bluuebunny/crossref_metadata_2025_split huggingface dataset:
Clinically amyopathic dermatomyositis manifested after the allogeneic haematopoietic stem cell transplantation: Case presentation and literature review
Authors: Miyagi Riko , Nakachi Sawako , Tamaki Yasutaro , Doi Mototsugu , Nakajima Tomo , Kitamura Sakiko , Tomori Shohei , Hanashiro Taeko , Tamaki Keita , Morichika Kazuho , Nishi Yukiko , Morishima Satoko , Fukushima Takuya , Masuzaki Hiroaki 
Published: August 2022

ABSTRACT
Clinically amyopathic dermatomyositis (CADM) lacks muscle symptoms, associated with rapidly progressive interstitial lung disease. Anti-melanoma differentiation-associated gene 5 (MDA-5) antibody has been identified as a disease-labelling autoantibody. We report two cases of CADM manifested after the allogeneic haematopoietic stem cell transplantation (allo-HSCT)—Case 1: a 56-year-old man with acute leukaemia received the allo-HSCT and Case 2: a 45-year-old female patient with lymphoma received the allo-HSCT. She received donor lymphocyte infusion because of a post-transplant relapse. After allo-HSCT or donor lymphocyte infusion, Gottron papules emerged, and both patients were diagnosed as CADM based on dermatological findings coupled with the positivity of anti-MDA-5 antibody, accompanied by interstitial shadows consistent with ILD on chest computed tomography. Case 2 was initially diagnosed as a kind of chronic graft versus host disease. Their symptoms were improved by the combination of immunosuppressive agents with a concomitant decrease in anti-MDA-5 antibody levels. For Case 2, rituximab was subsequently started for relapse of lymphoma, resulting in a substantial decrease in the level of anti-MDA-5 antibody and improvement in rash and ILD. Our cases raise a possibility that CADM emerges after the HSCT, highlighting the importance of early diagnosis to avoid fated progression into ILD.
Development of IgG4-related pancreatitis and kidney disease 7 years after the onset of undiagnosed lymphadenopathy: A case report
Authors: Yoshida Misaki , Mizushima Ichiro , Tsuge Shunsuke , Takahashi Yoshinori , Zoshima Takeshi , Nishioka Ryo , Hara Satoshi , Ito Kiyoaki , Kawano Mitsuhiro 
Published: August 2022

ABSTRACT
This report describes a patient diagnosed with immunoglobulin G4 (IgG4)-related pancreatitis and kidney disease 7 years after the onset of undiagnosed lymphadenopathy. A 48-year-old Japanese woman presented with fatigue and leg oedema. Computed tomography showed perigastric lymphadenopathy, for which she underwent a laparoscopic biopsy of the perigastric lymph nodes. Although histopathological examination of the lymph nodes did not lead to a definitive diagnosis, serological tests revealed elevated serum IgG4 levels (558 mg/dl) and IgG4 immunostaining of the lymph nodes showed IgG4-positive plasma cell infiltration, leading to the suspicion of IgG4-related disease. Further workup revealed no organ lesion other than lymphadenopathy. At age 55 years, despite having no subjective symptoms, contrast-enhanced computed tomography showed low-density lesions in the tail of the pancreas and the left kidney. Histopathological examination showed lymphocyte infiltration, consisting of a mixture of plasma cells and eosinophils, in both organs and obliterative phlebitis in the pancreas. IgG4 immunostaining of the kidney specimens showed 160 IgG4-positive cells per high-powered field, with the IgG4+/IgG+ cell ratio being almost 100%, leading to a diagnosis of IgG4-related pancreatitis and kidney disease. Treatment with prednisolone for 2 months resulted in lesion improvement. Although the diagnosis of IgG4-related lymphadenopathy is often challenging in patients with lymphadenopathy alone, findings in the present patient emphasise the importance of long-term follow-up, as it may allow early detection of involvement of other organs by IgG4-related disease.
Transcription factor NTL9 negatively regulates Arabidopsis vascular cambium development during stem secondary growth
Authors: Sugimoto Hiroki , Tanaka Tomoko , Muramoto Nobuhiko , Kitagawa-Yogo Ritsuko , Mitsukawa Norihiro 
Published: August 2022

