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Organic electrode materials (OEMs) have gathered extensive attention for aqueous zinc-ion batteries (AZIBs) due to their structural diversity and molecular designability. However, the reported research mainly focuses on the design of the planar configuration of OEMs and does not take into account the important influence of the spatial structure on the electrochemical properties, which seriously hamper the further performance liberation of OEMs. Herein, this work has designed a series of thioether-linked naphthoquinone-derived isomers with tunable spatial structures and applied them as the cathodes in AZIBs. The incomplete conjugated structure of the elaborately engineered isomers can guarantee the independence of the redox reaction of active groups, which contributes to the full utilization of active sites and high redox reversibility. In addition, the position isomerization of naphthoquinones on the benzene rings changes the zincophilic activity and redox kinetics of the isomers, signifying the importance of spatial structure on the electrochemical performance. As a result, the 2,2'-(1,4-phenylenedithio) bis(1,4-naphthoquinone) (p-PNQ) with the smallest steric hindrance and the most independent redox of active sites exhibits a high specific capacity (279 mAh g -1 ), an outstanding rate capability (167 mAh g -1 at 100 A g -1 ), and a long-term cycling lifetime (over 2800 h at 0.05 A g -1 )., (© 2024 Wiley‐VCH GmbH.)

Backgroup: Currently, aromatherapy is being increasingly utilized in clinical practice, particularly in managing the side effects associated with radiotherapy and chemoradiotherapy. However, it remains to be established whether aromatherapy can effectively alleviate these symptoms., Objective: To investigate the effects of aromatherapy on the physical and mental health of patients with cancer undergoing radiotherapy and chemotherapy., Methods: Seven databases were researched from inception until September 29, 2023, including PubMed, Scopus, and Web of Science, Chinese National Knowledge Infrastructure, Wanfang database, China Biology Medicine disc and VIP Chinese Medical Journal Database. Review Manager version 5.3 was utilized for data analysis. The Cochrane Risk of Bias tool RoB2 was employed to evaluate the quality of the literature included in the study. Evidence quality rating was assessed using the Grading of Recommendations Assessment, Development, and Evaluation (GRADE) approach through the GRADEpro GDT online tool., Results: Nineteen studies involving 1,541 patients were included. Aromatherapy can alleviate nausea [relative risk (RR)=0.64, 95% confidence interval (CI): 0.53 to 0.78, P<0.05, I 2 =46%; standardized mean difference (SMD)=-0.86, 95% CI: -1.21 to -0.51, P<0.05, I 2 =64%] and vomiting (RR=0.54, 95% CI: 0.42 to 0.69, P<0.05, I 2 =35%; SMD=-1.28, 95% CI: -1.52 to -1.03, P<0.05, I 2 =92%), improve sleep disorders [mean difference (MD)=-3.39, 95% CI: -3.95 to -2.84, P<0.05, I 2 =0%], relieve pain (SMD=-1.58, 95% CI: -1.96 to -1.21, P<0.05, I 2 =0%), mitigate fatigue (SMD=-1.28, 95% CI: -2.44 to -0.11, P<0.05, I 2 =93%) and enhance quality of life (SMD=0.50, 95% CI: 0.22 to 0.79, P<0.05, I 2 =0%) in cancer patients after radiotherapy and chemotherapy, but it may not have a significant effect on anxiety. The risk of bias was high in the included studies using the Cochrane Risk of Bias tool RoB2, and no studies were considered to be of high grade according to the GRADE system., Conclusions: Aromatherapy is an efficacious, safe and economic adjunctive therapy for cancer patients, which can mend the physical symptoms and mental health of cancer patients. However, more high-quality studies are needed to verify it. (PROSPERO registration No. CRD42023390171)., (© 2024. The Chinese Journal of Integrated Traditional and Western Medicine Press and Springer-Verlag GmbH Germany, part of Springer Nature.)

