MedNexus
Volume 130 · Issue 15 · 2017
MedNexus
- Sections
- Editorial
- Original Article
- Clinical Guidelines
- Expert Consensus
- Review Article
- Clinical Observation
- Clinical Challenge
- Correspondence
- Author’s Reply
肌萎缩侧索硬化(ALS)的特征是上下运动神经元的进行性变性。ALS的典型临床特征是肢体瘫痪、肌肉萎缩、吞咽困难、构音障碍、气短和呼吸衰竭。大约90%的ALS病例被归类为散发性ALS,其余10%被归类为家族性ALS。[
Progressive bulbar palsy (PBP) is a classic phenotype of bulbar onset amyotrophic lateral sclerosis (ALS) with more rapid progression and worse prognosis. However, as an often under-understood variant of ALS, isolated bulbar palsy (IBP) appears to progress more slowly and has a relatively benign prognosis. This study aimed to investigate the natural course and clinical features of IBP in Chinese population and to compare them with those of PBP.
The clinical data of patients with bulbar onset ALS were collected from January 2009 to December 2013. Revised ALS Functional Rating Scale (ALSFRS-R), forced vital capacity (FVC), and follow-up evaluation were performed, and the differences in basic clinical features, ALSFRS-R, FVC, and primary outcome measures between IBP and PBP were analyzed. The independent t-test, Chi-square test, Mann-Whitney U-test, and Kaplan-Meier analysis were used.
Totally 154 patients with bulbar onset ALS were categorized into two groups, 33 with IBP and 121 with PBP. In the IBP group, the male to female ratio was 0.7 to 1.0, and the mean onset age was 58.5 years. The mean duration from the onset was 16.0 months, and the mean ALSFRS-R score was 43.4 at patients’ first visit to our hospital. In 14 IBP patients performing FVC examination, the mean FVC value was 90.5% and there were only two cases with abnormal FVC. In 26 IBP patients completing follow-up, 15 (58%) suffered death or tracheotomy and the mean survival time was 40.5 months. Significant differences were noted in sex ratio, onset age, ALSFRS-R score, upper motor neuron limb signs, pure lower motor neuron (LMN) bulbar signs, FVC, and survival time between IBP and PBP.
IBP was evidently different from PBP, which was characterized with the predominance of female, pure LMN bulbar signs, an older onset age, a relative preservation of respiratory function, and a better prognosis.
The 9-hole peg test (9-HPT) and 10-meter walk test (10-MWT) are commonly used to test finger motor function and walking ability. The aim of this present study was to investigate the efficacy of these tests for evaluating functional loss in Chinese Charcot-Marie-Tooth (CMT) disease.
Thirty-four Chinese CMT patients (CMT group) from August 2015 to December 2016 were evaluated with 9-HPT, 10-MWT, CMT disease examination score, overall neuropathy limitation scale (ONLS), functional disability score, and Berg Balance Scale (BBS). Thirty-five age- and gender-matched healthy controls (control group) were also included in the study. Student’s nonpaired or paired t-test were performed to compare data between two independent or related groups, respectively. The Pearson test was used to examine the correlations between recorded parameters.
The mean 9-HPT completion time in the dominant hand of CMT patients was significantly slower than that in the healthy controls (29.60 ± 11.89 s vs. 19.58 ± 3.45 s; t = −4.728, P < 0.001). Women with CMT completed the 9-HPT significantly faster than men with CMT (dominant hand: 24.74 ± 7.93 s vs. 33.01 ± 13.14 s, t = 2.097, P = 0.044). The gait speed of the average self-selected velocity and the average fast-velocity assessed using 10-MWT for CMT patients were significantly slower than those in the control group (1.03 ± 0.18 m/s vs. 1.44 ± 0.17 m/s, t = 9.333, P < 0.001; 1.31 ± 0.30 m/s vs. 1.91 ± 0.25 m/s, t = 8.853, P < 0.001, respectively). There was no difference in gait speed between men and women. Both 9-HPT and 10-MWT were significantly correlated with the ONLS, functional disability score, and BBS (P < 0.05 for all).
The 9-HPT and 10-MWT might be useful for functional assessment in Chinese patients with CMT.