AbstractIn plant stems, secondary vascular development is established through the differentiation of cylindrical vascular cambium, producing secondary xylem (wood) and phloem (bast), which have economic importance. However, there is a dearth of knowledge on the genetic mechanism underlying this process. NAC with Transmembrane Motif 1-like transcription factor 9 (NTL9) plays a central role in abiotic and immune signaling responses. Here, we investigated the role of NTL9 in vascular cambium development in Arabidopsis (Arabidopsis thaliana) inflorescence stems by identifying and characterizing an Arabidopsis phloem circular-timing (pct) mutant. The pct mutant exhibited enhanced vascular cambium formation following secondary phloem production. In the pct mutant, although normal organization in vascular bundles was maintained, vascular cambium differentiation occurred at an early stage of stem development, which was associated with increased expression of cambium-/phloem-related genes and enhanced cambium activity. The pct mutant stem phenotype was caused by a recessive frameshift mutation that disrupts the transmembrane (TM) domain of NTL9. Our results indicate that NTL9 functions as a negative regulator of cambial activity and has a suppressive role in developmental transition to the secondary growth phase in stem vasculature, which is necessary for its precise TM domain-mediated regulation.
Sobolev regularity for linear growth functionals acting on ℂ-elliptic operators
Authors: Wozniak Piotr 
Published: August 2022

AbstractIn this paper, we prove the higher Sobolev regularity of minimizers for convex integral functionals evaluated on linear differential operators of order one. This work intends to generalize the already existing theory for the cases of full and symmetric gradients to the entire class of ${\mathbb C}$-elliptic operators therein including the trace-free symmetric gradient for dimension $n \geq 3$.
Comparing MRI and conventional radiography for the detection of structural changes indicative of axial spondyloarthritis in the ASAS cohort
Authors: Protopopov Mikhail , Proft Fabian , Wichuk Stephanie , Machado Pedro M , Lambert Robert G , Weber Ulrich , Juhl Pedersen Susanne , Østergaard Mikkel , Sieper Joachim , Rudwaleit Martin , Baraliakos Xenofon , Maksymowych Walter P , Poddubnyy Denis 
Published: August 2022

AbstractObjectivesTo compare MRI and conventional radiography of SI joints for detection of structural lesions typical for axial spondyloarthritis (axSpA).MethodsAdult patients from the Assessment of SpondyloArthritis international Society (ASAS) cohort with symptoms suggestive of axSpA and both SI joint MRI and radiographs available for central reading were included. Radiographs were evaluated by three readers according to the modified New York (mNY) criteria grading system. The presence of structural damage on radiographs was defined as fulfilment of the radiographic mNY criterion and, additionally, a lower threshold for sacroiliitis of at least grade 2 unilaterally. MRI scans were assessed for the presence of structural changes indicative of axSpA by seven readers. Diagnostic performance [sensitivity, specificity, positive and negative predictive values (PPV and NPV) and positive and negative likelihood ratios (LR+ and LR−)] of MRI and radiographs (vs rheumatologist’s diagnosis of axSpA) were calculated.ResultsOverall, 183 patients were included and 135 (73.7%) were diagnosed with axSpA. Structural lesions indicative of axSpA on MRI had sensitivity 38.5%, specificity 91.7%, PPV 92.9%, NPV 34.6%, LR+ 4.62 and LR− 0.67. Sacroiliitis according to the mNY criteria had sensitivity 54.8%, specificity 70.8%, PPV 84.1%, NPV 35.8%, LR+ 1.88 and LR− 0.64. Radiographic sacroiliitis of at least grade 2 unilaterally had sensitivity 65.2%, specificity 50.0%, PPV 78.6%, NPV 33.8%, LR+ 1.30 and LR− 0.69.ConclusionStructural lesions of the SI joint detected by MRI demonstrated better diagnostic performance and better interreader reliability compared with conventional radiography.
The effect of group-based cognitive behavioural therapy for insomnia in patients with rheumatoid arthritis: a randomized controlled trial
Authors: Latocha Kristine M , Løppenthin Katrine B , Østergaard Mikkel , Jennum Poul J , Hetland Merete L , Røgind Henrik , Lundbak Tine , Midtgaard Julie , Christensen Robin , Esbensen Bente A 
Published: August 2022

