Multifaceted superoxide dismutase 1 expression in amyotrophic lateral sclerosis patients:a rare occurrence?

Amyotrophic lateral sclerosis(ALS)is a neuromuscular condition resulting from the progressive degeneration of motor neurons in the cortex,brainstem,and spinal cord.While the typical clinical phenotype of ALS involves both upper and lower motor neurons,human and animal studies over the years have highlighted the potential spread to other motor and non-motor regions,expanding the phenotype of ALS.Although superoxide dismutase 1(SOD1)mutations represent a minority of ALS cases,the SOD1 gene remains a milestone in ALS research as it represents the first genetic target for personalized therapies.Despite numerous single case reports or case series exhibiting extramotor symptoms in patients with ALS mutations in SOD1(SOD1-ALS),no studies have comprehensively explored the full spectrum of extramotor neurological manifestations in this subpopulation.In this narrative review,we analyze and discuss the available literature on extrapyramidal and non-motor features during SOD1-ALS.The multifaceted expression of SOD1 could deepen our understanding of the pathogenic mechanisms,pointing towards a multidisciplinary approach for affected patients in light of new therapeutic strategies for SOD1-ALS.

Effects of Women’s Autonomy and Male Involvement on Reproductive and Child Health (RCH) Service Utilization in Uttar Pradesh

Introduction: Effective utilization of reproductive and child health (RCH) services is important to reduce morbidity and mortality among mothers as well as children under-five. After International Conference on Population and Development (ICPD), 1994 India has increasingly integrated male participation in women’s reproductive health to improve the women’s health care during pregnancy and child birth. This study examined effect of women’s autonomy and male involvement on RCH services utilization in Uttar Pradesh State of India. Methodology: State level data of Uttar Pradesh from National Family Health Survey (NFHS-3), India for currently married women and men aged 15 - 49 years was considered. The study was restricted to couples whose youngest child was born during the three years preceding the survey (N = 2685). Pearson chi-square test was used to determine the association of background characteristics with woman’s autonomy and male involvement in RCH utilization. Logistic regression was used to understand the effects of male involvement and women’s autonomy, using both computed indices after controlling for socio-economic and background characteristics of women and their husbands. Subsequently, the exercise was extended using individual component of both the indices. All findings were reported for 95% CI and p < 0.05. Results: Findings show that except religion, other socio-economic and demographic variables such as age of women, place of residence, number of living children, caste/tribe, women’s work status, education, wealth index, household structure and exposure to mass media, husband’s age, education, and occupation were statistically significant and associated with women’s autonomy and male involvement. Multivariate analysis indicated women’s autonomous decision making and support from their husbands (male involvement) significantly influenced their utilization of RCH services after controlling for all socio-economic and demographic variables. Conclusion: To improve RCH service utilization and overall health status of women and children male involvement and women’s autonomous decision-making should be addressed in all future RCH programs in the State.

Preventive behaviors for maintenance of brain health: an online quasi-experimental study on the influence of involvement and message framing

Objective:The prevalence and economic burden of Alzheimer's Disease(AD)are consistently increasing.Individuals are encouraged to engage in cognitive preventive behaviors.However,the effectiveness of information provided,depends on factors including the level of personal relevance to the issue and the framing of the information.Our objective in this study was to examine the effect of involvement and message framing on an individual's intention to initiate preventive behaviors toward AD.Methods:An online quasi-experimental scenario-based study was implemented.Vignettes were prepared consisting of information on preventive behaviors to reduce the risk of AD.In the vignettes,scenarios were presented to influence the participant's involvement level(low/high).At the same time,the information was framed positively(low level of fear)or negatively(high level of fear).After exposure to the vignettes,the involvement level and framing perception(fear level)were evaluated.The effects of the two factors on perceived severity,perceived vulnerability,response-efficacy,self-efficacy,and intention to engage in recommended preventive behaviors(based on the Protection Motivation Theory)were then measured.Results:Overall,368 individuals with a mean age of 51.64(±5.48)years participated.Adjusted multiple linear regression indicated that higher involvement was associated with significantly higher perceived severity,perceived vulnerability,and intention to engage in preventive behaviors.Negative message framing was associated with significantly higher perceived severity,perceived vulnerability,and self-efficacy.Conclusion:Our findings indicated that involvement and message framing are essential factors to consider when designing communication materials for promoting health behaviors.Their effects may depend on the underlying drivers of intention toward the recommended health behaviors.

