bluuebunny/crossref_metadata_2025_split huggingface dataset:
Increased fat mass and obesity risk after elexacaftor–tezacaftor–ivacaftor therapy in young adults with cystic fibrosis
Authors: Merino Sánchez-Cañete Ana , López Cárdenes Concepción Marina , Vicente Santamaría Saioa , Gutiérrez Martínez José Ramón , Suárez González Marta , Álvarez Merino María , González Jiménez David 
Published: November 2024

BackgroundWhen people with cystic fibrosis (PwCFs) are treated with cystic fibrosis transmembrane conductance regulator protein modulator (CFTRm), it leads to changes in body composition. An easy, non-invasive, and reproducible method to assess this is by means of electrical bioimpedance measurement, which complements the information provided by the already-known anthropometric parameters.MethodsSeventeen adults with cystic fibrosis in treatment with elexacaftor–tezacaftor–ivacaftor (ETI) were recruited for a prospective, longitudinal, single-center study over 12 months. Study variables included weight, body mass index (BMI), and body composition by bioelectrical impedance analysis (BIA) [including fat mass (FM) and fat-free mass (FFM)].ResultsAt 12 months, there was an increase in overweight patients (5.9% vs. 23.5%) and a statistically significant increase in BMI at 6 and 12 months. An increase in FM and FFM was observed at 12 months. The increase was greater in FM (95% CI) from a baseline of 1.77% (0.00–3.54; p = 0.050) at 6 months and 2.64% (0.48–4.81; p = 0.020) at 12 months.ConclusionAfter treatment with ETI, there was an increase in weight, BMI, and FM more than FFM in PwCF. These changes would be confirmed in long-term studies to improve nutritional management.
The effect of a low-carbohydrate diet on subcutaneous adipose tissue in females with lipedema
Authors: Lundanes Julianne , Gårseth Mari , Taylor Shannon , Crescenzi Rachelle , Pridmore Michael , Wagnild Rune , Hyldmo Åsne Ask , Martins Catia , Nymo Siren 
Published: November 2024

IntroductionLipedema is a common, yet underdiagnosed, subcutaneous adipose tissue (SAT) disorder. The main characteristics are SAT expansion in the lower extremities and arms, pain, and tenderness to palpation. It remains unknown if a low-carbohydrate diet (LCD) influences SAT in females with lipedema.ObjectivesTo evaluate the effect of a LCD low-energy diet, compared to a low-fat isoenergetic control diet, on calf subcutaneous adipose tissue area, muscle area, SAT/muscle ratio, calf circumference and body composition in females with lipedema.Subjects/methodsAdult females with obesity and lipedema were randomized to 1,200 kcal/day diets, either LCD or control (75 and 180 g/day of carbohydrates, respectively) for 8 weeks. Body composition was measured with bioelectrical impedance analysis, calf SAT area, muscle area, and circumference with magnetic resonance imaging and pain with brief pain inventory, before and after the intervention.ResultsThirteen participants were included (five in the LCD group), with a mean age of 46 ± 12 years and a BMI of 37 ± 6 kg/m2. A significant reduction in calf SAT area, calf circumference, and pain was observed in the LCD group only. Both LCD and control groups experienced a significant reduction body weight, fat mass, fat free mass, and muscle area, with no differences between groups. No significant changes over time were found for SAT/muscle ratio.ConclusionA LCD has the potential to reduce SAT and pain in females with lipedema, despite a reduction in muscle mass in lipedema affected areas in both diet groups. Further studies are needed to confirm these findings and explore potential mechanisms.Clinical trial registrationNCT04632810: Effect of ketosis on pain and quality of life in patients with lipedema (Lipodiet). https://clinicaltrials.gov/study/NCT04632810.
Spleen tyrosine kinase inhibition mitigates radiation-induced lung injury through anti-inflammatory effects and downregulation of p38 MAPK and p53
Authors: Zhang Guoxing , Sun Ni , Li Xiaohua 
Published: November 2024