Internal tandem duplication mutations in fms-like tyrosine kinase 3 (FLT3-ITD) are recurrent in acute myeloid leukemia (AML) and increase the risk of relapse. Clinical responses to FLT3 inhibitors (FLT3i) include myeloid differentiation of the FLT3-ITD clone in nearly half of patients through an unknown mechanism. We identified enhancer of zeste homolog 2 (EZH2), a component of polycomb repressive complex 2 (PRC2), as a mediator of this effect using a proteomic-based screen. FLT3i downregulated EZH2 protein expression and PRC2 activity on H3K27me3. FLT3-ITD and loss-of-function mutations in EZH2 are mutually exclusive in human AML. We demonstrated that FLT3i increase myeloid maturation with reduced stem/progenitor cell populations in murine Flt3-ITD AML. Combining EZH1/2 inhibitors with FLT3i increased terminal maturation of leukemic cells and reduced leukemic burden. Our data suggest that reduced EZH2 activity following FLT3 inhibition promotes myeloid differentiation of FLT3-ITD leukemic cells, providing a mechanistic explanation for the clinical observations. These results demonstrate that in addition to its known cell survival and proliferation signaling, FLT3-ITD has a second, previously undefined function to maintain a myeloid stem/progenitor cell state through modulation of PRC2 activity. Our findings support exploring EZH1/2 inhibitors as therapy for FLT3-ITD AML., (© 2024. The Author(s), under exclusive licence to Springer Nature Limited.)

The slow reaction kinetics and structural instability of organic electrode materials limit the further performance improvement of aqueous zinc-organic batteries. Herein, we have synthesized a Z-folded hydroxyl polymer polytetrafluorohydroquinone (PTFHQ) with inert hydroxyl groups that could be partially oxidized to the active carbonyl groups through the in situ activation process and then undertake the storage/release of Zn 2+ . In the activated PTFHQ, the hydroxyl groups and S atoms enlarge the electronegativity region near the electrochemically active carbonyl groups, enhancing their electrochemical activity. Simultaneously, the residual hydroxyl groups could act as hydrophilic groups to enhance the electrolyte wettability while ensuring the stability of the polymer chain in the electrolyte. Also, the Z-folded structure of PTFHQ plays an important role in reversible binding with Zn 2+ and fast ion diffusion. All these benefits make the activated PTFHQ exhibit a high specific capacity of 215 mAh g -1 at 0.1 A g -1 , over 3400 stable cycles with a capacity retention of 92 %, and an outstanding rate capability of 196 mAh g -1 at 20 A g -1 ., (© 2023 Wiley-VCH GmbH.)

In additive manufacturing (AM), the mechanical properties of manufactured parts are often insufficient due to complex defects and residual stresses, limiting their use in high-value or mission-critical applications. Therefore, the research and application of nondestructive testing (NDT) technologies to identify defects in AM are becoming increasingly urgent. This article reviews the recent progress in online detection technologies in AM, a special introduction to the high-speed synchrotron X-ray technology for real-time in situ observation, and analysis of defect formation processes in the past 5 years, and also discusses the latest research efforts involving process monitoring and feedback control algorithms. The formation mechanism of different defects and the influence of process parameters on defect formation, important parameters such as defect spatial resolution, detection speed, and scope of application of common NDT methods, and the defect types, advantages, and disadvantages associated with current online detection methods for monitoring three-dimensional printing processes are summarized. In response to the development requirements of AM technology, the most promising trends in online detection are also prospected. This review aims to serve as a reference and guidance for the work to identify/select the most suitable measurement methods and corresponding control strategy for online detection., Competing Interests: No competing financial interests exist., (Copyright 2023, Mary Ann Liebert, Inc., publishers.)

Organic materials have attracted much attention in aqueous zinc-ion batteries (AZIBs) due to their sustainability and structure-designable, but their further development is hindered by the high solubility, poor conductivity, and low utilization of active groups, resulting in poor cycling stability, terrible rate capability, and low capacity. In order to solve these three major obstacles, a novel organic host, benzo[b]naphtho[2',3':5,6][1,4]dithiino[2,3-i]thianthrene-5,7,9,14,16,18-hexone (BNDTH), with abundant electroactive groups and stable extended π-conjugated structure is synthesized and composited with reduced graphene oxide (RGO) through a solvent exchange composition method to act as the cathode material for AZIBs. The well-designed BNDTH/RGO composite exhibits a high capacity of 296 mAh g -1 (nearly a full utilization of the active groups), superior rate capability of 120 mAh g -1 , and a long lifetime of 58 000 cycles with a capacity retention of 65% at 10 A g -1 . Such excellent performance can be attributed to the ingenious structural design of the active molecule, as well as the unique solvent exchange composition strategy that enables effective dispersion of excess charge on the active molecule during discharge/charge process. This work provides important insights for the rational design of organic cathode materials and has significant guidance for realizing ideal high performance in AZIBs., (© 2023 Wiley-VCH GmbH.)