The Association between Home Literacy Environment and Children Literacy Outcomes: Fathers' involvement

Home Literacy Environment(HLE)is an important factor to influence children’s literacy abilities.The study was to look into previous studies on HLE and to propose the future direction on the relationship between fathers’involvement in HLE and children’s literacy outcomes.

Crohn's disease with gastroduodenal involvement: Diagnostic approach

Crohn's disease(CD) is a chronic idiopathic inflammatory disease of gastrointestinal tract characterizedby segmental and transmural involvement of gastrointestinal tract. Ileocolonic and colonic/anorectal is a most common and account for 40% of cases and involvement of small intestine is about 30%. Isolated involvement of stomach is an extremely unusual presentation of the disease accounting for less than 0.07% of all gastrointestinal CD. To date there are only a few documented case reports of adults with isolated gastric CD and no reports in the pediatric population. The diagnosis is difficult to establish in such cases with atypical presentation. In the absence of any other source of disease and in the presence of nonspecific upper gastrointestinal endoscopy and histological findings, serological testing can play a vital role in the diagnosis of atypical CD. Recent studies have suggested that perinuclear anti-neutrophil cytoplasmic antibody and anti-Saccharomycescervisia antibody may be used as additional diagnostic tools. The effectiveness of infliximab in isolated gastric CD is limited to only a few case reports of adult patients and the long-term outcome is unknown.

Tracheobronchial nodules and pulmonary infiltrates in a patient with Crohn's disease

Crohn's disease is a granulomatous systemic disorder of unknown etiology. Obvious pulmonary involvement is exceptional. Tracheal involvement in Crohn's disease is even more unusual, only a few cases have been report-ed to date. We herein report a rare case of tracheobron-chial nodules and pulmonary infiltrates in both lungs as a complication of Crohn's disease. A 42-year-old man underwent pancolectomy for multiple broken colon caused by Crohn's disease. Forty days later pulmonary symptoms and radiologic abnormalities were noted. A search for bacterial (including mycobacteria) and fungal in the repeated sputum proved negative. The treatment consisted of intravenous antimicrobials for one month, but there was no improvement in pyrexia or cough and radiologic abnormalities. Fibreoptic bronchoscopy (FOB) was performed and revealed nodes in the trachea and the right upper lobe opening. Histopathology of tracheo-bronchial nodules and bronchial mucosa biopsy specimen both showed granulomatous inflammation with proliferation of capillaries and inflammatory cells. Oral steroid and salicylazosulfapyridine were commenced and led to marked improvement in symptoms and an almost complete resolution of his chest radiograph. Repeated FOB showed that nodes in the trachea disappeared and the ones in the right upper lobe opening diminished obviously. Crohn's disease can be associated with several respiratory manifestations. The form of tracheal and bronchopulmonary involvement in Crohn's disease is rare and responded well to steroids.

Spectrum of gastrointestinal involvement in Stevens-Johnson syndrome

Stevens-Johnson syndrome(SJS) or toxic epidermal necrolysis(TEN) is a severe adverse drug reaction associated with involvement of skin and mucosal membranes, and carries significant risk of mortality and morbidity. Mucus membrane lesions usually involve the oral cavity, lips, bulbar conjunctiva and the anogenitalia. The oral/anal mucosa and liver are commonly involved in SJS or TEN. However, intestinal involvement is distinctly rare. We herein review the current literature regarding the gastrointestinal involvement in SJS or TEN. This review focuses mainly on the small bowel and colonic involvement in patients with SJS or TEN.

A Corpus-Based Analytical Study on Military and Security Developments Involving People＇s Republic of China

This paper contrasts Military Power of the People＇s Republic of China from the year of 2000 to 2009 and Military and Security Developments Involving People＇s Republic of China from the year 2010 to 2013 with corpus analysis. It distinguishes the new reports＇ differences from the old ones and tries to explore American＇s discourse colonization and discourse hegemony through corpus software WordSmith 5.0-Keywords and Concordance analysis of high frequency words. The significance of the study is that it enlightens military texts which will influence audiences＇ viewpoints and provides a new perspective for strategies replying American media war.