Charcot-Marie-Tooth disease (CMT) is the most common inherited peripheral neuropathy. A great number of causative genes have been described in CMT, and among them, the heterozygous duplication of peripheral myelin protein-22 (PMP22) is the major cause. Although the missense mutation in PMP22 is rarely reported, it has been demonstrated to be associated with CMT. This study described a novel missense mutation of PMP22 in a Chinese family with CMT phenotype.
Targeted next-generation sequencing (NGS) was used to screen the causative genes in a family featured with an autosomal dominant demyelinating form of CMT. The potential variants identified by targeted NGS were verified by Sanger sequencing and classified according to the American College of Medical Genetics and Genomics standards and guidelines. Further cell transfection studies were performed to characterize the function of the novel variant.
Using targeted NGS, a novel heterozygous missense variant in PMP22 (c.320G>A, p.G107D) was identified. In vitro cell functional studies revealed that mutant PMP22 protein carrying p.G107D mutation lost the ability to reach the plasma membrane, was mainly retained in the endoplasmic reticulum, and induced cell apoptosis.
This study supported the notion that missense mutations in PMP22 give rise to a CMT phenotype, possibly through a toxic gain-of-function mechanism.
Some esophageal cancer patients complicated with depression exhibit cognitive impairments. Frontal electroencephalogram (EEG) may be used as a reliable biomarker for prefrontal-mediated cognitive functions. This study was to investigate alterations of EEG and frontal cognitive impairment in esophageal cancer patients complicated with depression and to assess their correlation.
Sixty-five esophageal cancer patients with depression (study group) and 62 healthy controls (control group) were included in this study. The study group were assigned into psychotic depressed (PD, n= 32) and nonpsychotic depressed (NPD, n= 33) subgroups based on complication with psychotic symptoms (Brief Psychiatric Rating Scale [BPRS] >35). EEG examination, Beck self-rating depression scale, and BPRS were used to assess clinical symptoms. Chi-square test, two independent sample t-test, one-way analysis of variance, and Kruskal-Wallis test were utilized to compare the variables between two groups. EEG abnormalities and scores of frontal cognitive function test were analyzed by partial correlation analysis in the PD and NPD subgroups.
Compared with control group, the study group displayed greater scores either in the Stroop test (19.89 ± 2.05 vs. 24.12 ± 2.19, P = 0.006) or Color Trails Test (CTT; 11.92 ± 1.01 vs. 15.02 ± 1.63, P = 0.008), and reduced score (35.05 ± 2.01 vs. 32.11 ± 2.38, P = 0.007) in the verbal fluency test (VFT). Compared to NPD subgroup, PD subgroup exhibited increased scores in Stroop test (22.89 ± 2.07 vs. 25.38 ± 2.32, P= 0.009) and CTT (13.16 ± 1.71 vs. 15.82 ± 1.13, P = 0.008). Moreover, increased scores in Stroop test and CTT as well as scores in VFT were associated with the severity of depression. The study group had an abnormal frontal EEG, such as α forward, α asymmetry, α moderation, and increased θ activity relative to control group. Similarly, compared with NPD subgroup, PD subgroup displayed α forward, α asymmetry, and α moderation. The correlation test revealed that α forward and α asymmetry were negatively associated with VFT score, but positively correlated with the scores of CTT and the Stroop test in PD subgroup. In addition, α asymmetry in NPD subgroup was positively related to CTT scores.
This study indicated that frontal cognitive impairment in esophageal cancer patients complicated with depression is associated with EEG alterations.
Inflammatory demyelinating disease of central nervous system (CNS) is an inflammatory disease characterized by a high childbearing female predominance. Labor-related alterations for postpartum demyelinating attacks are not entirely clear. This study aimed to summarize clinical features of female patients of reproductive age with initial CNS inflammatory demyelinating attacks during puerperium.
Fourteen female patients with initial demyelinating events during puerperium between January 2013 and December 2016 were retrospectively studied. Records of clinical features, neuroimaging, serum antibodies, cerebrospinal fluid (CSF) findings, annualized relapse rate (ARR), and treatment were analyzed.