Pediatric brain and spinal cancers are collectively the leading disease-related cause of death in children; thus, we urgently need curative therapeutic strategies for these tumors. To accelerate such discoveries, the Children's Brain Tumor Network (CBTN) and Pacific Pediatric Neuro-Oncology Consortium (PNOC) created a systematic process for tumor biobanking, model generation, and sequencing with immediate access to harmonized data. We leverage these data to establish OpenPBTA, an open collaborative project with over 40 scalable analysis modules that genomically characterize 1,074 pediatric brain tumors. Transcriptomic classification reveals universal TP53 dysregulation in mismatch repair-deficient hypermutant high-grade gliomas and TP53 loss as a significant marker for poor overall survival in ependymomas and H3 K28-mutant diffuse midline gliomas. Already being actively applied to other pediatric cancers and PNOC molecular tumor board decision-making, OpenPBTA is an invaluable resource to the pediatric oncology community., Competing Interests: C.S.G.’s spouse was an employee of Alex’s Lemonade Stand Foundation, which was a sponsor of this research. J.A.S., C.L.S., C.J.B., S.J.S., and J.N.T. are or were employees of Alex’s Lemonade Stand Foundation, a sponsor of this research. A.J.W. is a member of the Scientific Advisory boards for Alexion and DayOne Biopharmaceuticals., (© 2023 The Author(s).)

Objective: To investigate the clinical features of peripherally inserted central catheter (PICC)-related thrombosis (PICCRT) within 2 weeks after PICC placement in cancer patients and its dynamic influence on the blood flow status of veins inserted with catheter, and to provide support for implementing thrombosis prevention and control measures., Methods: Between May 2019 and July 2020, patients who had solid tumors and who had PICC were prospectively enrolled at West China Hospital, Sichuan University. Scheduled color Doppler imaging was performed to examine the status of PICCRT formation at 8 points of time, with the first one conducted one day before the insertion of PICC and the other 7 completed within 2 weeks after the insertion of PICC. Then, based on whether patients had PICCRT, the patients were divided into two groups, a non-PICCRT group and a PICCRT group. The PICCRT group was further divided into two subgroups, an asymptomatic PICCRT group and a symptomatic PICCRT group, according to whether the patients had thrombosis-related symptoms and signs. Comparisons were made to study the incidence of PICCRT and the vascular diameter and the blood flow velocity in the veins inserted with catheters at different points of time in the patients of different groups., Results: Among 173 cancer patients in the cohort, 126 (72.8%) developed PICCRT, all of which occurred within 1 week after PICC insertion. There were 95 cases of asymptomatic PICCRT and 31 cases of symptomatic PICCRT. Before and after PICC insertion, the vascular diameter of both the asymptomatic and symptomatic PICCRT groups was significantly smaller than that of the non-PICCRT group and the blood flow velocity was significantly slower than that of the non-PICCRT group, with the difference continuing to increase with the prolongation of catheter indwelling time., Conclusion: Inserting catheters in veins with bigger vascular diameter and faster blood flow velocity may help reduce the incidence of PICCRT. The first week post catheter insertion is the key intervention period for the prevention of PICCRT., (Copyright© by Editorial Board of Journal of Sichuan University (Medical Sciences).)

To study the quality control of three traditional Chinese medicines derived from Gleditsia sinensis [Gleditsiae Sinensis Fructus(GSF), Gleditsiae Fructus Abnormalis(GFA), and Gleditsiae Spina(GS)], this paper established a multiple reaction monitoring(MRM) approach based on ultra-high performance liquid chromatography-triple quadrupole-linear ion-trap mass spectrometry(UHPLC-Q-Trap-MS). Using an ACQUITY UPLC BEH C&#95;(18) column(2.1 mm × 100 mm, 1.7 μm), gradient elution was performed at 40 ℃ with water containing 0.1% formic acid-acetonitrile as the mobile phase running at 0.3 mL·min~(-1), and the separation and content determination of ten chemical constituents(e.g., saikachinoside A, locustoside A, orientin, taxifolin, vitexin, isoquercitrin, luteolin, quercitrin, quercetin, and apigenin) in GSF, GFA, and GS were enabled within 31 min. The established method could quickly and efficiently determine the content of ten chemical constituents in GSF, GFA, and GS. All constituents showed good linearity(r&gt;0.995), and the average recovery rate was 94.09%-110.9%. The results showed that, the content of two alkaloids in GSF(2.03-834.75 μg·g~(-1)) was higher than that in GFA(0.03-10.41 μg·g~(-1)) and GS(0.04-13.66 μg·g~(-1)), while the content of eight flavonoids in GS(0.54-2.38 mg·g~(-1)) was higher than that in GSF(0.08-0.29 mg·g~(-1)) and GFA(0.15-0.32 mg·g~(-1)). These results provide references for the quality control of G. sinensis-derived TCMs.