AbstractObjectivesThe primary objective was to compare the effect of cognitive behavioural therapy for insomnia (CBT-I) to usual care on sleep efficiency, measured by polysomnography (PSG) immediately after the intervention at week 7. Secondary objectives included comparing the longer-term effect on sleep- and RA-related outcomes at week 26.MethodsIn a randomized controlled trial using a parallel group design, the experimental intervention was 6 weeks’ nurse-led group-based CBT-I; the comparator was usual care. Analyses were based on the intention-to-treat (ITT) principle; missing data were statistically modelled using repeated-measures linear mixed effects models adjusted for the level at baseline.ResultsThe ITT population consisted of 62 patients (89% women), with an average age of 58 years and an average sleep efficiency of 83.1%. At primary end point, sleep efficiency was 88.7% in the CBT-I group, compared with 83.7% in the control group (difference: 5.03 [95% CI −0.37, 10.43]; P = 0.068) measured by PSG at week 7. Key secondary outcomes measured with PSG had not improved at week 26. However, for all the patient-reported key secondary sleep- and RA-related outcomes, there were statistically highly significant differences between CBT-I and usual care (P &lt; 0.0001), e.g. insomnia (Insomnia Severity Index: −9.85 [95% CI −11.77, −7.92]) and the RA impact of disease (RAID: −1.36 [95% CI −1.92, −0.80]) at week 26.ConclusionNurse-led group-based CBT-I did not lead to an effect on sleep efficiency objectively measured with PSG. However, CBT-I showed improvement on all patient-reported key secondary sleep- and RA-related outcomes measured at week 26.Trial registrationClinicalTrials.gov, https://clinicaltrials.gov, NCT03766100.
Height Gain After Spinal Fusion for Idiopathic Scoliosis: Which Model Fits Best?
Authors: Ikwuezunma Ijezie , Wang Kevin , Raymond Seye , Badin Daniel , Kreulen R. Timothy , Jain Amit , Sponseller Paul D. , Margalit Adam 
Published: August 2022

Background:
Patients will often inquire about the magnitude of height gain after scoliosis surgery. Several published models have attempted to predict height gain using preoperative variables. Many of these models reported good internal validity but have not been validated against an external cohort. We attempted to test the validity of 5 published models against an external cohort from our institution. Models included were Hwang, Van Popta, Spencer, Watanabe, and Sarlak models.

Methods:
We retrospectively queried our institution’s records from 2006 to 2019 for patients with adolescent idiopathic scoliosis treated with posterior spinal fusion. We recorded preoperative and postoperative variables including clinical height measurements. We also performed radiographic measurements on preoperative and postoperative radiographic studies. We then tested the ability of the models to predict height gain by evaluating Pearson correlation coefficient, root mean square error, Akaike Information Criterion for each model.

Results:
A total of 387 patients were included. Mean clinical height gain was 3.1 (±1.7) cm.
All models demonstrated a moderate positive Pearson correlation coefficient, except the Hwang model, which demonstrated a weak correlation. The Spencer model was the only model with acceptable root mean square error (≤0.5) and was also the best fitting with the lowest Akaike Information Criterion (−308). The mean differences in height gain predictions between all models except the Hwang model was ≤1 cm.

Conclusions:
Four of the 5 models demonstrated moderate correlation and had good external validity compared with their development cohorts. Although the Spencer model was the best fitting, the clinical significance of the difference in height predictions compared with other models was low. The Watanabe model was the second best fitting and had the simplest formula, making it the most convenient to use in a clinical setting. We offer a simplified equation to use in a preoperative clinical setting based on this data—ΔHeight (mm)=0.77*(preoperative coronal angle−postoperative coronal angle).

Level of Evidence:
Not Applicable.
Risk Factors for Avascular Necrosis After Closed Reduction for Developmental Dysplasia of the Hip
Authors: Bian Zhen , Guo Yuan , Lyu XueMin , Zhu ZhenHua , Yang Zheng , Wang YuKun 
Published: August 2022

Background:
Avascular necrosis (AVN) is a major complication after closed reduction for developmental dysplasia of the hip. The factors that predispose to AVN remain controversial. The purpose of this study was to analyze the risk factors, especially patient factors, such as age at reduction, grade of dislocation, and ossific nucleus development, related to AVN.

Materials and Methods:
We retrospectively reviewed children with dysplasia of the hip treated by closed reduction between 1997 and 2006. AVN was evaluated using Salter criteria and Kalamchi and MacEwen classification. Related factors were analyzed.

Results:
One hundred and eight children (140 hips) with an average age of 16.6 months at closed reduction (range: 6-24 mo) were included in the study. For an average duration of 10.1 years (range 7–16 y) of follow-up, 44 hips (31.4%) developed AVN. Grade II or higher AVN occurred in 14 hips (10%). The incidence of AVN increased with the grade of dislocation (P=0.022) and underdevelopment of the ossific nucleus (P<0.001). Underdevelopment of the ossific nucleus was also found to be positively correlated with the dislocation grade (P=0.047). The age at the time of reduction, sex, and side were not significant factors. Children who underwent secondary operation were all older than 1 year at reduction.

