bluuebunny/crossref_metadata_2025_split huggingface dataset:
Face validity and reliability test of the Danish version of the compliance questionnaire rheumatology in patients with early rheumatoid arthritis
Authors: Knudsen Line Raunsbæk , de Thurah Annette 
Published: October 2023

Abstract
Background
Supporting adherence to medication is an essential part of the treatment and care of patients with rheumatic and musculoskeletal diseases. The Compliance Questionnaire Rheumatology (CQR) measures adherence in rheumatic diseases through 19 items covering drug-taking behaviour to identify the reasons for adhering to treatment and the factors that contribute to suboptimal adherence. The objective of this study was to present the translation of the CQR into Danish and the face validity and reliability test.

Methods
The CQR was translated into Danish according to international guidelines, followed by a face validity test among 10 patients with rheumatoid arthritis in 2009. The test–retest reliability of the Danish CQR was evaluated in 49 patients with rheumatoid arthritis in 2020 - 2021 using the standard error of the measurement (SEM) converted into the minimally detectable change (MDC) and the intraclass correlation coefficient (ICC). Questionnaires were administered with a minimum of 10 days between assessments.

Results
The participants in the reliability test had a mean age of 57.4 years (SD 16.1) and a mean disease duration of 1.13 years (range 2 months–2 years). The mean CQR score in the test and retest was 62.7 (confidence interval (CI) 58.8; 66.6) and 62.5 (CI 58.9; 66.1), respectively, with a SEM of 8.59 (7.16; 10.73) and an MDC of 16.83. A satisfactory test–retest reliability was confirmed by an ICC value of 0.79 (CI 0.68; 0.89).

Conclusion
The Danish CQR has satisfactory test–retest reliability in patients newly diagnosed with rheumatoid arthritis and is considered a reliable tool to measure adherence in this group.
Computer-guided cranioplasty using ultra-high molecular weight polyethylene patient-specific implants: a case series
Authors: Abdelwahed Mariam S. K. , Ahmed Mamdouh S. , Abdelwahed Ahmed S. Kamel , Aziz Mohamed M. 
Published: October 2023

Abstract
Introduction
Cranioplasty is the surgical correction of a cranial defect. Three-dimensional software designs have allowed patient-specific single-step cranial reconstruction for neuroprotection and cosmesis. Ultra-high molecular weight polyethylene for partial or total bone replacement has been recently introduced as a promising material for cranioplasty. The objectives of this study are to evaluate the complications and esthetic results concerning the use of ultra-high molecular weight polyethylene patient-specific implants in craniectomy patients.

Methods
We report a series of nine patients with cranial defects from a previous craniectomy, or patients eligible for simultaneous craniectomy and cranioplasty via computer designed ultra-high molecular weight polyethylene patient-specific implants. We have analyzed the complications and cosmetic outcomes over a course of six months.

Results
None of the cases developed infection, extra/subdural hematoma, cerebrospinal fluid leak, or implant failure. Three cases had postoperative sequalae: The first patient had mild postoperative seroma which subsided after medical therapy and compression, the second showed wound breakdown due to tumor metastasis and recurrence but did not necessitate implant removal, while the third sequela was a subgaleal hematoma which was aspirated, and the patient healed uneventfully afterward. Esthetic results were highly satisfactory in 75% of the patients (good patient acceptance without touch-ups).

Conclusion
Ultra-high molecular weight polyethylene is in all respects suitable for primary and secondary cranioplasty, combined with computer-aided manufacturing–computer-aided design techniques, excellent esthetic and functional results were achieved. However, proper preoperative planning is important, and we recommend further prospective studies with larger number of patients followed up for longer periods for better assessment.
Efficacy and safety of transdermal electrical stimulation in patients with nonarteritic anterior ischemic optic neuropathy
Authors: Miura Gen , Fujiwara Tadami , Ozawa Yoshihito , Shiko Yuki , Kawasaki Yohei , Nizawa Tomohiro , Tatsumi Tomoaki , Kurimoto Takuji , Mori Sotaro , Nakamura Makoto , Hanaoka Hideki , Baba Takayuki , Yamamoto Shuichi 
Published: October 2023

Abstract
Background
No effective treatment for NAION with strong evidence has been established till date. The aim of this investigator-led, prospective, non-randomized, open-label, uncontrolled multi-center exploratory clinical trial is to evaluate the efficacy and safety of transdermal electrical stimulation (TdES) using skin electrodes in patients with NAION.

