bluuebunny/crossref_metadata_2025_split huggingface dataset:
Pediatric rheumatologists’ perspectives on diagnosis, treatment, and outcomes of Sjögren disease in children and adolescents
Authors: Randell Rachel L. , Stern Sara M. , Van Mater Heather , Schanberg Laura E. , Lieberman Scott M. , Basiaga Matthew L. ,   CARRA Sjögren Workgroup,   CARRA Investigators
Published: September 2022

Abstract
Background
Sjögren disease in children and adolescents (pedSD) presents differently than adult disease. Diagnosis and classification are controversial, optimal treatment is unknown and outcomes are poorly understood. Here, we describe the current perspectives of pediatric rheumatologists on diagnosis, treatment, and outcomes of pedSD.

Methods
A voluntary, 17-question survey was distributed to providers in the Childhood Arthritis and Rheumatology Research Alliance and/or the American College of Rheumatology Childhood Sjögren’s Study Group at the 2020 Convergence Virtual Conference. Findings are reported using descriptive statistics and chi-square testing.

Results
Of 465 eligible providers, 157 (34%) responded with 135 (29%) completing the survey. The majority (85%) saw five or fewer patients with pedSD in the past year. Parotitis, dry eye and/or dry mouth, and constitutional symptoms were among the most specific and common clinical features. Most providers (77%) used clinical judgment guided by adult criteria for diagnosis. The vast majority (86–99%) of survey participants indicated routine use of serologic testing, while salivary gland ultrasound, minor salivary gland biopsy and other diagnostic tests were less often used. The most commonly prescribed systemic immunomodulators were hydroxychloroquine, corticosteroids, methotrexate, rituximab, and mycophenolate. Seven providers reported malignancy in a patient with pedSD, including one death.

Conclusions
Pediatric rheumatologists diagnose and treat pedSD; however, most only see a few patients per year and rely on clinical judgment and laboratory testing for diagnosis. Treatment frequently includes systemic immunomodulators and malignancies are reported. More studies are needed to better understand natural history, risk factors, and the impact of interventions on outcomes.
The impact of the COVID-19 pandemic on stress and other psychological factors in pregnant women giving birth during the first wave of the pandemic
Authors: Hübner Theresa , Wolfgang Tanja , Theis Ann-Catrin , Steber Magdalena , Wiedenmann Lea , Wöckel Achim , Diessner Joachim , Hein Grit , Gründahl Marthe , Kämmerer Ulrike , Kittel-Schneider Sarah , Bartmann Catharina 
Published: September 2022

Abstract
Background
The onset of mental illness such as depression and anxiety disorders in pregnancy and postpartum period is common. The coronavirus induced disease 2019 (COVID-19) pandemic and the resulting public policy responses represent an exceptional situation worldwide and there are hints for adverse psychosocial impact, hence, the study of psychological effects of the pandemic in women during hospitalization for delivery and in the postpartum period is highly relevant.

Methods
Patients who gave birth during the first wave of the COVID-19 pandemic in Germany (March to June 2020) at the Department of Obstetrics and Gynecology, University of Würzburg, Germany, were recruited at hospital admission for delivery. Biosamples were collected for analysis of SARS-CoV-2 infection and various stress hormones and interleukin-6 (IL-6). In addition to sociodemographic and medical obstetric data, survey questionnaires in relation to concerns about and fear of COVID-19, depression, stress, anxiety, loneliness, maternal self-efficacy and the mother–child bonding were administered at T1 (delivery stay) and T2 (3–6 months postpartum).

Results
In total, all 94 recruited patients had a moderate concern of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) at T1 with a significant rise at T2. This concern correlated with low to low-medium general psychosocial stress levels and stress symptoms, and the women showed a significant increase of active coping from T1 to T2. Anxiety levels were low and the Edinburgh Postnatal Depression Scale showed a medium score of 5 with a significant (T1), but only week correlation with the concerns about SARS-CoV-2. In contrast to the overall good maternal bonding without correlation to SARS-CoV-2 concern, the maternal self-efficiency correlated negatively with the obstetric impairment caused by the COVID-19 pandemic.