Conclusions:
High-grade dislocation correlates with the underdevelopment of the ossific nucleus. Patients with these 2 characteristics are predisposed to AVN. As underdevelopment of the ossific nucleus occurred regardless of age, it is not advisable to delay reduction because it does not alter the AVN rate, and instead, it increases the secondary operation rate.

Level of Evidence:
Level IV case series.
Redislocation After Posteromedial Open Reduction in Developmental Dysplasia of the Hip: Analyzing the Risk Factors and Determining the Optimal Treatment Method
Authors: Gencer Batuhan , Doğan Özgür , Çulcu Ahmet , Biçimoğlu Ali 
Published: August 2022

Background:
Redislocation, which is one of the most serious problems in developmental dysplasia of the hip (DDH), might occur because of several reasons. Regardless of the treatment, redislocations are reported to be associated with high complication rates in the literature. Our objective was to identify the risk factors for redislocation in the patients treated for DDH and to determine the optimal treatment method for redislocations.

Methods:
Patients with DDH who were treated with the limited posteromedial approach in our clinic between 1993 and 2021 and followed up prospectively were examined in this single-centered study. The participants were assigned into 2 groups: a study group consisting of 25 hips of 17 patients with redislocation and a control group consisting of 502 hips of 390 patients without redislocation. To determine the risk factors for redislocation, demographic data, known risk factors for DDH, preoperative Tönnis stage, and whether the capsule was opened or not were evaluated. To determine the optimal treatment method, a subgroup analysis based on applied treatment (closed reduction and cast replacement vs. repeating open reduction) was conducted, and recurrent redislocation, complication, and secondary surgery rates were evaluated.

Results:
Bilaterality and high-grade hip dislocations were found to be associated with higher odds ratio (OR) for redislocation [P=0.007, OR=3.64, 95% confidence interval (CI), 1.3 to 8.8; and P=0.006, OR=4.52, 95% CI, 1.37 to 14.91, respectively]. Recurrent redislocation and complication rates were found to be significantly higher in redislocations treated with closed reduction and cast replacement (P=0.007 and P=0.015, respectively).

Conclusions:
Bilaterality and higher preoperative Tönnis stage are critical risk factors for redislocation after open reduction in DDH. It should be kept in mind that closed reduction and cast replacement is associated with higher rates of recurrent redislocation and complications, and redislocation cases should be treated by repeating open reduction.

Level of Evidence:
Level II—therapeutic study.
Elimination of Herpes Simplex Virus-2 and Epstein-Barr Virus With Seraph 100 Microbind Affinity Blood Filter and Therapeutic Plasma Exchange: An Explorative Study in a Patient With Acute Liver Failure
Authors: Andermatt Rea , Bloemberg Guido V. , Ganter Christoph C. , Mueller Nicolas J. , Mueller Antonia M. S. , Muellhaupt Beat , Kielstein Jan T. , David Sascha 
Published: August 2022

OBJECTIVES:
Herpes simplex virus (HSV)-2 is a rare cause of hepatitis that can lead to acute liver failure (ALF) and often death. The earlier the initiation of acyclovir treatment the better the survival. With regard to ALF, controlled randomized data support the use of therapeutic plasma exchange (TPE) both as bridge to recovery or transplantation—possibly by modulating the systemic inflammatory response and by replacing coagulation factors. Seraph 100 Microbind Affinity Blood Filter (Seraph; Ex Thera Medical, Martinez, CA), a novel extracorporeal adsorption device, removes living pathogens by binding to a heparin-coated surface was shown to efficiently clear HSV-2 particles in vitro. Here, we tested the combination of Seraph with TPE to reduce a massive HSV-2 viral load to reach a situation in that liver transplantation would be feasible.

DESIGN:
Explorative study.

SETTING:
Academic tertiary care transplant center.

PATIENT:
Single patient with HSV-2–induced ALF.

INTERVENTIONS:
TPE + Seraph 100 Microbind Affinity Blood Filter.