The functions of the influenza virus neuraminidase has been well documented but those of the mammalian neuraminidases remain less explored. Here, we characterize the role of neuraminidase 1 (NEU1) in unilateral ureteral obstruction (UUO) and folic acid (FA)-induced renal fibrosis mouse models. We find that NEU1 is significantly upregulated in the fibrotic kidneys of patients and mice. Functionally, tubular epithelial cell-specific NEU1 knockout inhibits epithelial-to-mesenchymal transition, inflammatory cytokines production, and collagen deposition in mice. Conversely, NEU1 overexpression exacerbates progressive renal fibrosis. Mechanistically, NEU1 interacts with TGFβ type I receptor ALK5 at the 160-200aa region and stabilizes ALK5 leading to SMAD2/3 activation. Salvianolic acid B, a component of Salvia miltiorrhiza, is found to strongly bind to NEU1 and effectively protect mice from renal fibrosis in a NEU1-dependent manner. Collectively, this study characterizes a promotor role for NEU1 in renal fibrosis and suggests a potential avenue of targeting NEU1 to treat kidney diseases., (© 2023. The Author(s).)

Pyrolysis is an important technology to achieve the harmlessness and recycling of contaminated biomass. In this study, the effects of oxygen-controlled atmosphere on the component properties and heavy metal accumulation characteristics of contaminated rice straw biochar were studied. The results showed that low-oxygen pyrolysis could effectively produce biochar using contaminated rice straw and improve the stability of heavy metals in biochar. Under the nitrogen atmosphere, the yield of rice straw biochar was 29.4%-34.9%. The aromatization index (SUVA 254 ) of dissolved organic matter (DOM) increased first and then decreased with the increase in pyrolysis temperature, whereas the fluorescent components were mainly humic-like acid substances. Meanwhile, Ca mainly existed in the form of CaCO 3 in biochar. Compared with the pure nitrogen condition, the biochar yield was reduced by 5.6%-13.5% and 14.9%-15.7% under the pyrolysis atmosphere containing 10% and 20% oxygen content, respectively. Ca existed in the form of CaO in biochar, which increased the pH value of the biochar by more than 0.5 units. The oxygen of the pyrolysis atmosphere accelerated the degradation of the lignin component, resulting in the gradual decrease in SUVA 254 of DOM. With the increase in oxygen content in the pyrolysis atmosphere, humic-like acid substances in DOM were transformed into fulvic-like acid substances. Under the conditions of 400℃ and a 10% oxygen-containing atmosphere, the exchangeable fractions of Cu, Cd, Pb, Ni, and As in biochar were decreased by 5.2%, 3.7%, 1.7%, 0.8%, and 0.7%, respectively, indicating that heavy metals are transformed into more stable states. The results suggested that the higher biochar yield and heavy metal stability could be obtained by introducing a proper amount of nitrogen into the air (controlling the oxygen content of approximately 10%) for pyrolysis treatment of contaminated rice straw, providing an economic and feasible technology for the achievement of harmlessness and recovery of contaminated rice straw.

Background and Aims: A key histopathological feature of inflammatory bowel disease is damage to the mucosa, including breakdown of the epithelial barrier. Human enteroids and colonoids are a critical bench-to-bedside tool for studying the epithelium in inflammatory bowel disease. The goal of the current study was to define transcriptional differences in healthy versus diseased subjects that are sustained in enteroids and colonoids, including from disease-spared tissue., Methods: Biopsies and matching enteroid or colonoid cultures from pediatric patients with ileal Crohn disease (N = 6) and control subjects (N = 17) were subjected to RNA sequencing followed by bioinformatic and machine learning analyses. Late passage enteroids were exposed to cytokines to assess durable transcriptional differences., Results: We observed substantial overlap of pathways upregulated in Crohn disease in enteroids and ileal biopsies, as well as colonoids and rectal biopsies. KEGG pathways for cytokine-cytokine receptor interaction, chemokine signaling, protein export, and Toll-like receptor signaling were upregulated in both ileal and rectal biopsies, as well as enteroids and colonoids. In vitro cytokine exposure reactivated genes previously increased in biopsies. Machine learning predicted biopsy location (100% accuracy) and donor disease status (83% accuracy). A random forest classifier generated using ileal enteroids identified rectal colonoids from ileal Crohn disease subjects with 80% accuracy., Conclusion: We confirmed transcriptional profiles of Crohn disease biopsies are expressed in enteroids and colonoids. Furthermore, transcriptomic data from disease-spared rectal tissue can identify patients with ileal Crohn disease. Our data support the use of patient enteroids and colonoids as critical translational tools for the study of inflammatory bowel disease., Competing Interests: Conflicts of Interest: The authors disclose no conflicts.