Conclusion
Obstetric patients` concerns regarding SARS-CoV-2 and the accompanying pandemic increased during the course of the pandemic correlating positively with stress and depression. Of note is the increase in active coping over time and the overall good mother–child-bonding. Maternal self-efficacy was affected in part by the restrictions of the pandemic.
Clinical trial registration DRKS00022506
Patient-specific instrumentation for total knee arthroplasty improves reproducibility in the planned rotational positioning of the tibial component
Authors: Sotozawa Masaichi , Kumagai Ken , Yamada Shunsuke , Nejima Shuntaro , Inaba Yutaka 
Published: September 2022

Abstract
Background
The purpose of this study was to evaluate the reproducibility of planned component positioning including tibial rotational alignment in patient-specific instrumentation (PSI) for total knee arthroplasty (TKA).

Methods
A total of 100 knees of 100 patients underwent TKA using PSI (n = 50) or the conventional method (n = 50). Full-length anteroposterior radiographs of the lower limb were taken in the standing position, and the coronal alignments of the femoral and tibial components were measured. Computed tomography (CT) images of the lower limb were obtained preoperatively and postoperatively, and the rotational alignments of the femoral and tibial components were measured. The difference from the preoperative planning in tibial rotational alignment was measured using three-dimensionally merged pre- and postoperative images. The mean values and rates of outliers in each measurement were compared between the PSI group and the conventional group.

Results
There were no significant differences in coronal alignment of the femoral and tibial components and rotational alignment of the femoral component between the two groups. With respect to rotational alignment of the tibial component from the preoperatively planned reference axis, the PSI group showed a lower rate of outliers (internal rotation > 10°) than the conventional group (p < 0.05).

Conclusions
This study demonstrated that the difference from the preoperative planning in tibial rotational positioning was accurately evaluated using novel three-dimensional measurement method, and PSI could reduce outliers in rotational alignment of the tibial component (internal rotation > 10°). PSI is a useful technique for improving the reproducibility of the planned tibial rotational positioning in TKA.
Method for generating transparent porcine tibia showing the intraosseous artery
Authors: Wang Hongyu , Wan Jiaming , Geng Kailong , Zhang Xiangnan , Hou Ruixing 
Published: September 2022

Abstract
Background
The occurrence of nonunion after tibial fracture surgery is mainly related to insufficient blood supply. Therefore, anatomical study of the internal and external tibial artery is very important, but there is no good method for displaying the intraosseous artery clearly and intuitively. This hinders the protection and reconstruction of it by surgeons, as well as the development of new instruments and techniques by researchers.

Objective
To develop a transparent specimen that could clearly display the intraosseous artery of the tibia.

Methods
In 10 isolated pig calves with popliteal vessels, the popliteal artery was exposed and a tube was placed. A casting agent was then injected at constant pressure, and the tissue around the blood vessel was preliminarily removed after solidification. The perivascular tissue and periosteum were further removed via alkali corrosion, and the tibia was fixed with an external fixator to protect the non-corrosive areas at both ends. Alternate acid corrosion and flushing were then applied until the intraosseous artery was completely exposed. The distribution and branches of intraosseous nutrient arteries were observed with the naked eye and via microscopy. Three-dimensional (3D) scanning and 3D printing filling techniques were used to make transparent tibia specimens with preservation of intraosseous arteries.

Results
A cast specimen of the intraosseous artery of porcine tibia was successfully generated via epoxy resin perfusion combined with acid–alkali etching, and the intraosseous artery was clearly visible. The 3D printing and filling technique successfully produced a transparent tibia specimen with preservation of internal bone arteries, and accurately restored the external shape of the tibia. The foramen of the nutrient artery appeared near the middle upper third of the lateral edge of the tibia. After entering the tibia, the nutrient artery proceeded forward, medial, and downward for a certain distance, twisted and turned near the midpoint of the medullary cavity, and divided into the ascending and descending branches. After going in the opposite direction for a distance, the ascending trunk sent out 2–3 branches, and the descending trunk sent out 2–3 branches.