Unicentric Castleman's Disease with Cardiovascular Involvement

CASTLEMAN＇S disease（CD）,a rare lymphoproliferative disorder of unknown etiology, was first described in 1956 as a benign mass in the mediastinum. Although CD can present anywhere in the body, 70% of the cases are in the chest along the tracheobronchial tree or hilum of the lung in the middle mediastinum; however, they can also occur in the anterior or posterior compartments. CD is classi-fied as unicentric （UCD） or multicentric （MCD） based on the anatomical distribution, and histologically as hya-line-vascular, plasma cell, or mixed subtypes.1 Although MCD is less common than UCD, it can be rapidly pro-gressive and often fatal.

Management and outcomes of surgical patients with intestinal Behçet’s disease and Crohn’s disease in southwest China

BACKGROUND Gastrointestinal involvement in Behçet's disease(GIBD)and Crohn’s disease(CD)are inflammatory diseases sharing a considerable number of similarities.However,different from CD,the operative and postoperative management of GIBD remains largely empirical because of the lack of comprehensive treatment guidelines.AIM To compare surgical patients with GIBD and those with CD in a medical center and identify notable clinical features and effective postoperative treatment for surgical patients with GIBD.METHODS We searched patients diagnosed with CD and GIBD who underwent operations for gastrointestinal complications from 2009 to 2015 at West China Hospital of Sichuan University.A total of 10 surgical patients with GIBD and 106 surgical patients with CD were recruited.Information including demographic data,medication,and operative and postoperative parameters were collected and analyzed.As the incidence of surgical GIBD is low,their detailed medical records were reviewed and compared to previous studies.Moreover,the prognoses of CD and GIBD were evaluated respectively between groups treated with biological and non-biological agents.RESULTS Indication for first surgery was often acute intestinal perforation for GIBDpatients (7/10 vs 0/106, P < 0.001), whereas intestinal fistulae (0/10 vs 44/106, P =0.013) and ileus (0/10 vs 40/106, P = 0.015) were the indications for surgical CDpatients. Approximately 40% of patients with GIBD and 23.6% of patients withCD developed postoperative complications, 50% of patients with GIBD and 38.7%of patients with CD had recurrence postoperatively, and 40% (4/10) of patientswith GIBD and 26.4% (28/106) of patients with CD underwent reoperations. Theaverage period of postoperative recurrence was 7.87 mo in patients with Behçet'sdisease (BD) and 10.43 mo in patients with CD, whereas the mean duration fromfirst surgery to reoperation was 5.75 mo in BD patients and 18.04 mo in CDpatients. Surgical patients with GIBD more often used corticosteroids (6/10 vs7/106, P < 0.001) and thalidomide (7/10 vs 9/106, P < 0.001) postoperatively,whereas surgical patients with CD often used infliximab (27/106), azathioprine,or 6-mercaptopurine (74/106) for maintenance therapy.CONCLUSIONPatients suffering GIBD require surgery mostly under emergency situations,which may be more susceptible to recurrence and reoperation and need moreaggressive postoperative treatment than patients with CD.

GPCR/endocytosis/ERK signaling/S2R is involved in the regulation of the internalization,mitochondria-targeting and-activating properties of human salivary histatin 1

Human salivary histatin 1(Hst1)exhibits a series of cell-activating properties,such as promoting cell spreading,migration,and metabolic activity.We recently have shown that fluorescently labeled Hst1(F-Hst1)targets and activates mitochondria,presenting an important molecular mechanism.However,its regulating signaling pathways remain to be elucidated.We investigated the influence of specific inhibitors of G protein-coupled receptors(GPCR),endocytosis pathways,extracellular signal-regulated kinases1/2(ERK1/2)signaling,p38 signaling,mitochondrial respiration and Na+/K+-ATPase activity on the uptake,mitochondria-targeting and-activating properties of F-Hst1.We performed a si RNA knockdown(KD)to assess the effect of Sigma-2 receptor(S2R)/Transmembrane Protein 97(TMEM97)—a recently identified target protein of Hst1.We also adopted live cell imaging to monitor the whole intracellular trafficking process of F-Hst1.Our results showed that the inhibition of cellular respiration hindered the internalization of F-Hst1.The inhibitors of GPCR,ERK1/2,phagocytosis,and clathrin-mediated endocytosis(CME)as well as siRNA KD of S2R/TMEM97 significantly reduced the uptake,which was accompanied by the nullification of the promoting effect of F-Hst1 on cell metabolic activity.Only the inhibitor of CME and KD of S2R/TMEM97 significantly compromised the mitochondria-targeting of Hst1.We further showed the intracellular trafficking and targeting process of F-Hst1,in which early endosome plays an important role.Overall,phagocytosis,CME,GPCR,ERK signaling,and S2R/TMEM97 are involved in the internalization of Hst1,while only CME and S2R/TMEM97 are critical for its subcellular targeting.The inhibition of either internalization or mitochondria-targeting of Hst1 could significantly compromise its mitochondria-activating property.