Heterozygous pathogenic variants in DNM1 cause developmental and epileptic encephalopathy (DEE) as a result of a dominant-negative mechanism impeding vesicular fission. Thus far, pathogenic variants in DNM1 have been studied with a canonical transcript that includes the alternatively spliced exon 10b. However, after performing RNA sequencing in 39 pediatric brain samples, we find the primary transcript expressed in the brain includes the downstream exon 10a instead. Using this information, we evaluated genotype-phenotype correlations of variants affecting exon 10a and identified a cohort of eleven previously unreported individuals. Eight individuals harbor a recurrent de novo splice site variant, c.1197-8G>A (GenBank: NM&#95;001288739.1), which affects exon 10a and leads to DEE consistent with the classical DNM1 phenotype. We find this splice site variant leads to disease through an unexpected dominant-negative mechanism. Functional testing reveals an in-frame upstream splice acceptor causing insertion of two amino acids predicted to impair oligomerization-dependent activity. This is supported by neuropathological samples showing accumulation of enlarged synaptic vesicles adherent to the plasma membrane consistent with impaired vesicular fission. Two additional individuals with missense variants affecting exon 10a, p.Arg399Trp and p.Gly401Asp, had a similar DEE phenotype. In contrast, one individual with a missense variant affecting exon 10b, p.Pro405Leu, which is less expressed in the brain, had a correspondingly less severe presentation. Thus, we implicate variants affecting exon 10a as causing the severe DEE typically associated with DNM1-related disorders. We highlight the importance of considering relevant isoforms for disease-causing variants as well as the possibility of splice site variants acting through a dominant-negative mechanism., Competing Interests: Declaration of interests The authors declare no competing interests., (Copyright © 2022 American Society of Human Genetics. Published by Elsevier Inc. All rights reserved.)

Acquired aplastic anemia (AA) is caused by autoreactive T cell-mediated destruction of early hematopoietic cells. Somatic loss of human leukocyte antigen (HLA) class I alleles was identified as a mechanism of immune escape in surviving hematopoietic cells of some patients with AA. However, pathogenicity, structural characteristics, and clinical impact of specific HLA alleles in AA remain poorly understood. Here, we evaluated somatic HLA loss in 505 patients with AA from 2 multi-institutional cohorts. Using a combination of HLA mutation frequencies, peptide-binding structures, and association with AA in an independent cohort of 6,323 patients from the National Marrow Donor Program, we identified 19 AA risk alleles and 12 non-risk alleles and established a potentially novel AA HLA pathogenicity stratification. Our results define pathogenicity for the majority of common HLA-A/B alleles across diverse populations. Our study demonstrates that HLA alleles confer different risks of developing AA, but once AA develops, specific alleles are not associated with response to immunosuppression or transplant outcomes. However, higher pathogenicity alleles, particularly HLA-B*14:02, are associated with higher rates of clonal evolution in adult patients with AA. Our study provides insights into the immune pathogenesis of AA, opening the door to future autoantigen identification and improved understanding of clonal evolution in AA.

