特发性视网膜前膜的发病机制及治疗进展

特发性视网膜前膜(iERM)在老年人群中常见,对于症状严重者可能出现视物模糊、视物扭曲、单眼复视和视物变大或变小等症状,严重影响患者的日常生活,因而了解其发病机制和有效治疗具有重要的意义。     

喜炎平注射液佐治儿童肺炎支原体肺炎的临床疗效评价及对血清炎症因子水平的影响

目的　观察喜炎平注射液佐治儿童肺炎支原体肺炎（MPP）的临床疗效及对患儿血清中相关炎症因子水平的影响。方法　随机选取2017年9月至2018年10月辽宁中医药大学附属医院儿科收治的53例MPP患儿为疾病组,将疾病组再随机分为对照组27例和观察组26例,随机选取同期15例健康体检儿童为正常组。对照组给予笔者医院常规中西医结合治疗方案,观察组在对照组基础上加用喜炎平注射液静点。对比观察组与对照组的病证总分变化、中医证候疗效及各组患儿治疗前后血清中γ干扰素（IFN-γ）、肿瘤坏死因子α（TNF-α）、白细胞介素（IL）-4、IL-17、IL-18炎症因子水平变化。结果　治疗后,两组患儿的临床症状及中医证候疗效均明显好转,但两组比较差异无统计学意义（P＞0.05）。两组患儿病证总积分均明显减少（P＜0.05）,观察组优于对照组,但差异无统计学意义（P＞0.05）。治疗前,与正常组比较,疾病组患儿血清中各炎症因子均升高,且差异均有统计学意义（P＜0.05）;与治疗前比较,观察组和对照组患儿治疗后各炎症因子均降低,其中IFN-γ、TNF-α、IL-4、IL-17差异均有统计学意义（P＜0.05）。与对照组治疗后比较,观察组治疗后TNF-α、IL-4、IL-17、IL-18炎症因子降幅更低,其中TNF-α、IL-4、IL-17差异有统计学意义（P＜0.05）。而IFN-γ水平相对升高,但差异无统计学意义（P＞0.05）。结论　喜炎平注射液佐治儿童MPP安全有效,可通过调节MPP患儿血清中IFN-γ、TNF-α、IL-4、IL-17、IL-18炎症因子水平,抑制MPP炎症反应,从而提高临床治疗效果。     

Paraneoplastic opsoclonus myoclonus syndrome associated with inflammatory myofibroblastic tumor in a pediatric patient

Opsoclonus myoclonus syndrome (OMS) is a rare neurological syndrome caused by a paraneoplastic autoimmune process that affects children with neuroblastic tumors. Treatment includes corticosteroids, intravenous gamma globulin (IVIG), rituximab, and other immunosuppressive therapies. Here, we describe a patient diagnosed with OMS associated with a localized inflammatory myofibroblastic tumor. The patient has no evidence of tumor recurrence following surgical resection with 8‐month follow‐up. The neurologic symptoms resolved with corticosteroids and IVIG. This case demonstrates that in children, neoplasms other than neuroblastoma may be associated with this paraneoplastic syndrome, and highlights the importance of evaluating patients with OMS for underlying malignancies.     

石膏矫形治疗幼儿特发性脊柱侧弯的临床研究

目的介绍简易石膏床方法行石膏矫形治疗幼儿特发性脊柱侧弯,并进行效果评价。方法选取2014年9月至2016年9月于河北省儿童医院住院治疗的25例幼儿特发性脊柱侧弯病例作为研究对象,平均年龄29.7个月(17~53个月),其中男童10例,女童15例,共行石膏矫形105次。应用去旋转石膏矫形方法并结合我院实际情况采用简易石膏床对患儿进行石膏矫形,测量并记录石膏矫形前后脊柱正位X线片上Cobb角改变情况,并对所有患儿按照性别及年龄是否≤2岁进行分组分析。结果 25例患儿石膏矫形治疗前脊柱侧弯Cobb角平均角度为(39.1±9.1)°,经过第1次石膏矫形后平均角度为(22.3±5.0)°,末次随访时平均角度为(20.3±4.3)°。第1次石膏矫形后Cobb角与矫形前相比差异有统计学意义(t=8.825,P<0.05)。随访终点时Cobb角与矫形前相比差异有统计学意义(t=9.653,P<0.05)。不同性别分组随访终点时Cobb角矫正度数差异无统计学意义(t=0.070,P=0.945)。不同年龄分组随访终点时Cobb角矫正度数差异无统计学意义(t=-0.733,P=0.471)。结论我们采用的简易石膏床方法结合去旋转技术进行石膏矫形可以纠正幼儿特发性脊柱侧弯患儿的Cobb角,性别和年龄因素未对矫正效果产生影响。     

Small fiber neuropathy in unexpected clinical settings: a review

Small fiber neuropathy (SFN) is being recognized with increasing frequency in neuromuscular practice due to improved diagnostic techniques. Although there are some common etiologies, up to one-third of cases are considered idiopathic. In recent years, several disorders have unexpectedly been reported in association with SFN, on clinical grounds and complementary investigations, including quantitative sensory testing, intraepidermal nerve fiber density and confocal corneal microscopy. Knowledge of these disorders is important in clinical practice as increased awareness enables prompt diagnosis of SFN in these settings and early optimal therapeutic management of affected patients. Furthermore, these new developments may lead to a better understanding of the pathophysiologic mechanisms underlying SFN in these different disorders as well as, in some cases, an expanded spectrum of affected organs and systems. This article reviews these reported associations, their possible pathophysiologic bases, and the potential resulting management implications.     