Conclusion
The cast specimen of pig intraosseous artery generated via the above-described perfusion corrosion method provides methodological guidance for the study of anatomical characteristics of the intraosseous artery, and a theoretical basis for the study of new methods of internal fixation and reconstruction of the blood supply of the lower tibia.
Phenotypic expression of swallowing function in Niemann–Pick disease type C1
Authors: Solomon Beth I. , Muñoz Andrea M. , Sinaii Ninet , Farhat Nicole M. , Smith Andrew C. , Bianconi Simona , Dang Do An , Backman Michael C. , Machielse Leonza , Porter Forbes D. 
Published: September 2022

Abstract
Background
Niemann–Pick disease type C1 (NPC1) is a rare autosomal recessive disease characterized by endolysosomal accumulation of unesterified cholesterol with progressive deterioration in swallowing, often leading to premature death. Although documented, the natural history of NPC1 swallowing dysfunction has yet to be delineated systematically. This manuscript aims to provide a comprehensive characterization of the phenotypic spectrum and progression of swallowing dysfunction in NPC1.

Methodology
The National Institutes of Health (NIH) NPC1 natural history study (NCT00344331) enrolled 120 patients, who underwent comprehensive interpretative swallow assessments for swallowing safety, dietary modifications, and aspiration risk. Longitudinal statistical modeling accounted for all outcomes with NPC1 disease covariates (first symptom onset, age at neurological symptom onset, seizure history, duration of neurological symptoms) as well as miglustat use (a glucosylceramide synthase inhibitor) and NIH study duration (NIHSD; the length of time an individual participated in the NIH study). Probabilities for disease progression and time to swallowing decline were conducted for the entire cohort.

Results
Time to swallowing decline with American Speech-Language-Hearing Association National Outcome Measure (ASHA-NOMS) and the NIH-adapted Penetration Aspiration Scale (NIH-PAS) were identified: $$\frac{7.7}{100}$$

7.7

100

person-years and $$\frac{9.8}{100}$$

9.8

100

person-years, respectively. NIHSD and seizure history consistently and significantly were associated with decline (ORNIHSD = 1.34–2.10, 95% CI 1.04–3.4, p = 0.001–0.026; ORSeizure = 3.26–18.22, 1.03–167.79; p = 0.001–0.046), while miglustat use revealed protection (ORMiglustat = 0.01–0.43, 0.007–0.98; p = 0.001–0.044). The probability of decline with NPC1 neurological severity scale and annual severity increment scale were established with the aforementioned covariates, varying amongst subgroups.

Conclusion
This study represents the most extensive collection of prospective, instrumental swallowing assessments in NPC1 to date with an interpretive analysis providing an improved understanding of NPC1 disease progression with swallowing function—serving as a foundation for clinical management and future NPC1 therapeutics.
Silencing of hypothalamic FGF11 prevents diet-induced obesity
Authors: Cho Jae Hyun , Kim Kyungchan , Cho Han Chae , Lee Jaemeun , Kim Eun-Kyoung 
Published: September 2022

AbstractFibroblast growth factor 11 (FGF11) is a member of the intracellular fibroblast growth factor family. Here, we report the central role of FGF11 in the regulation of metabolism. Lentiviral injection of Fgf11 shRNA into the arcuate nucleus of the mouse hypothalamus decreased weight gain and fat mass, increased brown adipose tissue thermogenesis, and improved glucose and insulin intolerances under high-fat diet conditions. Fgf11 was expressed in the NPY–expressing neurons, and Fgf11 knockdown considerably decreased Npy expression and projection, leading to increased expression of tyrosine hydroxylase in the paraventricular nucleus. Mechanistically, FGF11 regulated Npy gene expression through the glycogen synthase kinase 3–cAMP response element-binding protein pathway. Our study defines the physiological significance of hypothalamic FGF11 in the regulation of metabolism in response to overnutrition such as high-fat diet.
Physical growth and intelligence development of discordant dizygotic twins from birth to preschool age: a prospective cohort study
Authors: Xiang Huiqiu , Huang Xianping , Zhu Jing , Chen Jiajia , Zhou Pangpang , Zhou Tong , Bao Jiale , Xu Zhangye 
Published: September 2022

Abstract
Background
The majority of studies are limited to adverse perinatal outcomes and poor cognitive abilities in the short term in discordant monochorionic twins.