The relationship between hemiplegic shoulder pain (HSP) and subluxation is unclear. This study aimed to determine the differences of magnetic resonance imaging (MRI) findings in HSP patients with or without subluxation after stroke, and to analyze the etiology of shoulder pain. This retrospective study included 53 patients with HSP after stroke from September 2013 to February 2020. Patients underwent MRI of the shoulder because of shoulder pain. Clinical characteristics, including age, sex, stroke duration, body mass index, stroke type, visual analog scale score, Brunnstrom stage, and MRI arthrography findings of the affected shoulder, were recorded. Patients were classified into the glenohumeral subluxation (GHS) group ( n = 27) or non-glenohumeral subluxation (nGHS) group ( n = 26). We found that patients with HSP may be prone to bursa effusion, rotator cuff injury, ligament injury, and cartilage injury, even though there was no significant difference between the GHS and nGHS groups. MRI revealed 14 cases of long bicipital tendon-glenoid labrum injury (51.8%) in the GHS group and 6 cases (23.1%) in the nGHS group ( p = 0.030). We also found 10 cases (37%) of glenoid labrum injury in the GHS group and 2 cases (7.7%) in the nGHS group ( p = 0.026). Eight cases (29.6%) and 1 case (3.8%) of bone marrow edema were found in the GHS and nGHS groups, respectively ( p = 0.033). Compared with painful hemiplegic shoulder patients without subluxation, patients with subluxation may be more susceptible to some injuries, such as long bicipital tendon-glenoid labrum injury, glenoid labrum injury, and bone marrow edema. During rehabilitation, physicians need to pay attention to these injuries., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Xie, Zhang, Xu, Wang, Wang, Jia and Zhang.)

The objective of this study was to investigate whether brain volume changes occur in patients with chronic ankle instability (CAI) using voxel-based morphometry and assessing correlations with clinical tests. Structural magnetic resonance imaging data were prospectively acquired in 24 patients with CAI and 34 healthy controls. CAI symptoms and pain intensity were assessed using the Foot and Ankle Ability Measure (FAAM), Cumberland Ankle Instability Tool (CAIT), American Orthopedic Foot and Ankle Society (AOFAS) ankle-hindfoot score, and visual analog scale (VAS). The gray matter volume (GMV) of each voxel was compared between the two groups while controlling for age, sex, weight, and education level. Correlation analysis was performed to identify associations between abnormal GMV regions and the FAAM score, AOFAS score, VAS score, disease duration, and body mass index. Patients with CAI exhibited reduced GMV in the right precentral and postcentral areas, right parahippocampal area, left thalamus, left parahippocampal area, and left postcentral area compared to that of healthy controls. Furthermore, the right parahippocampal (r = 0.642, p = 0.001), left parahippocampal (r = 0.486, p = 0.016), and left postcentral areas (r = 0.521, p = 0.009) were positively correlated with disease duration. The left thalamus was positively correlated with the CAIT score and FAAM activities of daily living score (r = 0.463, p = 0.023 and r = 0.561, p = 0.004, respectively). A significant positive correlation was found between the local GMV of the right and left parahippocampal areas (r = 0.487, p = 0.016 and r = 0.763, p < 0.001, respectively) and the AOFAS score. Neural plasticity may occur in the precentral and postcentral areas, parahippocampal area, and thalamus in patients with CAI. The patterns of structural reorganization in patients with CAI may provide useful information on the neuropathological mechanisms of CAI., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Xie, Xing, Chen, Zhang, Qiu, Jia, Zhang and Yu.)

Angiosarcoma is a rare, devastating malignancy with few curative options for disseminated disease. We analyzed a recently published genomic data set of 48 angiosarcomas and noticed recurrent amplifications of HOXA -cluster genes in 33% of patients. HOXA genes are master regulators of embryonic vascular development and adult neovascularization, which provides a molecular rationale to suspect that amplified HOXA genes act as oncogenes in angiosarcoma. HOXA amplifications typically affected multiple pro-angiogenic HOXA genes and co-occurred with amplifications of CD36 and KDR, whereas the overall mutation rate in these tumors was relatively low. HOXA amplifications were found most commonly in angiosarcomas located in the breast and were rare in angiosarcomas arising in sun-exposed areas on the head, neck, face and scalp. Our data suggest that HOXA -amplified angiosarcoma is a distinct molecular subgroup. Efforts to develop therapies targeting oncogenic HOX gene expression in AML and other sarcomas may have relevance for HOXA -amplified angiosarcoma.