Methods
Five patients with monocular NAION underwent TdES (10-ms biphasic pulses, 1.0 mA, 20 Hz, 30 min) of the affected eye six times at 2-week intervals. The primary endpoint was the logarithm of the mini-mum angle of resolution (logMAR) visual acuity at 12 weeks compared with 0 weeks. The secondary endpoints were changes in the best-corrected logMAR visual acuity, Early Treatment of Diabetic Retinopathy Study (ETDRS) visual acuity, and mean deviation (MD) of the Humphrey field analyzer (HFA) 10–2 and HFA Esterman test scores. Additionally, the safety of TdES was evaluated.

Results
LogMAR visual acuity improved by ≥ 0.1 in two eyes, and ETDRS visual acu-ity improved by ≥ 5 characters in one eye. The mean change in logMAR visual acuity from week 0 showed an increasing trend. The mean MD of HFA 10–2 showed no obvious change, while HFA Esterman score improved in four eyes. All patients completed the study according to the protocol, and no treatment-related adverse events were observed.

Conclusions
TdES treatment may have improved visual acuity and visual field in some patients. Further sham-controlled study in larger cohort is needed on its effectiveness.

Trial registration
UMIN, UMIN000036220. Registered 15 March, 2019, https://center6.umin.ac.jp/cgi-open-bin/ctr/ctr_view.cgi?recptno=R000041261.
Microsatellite instability screening in colorectal carcinoma: immunohistochemical analysis of MMR proteins in correlation with clinicopathological features and Ki-67 protein expression
Authors: Yassen Noha N. , Abouelfadl Dalia M. , Abbas Naglaa F. , Soliman Ahmed S. A. , Shabana Marwa E. 
Published: October 2023

Abstract
Background
Defects in mismatch repair (MMR) system or microsatellite instability (MSI) and detected in colorectal carcinoma (CRC), both in sporadic and more frequently in hereditary cases. Immunohistochemistry (IHC) is the most frequent method for MMR protein deficiency screening in CRCs. In this study, we aimed to evaluate immunohistochemical expression of MMR and Ki-67 in colorectal carcinoma with clinicopathological features.

Methods
In this study, we evaluated the immunohistochemical expression of MMR proteins including MSH6, MSH2, PMS2 and MLH1 in 50 resection materials with colorectal carcinoma. Their expression is correlated with clinicopathological features of patients together, with Ki-67 protein expression in attempt to screen the most significant predictor of microsatellite instability.

Results
Of the 50 cases of cancer colon, 28% were classified as MSI-H, 20% were MSI-L, and 52% were MSS. The most frequent pattern in MSI-H tumors was concurrent loss of MSH6 and PMS2 proteins. There was a significant correlation between MMR protein expression pattern with tumor size, grade, T-classification and stage (0.015, 0.0515, 0.0162 and 0.0391), respectively. MSI-H tumors were located more frequently in right colon, early TNM stage and poorly differentiated and infrequent distant metastases. There was a significant correlation between Ki-67 high expression and MSI status patterns in their common biological aspects distinct from MSI-negative tumors.

Conclusions
Mismatch repair defective colorectal carcinoma has characteristics clinicopathological features different from MSS tumors. The role of immunohistochemistry (IHC) for MSI evaluation is the easiest and effective way for evaluation of MMR deficiency in colorectal carcinoma.
Progression of pancreatic branch duct-type intraductal papillary mucinous neoplasms (BD-IPMNs) after surgery for extrapancreatic malignancies
Authors: Imoto Akira , Ogura Takeshi , Masuda Daisuke , Narabayashi Ken , Okada Toshihiko , Abe Yosuke , Takeuchi Toshihisa , Inoue Takuya , Ishida Kumi , Nouda Sadaharu , Higuchi Kazuhide , Abdelaal Usama M. 
Published: October 2023

Abstract
Background/aims
The natural history of pancreatic branch duct-type intraductal papillary mucinous neoplasms (BD-IPMNs) is still unknown. This retrospective study aims to determine the morphological changes of BD-IPMNs with attention to the history of surgical resection for extrapancreatic malignancies.

Methods and materials
This study included 427 subjects with BD-IPMN at the Osaka Medical College from January 2001 to December 2019; 134 patients were included. The predictive factors for the progression of BD-IPMN based on morphological changes were evaluated by univariate as well as multivariate analyses. Furthermore, the clinical features of BD-IPMNs with progressive lesions during follow-up were investigated.