Comparative value and determinants of suitability of outcome measures in treated chronic inflammatory demyelinating polyneuropathy

Coronavirus disease 2019 has created unprecedented challenges for amyotrophic lateral sclerosis (ALS) clinical care and research in the United States. Traditional evaluations for making an ALS diagnosis, measuring progression, and planning interventions rely on in-person visits that may now be unsafe or impossible. Evidence- and experience-based treatment options, such as multidisciplinary team care, feeding tubes, wheelchairs, home health, and hospice, have become more difficult to obtain and in some places are unavailable. In addition, the pandemic has impacted ALS clinical trials by impairing the ability to obtain measurements for trial eligibility, to monitor safety and efficacy outcomes, and to dispense study drug, as these also often rely on in-person visits. We review opportunities for overcoming some of these challenges through telemedicine and novel measurements. These can reoptimize ALS care and research in the current setting and during future events that may limit travel and face-to-face interactions.     

Management challenges for chronic dysimmune neuropathies during the COVID-19 pandemic

Since March 2020, the COVID-19 pandemic has led to the need to re-think the delivery of services to patients with chronic dysimmune neuropathies. Telephone/video consultations have become widespread but have compounded concerns about objective evaluation. Therapeutic decisions need, more than ever before, to be considered in the best interests of both patients, and society, while not denying function-preserving/restoring treatment. Immunoglobulin therapy and plasma exchange, for those treated outside of the home, expose patients to the hazards of hospital or outpatient infusion centers. Steroid therapy initiation and continuation pose increased infectious risk. Immunosuppressant therapy similarly becomes highly problematic, with the risks of treatment continuation enhanced by uncertainties regarding duration of the pandemic. The required processes necessitate considerable time and effort especially as resources and staff are re-deployed to face the pandemic, but are essential for protecting this group of patients and as an integral part of wider public health actions.     

Prospective analysis of gait characteristics in chronic inflammatory demyelinating polyradiculoneuropathy

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a disorder that may lead to functional impairment, including gait abnormalities. Our aim was to analyze gait characteristics in patients with CIDP compared to healthy controls (HC). Moreover, we sought to determine changes of gait parameters after six-month follow-up period. Twenty-four patients with CIDP and 24 HCs performed basic walking task, dual-motor task, dual-mental task, and combined task using the same GAITRite system. Lower limb MRC-SS and lower limb INCAT disability score were assessed. Fourteen patients were retested after six months. Majority of gait parameters showed significant differences in all experimental conditions when compared between CIDP and HCs. The most consistent findings in CIDP were shorter stride length (SL), prolonged cycle time (CT) and double support time (DS), as well as increased variation of SL and of swing time (ST) (p < 0.05). During follow-up, INCAT improved in nine (64.3%) of 14 patients and MRC-SS improved in eight (57.1%) patients. Six-month changes of CT and its variation during combined task significantly differentiated patients with improved vs. non-improved INCAT (p < 0.05). In conclusion, patients with CIDP had slower gait with prolonged DS and with shorter SL compared to HCs. Increased variation of SL and of ST in CIDP may suggest a potential risk for instability and falls. Shorter CT duration and less CT variation during time correlated well with improvement in disability.     

Investigation of the miRNA146a and miRNA155 gene expression levels in patients with multiple sclerosis

Multiple sclerosis (MS) is a chronic inflammatory disease and the most common neurodegenerative status. MicroRNAs play an important role in macrophage response to inflammatory processes, and alterations in miRNA levels trigger the inactivation of specific T lymphocytes. As a result, these factors can lead to autoimmune diseases such as MS. Therefore, to determine the role of MicroRNA-146a and MicroRNA-155 in MS patients, their expression levels in serum of MS patients were compared with healthy controls.In this study, the expression levels of MicroRNA-146a and MicroRNA-155 in 30 serum samples of MS and healthy patients as a control group. MicroRNA extraction and cDNA synthesis was performed according manufacture protocols. The expression levels of MicroRNAs were evaluated by Real Time-PCR.MicroRNA-146a and MicroRNA-155 levels were increased in patients with MS compared to controls. The results demonstrated that EDSS score are increased with increasing level of MicroRNA-146a and MicroRNA-155. ROC curve analysis showed that the area under curve (AUC) was significant for MicroRNA-146a and MicroRNA-155.Increased expression levels of MicroRNA-146a and MicroRNA-155 may be associated with the pathogenesis of MS disease. If this study is conducted in a larger sample population and the above results can be used to identify patients or control patients who are under medical care.