MEASUREMENTS AND MAIN RESULTS:
We report Seraph clearance data of HSV-2 and of Epstein-Barr virus (EBV) in vivo as well as total viral elimination by TPE. Genome copies/mL of HSV-2 and EBV in EDTA plasma were measured by polymerase chain reaction every 60 minutes over 6 hours after starting Seraph both systemically and post adsorber. Also, HSV-2 and EBV were quantified before and after TPE and in the removed apheresis plasma. We found a total elimination of 1.81 × e11 HSV-2 copies and 2.11 × e6 EBV copies with a single TPE (exchange volume of 5L; 1.5× calculated plasma volume). Whole blood clearance of HSV-2 in the first 6 hours of treatment was 6.64 mL/min (4.98–12.92 mL/min). Despite much lower baseline viremia, clearance of EBV was higher 36.62 mL/min (22.67–53.48 mL/min).

CONCLUSIONS:
TPE was able to remove circulating HSV-2 copies by 25% and EBV copies by 40% from the blood. On the other hand, clearance of HSV-2 by Seraph was clinically irrelevant, but Seraph seemed to be far more effective of removing EBV, implicating a possible use in EBV-associated pathologies, but this requires further study.
Regulated cell death in cancer: from pathogenesis to treatment
Authors: Gong Linjing , Huang Dong , Shi Yujun , Liang Zong’an , Bu Hong 
Published: August 2022

Abstract
Regulated cell death (RCD), including apoptosis, pyroptosis, necroptosis, and ferroptosis, is regulated by a series of evolutionarily conserved pathways, and is required for development and tissue homeostasis. Based on previous genetic and biochemical explorations of cell death subroutines, the characteristics of each are generally considered distinctive. However, recent in-depth studies noted the presence of crosstalk between the different forms of RCD; hence, the concept of PANoptosis appeared. Cancer, a complex genetic disease, is characterized by stepwise deregulation of cell apoptosis and proliferation, with significant morbidity and mortality globally. At present, studies on the different RCD pathways, as well as the intricate relationships between different cell death subroutines, mainly focus on infectious diseases, and their roles in cancer remain unclear. As cancers are characterized by dysregulated cell death and inflammatory responses, most current treatment strategies aim to selectively induce cell death via different RCD pathways in cancer cells. In this review, we describe five types of RCD pathways in detail with respect to tumorigenesis and cancer progression. The potential value of some of these key effector molecules in tumor diagnosis and therapeutic response has also been raised. We then review and highlight recent progress in cancer treatment based on PANoptosis and ferroptosis induced by small-molecule compounds, immune checkpoint inhibitors, and nanoparticles. Together, these findings may provide meaningful evidence to fill in the gaps between cancer pathogenesis and RCD pathways to develop better cancer therapeutic strategies.
Roles of organelle-specific autophagy in hepatocytes in the development and treatment of non-alcoholic fatty liver disease
Authors: Zhang Yizhi , Chen Yu 
Published: August 2022

Abstract
Non-alcoholic fatty liver disease (NAFLD) is a disorder of lipid metabolism. The lipotoxic intermediates of lipid metabolism cause mitochondrial dysfunction and endoplasmic reticulum stress. Organelle-specific autophagy is responsible for the removal of dysfunctional organelles to maintain intracellular homeostasis. Lipophagy contributes to lipid turnover by degrading lipid droplets. The level of autophagy changes during the course of NAFLD, and the activation of hepatocyte autophagy might represent a method of treating NAFLD.
Inactivation of tumor suppressor TAp63 by hepatitis B virus X protein in hepatocellular carcinoma
Authors: Xie Bangxiang , Hao Qian , Zhou Xiang , Chen Dexi 
Published: August 2022

Abstract

Background:
The hepatitis B virus X (HBx) protein plays a critical role in the initiation and progression of hepatitis B virus (HBV)-associated hepatocellular carcinoma (HCC). In the early stage of the disease, HBx facilitates tumor onset by inactivating the tumor suppressor p53. The p53-encoding gene, however, is frequently mutated or deleted as the cancer progresses to the late stage and, under such circumstance, the p53 homolog TAp63 can harness HCC growth by transactivating several important p53-target genes.

Methods:
To determine whether HBx regulates TAp63, we performed co-immunoprecipitation assay, real-time quantitative polymerase chain reaction, immunoblotting, and flow cytometry analysis in p53-null cancer cell lines, Hep3B and H1299.

Results:
HBx interacts with the transactivation domain of TAp63, as HBx was co-immunoprecipitated with TAp63 but not with ΔNp63. The interaction between HBx and TAp63 abolished transcriptional activity of TAp63, as evidenced by the reduction of the levels of its target genes p21 and PUMA, consequently leading to restricted apoptosis and augmented proliferation of HCC cells.

Conclusion:
HBV induces progression of HCC that harbors defective p53 by inhibiting the tumor suppressor TAp63.