Results
The average interval of follow-up was 35.8 months (with a range between 12.1 and 157 months). Disease progression occurred in 6 subjects (4.5%). In two of them (1.5%), IPMN-related invasive carcinoma was found. Multivariate analysis demonstrated that surgical resection for extrapancreatic malignancies was a significant predictor of BD-IPMN progression.

Conclusion
The history of resection of extrapancreatic malignancies should be considered during the follow-up of BD-IPMN.
Connectedness and spillover between African equity, commodity, foreign exchange and cryptocurrency markets during the COVID-19 and Russia-Ukraine conflict
Authors: Anyikwa Izunna , Phiri Andrew 
Published: October 2023

AbstractSince the onset of the COVID-19 pandemic, financial and commodity markets have exhibited significant volatility and displayed fat tail properties, deviating from the normal probability curve. The recent Russia-Ukraine war has further disrupted these markets, attracting considerable attention from both researchers and practitioners due to the occurrence of consecutive black swan events within a short timeframe. In this study, we utilized the Quantile-VAR technique to examine the interconnectedness and spillover effects between African equity markets and international financial/commodity assets. Daily data spanning from January 3, 2020, to September 6, 2022, was analyzed to capture tail risks. Our main findings can be summarized as follows. Firstly, the level of connectedness in returns is more pronounced in the lower and upper tails compared to the median. Secondly, during times of crisis, African equity markets primarily serve as recipients of systemic shocks. Lastly, assets such as Silver, Gold, and Natural Gas exhibit greater resilience to systemic shocks, validating their suitability as hedging instruments for African equities, in contrast to cryptocurrencies and international exchange rates. These findings carry significant implications for policymakers and investors in Africa equities.
Effect of physicochemical parameters on the L-methioninase activity of Methylobacterium sp. and its in vitro anticancer activity in combination with tamoxifen citrate
Authors: Dayanand Kavya , Nadumane Varalakshmi Kilingar 
Published: October 2023

Abstract
Background
Methionine dependence is a metabolic abnormality observed exclusively in cancer cells. Methionine depletion using methioninase is therefore an attractive strategy for cancer treatment. The current study focuses on the purification of L-Methioninase from a bacterial isolate, Methylobacterium sp. JUBTK33, for its anticancer application in conjunction with Tamoxifen in MCF-7, HepG2, and HeLa cancer cell lines.

Results
L-methioninase was purified from Methylobacterium sp. JUBTK33 using a DEAE-Sephadex G-200 column, resulting in a 6.15-fold purification with a specific activity of 17.89 U/mg. At 40 °C and pH 8.5, the enzymatic biochemical characteristics demonstrated increased enzyme activity. Na+ ions (1 mM) significantly enhanced the enzyme’s activity, while Li+, Mn++, Ni++, Fe++, and K+ had little impact. The highest activity was observed at a 225 µM (2.5%) substrate concentration of methionine, with Vmax and Km values of 0.48 U/mL/min and 48.23 µM, respectively. The enzyme’s potential anticancer effect in combination with TAM was evaluated on HepG2, MCF-7, and HeLa cell lines. It was found to be highly effective on MCF-7 cell lines, with a combination of L-MET-TAM (5 and 10 µg/mL) resulting in 3.72% and 1.0% cell viabilities, and IC50 values of 9.701 µg/mL and 5.72 µg/mL, respectively. On the normal HEK-293 cell line, the combination of L-MET-TAM (10 µg/mL) demonstrated approximately an 18% protective effect compared to TAM alone.

Conclusion
The combination approach demonstrated remarkable success against cancer cells in vitro, highlighting the need for further investigations to develop it into an effective treatment strategy.
Management practices and technologies for efficient biological sample collection from domestic animals with special reference to Indian field conditions
Authors: Ragavan Gokula Kannan , Selvaraj Purushothaman , Murugesan Dharman , Krishnaswamy Gopalan Tirumurugaan , Chinnaiyan Soundararajan , Gopal Dhinakar Raj , Ramasamy Parthiban Aravindh Babu , Kumaragurubaran Karthik , Palavesam Azhahianambi 
Published: October 2023

