rating Scale (NRS) at rest and with movement. Secondary endpoints included additional pain ratings such as global perceived effect (GPE), Neuropathic Pain Symptom Inventory scores (NPSI), daily sleep interference score (DSIS), Quality of life (QOL) as per WHO QOL‐BREF Questionnaire scores, patient and clinician ratings of global improvement. The 2 groups were compared on the basis of pain intensity scores, encompassing primary as well as secondary endpoints, and QOL after 28 days of the treatment regimen. Results: Pain intensity scores measured by NRS (at resting and with movement), NPSI, and DSIS were consistently reduced (P < 0.001) over 28 days at varying intervals in both the groups, but the magnitude of reduction was higher in responders than non‐responders. A concomitant improvement (P < 0.001) was observed in GPE on days 3, 14, and 28 as compared to the respective baseline scores in both the groups. Although the WHO QOL‐BREF scores showed significant (P < 0.001) improvement in QOL of PHN patients at days 14 and 28 in both the groups, the magnitude of improvement was higher in responders as compared to non‐responders. Significant improvement in pain intensity scores and QOL in non‐responders is mainly attributed to the use of rescue analgesia for 28 days rather than recommended tramadol therapy. Conclusions: Treatment with tramadol 50 to 200 mg per day was associated with significant pain reduction in terms of enhanced pain relief, reduced sleep interference, greater global improvement, diminished side‐effect profile, and improved QOL in PHN patients from North India. Further categorization of PHN patients may be helpful so that additional or alternative therapy may be prescribed to non‐responders.     

J wave syndromes: Molecular and cellular mechanisms

An early repolarization (ER) pattern in the ECG, consisting of J point elevation, distinct J wave with or without ST segment elevation or slurring of the terminal part of the QRS, was long considered a benign electrocardiographic manifestation. Experimental studies a dozen years ago suggested that an ER is not always benign, but may be associated with malignant arrhythmias. Validation of this hypothesis derives from recent case–control and population-based studies showing that an ER pattern in inferior or infero-lateral leads is associated with increased risk for life-threatening arrhythmias, termed early repolarization syndrome (ERS). Because accentuated J waves characterize both Brugada syndrome (BrS) and ERS, these syndromes have been grouped under the heading of J wave syndromes. BrS and ERS appear to share common ECG characteristics, clinical outcomes, risk factors as well as a common arrhythmic platform related to amplification of I_to-mediated J waves. However, they differ with respect to the magnitude and lead location of abnormal J waves and can be considered to represent a continuous spectrum of phenotypic expression. Recent studies support the hypothesis that BrS and ERS are caused by a preferential accentuation of the AP notch in right or left ventricular epicardium, respectively, and that this repolarization defect is accentuated by cholinergic agonists. Quinidine, cilostazol and isoproterenol exert ameliorative effects by reversing these repolarization abnormalities. Identifying subjects truly at risk is the challenge ahead. Our goal here is to review the clinical and genetic aspects as well as the cellular and molecular mechanisms underlying the J wave syndromes.     