BackgroundTo explore new modulatory intervention targets for radiation-induced lung injury, bioinformatics analysis technology was used to search for the core driving genes in the pathogenesis of radiation pneumonitis, and the results were verified by a radiation-induced murine lung injury model to find possible new targets for the treatment of radiation lung injury.MethodGene Expression Omnibus Database was used to identify differentially expressed genes in radiation pneumonitis. DAVID database was used for gene ontology (GO) and Kyoto Encyclopedia of Genes and Genome (KEGG) enrichment analysis. Gene Set Enrichment Analysis was used to analyze abnormal expressions. Protein–protein interaction networks were constructed using STRING and Cytoscape. Discovery Studio 4.5 software was used to find the preferred inhibitor of the specific gene. A radiation-induced lung injury model was induced in female C57BL/6N mice. The specific inhibitors were administered by intraperitoneal injection 24 h before and for 7 consecutive days after radiation. Lungs were harvested for further analysis 14 days and 10 weeks post-irradiation.ResultsWe screened Syk as one of the most important driver genes of radiation pneumonitis by bioinformatics analysis and screened the preferred Syk inhibitor fostamatinib from the drug database. Syk was highly expressed in irradiated lung tissue, and fostamatinib inhibited the level of Syk expression. Syk inhibitor significantly alleviated the radiation-induced lung injury and downregulated the increased expression of p38 MAPK, p53, IL-1β, and IL-6 in lung tissue at 2 weeks after radiation. The levels of TGF-β, COL1A1, and α-SMA and degree of pulmonary fibrosis at 10 weeks after radiation were also decreased by Syk inhibitor.ConclusionSyk inhibitor may have a potential to be used as a targeted drug to mitigate radiation pneumonitis and inhibit radiation-induced pulmonary fibrosis.
Combination of ultrasound guided superficial cervical plexus block and local infiltration for oromaxillofacial surgeries: a case series
Authors: Zhao Hao-ran , Hao Jian-shuai , Xue Ling-fa , Zhao Jin-ze , Wang Yi-chen , Xiao Wen-lin 
Published: November 2024

IntroductionWhen elderly patients have underlying diseases combined with oromaxillofacial diseases requiring surgical treatment, the application of conventional general anesthesia (GA) for oromaxillofacial surgical diseases has become a risk due to underlying disease reasons. The objective of this study was to evaluate the efficacy and safety of ultrasound-guided superficial cervical plexus block (SCPB) anesthesia combined with local infiltration anesthesia (LIA) for partial oral and maxillofacial surgery (OMFS) in patients who with risk for GA due to underlying disease.MethodsThe clinical data of 7 high risk patients with OMFS treated with SCPB anesthesia combined with LIA were retrospectively analyzed. All seven surgeries were performed on one side of the neck. All patients were given ultrasound-guided SCPB anesthesia by the same anesthesiologist, LIA by the same surgeon, and surgery was performed under continuous Electrocardiogram (ECG) monitoring.ResultsSeven patients had stable vital signs and no significant postoperative complications. The results of this study indicated that SCPB anesthesia combined with LIA is a safe and effective anesthesia technique with a high success rate and patient tolerance.DiscussionFor patients with OMFS who have a risk for GA due to underlying diseases, ultrasound-guided cervical superficial plexus block anesthesia combined with LIA is a safe and effective alternative to conventional GA.
Commissioning of a novel gantry-less proton therapy system
Authors: Feldman Jon , Pryanichnikov Alexander , Achkienasi Alejandro , Polyansky Ilya , Hillman Yair , Raskin Stas , Blumenfeld Philip , Popovtzer Aron , Marash Michael 
Published: November 2024