Among 14 patients, 5 patients were diagnosed with multiple sclerosis (MS), four as neuromyelitis optica (NMO), two as longitudinal extensive transverse myelitis, two as clinical isolated syndrome (CIS), and one as acute brainstem syndrome. All the 14 puerperal female patients presented with more than one manifestation of hemiplegia, paraplegia, uroschesis, visual loss or dysarthria, and with mild to moderate abnormalities of CSF. Attacks occurred during the first trimester postpartum and cesarean section was the main delivery way (n = 10). Median Expanded Disability Status Scale (EDSS) scores were 5.0 (range: 2.0–9.0) at the onset and 2.5 (range: 0–7.0) at the end of follow-ups. Patients with MS and CIS had a significantly lower EDSS scores than patients with NMO spectrum disorders (P < 0.05). Median ARR was 0.46 (range: 0–1.16); all patients had a low ARR (0.49 ± 0.34, 95% confidence interval: 0.29–0.69) with standardized treatments.
Labor-related alterations in the mother’s immune system might result in newly-onset demyelinating diseases of central nervous system.
Glehnia littoralis, as a traditional herbal medicine to heal various health ailments in EastAsia, displays various therapeutic properties including antioxidant effects. However, neuroprotective effects of G. littoralis against cerebral ischemic insults have not yet been addressed. Therefore, in this study, we first examined its neuroprotective effects in the hippocampus using a gerbil model of transient global cerebral ischemia (TGCI).
Gerbils were subjected to TGCI for 5 min. G. littoralis extract (GLE; 100 and 200 mg/kg) was administrated orally once daily for 7 days before ischemic surgery. Neuroprotection was examined by neuronal nuclear antigen immunohistochemistry and Fluoro-Jade B histofluorescence staining. Gliosis was observed by immunohistochemistry for glial fibrillary acidic protein and ionized calcium-binding adapter molecule 1. For neuroprotective mechanisms, immunohistochemistry for superoxide dismutase (SOD) 1 and brain-derived neurotrophic factor (BDNF) was done.
Pretreatment with 200 mg/kg of GLE protected pyramidal neurons in the cornu ammonis 1 (CA1) area from ischemic insult area (F = 29.770, P < 0.05) and significantly inhibited activations of astrocytes (F = 22.959, P < 0.05) and microglia (F = 44.135, P < 0.05) in the ischemic CA1 area. In addition, pretreatment with GLE significantly increased expressions of SOD1 (F = 28.561, P < 0.05) and BDNF (F = 55.298, P < 0.05) in CA1 pyramidal neurons of the sham- and ischemia-operated groups.
Our findings indicate that pretreatment with GLE can protect neurons from ischemic insults, and we suggest that its neuroprotective mechanism may be closely associated with increases of SOD1 and BDNF expressions as well as attenuation of glial activation.
Galectin-3 (Gal-3) plays a role in the mechanisms underlying ocular venous malformation. We conducted this study to investigate the effect of pingyangmycin pretreatment on the Gal-3 expressions and biological behavior of ocular venous malformation.
Tissue samples were collected from 136 patients with ocular venous malformation. Patients were randomly divided into pingyangmycin (n = 69) and nonpingyangmycin group (n = 67). Patients in the pingyangmycin group received a local injection of 0.02% pingyangmycin once every 2 days for 2 weeks (7 doses) before removal surgery, whereas patients in the nonpingyangmycin group underwent removal surgery without local injection. The protein and messenger RNA (mRNA) expression of Gal-3 were detected by using immunohistochemistry and in situ hybridization.
Gal-3 protein was expressed in 35 (52%) of 67 samples in the nonpingyangmycin group and in 19 (28%) of 69 samples in the pingyangmycin group (P < 0.05). Gal-3 mRNA expression was detected in 39 (58%) of 67 samples in the nonpingyangmycin group and 22 (32%) of 69 samples in the pingyangmycin group (P < 0.05). The higher Gal-3 expressions were detected in samples with deeper invasiveness than those with superficial invasiveness before (χ2 = 12.720 and 13.369, respectively, both P < 0.05) and after pingyangmycin treatment (χ2 = 8.429 and 4.590, respectively, both P < 0.05). It was more frequently detected in mesh-like lesions with unclear boundary than round lesions with clear boundary before (χ2 = 30.291 and 41.466, respectively, both P < 0.05) and after pingyangmycin treatment (χ2 = 14.619 and 15.130, respectively, both P < 0.05). Pingyangmycin treatment led to a significant difference in Gal-3 expressions at both protein and mRNA levels (χ2 = 8.664 and 9.524, respectively, both P < 0.05).