AbstractManagement strategies and the use of advanced technologies are equally important for determining the sample number and sampling frame for successful field sampling for animal disease prevalence studies. The quality of the biological samples collected in the field has a direct bearing on the integrity of the data generated, prevalence estimates and subsequent policy decisions on disease control. Hence, compromising the quality of biological samples collected in the field could potentially undermine the priority setting principles in disease control strategies. Biological samples collected from domestic animals in the field are precious materials and require meticulous planning for sample collection, sample storage in the field, transportation, and storage in the laboratory. Poorly managed field sampling has a significant detrimental impact on the sample quality and quantity and directly affects the accuracy of disease prevalence data. A bad choice of sampling tools, containers, storage and transport all have a negative impact on the integrity of the sample and consequently have an impact on the outcome. Over the last two years, as part of our one health animal sampling work in India, we have observed challenges and opportunities in the field sampling of animals for disease prevalence studies. This paper aims to provide information on management practices and technologies for efficient biological sample collection from the field and ensure that good quality samples are available for testing.
Complications of unintentional dural puncture during labour epidural analgesia: a 10-year retrospective observational study
Authors: Poma S. , Bonomo M. C. , Gazzaniga G. , Pizzulli M. , De Silvestri A. , Baldi C. , Broglia F. , Ciceri M. , Fuardo M. , Morgante F. , Pellicori S. , Roldi E. M. , Delmonte M. P. , Mojoli F. , Locatelli A. 
Published: October 2023

Abstract
Introduction
Unintentional dural puncture (UDP) occurs in 0.5–1.5% of labour epidural analgesia cases. To date, little is known about evidence of UDP-related complications. This work aimed to assess the incidence of intrapartum and postpartum complications in parturients who experienced UDP.

Methods
This is a 10-year retrospective observational study on parturients admitted to our centre who presented UDP. Data collection gathered UDP-related complications during labour and postpartum. All women who displayed UDP received medical therapy and bed rest. An epidural blood patch (EBP) was not used in this population. Once asymptomatic, patients were discharged from the hospital.

Results
Out of 7718 neuraxial analgesia cases, 97 cases of UDP occurred (1.25%). During labour, complications appeared in a small percentage of analgesia procedures performed, including total spinal anaesthesia (1.0%), extended motor block (3%), hypotension (4.1%), abnormal foetal heart rate (2%), inadequate analgesia (14.4%), and general anaesthesia following neuraxial anaesthesia failure (33.3% of emergency caesarean sections). During the postpartum period, 53.6% of parturients exhibited a postdural puncture headache, 13.4% showed neurological symptoms, and 14.4% required neurological consultation and neuroimaging. No patient developed subdural hematoma or cerebral venous sinus thrombosis; one woman presented posterior reversible encephalopathy syndrome associated with eclampsia. Overall, 82.5% of women experienced an extension of hospital stay.

Conclusion
Major complications occurred in a small percentage of patients during labour. However, since they represent high-risk maternal and neonatal health events, a dedicated anaesthesiologist and a trained obstetric team are essential.
No major neurological complications were registered postpartum, and EBP was not performed. Nevertheless, all patients with UDP were carefully monitored and treated until complete recovery before discharge, leading to an extension of their hospitalization.
Single-Exon Deletions of ZNRF3 Exon 2 Cause Congenital Adrenal Hypoplasia
Authors: Amano Naoko , Narumi Satoshi , Aizu Katsuya , Miyazawa Mari , Okamura Kohji , Ohashi Hirofumi , Katsumata Noriyuki , Ishii Tomohiro , Hasegawa Tomonobu 
Published: October 2023

Abstract

Context
Primary adrenal insufficiency (PAI) is a life-threatening condition characterized by the inability of the adrenal cortex to produce sufficient steroid hormones. E3 ubiquitin protein ligase zinc and ring finger 3 (ZNRF3) is a negative regulator of Wnt/β-catenin signaling. R-spondin 1 (RSPO1) enhances Wnt/β-catenin signaling via binding and removal of ZNRF3 from the cell surface.

Objective
This work aimed to explore a novel genetic form of PAI.

Methods
We analyzed 9 patients with childhood-onset PAI of biochemically and genetically unknown etiology using array comparative genomic hybridization. To examine the functionality of the identified single-exon deletions of ZNRF3 exon 2, we performed three-dimensional (3D) structure modeling and in vitro functional studies.

Results
We identified various-sized single-exon deletions encompassing ZNRF3 exon 2 in 3 patients who showed neonatal-onset adrenal hypoplasia with glucocorticoid and mineralocorticoid deficiencies. Reverse-transcriptase polymerase chain reaction (RT-PCR) analysis showed that the 3 distinct single-exon deletions were commonly transcribed into a 126-nucleotide deleted mRNA and translated into 42-amino acid deleted protein (ΔEx2-ZNRF3). Based on 3D structure modeling, we predicted that interaction between ZNRF3 and RSPO1 would be disturbed in ΔEx2-ZNRF3, suggesting loss of RSPO1-dependent activation of Wnt/β-catenin signaling. Cell-based functional assays with the TCF-LEF reporter showed that RSPO1-dependent activation of Wnt/β-catenin signaling was attenuated in cells expressing ΔEx2-ZNRF3 as compared with those expressing wild-type ZNRF3.