Methods
To determine whether small and large discordant dizygotic twins differ in physical growth and intelligence development and weight and height from birth up to 6 years of age were measured in 34 dizygotic twin pairs with ≥ 20% birth weight discordance. Mental developmental index (MDI) and psychomotor developmental index (PDI) were calculated at 1 year, while the Wechsler Intelligence Scale for Children-IV (WISC-IV) full-scale intelligence quotient (IQ) was assessed at the age of 6.

Results
The difference in height and weight in each stage differed significantly from birth to 72-months-old (P < 0.05), although there was disappointing catch-up growth in smaller twins. PDI but not MDI at 1 year of age was significantly different between the two groups (P < 0.05), and smaller twins experienced higher psychomotor retardation rates (P < 0.05). Also, the influence of height and weight on PDI was statistically significant (P < 0.05). No significant difference was detected in the WISC-IV full-scale IQ at the age of 6; however, the full-scale IQ may be affected by the history of suffocation and the S/D value (P = 0.011, P = 0.022).

Conclusions
Intrauterine fetal growth and development lead to birth weight differences in twins and sustain an impact on the children’s physical growth in height and weight from birth to preschool age, causing psychomotor developmental differences at 1 year of age. However, the differences in psychomotor development decrease gradually by the age of 6.
Lingual laser frenotomy in newborns with ankyloglossia: a prospective cohort study
Authors: Dell’Olio Fabio , Baldassarre Maria Elisabetta , Russo Fabio Giovanni , Schettini Federico , Siciliani Rosaria Arianna , Mezzapesa Pietro Paolo , Tempesta Angela , Laforgia Nicola , Favia Gianfranco , Limongelli Luisa 
Published: September 2022

Abstract
Background
The study aims to describe the lingual laser frenotomy perioperative protocol for newborns with ankyloglossia with or without breastfeeding difficulties developed by Odontostomatology and Neonatology and Neonatal Intensive Care Units of the Aldo Moro University of Bari.

Methods
Authors carried out a prospective observational cohort study. Newborns with ankyloglossia (classified by using both Coryllos’ and Hazelbaker’s criteria) with or without difficult breastfeeding (according to Infant Breastfeeding Assessment Tool) underwent diode laser frenotomy (800 ± 10 nm; 5 W; continuous wave mode; contact technique; under topical anesthesia) and follow-up visits after seven and thirty days postoperatively. The authors analyzed as main outcomes the perioperative pain intensity measured by the C.R.I.E.S. scale, the occurrence of complications and quality of healing, the quality of breastfeeding, newborn’s postoperative weight gain, maternal nipple pain, and the presence of lesions as secondary outcomes.

Results
Fifty-six newborns were included in the current study. Intraoperative mean pain intensity was 5.7 ± 0.5 points, resolved within thirty postoperative minutes. Observed complications were mild punctuating bleeding, carbonization of the irradiated site, and transitory restlessness. All wounds were completely healed within the thirtieth postoperative day. During follow-up, a significant breastfeeding improvement was evident with satisfactory newborns’ weight gain and a significant reduction of nipple pain and lesions (p < .05).

Conclusion
Our lingual laser frenotomy protocol provided significant breastfeeding improvement in the mother-newborn dyads with low intraoperative pain and no significant complications.
Right dose, right now: bedside, real-time, data-driven, and personalised antibiotic dosing in critically ill patients with sepsis or septic shock—a two-centre randomised clinical trial
Authors: Roggeveen Luca F. , Guo Tingjie , Fleuren Lucas M. , Driessen Ronald , Thoral Patrick , van Hest Reinier M. , Mathot Ron A. A. , Swart Eleonora L. , de Grooth Harm-Jan , van den Bogaard Bas , Girbes Armand R. J. , Bosman Rob J. , Elbers Paul W. G. 
Published: September 2022

Abstract
Background
Adequate antibiotic dosing may improve outcomes in critically ill patients but is challenging due to altered and variable pharmacokinetics. To address this challenge, AutoKinetics was developed, a decision support system for bedside, real-time, data-driven and personalised antibiotic dosing. This study evaluates the feasibility, safety and efficacy of its clinical implementation.