Right Ventricular Thrombus and Tricuspid Valve Dysfunction in a Patient with Behçet’s Disease

Background: Behçet’s disease (BD) is a multisystemic, chronic inflammatory disorder with a broad range of manifestations including within the cardiovascular system. Cardiac involvement like intracardiac thrombus (ICT) and valvular involvement in BD are rarely seen entities and often associated with poor prognosis. Case Presentation: We present the case of a young patient with vascular-Behçet diagnosed by the presence of intracardiac thrombus in the right ventricle, unresponsive to medical treatment. Even though the intracardiac lesion was successfully treated by surgical excision, he presented a severe tricuspid dysfunction some years later due to the extension of fibrosis with no surgical therapeutic option. Conclusion: Intracardiac thrombosis is a rare but early manifestation of Behçet’s disease, which is difficult to diagnose and has a poor prognosis.

Some Implications of the Gessel Identity

We generalize the congruences of Friedmann-Tamarkine (1909), Lehmer (1938), and Ernvall-Metsänkyla (1991) on the sums of powers of integers weighted by powers of the Fermat quotients to the next Fermat quotient power, namely to the third power of the Fermat quotient. Using this result and the Gessel identity (2005) combined with our past work (2021), we are able to relate residues of some truncated convolutions of Bernoulli numbers with some Ernvall-Metsänkyla residues to residues of some full convolutions of the same kind. We also establish some congruences concerning other related weighted sums of powers of integers when these sums are weighted by some analogs of the Teichmüller characters.

县域地区父母卷入对儿童希望感的影响——基于西部N县数据的实证研究

聚焦西部县域儿童希望感,基于课题组在N县23所学校开展的中学生调查数据,运用多元线性回归分析了父母卷入对儿童希望感的影响,并纳入交互项,从性别和留守视角分析父母卷入对儿童希望感影响的异质性。研究发现:(1)父母的情感卷入和母亲认知卷入均会对儿童希望感产生显著的正向影响;(2)性别视角下,父母卷入对儿童希望感的影响不存在显著的性别差异;(3)留守状态视角下父母卷入的影响效应差异并不显著;(4)留守距离视角下,对于双留守而言,父母一方在省内会显著降低父亲的行为卷入,但会显著提升母亲的行为卷入。在此基础上,提出应从强化家庭教育宣传引导,促进特色产业发展,通过学校、社区的力量,加强对留守家庭的支持,促进西部县域儿童希望感的发展等方面的政策建议。

基于胸部CT及临床特征构建原发性干燥综合征患者肺脏受累的风险预测模型

目的基于胸部CT及临床特征构建原发性干燥综合征(primary Sjogren′s syndrome,pSS)患者肺脏受累的风险预测模型,并探讨模型的风险预测价值。方法回顾性选取邯郸市中医院于2020年10月至2023年8月收治的360例pSS患者为研究对象,按照7∶3的分配比例分为建模组252例和验证组108例。建模组患者根据肺脏受累与否分为对照组201例和受累组51例。收集建模组患者临床特征资料与胸部高分辨CT(high resolution CT,HRCT)特点,行组间单因素分析确定收集信息中影响pSS患者肺脏受累的相关因素。对相关因素行二元logistic回归分析以筛选独立危险因素,并以独立危险因素建立预测模型,通过验证组资料收集配合完成列线图预测模型的验证与价值分析。结果患者年龄、病程、咳嗽、雷诺现象、C反应蛋白(C-reactive protein,CRP)、抗SSA抗体、HRCT等为影响pSS患者肺脏受累的相关因素(P<0.05)。进一步行二元logistic回归分析发现,患者年龄大、病程长、咳嗽及HRCT异常为影响SS患者肺脏受累的独立危险因素(P<0.05)。以独立影响因素构建列线图风险预测模型,模型验证结果提示,校准图显示预测模型性能良好;建模组受试者工作特征(ROC)曲线的曲线下面积(AUC)为0.993;验证组ROC的AUC为0.995。结论pSS患者临床特征与胸部CT结果与患者肺脏受累密切相关,其中患者年龄大、病程长、咳嗽及HRCT异常为影响pSS患者肺脏受累的独立危险因素,以此为基础建立预测模型对患者后装放疗是否发生肺脏受累具有较高预测价值。