Conclusion
We provided genetic evidence linking deletions encompassing ZNRF3 exon 2 and congenital adrenal hypoplasia, which might be related to constitutive inactivation of Wnt/β-catenin signaling by ΔEx2-ZNRF3.
Predictors of Cardiovascular Morbidities in Adults With 21-Hydroxylase Deficiency Congenital Adrenal Hyperplasia
Authors: Charoensri Suranut , Auchus Richard J 
Published: October 2023

Abstract

Context
The prevalence of cardiovascular and metabolic complications among adults with 21-hydroxylase deficiency (21OHD) is unknown.

Objective
We sought to determine the prevalence of cardiovascular and metabolic morbidities among adults with 21OHD and to identify clinical factors and biomarkers associated with cardiovascular outcomes.

Methods
A 10-year retrospective cross-sectional analysis was conducted on adult patients with confirmed 21OHD, aged 18 to 70 years, who had at least one clinical visit for assessment at the University of Michigan. The presence of cardiovascular diseases (CVDs) and other metabolic comorbidities was extracted from medical records based on International Classification of Diseases (ICD) codes. Medical treatments, glucocorticoid (GC) and mineralocorticoid doses, as well as specific biomarkers of disease control since age 18, were collected for analysis.

Results
A total of 254 patients with 21OHD, median age of 35 years (interquartile range, 28.25-46 y), were included in the analysis. The prevalence of CVDs in the entire cohort was 7.5%. An increase in prevalence was seen from early adulthood, reaching 25% in patients older than 60 years. Increasing age (adjusted odds ratio [OR], 1.05; 95% CI, 1.01-1.09), hypertension (OR, 4.27; 95% CI, 1.41-12.92), and higher GC doses (OR, 1.51; 95% CI, 1.11-2.06) were significantly associated with prevalent CVDs. Higher plasma renin activity was significantly associated with CVDs (OR, 1.07; 95% CI, 1.01-1.15) but not other biochemical markers of disease.

Conclusion
Cardiometabolic morbidities are prevalent among adults with 21OHD. Hypertension, age, and GC exposure are the main predictive factors of established CVDs in our cohort.
A Cross-Sectional Analysis of Cardiovascular and Bone Health Care Utilization During Treatment With Thyroid Hormone
Authors: Penna Gustavo C , Bianco Antonio C , Ettleson Matthew D 
Published: October 2023

Abstract

Context
Combination therapy with levothyroxine and liothyronine (LT4 + LT3) and desiccated thyroid extract (DTE) make up &gt;10% of new thyroid hormone (TH) prescriptions in the United States.

Objective
To assess health care utilization related to cardiovascular disease (CVD) and bone health (BH) events (atrial fibrillation [AF], heart failure [HF], myocardial infarction [MI], stroke, and osteoporosis/fractures [FX]) in participants taking LT4+LT3 or DTE surveyed in the Medical Expenditure Panel Survey database.

Materials and Methods
Multi-year cross-sectional analysis examining 5437 participants (≥18 years old) treated with LT4, LT4+LT3, or DTE between 2016 and 2020. Health care utilization was assessed through outpatient, emergency, and hospital visits for AF, HF, MI, stroke, FX, and a composite index. A weighted analysis provided national estimates of health care utilization parameters. Utilization was re-analyzed following propensity score–based matching to balance sociodemographic and clinical covariates between treatment groups. Additionally, provider type and specialty data were obtained from visits associated with TH prescriptions.

Results
5106 participants were treated with LT4 monotherapy, 252 with DTE, and 79 with LT4 + LT3. Prevalence of combined outpatient CVD and BH-related care utilization was lower among DTE/LT4+LT3 vs LT4 users (3.5% vs 7.7%; P = .008). There were no differences in emergency/hospital events. After covariate balancing, CVD and BH-related care utilization was similar between groups in outpatient and emergency/hospital settings. LT3 and DTE made up 7.6% of all TH prescriptions. For visits associated with DTE prescriptions, nurse practitioners and alternative medicine professionals were more likely to be identified as the primary provider type.

Conclusion
No significant differences in CVD- and BH-related health care utilization were identified between LT4 and DTE/LT4+LT3 users after covariate balancing. Non-MD providers were more likely to prescribe DTE.