PurposeThe focus of this article is to describe the configuration, testing, and commissioning of a novel gantry-less synchrotron-based proton therapy (PT) facility.Materials and methodsThe described PT system delivers protons with a water equivalent range between 4 and 38 cm in 1800 energy layers. The fixed beam delivery permits a maximum field size of 28 × 30 cm2. The patient positioning and imaging system includes a six-degree-of-freedom robotic arm, a convertible patient chair, a vertical 4DCT, and an orthogonal 2D X-ray imaging system.ResultsThe spot positioning reproducibility was consistent within ±1 mm. The width (σ) of the beam profile at the isocenter was energy dependent and ranged from 2.8 mm to 7.7 mm. Absolute dose reproducibility was measured and deviations were found to be &lt;0.62% for all possible beam scenarios. The built-in dose monitoring system was successfully tested for its ability to generate interlocks under specific conditions (beam spot deviation ≥2 mm, individual spot dose ≥10% or ≥0.25 Gy, spot energy deviation ≥0.5 MeV). The robot positioning exhibited a consistent reproducibility within ±1 mm. All tested scenarios achieved laser-free initial 3D/3D image-guided positioning within ±5 mm. Subsequent 2D/3D positioning showed an accuracy of ±1 mm. A single 2D/3D image registration event corrected positions in all cases. Results of gamma analysis (3%, 3 mm) demonstrated pass rates greater than 95% for head and neck, thorax, abdomen treatment plans.ConclusionsWe report on the performance of a novel single-room gantry-less PT system comprised of a compact synchrotron and an adjustable (from nearly horizontal to almost vertical) patient positioning system. The commissioning results show high accuracy and reproducibility of the main proton beam parameters and the patient positioning system. The new PT facility started patient treatments in March 2023, which were the first in Israel and the Middle Eastern region.
Efficacy and safety of Toliparibumab for the treatment of non-small cell lung cancer: a systematic review and meta-analysis
Authors: Liu Yihao , Yang Liangyu , Duan Zhixuan , Cheng Qian , Liu Minghui , Zhang HongBing , Zhao HongLin 
Published: November 2024

PurposeThis research intends to investigate the treatment of non-small cell lung cancer (NSCLC) using Toripalimab, focusing on its effectiveness and safety profile. Efficacy refers to the survival prognosis, while safety pertains to the occurrence of adverse events in our study. It also aims to provide reference information for neoadjuvant and postoperative therapies.MethodsUp to March 20, 2024, studies on randomized controlled trials and single-arm trials involving Toripalimab for NSCLC were sourced from the Cochrane Library, Embase, PubMed, and Web of Science databases. Data extraction and analysis were independently conducted by two researchers utilizing Stata 15.0 and R software.ResultsA total of 8 studies were analyzed, including 6 single-arm studies and 2 randomized controlled trials (RCTs). Toripalimab treatment in the RCTs showed an overall survival (OS) of [HR=0.67, 95% CI (0.53, 0.85); p=0.71]. The objective response rate (ORR) from single-arm studies was reported as [ES=0.59, 95% CI (0.36, 0.81); p&lt;0.01], and progression-free survival (PFS) was [ES=4.89, 95% CI (2.65, 9.02); p&lt;0.01]. Furthermore, observed adverse effects included Anemia [OR=0.53, 95% CI (0.26, 0.79); p&lt;0.01], Neutropenia [OR=0.43, 95% CI (0.20, 0.68); p&lt;0.01], and Thrombocytopenia [OR=0.28, 95% CI (0.18, 0.43); p&lt;0.01].ConclusionsToripalimab, being China’s first domestically developed anti-tumor PD-1 antibody drug, shows potential advantages over traditional chemotherapy in possibly prolonging patients’ survival times. However, the limited number of studies included indicates the need for additional single-arm and RCT studies to further validate these findings.Systematic review registrationhttps://www.crd.york.ac.uk/PROSPERO/, identifier (CRD42024519806).
Orbital granular cell tumor involving the superior rectus muscle: a case report
Authors: Wang Pei , Han Zijian , Peng Li , Li Xiuhong , Yuan Hongfeng 
Published: November 2024