Gal-3 expression may be involved in the development and invasiveness of ocular venous malformation, and pingyangmycin can inhibit Gal-3 expression, indicating a role of pingyangmycin treatment before the removal of ocular venous malformation.
Hepatitis B is an immune response-mediated disease. The aim of this study was to explore the differences of ratios of T-helper (Th) 2 cells to Th1 cells and cytokine levels in acute hepatitis B (AHB) patients and chronic hepatitis B virus (HBV)-infected patients in immune-tolerance and immune-active phases.
Thirty chronic HBV-infected patients in the immune-tolerant phase (IT group) and 50 chronic hepatitis B patients in the immuneactive (clearance) phase (IC group), 32 AHB patients (AHB group), and 13 healthy individuals (HI group) were enrolled in the study. Th cell proportions in peripheral blood, cytokine levels in plasma, and serum levels of HBV DNA, hepatitis B surface antigen, and hepatitis B e antigen were detected.
The Th1 cell percentage and Th2/Th1 ratio in the HBV infection group (including IT, IC, and AHB groups) were significantly different from those in HI group (24.10% ± 8.66% and 1.72 ± 0.61 vs. 15.16% ± 4.34% and 2.40 ± 0.74, respectively; all P < 0.001). However, there were no differences in the Th1 cell percentages and Th2/Th1 ratios among the IT, IC, and AHB groups. In HBV infection group, the median levels of Flt3 ligand (Flt3L), interferon (IFN)-γ, and interleukin (IL)-17A were significantly lower than those in HI group (29.26 pg/ml, 33.72 pg/ml, and 12.27 pg/ml vs. 108.54 pg/ml, 66.48 pg/ml, and 35.96 pg/ml, respectively; all P < 0.05). IFN-α2, IL-10, and transforming growth factor (TGF)-β2 median levels in hepatitis group (including patients in AHB and IC groups) were significantly higher than those in IT group (40.14 pg/ml, 13.58 pg/ml, and 557.41 pg/ml vs. 16.74 pg/ml, 6.80 pg/ml, and 419.01 pg/ml, respectively; all P < 0.05), while patients in hepatitis group had significant lower Flt3L level than IT patients (30.77 vs. 59.96 pg/ml, P = 0.021). Compared with IC group, patients in AHB group had significant higher median levels of IL-10, TGF-β1, and TGF-β2 (22.77 pg/ml, 10,447.00 pg/ml, and 782.28 pg/ml vs. 8.66 pg/ml, 3755.50 pg/ml, and 482.87 pg/ml, respectively; all P < 0.05).
Compared with chronic HBV-infected patients in immune-tolerance phase, chronic HBV-infected patients in immune-active phase and AHB patients had similar Th2/Th1 ratios, significantly higher levels of IFN-α2, IL-10, and TGF-β. AHB patients had significantly higher IL-10 and TGF-β levels than chronic HBV-infected patients in immune-active phase.
Foreign bodies within the sinuses, orbit, and skull base (FBSOS) are rare; hence, diagnosis and management guidelines are lacking. Endoscopic sinus surgery (ESS) removal is preferred because of the less invasiveness and minimal morbidity. This study was designed to summarize clinical experience with ESS management of FBSOS.
We retrospectively reviewed clinical manifestations, imaging findings, treatment, and outcomes in consecutive patients with ESS removal of FBSOS between 2004 and 2015 at a tertiary academic medical center. The Chi-square test was performed to compare the infection rate between wooden and nonwooden FBSOS.
There were 23 male and five female patients, with median age of 11 years. FBSOS were located within the sinuses (86%), orbit (75%), and skull base/intracranial region (46%). Wooden FBSOS had a significantly higher risk of infection (78%) compared with nonwooden FBSOS (5%, P < 0.05). Contrast-enhanced computed tomography (CT) plus three-dimensional reconstruction was sensitive in all cases. Twenty-seven (96%) FBSOS were removed by ESS alone, while 1 (4%) FBSOS was removed using the combined ESS and lateral cervical approach. Four of the nine intracranial penetrating FBSOS patients had intraoperative cerebrospinal fluid (CSF) leak and received endoscopic CSF leak repair. Twelve (43%) patients suffered complications (meningitis, diplopia, and vision loss).