Objective: The purpose of this study was to investigate the effects of different durations of static progressive stretching (SPS) on posttraumatic knee contracture in rats, including range of motion (ROM), gait analysis, myofibroblast proliferation, and collagen regulation., Methods: The posttraumatic knee contracture model was established, and male Wistar rats were randomly divided into the 20-minute SPS treatment, 30-minute SPS treatment (S30), 40-minute SPS treatment, untreated, immobilization, and control groups. At Week 1, 2, and 4 of treatment intervention, joint ROM and gait were measured and compared. Knee joint samples stained with hematoxylin and eosin and Masson trichrome were used to observe alterations in pathological structures. Collagen density and cell numbers in the posterior joint capsule were used to assess joint capsule fibrosis and inflammation. Immunohistochemistry was used to detect type I collagen and α-smooth muscle actin expression., Results: The S30 group improved the most; ROM, stance, mean intensity, print area, and stride length were 115 (SD = 5) degrees, 0.423 (SD = 0.074) seconds, 156.020 (SD = 7.952), 2.116 (SD = 0.078) cm2, and 11.758 (SD = 0.548) cm, respectively. The numbers of myofibroblasts, fibroblasts, and inflammatory cells decreased, and collagen proliferation was significantly suppressed in the S30 group compared with the other groups., Conclusion: S30 significantly improved posttraumatic knee contracture in rats, with reduced type I collagen and α-smooth muscle actin expression, decreased the numbers of myofibroblasts and inflammatory cells, suppressed fibrotic and inflammatory changes in the joint capsule, and increased joint mobility. This study provided basic evidence for an optimal standard-of-care treatment approach for posttraumatic knee joint contracture in rats, which may have significance for humans., (© The Author(s) 2021. Published by Oxford University Press on behalf of the American Physical Therapy Association. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Objective: To evaluate the effectiveness of mirror therapy (MT) for phantom limb pain (PLP)., Data Sources: PubMed, EMBASE, Ovid MEDLINE, Scopus, Cochrane Library, Physiotherapy Evidence Database, CNKI, and WanFang Data were used to search for studies published up to March 31, 2021., Study Selection: Randomized controlled trials (RCTs) comparing the pain intensity of MT for PLP were performed. A total of 2094 articles were found. Among them, 10 were eligible for the final analysis., Data Extraction: The quality of the RCTs was assessed using the Physiotherapy Evidence Database (PEDro) scale by 2 independent reviewers. Outcome data were pooled according to follow-up intervals (1, 3, 6, and 12mo). Duration times were used as a basis for distinguishing subgroups. The primary evaluation was by visual analog scale. The PEDro scale was used to assess the methodological quality of studies., Data Synthesis: Meta-analysis revealed a statistically significant decrease in pain in the MT group vs the control group within 1 month (I 2 =0%; standardized mean difference [SMD]=-0.46, 95% confidence interval [CI], -0.79 to -0.13; P = .007). The patients with pain for longer than 1 year benefited more from MT (I 2 =0%; SMD=-0.46; 95% CI, -0.85 to -0.07; P = .02)., Conclusions: MT has beneficial effects for patients with PLP in the short-term, as evidenced by their improved pain scores. There was no evidence that MT had a long-term effect, but that may be a product of limited data. For patients with long-term PLP, MT may be an effective treatment., (Copyright © 2021 American Congress of Rehabilitation Medicine. Published by Elsevier Inc. All rights reserved.)

Glial cells play an important role in signal transduction, energy metabolism, extracellular ion homeostasis and neuroprotection of the central nervous system. However, few studies have explained the potential effects of exosomes from glial cells on central nervous system health and disease. In this study, the genes expressed in exosomes from astrocytes and microglia were identified by deep RNA sequencing. Kyoto Encyclopedia of Genes and Genomes analysis indicated that several pathways in these exosomes are responsible for promoting neurodegenerative diseases, including Alzheimer's disease, Parkinson's disease and Huntington's disease. Gene ontology analysis showed that extracellular exosome, mitochondrion and growth factor activity were enriched in exosomes from the unique astrocyte group, while extracellular exosome and mitochondrion were enriched in exosomes from the unique microglia group. Next, combined with the screening of hub genes, the protein-protein interaction network analysis showed that exosomes from astrocytes influence neurodegenerative diseases through metabolic balance and ubiquitin-dependent protein balance, whereas exosomes from microglia influence neurodegenerative diseases through immune inflammation and oxidative stress. Although there were differences in RNA expression between exosomes from astrocytes and microglia, the groups were related by the hub genes, ubiquitin B and heat shock protein family A (Hsp70) member 8. Ubiquitin B appeared to be involved in pleiotropic regulatory functions, including immune regulation, inflammation inhibition, protein catabolism, intracellular protein transport, exosomes and oxidative stress. The results revealed the clinical significance of exosomes from glia in neurodegenerative diseases. This study was approved by the Animal Ethics Committee of Nantong University, China (approval No. S20180102-152) on January 2, 2018., Competing Interests: None