ObjectiveThe aim of this case report is to assess the clinicopathological characteristics and differential diagnosis of orbital granular cell tumor (GCT).MethodsClinical and imaging data of a rare case of orbital GCT involving the superior rectus muscle were collected. Its clinical characteristics, imaging, and histopathological features were observed.ResultsA 36-year-old female patient presented with a 2-year history of left eye proptosis. Magnetic resonance imaging (MRI) enhancement suggested a space-occupying lesion in the left superior rectus muscle region. On T1-weighted and T2-weighted MRI, the tumor was isointense to gray matter and significantly enhanced on the enhanced scan. Microscopic examination revealed that most tumor cells exhibited diffuse growth with unclear boundaries, and some cells were arranged in small nests. The tumor cells were large, with abundant, coarse eosinophilic granules in the cytoplasm. Occasional cells contained larger round eosinophilic droplets in the cytoplasm. Focal areas showed foamy cells, small and central round or oval nuclei with occasional nuclear enlargement and mild atypia, inconspicuous nucleoli, rare mitoses, and low proliferative activity. Immunohistochemistry results were Vimentin (+), S-100 (+), CD68 (+), Ki67 (2%+), Inhibin-a (−), CK (−), SMA (−), and Desmin (−). The pathological examination of a specimen harvested from the mass corresponded to a GCT.ConclusionOrbital GCT is rare and should be considered in the differential diagnosis of orbital tumors. It is essential to distinguish it from thyroid-associated ophthalmopathy, inflammatory pseudotumor, and myohemangioma. Definitive diagnosis requires a comprehensive analysis of clinical, histopathological, and immunohistochemical findings. Surgical excision is the primary treatment for orbital GCTs. For patients with incomplete tumor resection, close follow-up is necessary. Proton beam radiation therapy can be considered to prevent recurrence or metastasis if needed.
Uterine smooth muscle tumors of uncertain malignant potential: a 13-year retrospective study
Authors: Liu Liuliu , Xiao Zhendong , Li Zhiwen , Zheng Jinyu , Xu Xiaofeng , Zhou Huaijun 
Published: November 2024

ObjectiveThe primary objective of this study was to provide valuable evidence for the management of patients diagnosed with uterine smooth muscle tumors of uncertain malignant potential (STUMP), with a focus on those with reproductive aspirations.MethodsWe conducted a retrospective analysis of clinical and pathological data from the medical records and slides of STUMP patients treated at Drum Tower Hospital, affiliated with Nanjing University Medical School, from January 2009 to December 2021.ResultsThirty-four patients were included in the study, with a median follow-up duration of 76 months (range: 13-157 months). After slide review, the diagnosis agreement rate was 77.3% (34/44 among initially considered cases). The consistency rate between our hospital’s diagnosis and those of other institutions was 75% (15/20). The accuracy rate of intraoperative frozen section diagnosis was low, at 21.4% (3/14). Half of the patients (17) underwent myomectomy, while the other half (17) received hysterectomy, including one subtotal hysterectomy. Two recurrences were observed (5.9%), one as STUMP and the other as leiomyosarcoma, with one recurrence in each surgical group. Notably, 4 of 9 patients with reproductive aspirations successfully underwent cesarean deliveries. Patients with single lesions appeared to exhibit potentially favorable fertility outcomes compared to those with multiple lesions.ConclusionThe diagnosis of STUMP was difficult. Myomectomy potentially could serve as an alternative for patients with reproductive needs. In selected cases with single lesions, it may indicate potentially favorable fertility outcomes.
Conformal proctectomy with sphincter preservation retains acceptable defecation functions in very low rectal cancer male patients
Authors: Chen Weijie , Zhang Xiao , Qiu Xiaoyuan , Zhou Jiaolin , Lin Guole 
Published: November 2024

BackgroundConformal proctectomy with sphincter preservation (CPSP) is designed to preserve the rectal wall as much as possible in very low rectal cancer patients. Evaluations of anal function and quality of life outcomes are lacking.MethodsThis study included male patients with very low (≤ 5 cm from the anal verge) rectal adenocarcinoma between January 1, 2020, and January 1, 2022. A LARS score questionnaire survey and EORTC-QLQ-CR38 questionnaire survey were administered.ResultsA total of 21 very low rectal cancer patients were enrolled in follow-up. The average age of the patients was 56.7 years, the tumors were 1.9 ± 0.6 cm in size, and the distance from the anal verge was 4.8 ± 0.5 cm. All patients were followed up, and the mean follow-up period was 2.7 ± 0.5 years. The LARS score increased significantly from 4.1 ± 2.8 before surgery to 19.1 ± 6.0 at the 1st year after surgery (P &lt; 0.001) and then decreased to 13.1 ± 4.2 (P &lt; 0.001) at the 2nd year. The quality of life of patients was also lower at the 1st year after surgery (61.1 ± 9.6 vs. 74.2 ± 11.2, P &lt; 0.001) and was restored at the 2nd year after surgery (80.6 ± 11.9 vs. 74.2 ± 11.2, P = 0.029). During standard follow-up at the outpatient department, no rectal tumor relapse was confirmed in these patients, although 2 patients were found to have suspected recurrence of local lymph node metastasis.ConclusionsThese results suggest that the CPSP technique preserves acceptable defecation function and is a safe and feasible option for male patients with very low rectal cancer.Clinical trial registrationhttps://www.chictr.org.cn/, identifier ChiCTR2100052094.
Liver transplantation in a child with sclerosing cholangitis due to Langerhans cell histiocytosis: a case report
Authors: Wang Xue-Lian , Fang Chun-Xiao , Chen Min-Xia , Yang Hua-Mei , She Lan-Hui , Gong Yu , Xu Yi , Xiao Wei-Qiang , Tian Jin-Sheng , Ai Bin , Huang Li , Li Xu-Fang 
Published: November 2024