ESS is a minimally invasive, safe, and promising surgical approach for FBSOS removal. Contrast-enhanced CT is effective in preoperative diagnosis and intraoperative guidance. Wooden FBSOS had higher risk of infection, thus antibiotics are recommended.
Nuclear mitotic apparatus protein 1 (NuMA1) had been reported to produce three groups of isoforms categorized as long, middle, and short groups, of which short NuMA displayed distinct localization patterns compared to long and middle isoforms. However, the function of short NuMA was not clear in the progress of cancer formation. This study aimed to unveil the role of short NuMA in cancer pathogenesis.
The expression levels of short isoforms were explored in paired gastric carcinoma (GC) samples and different cell lines. Furthermore, the short isoform behaved as a putative tumor suppressor based on cell proliferation and cell colony formation assays. Pull-down assay and whole-genome gene expression analysis were carried out to search candidate interaction partners of short NuMA.
The expression of short NuMA was highly expressed in S and G2 phases of the cell cycle; compared with nontumor tissues, short NuMA downregulated in nine GCs (GC1 [0.131, P = 5 × 10−4]; GC2 [0.316, P = 3 × 10−5]; GC3 [0.111, P = 6 × 10−4]; GC4 [0.456, P = 0.011]; GC5 [0.474, P = 0.001]; GC6 [0.311, P = 0.004]; GC7 [0.28, P = 3 × 10−5]; GC8 [0.298, P = 0.007]; and GC9 [0.344, P = 0.002]). Besides, high expression of short NuMA significantly inhibits cell growth (2.43 × 105 vs. 2.97 × 105, P = 0.0029) and cell clone information in vitro (70 vs. 2, P = 1.67 × 10−45). Short NuMA could bind with alpha–actinin-4 (ACTN4), a putative tumor promoting gene. Overexpression of short NuMA could tremendously decrease the expression of MYB proto-oncogene like 2 (MYBL2) of about 92-fold, which played an important role in the cell cycles.
Short isoform of NuMA might be functioned as a putative role of tumor suppressor. Further studies should be made to illuminate the relationship between ACTN4, MYBL2, and tumor progression.
肿瘤性脱髓鞘病变、[
自20世纪50年代以来,脑电图(EEG)已广泛应用于神经重症监护病房(NCU)。本世纪初,中国NCU经历了快速发展。根据一份不完全统计报告,[
The aim of this study was to summarize recent studies on nondopaminergic options for the treatment of motor symptoms in Parkinson’s disease (PD).
Papers in English published in PubMed, Cochrane, and Ovid Nursing databases between January 1988 and November 2016 were searched using the following keywords: PD, nondopaminergic therapy, adenosine, glutamatergic, adrenergic, serotoninergic, histaminic, and iron chelator. We also reviewed the ongoing clinical trials in the website of clinicaltrials.gov.
Articles related to the nondopaminergic treatment of motor symptoms in PD were selected for this review.
PD is conventionally treated with dopamine replacement strategies, which are effective in the early stages of PD. Long-term use of levodopa could result in motor complications. Recent studies revealed that nondopaminergic systems such as adenosine, glutamatergic, adrenergic, serotoninergic, histaminic, and iron chelator pathways could include potential therapeutic targets for motor symptoms, including motor fluctuations, levodopa-induced dyskinesia, and gait disorders. Some nondopaminergic drugs, such as istradefylline and amantadine, are currently used clinically, while most such drugs are in preclinical testing stages. Transitioning of these agents into clinically beneficial strategies requires reliable evaluation since several agents have failed to show consistent results despite positive findings at the preclinical level.
Targeting nondopaminergic transmission could improve some motor symptoms in PD, especially the discomfort of dyskinesia. Although nondopaminergic treatments show great potential in PD treatment as an adjunct therapy to levodopa, further investigation is required to ensure their success.
UEscope is a new angulated videolaryngoscope (VL). This review aimed to describe the features of UEscope and provide clinical evidences regarding the efficacy and safety of this video device in adult tracheal intubation and its roles in airway management teaching.