特发性肺纤维化患者辅助性T细胞1/辅助性T细胞2的研究

目的 对特发性肺纤维化(IPF)患者辅助性T细胞1(Th1)/辅助性T细胞2(Th2)进行研究,探讨Th1/Th2能否反映疾病的严重性,能否预测疾病的进展.方法 入选83例IPF患者,应用酶联免疫吸附测定法检测所有受试者血清、支气管肺泡灌洗液(BALF)中γ干扰素(IFNγ)、白细胞介素-4(IL-4)水平,并进行相关性分析.结果 (1)基线资料:IPF患者血清、BALF中IFNγ/IL-4比值(0.8±0.3;0.8±0.3)较对照组(1.4±0.2;1.4±0.2)显著降低;血清、BALF中IFNγ/IL-4比值与病程、呼吸困难评分、第1秒用力呼气容积(FEV1)占预计值百分比、用力肺活量(FVC)占预计值百分比、肺总量(TLC)占预计值百分比、最大去氧饱和度、6分钟步行距离(6MWD)、CT间质纤维化评分显著相关(血清:r值分别为-0.426、-0.623、0.487、0.455、0.517、-0.491、0.263、-0.569,P值均＜0.05;BALF:r值分别为-0.434、-0.637、0.480、0.456、0.501、-0.507、0.253、-0.605,P值均＜0.05);血清中IFNγ/IL-4比值与CT磨玻璃影评分呈正相关(r=0.340,P＜0.01).(2)随访:IPF患者血清中IFNγ、IL-4、IFNγ/IL-4在糖皮质激素治疗者与非糖皮质激素治疗者间差异无统计学意义.随访6个月后的呼吸困难评分、FEV1占预计值百分比、TLC占预计值百分比、CT磨玻璃影评分、CT间质纤维化评分、IFNγ和IL-4较基线值显著恶化.血清中IFNγ/IL-4比值变化与呼吸困难评分、FVC占预计值百分比、TLC占预计值百分比、肺一氧化碳弥散量占预计值百分比、6MWD、CT间质纤维化评分的变化显著相关(r值分别为-0.297、0.462、0.315、0.353、0.420、-0.307,P值均＜0.05).结论 IPF患者血清和BALF中Th1/Th2存在失衡,Th1/Th2可能反映IPF的严重性,随访时血清中Th1/Th2的变化可能会预测IPF的疾病进展.     

特发性左心室心动过速致心律失常基质的电解剖差异

目的 探索特发性左心室心动过速（ILVT）和房室折返性心动过速（AVRT）患者左心室传导系统和缓慢传导区电解剖变异情况.方法 选取2009年5月至2011年12月20例成功消融的ILVT患者（ILVT组）,年龄20～51（37±7）岁,男16例;26例AVRT患者（对照组）,年龄25～51（38±8）岁,男20例,窦性心律下分别建立左心室三维电解剖标测,标记左心室传导系统、缓慢传导区及其交汇区,分析变异情况.ILVT组患者依据消融关键区和拖带刺激进行消融.结果 根据浦肯野电位分布将传导系统变异分为3个亚型：两分支、三分支和扇形分布于左心室间隔,两组间各分支长度差异无统计学意义（P＞0.05）.缓慢传导区在ILVT中亦存在变异：17例位于后下间隔,1例位于下间隔近心尖处,2例于中、后间隔处.浦肯野电位和舒张期电位间存在面积约（1.5±0.4） cm2的交汇区,该处拖带和消融均取得成功.6例AVRT患者于后下间隔处记录到缓慢传导区,长度[12.0～28.7 （20.4±4.7） mm对11.8～ 20.3（16.1±3.3） mm,t=2.1,P=0.048]、面积[1.6～ 3.5（2.5±0.5） cm2对1.4～2.1 （1.8±0.3） cm2,t=3.0,P=0.006]显著小于ILVT组.结论 左心室传导系统和缓慢传导区存在多种变异,使ILVT折返机制更为复杂.     

大剂量丙种球蛋白治疗婴幼儿特发性心内膜弹力纤维增生症临床分析

目的:分析大剂量丙种球蛋白(IVIG)治疗婴幼儿特发性心内膜弹力纤维增生症的临床疗效。方法:回顾性分析1984年8月至2010年2月,在首都医科大学附属北京安贞医院小儿心脏科住院的53例特发性婴幼儿心内膜弹力纤维增生症患儿临床资料,应用大剂量丙种球蛋白治疗组患儿24例(2 g.kg-1.次-1,分2 d使用,每月1次连用3次,以后每3个月重复1次)未使用大剂量丙种球蛋白治疗组患儿29例作为对照。对比两组治疗前后1年的临床疗效。结果:两组患者发病月龄、发病时左心室射血分数、舒张末径之间差异无统计学意义(P>0.05),治疗后1年左心室射血分数、舒张末径的改善情况,两组之间差异无统计学意义(P>0.05),但IVIG组环磷酰胺的使用率明显低于非IVIG组,分别为(33.3%vs.62.1%,χ2=4.339,P=0.037)差异有统计学意义。结论:大剂量丙种球蛋白治疗婴幼儿特发性心内膜弹力纤维增生症有一定临床疗效,并能显著降低环磷酰胺的使用率。