BackgroundLangerhans cell histiocytosis (LCH) is a systemic neoplasia with diverse clinical manifestations, predominantly affecting bone and skin. However, in children, LCH presenting primarily with cholestasis is rare.Case summaryWe present the case of a 22-month-old boy who was admitted to our hospital with a history of intermittent fever and abdominal distension for over 2 months, and jaundice for over 1 month. Prior to admission, the child had been managed with anti-infective and anti-inflammatory drugs and supportive care at multiple hospitals without significant improvement. He was then referred to our facility for further treatment. Upon admission, a series of laboratory tests, imaging studies, and pathological examinations were conducted, revealing the presence of diabetes insipidus, sclerosing cholangitis (SC), and liver cirrhosis. These findings led to a clinical diagnosis of LCH. Given the absence of definitive pathological evidence, his progression to decompensated liver cirrhosis and his pronounced growth retardation, the child was deemed a candidate for living donor liver transplantation. Following the liver transplant, pathological examination of the explanted liver tissue confirmed the clinical diagnosis of LCH. The child received postoperative chemotherapy, which resolved his systemic symptoms and normalized liver function. There was no evidence of LCH recurrence. The symptoms of diabetes insipidus were controlled with desmopressin acetate, however, the existing destructive lesions in the pituitary gland persisted.ConclusionSclerosing cholangitis is a late and severe form of liver involvement in LCH that may be accompanied by lesions in other tissues or organs. Along with pathological evidence, a diagnosis should be made based on characteristic rashes, osteolytic lesions, and thickening of the pituitary stalk. In children with LCH complicated by SC for whom pathological diagnostic evidence cannot be obtained, liver transplantation may be considered once signs of decompensated liver cirrhosis appear, followed by systemic chemotherapy to control active disease. This strategy represents a therapeutic approach with the potential to achieve a better prognosis in children with LCH.
The role of SIRT1-FXR signaling pathway in valproic acid induced liver injury: a quantitative targeted metabolomic evaluation in epileptic children
Authors: Zhao Mingming , Li Guofei , Zhao Limei 
Published: November 2024

AimThis study aimed to gain deeper insights into the hepatotoxicity mechanisms of valproic acid (VPA), as well as to identify potential risk markers for VPA-induced hepatotoxicity.MethodsTwenty-two children with epilepsy treated with VPA monotherapy were divided into a normal liver function (NLF) group, a mild abnormal liver function (ANLF1) group, and a serious abnormal liver function (ANLF2) group based on their liver function indicator levels. The full quantitative targeted metabolomics technique was used to systematically investigate how the differential endogenous metabolic components change with the development of liver injury.ResultsA total of 195 metabolic components were quantitatively analyzed. Nineteen identified metabolites, including five organic acids, four short-chain fatty acids, four amino acids, three fatty acids, and three benzenoids, differed significantly among the three groups, showing a strong association with VPA-induced hepatotoxicity. Only three bile acid metabolites, taurodeoxycholic acid, taurochenodeoxycholic acid, and deoxycholic acid, were significantly different between the ANLF1 and ANLF2 groups, increasing at first and then decreasing with the aggravation of liver injury. The mechanistic evaluation showed that SRT1720 activation could alleviate the severity of liver function abnormalities induced by VPA. Immunocoprecipitation indicated that VPA significantly increased the acetylation level of FXR, and the application of agonist SRT1720 can antagonize the acetylation of FXR by VPA.ConclusionNineteen identified metabolites showed a strong association with hepatotoxicity and three bile acid metabolites changed with the development of liver injury. The SIRT1–FXR pathway was firstly proposed to participate in VPA-induced hepatotoxicity.