bluuebunny/crossref_metadata_2025_split huggingface dataset:
Long-term results of additional pulmonary blood flow with bidirectional cavopulmonary shunt
Authors: Kowatari Ryosuke , Suzuki Yasuyuki , Daitoku Kazuyuki , Fukuda Ikuo 
Published: September 2020

Abstract
Objective
We evaluated additional pulmonary blood flow at the time of bidirectional cavopulmonary shunt and its effects on the Fontan procedure and long-term outcome of Fontan circulation and liver function.

Methods
We included 22 patients (16 boys, 6 girls) having undergone bidirectional cavopulmonary shunt with additional pulmonary blood flow between April 2002 and January 2016. Mean age and body weight were 20 ± 13 months and 7.5 ± 6.5 kg, respectively. We retrospectively evaluated the patients’ clinical data, including cardiac catheterization data, liver function, and liver fibrosis markers.

Results
All patients were alive with a New York Heart Association status of I at the long-term follow-up. Changes between pre-bidirectional cavopulmonary shunt and 101 months after the Fontan procedure included the following: the cardiothoracic ratio of chest X-ray decreased from 52.2 ± 3.9% to 41.8 ± 5.9% (p < 0.001); systemic ventricle end-diastolic pressure decreased from 11.4 ± 3.2 mmHg to 6.9 ± 3.6 mmHg (p < 0.001); and the pulmonary artery index decreased from 485.1 ± 272.3 to 269.5 ± 100.5 (p = 0.02). Type IV collagen, hyaluronic acid, and procollagen levels increased over the normal range 116 months after the Fontan procedure.

Conclusions
The additional pulmonary blood flow at the time of bidirectional cavopulmonary shunt may contribute to pulmonary arterial growth at the Fontan procedure with low pulmonary arterial resistance and without ventricle volume overload. The Fontan circulation was well-maintained at the long-term follow-up, while liver fibrosis markers were above their normal values.
Phenotypic diversity in an international Cure VCP Disease registry
Authors: Ikenaga Chiseko , Findlay Andrew R. , Seiffert Michelle , Peck Allison , Peck Nathan , Johnson Nicholas E. , Statland Jeffrey M. , Weihl Conrad C. 
Published: September 2020

Abstract
Background
Dominant mutations in valosin-containing protein (VCP) gene cause an adult onset inclusion body myopathy, Paget’s disease of bone, and frontotemporal dementia also termed multisystem proteinopathy (MSP). The genotype-phenotype relationships in VCP-related MSP are still being defined; in order to understand this better, we investigated the phenotypic diversity and patterns of weakness in the Cure VCP Disease Patient Registry.

Methods
Cure VCP Disease, Inc. was founded in 2018 for the purpose of connecting patients with VCP gene mutations and researchers to help advance treatments and cures. Cure VCP Disease Patient Registry is maintained by Coordination of Rare Diseases at Sanford. The results of two questionnaires with a 5-point Likert scale questions regarding to patients’ disease onset, symptoms, and daily life were obtained from 59 participants (28 males and 31 females) between June 2018 and May 2020. Independent of the registry, 22 patients were examined at the Cure VCP Disease annual patient conference in 2019.

Results
In the questionnaires of the registry, fifty-three patients (90%) reported that they were with inclusion body myopathy, 17 patients (29%) with Paget’s disease of bone, eight patients (14%) with dementia, two patients (3%) with amyotrophic lateral sclerosis, and a patient with parkinsonism. Thirteen patients (22%) reported dysphagia and 25 patients (42%) reported dyspnea on exertion. A self-reported functional rating scale for motor function identified challenges with sit to stand (72%), walking (67%), and climbing stairs (85%). Thirty-five (59%) patients in the registry answered that their quality of life is more than good. As for the weakness pattern of the 22 patients who were evaluated at the Cure VCP Disease annual conference, 50% of patients had facial weakness, 55% had scapular winging, 68% had upper proximal weakness, 41% had upper distal weakness, 77% had lower proximal, and 64% had lower distal weakness.

Conclusions
The Cure VCP Disease Patient Registry is useful for deepening the understanding of patient daily life, which would be a basis to develop appropriate clinical outcome measures. The registry data is consistent with previous studies evaluating VCP patients in the clinical setting. Patient advocacy groups are essential in developing and maintaining disease registries.
Predictors of response to foot orthoses and corticosteroid injection for plantar heel pain
Authors: Whittaker Glen A. , Landorf Karl B. , Munteanu Shannon E. , Menz Hylton B. 
Published: September 2020