The Wan Fang Data, CNKI, PubMed, Embase, Cochrane Library, and Google Scholar were searched for relevant English and Chinese articles published up to January 15, 2017, using the following keywords: "HC video laryngoscope" , "UE videolaryngoscope" , "video laryngoscope" , and "videolaryngoscopy" .
Human case reports, case series, observable studies, and randomized controlled clinical trials were included in our search. The results of these studies and their reference lists were cross-referenced to identify a common theme.
UEscope features the low-profile portable design, intermediate blade curvatures, all-angle adjustable monitor, effective anti-fog mechanisms, and built-in video recording function. During the past 5 years, there have been a number of clinical studies assessing the application and roles of UEscope in airway management and education. As compared with direct laryngoscope, UEscope improves laryngeal visualization, decreases intubation time (IT), and increases intubation success rate in adult patients with normal and difficult airways. These findings are somewhat different from the previous results regarding the other angulated VLs; they can provide an improved laryngeal view, but no conclusive benefits with regard to IT and intubation success rate. Furthermore, UEscope has extensively been used for intubation teaching and shown a number of advantages.
UEscope can be used as a primary intubation tool and may provide more benefits than other VLs in patients with normal and difficult airways. However, more studies with large sample are still needed to address some open questions about clinical performance of this new VL.
近来,随着放射治疗(RT)技术和综合治疗的提高,头颈癌患者的生存率有所提高。放疗后的癌症幸存者发生脑血管事件的风险增加。[
对于老年患者来说,原发性入口撕裂位于升主动脉内曲线的A型主动脉夹层(TAAD)的治疗是一个巨大的挑战。体外循环和深低温停循环的常规手术修复与高死亡率风险相关。[
随着尿流动态监测和留置导尿管技术在动物实验中的普及,导尿已成为科学研究中的常用技术。小型猪被认为是科学研究中使用的主要动物物种之一,并且越来越多地被用作狗或猴子的替代品,作为药物临床前毒理学试验中的非啮齿动物物种的选择。[
致编者:2013年6月,我院收治一名17岁患者,因盆腔包块合并原发性闭经。患者的堂兄有类似的临床表现,但没有明确的诊断。体格检查发现一位表型女性,身体细长,乳腺发育不良,外阴幼稚,无阴毛,阴道口和前庭外观正常。发现一个直径约12厘米的坚硬实性腹部肿块。阴道长约3.0 cm,盲端通过棉签测量。人绒毛膜促性腺激素(HCG)(40.64 mU/ml)和乳酸脱氢酶(LDH)(431U/ml)水平升高。卵泡刺激素(111 mU/ml)和促黄体生成素(27.8 mU/ml)水平也升高,而E2、T和A水平低于参考值。日间皮质醇和促肾上腺皮质激素(ACTH)水平正常。然后进行染色体核型分析,结果为46XY。盆腔计算机断层扫描显示子宫后部多个肿块,累及腹膜后腹主动脉至左肾静脉水平。
编者:眶内电针(IEA)疗法在治疗眼球运动障碍方面非常有效,但它很少用于治疗甲状腺相关眼病(TAO)。在此,我们报告一个成功的案例。
致编辑:医疗保健重新设计似乎是美国的一个流行词。然而,特朗普总统最近反对奥巴马医改的一些部分。没有人怀疑“奔月”和精准医疗在癌症治疗中的重要性。全方位医疗保健通过最先进的技术产生了重大的有益影响,如大数据、互联网医疗保健服务、智能医疗设备和微创技术。因此,医疗保健正在进入一个新的第三时代;新兴技术和旧护理重新设计观念之间的碰撞。这个新时代也引起了从全球领先专家到普通大众更广泛的兴趣。
编辑:胫骨前粘液水肿(PTM)是一种局限性甲状腺相关皮肤病,其特征是皮肤局部增厚和重塑,通常与一组自身免疫性甲状腺疾病(ATDs)有关。[
致编者:我们怀着极大的兴趣阅读了Seo的文章《选择性环氧合酶-2抑制剂诱导急性心肌梗死一例》等人.[
我谨代表题为“选择性环氧合酶-2抑制剂诱导的急性心肌梗死一例”的文章的作者,感谢读者的评论。
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