Methods
In this two-centre randomised clinical trial, critically ill patients with sepsis or septic shock were randomised to AutoKinetics dosing or standard dosing for four antibiotics: vancomycin, ciprofloxacin, meropenem, and ceftriaxone. Adult patients with a confirmed or suspected infection and either lactate > 2 mmol/L or vasopressor requirement were eligible for inclusion. The primary outcome was pharmacokinetic target attainment in the first 24 h after randomisation. Clinical endpoints included mortality, ICU length of stay and incidence of acute kidney injury.

Results
After inclusion of 252 patients, the study was stopped early due to the COVID-19 pandemic. In the ciprofloxacin intervention group, the primary outcome was obtained in 69% compared to 3% in the control group (OR 62.5, CI 11.4–1173.78, p < 0.001). Furthermore, target attainment was faster (26 h, CI 18–42 h, p < 0.001) and better (65% increase, CI 49–84%, p < 0.001). For the other antibiotics, AutoKinetics dosing did not improve target attainment. Clinical endpoints were not significantly different. Importantly, higher dosing did not lead to increased mortality or renal failure.

Conclusions
In critically ill patients, personalised dosing was feasible, safe and significantly improved target attainment for ciprofloxacin.
Trial registration: The trial was prospectively registered at Netherlands Trial Register (NTR), NL6501/NTR6689 on 25 August 2017 and at the European Clinical Trials Database (EudraCT), 2017-002478-37 on 6 November 2017.
Efficacy and safety of unrestricted visiting policy for critically ill patients: a meta-analysis
Authors: Wu Yuchen , Wang Guoqiang , Zhang Zhigang , Fan Luo , Ma Fangli , Yue Weigang , Li Bin , Tian Jinhui 
Published: September 2022

Abstract
Aim
To compare the safety and effects of unrestricted visiting policies (UVPs) and restricted visiting policies (RVPs) in intensive care units (ICUs) with respect to outcomes related to delirium, infection, and mortality.

Methods
MEDLINE, Cochrane Library, Embase, Web of Science, CINAHL, CBMdisc, CNKI, Wanfang, and VIP database records generated from their inception to 22 January 2022 were searched. Randomized controlled trials and quasi-experimental studies were included. The main outcomes investigated were delirium, ICU-acquired infection, ICU mortality, and length of ICU stay. Two reviewers independently screened studies, extracted data, and assessed risks of bias. Random‑effects and fixed-effects meta‑analyses were conducted to obtain pooled estimates, due to heterogeneity. Meta-analyses were performed using RevMan 5.3 software. The results were analyzed using odds ratios (ORs), 95% confidence intervals (CIs), and standardized mean differences (SMDs).

Results
Eleven studies including a total of 3741 patients that compared UVPs and RVPs in ICUs were included in the analyses. Random effects modeling indicated that UVPs were associated with a reduced incidence of delirium (OR = 0.4, 95% CI 0.25–0.63, I2 = 71%, p = 0.0005). Fixed-effects modeling indicated that UVPs did not increase the incidences of ICU-acquired infections, including ventilator-associated pneumonia (OR = 0.96, 95% CI 0.71–1.30, I2 = 0%, p = 0.49), catheter-associated urinary tract infection (OR 0.97, 95% CI 0.52–1.80, I2 = 0%, p = 0.55), and catheter-related blood stream infection (OR = 1.15, 95% CI 0.72–1.84, I2 = 0%, p = 0.66), or ICU mortality (OR = 1.03, 95% CI 0.83–1.28, I2 = 49%, p = 0.12). Forest plotting indicated that UVPs could reduce the lengths of ICU stays (SMD =  − 0.97, 95% CI − 1.61 to 0.32, p = 0.003).

Conclusion
The current meta-analysis indicates that adopting a UVP may significantly reduce the incidence of delirium in ICU patients, without increasing the risks of ICU-acquired infection or mortality. Further large-scale, multicenter studies are needed to confirm these indications.