韦氏黄斑水肿方治疗痰瘀互结型中心累及型糖尿病黄斑水肿的临床研究

目的评价韦氏黄斑水肿方治疗痰瘀互结型中心累及型糖尿病黄斑水肿的临床疗效。方法采用前瞻性随机对照试验的方法(伦理审批号:JDF-IRB-20220530002),观察2022年6月—2023年1月在北京中医药大学东方医院眼科就诊的糖尿病黄斑水肿患者42例,随机将受试者分入试验组和对照组各21例,试验组给予韦氏黄斑水肿方中药颗粒口服4周联合雷珠单抗玻璃体腔注药术1次,对照组给予雷珠单抗玻璃体腔注药术1次。在治疗开始前、治疗2周、治疗4周时分别测量患者的最佳矫正视力、视网膜中央子区厚度、立方体体积、立方体平均厚度、非接触眼压;在治疗前、治疗4周时评估两组痰瘀互结中医证候评分。再予以受试者合并疾病的初步探析;最后进行整体有效率评价与安全性评价。结果治疗后两组患者最佳矫正视力、中央子区厚度、立方体体积、立方体平均厚度较治疗前均有明显改善(P<0.05);两组的中央子区厚度、立方体平均厚度在治疗4周时出现组间差异(P<0.05);治疗4周后试验组中医证候评分变化具有显著统计学意义(P<0.05)。试验组总体有效率达95%,与对照组有效率之间差异具有统计学意义(P=0.049);合并疾病中,患者最多合并罹患的疾病是高血压病。整个试验期间,仅对照组1人出现球结膜下出血,余未发生不良结局。结论韦氏黄斑水肿方可提高痰瘀互结型中心累及型糖尿病黄斑水肿患者的最佳矫正视力,降低患者黄斑相关厚度指标,显著改善患者的中医证候指标,具备临床安全性。

Pulmonary manifestations of Crohn's disease

Crohn&#x02019;s disease (CD) is a systemic illness with a constellation of extraintestinal manifestations affecting various organs. Of these extraintestinal manifestations of CD, those involving the lung are relatively rare. However, there is a wide array of lung manifestations, ranging from subclinical alterations, airway diseases and lung parenchymal diseases to pleural diseases and drug-related diseases. The most frequent manifestation is bronchial inflammation and suppuration with or without bronchiectasis. Bronchoalveolar lavage findings show an increased percentage of neutrophils. Drug-related pulmonary abnormalities include disorders which are directly induced by sulfasalazine, mesalamine and methotrexate, and opportunistic lung infections due to immunosuppressive treatment. In most patients, the development of pulmonary disease parallels that of intestinal disease activity. Although infrequent, clinicians dealing with CD must be aware of these, sometimes life-threatening, conditions to avoid further impairment of health status and to alleviate patient symptoms by prompt recognition and treatment. The treatment of CD-related respiratory disorders depends on the specific pattern of involvement, and in most patients, steroids are required in the initial management.