Rationale: Hemangioblastomas (HGBMs) are very rare, and the cerebellum is usually the most common site of occurrence. HGBMs with ring-enhanced walls are often misdiagnosed as metastases, abscesses, glioblastomas, tuberculomas, and demyelinating diseases. Thus, we present a rare case of HGBM masquerading as a ring-enhancing lesion in the cerebellum., Patient Concerns: We present a 33-year-old female who was admitted to our department because of headaches, unstable walking, and visual loss in both eyes. Cranial nerve examination revealed deficits in cranial nerve II., Diagnosis: Magnetic resonance imaging revealed 2 cystic lesions in the cerebellum, with irregular ring-enhanced cyst walls composed of smaller nodular parts. Immunohistochemical staining of resected specimens established HGBM., Interventions: The lesions were completely resected using a right retrosigmoid approach., Outcomes: Two years of follow-up revealed no recurrence of her symptoms or tumor. She is currently well and performs her daily duties., Lessons: HGBMs with enhanced cysts are often misdiagnosed by radiology because of their ring-enhanced nature. Computed tomography angiography may be the best modality for differentiating cerebellar HGBM from other ring-enhancing lesions. Surgery is the gold standard of treatment for these lesions., Competing Interests: The authors have no funding and conflicts of interest to disclose., (Copyright © 2022 the Author(s). Published by Wolters Kluwer Health, Inc.)

Bone marrow (BM) niche-derived signals are critical for facilitating engraftment after hematopoietic stem cell (HSC) transplantation (HSCT). HSCT is required for restoration of hematopoiesis in patients with inherited BM failure syndromes (iBMFSs). Shwachman-Diamond syndrome (SDS) is a rare iBMFS associated with mutations in SBDS. Previous studies have demonstrated that SBDS deficiency in osteolineage niche cells causes BM dysfunction that promotes leukemia development. However, it is unknown whether BM niche defects caused by SBDS deficiency also impair efficient engraftment of healthy donor HSC after HSCT, a hypothesis that could explain morbidity noted after clinical HSCT for patients with SDS. Here, we report a mouse model with inducible Sbds deletion in hematopoietic and osteolineage cells. Primary and secondary BM transplantation (BMT) studies demonstrated that SBDS deficiency within BM niches caused poor donor hematopoietic recovery and specifically poor HSC engraftment after myeloablative BMT. We have also identified multiple molecular and cellular defects within niche populations that are driven by SBDS deficiency and are accentuated by or develop specifically after myeloablative conditioning. These abnormalities include altered frequencies of multiple niche cell subsets, including mesenchymal lineage cells, macrophages, and endothelial cells; disruption of growth factor signaling, chemokine pathway activation, and adhesion molecule expression; and p53 pathway activation and signals involved in cell cycle arrest. Taken together, this study demonstrates that SBDS deficiency profoundly impacts recipient hematopoietic niche function in the setting of HSCT, suggesting that novel therapeutic strategies targeting host niches could improve clinical HSCT outcomes for patients with SDS., (© 2022 by The American Society of Hematology. Licensed under Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International (CC BY-NC-ND 4.0), permitting only noncommercial, nonderivative use with attribution. All other rights reserved.)

Rationale: Glioma in the petroclival region is very rare. Also, very few cases of primary gliomas have been reported to have radiographic as well as intraoperative features of extra-axial lesions resulting in diagnostic dilemma in the literature. We present a rare case of petroclival glioma mimicking trigeminal schwannoma in a young female., Patient Concerns: We present a 21-years old female with a 3-month history of pain in the right eye with no visual impairment. Cranial nerves examination revealed mild deficits in the trigeminal nerve, facial nerve, auditory nerve, oculomotor as well as the trochlear nerve., Diagnoses: Magnetic resonance imaging detected an extra-axial mass with mixed signal intensities in the right petroclivus area. Immunohistochemical established glioma with world health organization (WHO) grade II., Interventions: The lesion was resected via 2 successive operations in 6 months interval. The patient was further treated with radiotherapy and post-radiotherapy temozolamide., Outcomes: Two years follow-up revealed no recurrence of the lesions and she is well. Nevertheless, he is still being followed diligently to uncover any recurrence., Lessons: The extra-axial nature as well as petroclival location of the glioma makes our case very unique and very rare. The imaging characteristics were very extraordinary for a glioma which resulted in diagnostic dilemma. Thus, the definitive diagnosis was based on the histopathological evaluation of the excised tumor., Competing Interests: The authors have no funding and conflicts of interests to disclose., (Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.)