Comparison of the use of wireless capsule endoscopy with magnetic resonance enterography in children with inflammatory bowel disease

BACKGROUND Magnetic resonance enterography (MRE) and wireless capsule endoscopy (WCE) are equally accepted modalities for noninvasive screening of small bowel involvement (SBI) in children with Crohn’s disease (CD) and indeterminate colitis (IC) albeit there is a paucity of data comparing the two and thereby guiding the clinician in selecting the ideal diagnostic approach. Therefore, the goal of this study is to provide additional evidence for capsule endoscopy role in the evaluation of established Crohn’s disease exacerbation compared to MRE in relation to Pediatric Crohn's Disease Activity Index (PCDAI), and histological indices. AIM To prospectively compare the findings of MRE and WCE and their agreement with PCDAI or histology in children with CD or IC. METHODS Consecutive patients diagnosed with CD and IC were screened for inclusion. After informed consent, patient’s demographic and clinical data was abstracted. The current pediatric disease activity index (PCDAI) and endoscopic findings were included. Patients underwent MRE and WCE including preprocedural patency capsule within a maximum of 7 d of each other. Pathological presence of active small bowel disease in ileal and duodenal biopsies were collected if the endoscopy was performed within 2 mo of the WCE study. Patients who failed to pass the PC were excluded from the study. WCE was read by two different experienced gastroenterologists (Attard TM and Colombo JM) blinded to each other's findings and to the findings on MRE (Mardis NJ). Agreement between WCE reviewers, WCE and MRE findings and concordance between positive PCDAI and SBI based on MRE compared with WCE was computed. RESULTS Forty-five patients were included in the study, 18 withdrew and 27 (20 males and 20 CD), mean age (standard deviation) 13.46 (2.4) years, completed the study protocol. There were no instances of capsule retention. Concordance between gastroenterologist reviewers was excellent for the diagnosis of small intestinal CD with good correlation between the two Lewis scores (r=0.875, P<0.001). Concordance between WCE and MRE was poor (69%). In CD patients, when both MRE and WCE were compared using PCDAI>10 as the standard reference reflecting active small intestinal CD, the sensitivity of MRE and WCE were 100% and 83% respectively and the specificity of MRE and WCE were 57.14% and 78.6%, respectively. If the histology in ileum or/and duodenum was used as the reference for active small bowel involvement, WCE had a higher specificity as compared to MRE (83.3% vs 50%). In patients with Crohn’s disease, those with a positive PCDAI (>10) were more likely to have a positive WCE as compared to those with a negative PCDAI (83% vs 21%;P=0.018). CONCLUSION We suggest that MRE and WCE have a complementary role in the assessment of SBI in CD. WCE detected SBI with a much higher specificity while MRE had a higher sensitivity.

Higher frequency of brain abnormalities in neuromyelitis optica spectrum disorder patients without primary Sjogren's syndrome

Neuromyelitis optica spectrum disorder often co-exists with primary Sjogreffs syndrome. We compared the clinical features of 16 neuro- myelitis optica spectrum disorder patients with （n = 6） or without primary Sjogreffs syndrome （n = 10）. All patients underwent extensive clinical, laboratory, and MRI evaluations. There were no statistical differences in demographics or first neurological involvement at onset between neuromyelitis optica spectrum disorder patients with and without primary Sjogren＇s syndrome. The laboratory findings of cerebrospinal fluid oligoclonal banding, serum C-reactive protein, antinudear autoantibody, anti-Sjogren＇s-syndrome-related antigen A an- tibodies, anti-Sjogren＇s-syndrome-related antigen B antibodies, and anti-Sm antibodies were significantly higher in patients with primary Sjogren＇s syndrome than those without. Anti-aquaporin 4 antibodies were detectable in 67% （4/6） of patients with primary Sjogren＇s syndrome and in 60% （6/10） of patients without primary Sj6gren＇s syndrome. More brain abnormalities were observed in patients without primary Sj6gren＇s syndrome than in those with primary Sj6gren＇s syndrome. Segments lesions （〉 3 centrum） were noted in 50% （5/10） of patients without primary Sj6gren＇s syndrome and in 67% （4/6） of patients with primary Sjogren＇s syndrome. These findings indicate that the clinical characteristics of neuromyelitis optica spectrum disorder patients with and without primary Sjogren＇s syndrome are similar. However, neu- romyelitis optica spectrum disorder patients without primary Sjogreffs syndrome have a high frequency of brain abnormalities.

Left intracardiac thrombosis: An exceptional complication revealing BehCet’s disease

Although cardiac involvement during Behcet syndrome is uncommon and intracardiac thrombosis is exceptional, it concerns the right ventricle much more than the left one. We report an illustrative case of left intracardiac thrombosis revealing Behcet’s disease in a 33-year-old Tunisian man.