AbstractBackgroundFoot orthoses and corticosteroid injection are common interventions used for plantar heel pain, however few studies have investigated the variables that predict response to these interventions.MethodsBaseline variables (age, weight, height, body mass index (BMI), sex, education, foot pain, foot function, fear‐avoidance beliefs and feelings, foot posture, weightbearing ankle dorsiflexion, plantar fascia thickness, and treatment preference) from a randomised trial in which participants received either foot orthoses or corticosteroid injection were used to predict change in the Foot Health Status Questionnaire foot pain and foot function subscales, and first‐step pain measured using a visual analogue scale. Multivariable linear regression models were generated for different dependent variables (i.e. foot pain, foot function and first‐step pain), for each intervention (i.e. foot orthoses and corticosteroid injection), and at different timepoints (i.e. weeks 4 and 12).ResultsFor foot orthoses at week 4, greater ankle dorsiflexion with the knee extended predicted reduction in foot pain (adjusted R2 = 0.16, p = 0.034), and lower fear‐avoidance beliefs and feelings predicted improvement in foot function (adjusted R2 = 0.43, p = 0.001). At week 12, lower BMI predicted reduction in foot pain (adjusted R2 = 0.33, p < 0.001), improvement in foot function (adjusted R2 = 0.37, p < 0.001) and reduction in first‐step pain (adjusted R2 0.19, p = 0.011). For corticosteroid injection at week 4, there were no significant predictors for change in foot pain or foot function. At week 12, less weightbearing hours predicted reduction in foot pain (adjusted R2 = 0.25, p = 0.004) and lower baseline foot pain predicted improvement in foot function (adjusted R2 = 0.38, p < 0.001).ConclusionsPeople with plantar heel pain who use foot orthoses experience reduced foot pain if they have greater ankle dorsiflexion and lower BMI, while they experience improved foot function if they have lower fear‐avoidance beliefs and lower BMI. People who receive a corticosteroid injection experience reduced foot pain if they weightbear for fewer hours, while they experience improved foot function if they have less baseline foot pain.
Epinephrine’s effects on cerebrovascular and systemic hemodynamics during cardiopulmonary resuscitation
Authors: Mavroudis Constantine D. , Ko Tiffany S. , Morgan Ryan W. , Volk Lindsay E. , Landis William P. , Smood Benjamin , Xiao Rui , Hefti Marco , Boorady Timothy W. , Marquez Alexandra , Karlsson Michael , Licht Daniel J. , Nadkarni Vinay M. , Berg Robert A. , Sutton Robert M. , Kilbaugh Todd J. 
Published: September 2020

Abstract
Background
Despite controversies, epinephrine remains a mainstay of cardiopulmonary resuscitation (CPR). Recent animal studies have suggested that epinephrine may decrease cerebral blood flow (CBF) and cerebral oxygenation, possibly potentiating neurological injury during CPR. We investigated the cerebrovascular effects of intravenous epinephrine in a swine model of pediatric in-hospital cardiac arrest. The primary objectives of this study were to determine if (1) epinephrine doses have a significant acute effect on CBF and cerebral tissue oxygenation during CPR and (2) if the effect of each subsequent dose of epinephrine differs significantly from that of the first.

Methods
One-month-old piglets (n = 20) underwent asphyxia for 7 min, ventricular fibrillation, and CPR for 10–20 min. Epinephrine (20 mcg/kg) was administered at 2, 6, 10, 14, and 18 min of CPR. Invasive (laser Doppler, brain tissue oxygen tension [PbtO2]) and noninvasive (diffuse correlation spectroscopy and diffuse optical spectroscopy) measurements of CBF and cerebral tissue oxygenation were simultaneously recorded. Effects of subsequent epinephrine doses were compared to the first.

Results
With the first epinephrine dose during CPR, CBF and cerebral tissue oxygenation increased by > 10%, as measured by each of the invasive and noninvasive measures (p < 0.001). The effects of epinephrine on CBF and cerebral tissue oxygenation decreased with subsequent doses. By the fifth dose of epinephrine, there were no demonstrable increases in CBF of cerebral tissue oxygenation. Invasive and noninvasive CBF measurements were highly correlated during asphyxia (slope effect 1.3, p < 0.001) and CPR (slope effect 0.20, p < 0.001).

Conclusions
This model suggests that epinephrine increases CBF and cerebral tissue oxygenation, but that effects wane following the third dose. Noninvasive measurements of neurological health parameters hold promise for developing and directing resuscitation strategies.
COVID-19: instruments for the allocation of mechanical ventilators—a narrative review
Authors: dos Santos Marcelo José , Martins Maristela Santini , Santana Fabiana Lopes Pereira , Furtado Maria Carolina Silvano Pacheco Corr , Miname Fabiana Cristina Bazana Remédio , Pimentel Rafael Rodrigo da Silva , Brito Ágata Nunes , Schneider Patrick , dos Santos Edson Silva , da Silva Luciane Hupalo 
Published: September 2020

AbstractAfter the World Health Organization declared COVID-19 to be a pandemic, the elaboration of comprehensive and preventive public policies became important in order to stop the spread of the disease. However, insufficient or ineffective measures may have placed health professionals and services in the position of having to allocate mechanical ventilators. This study aimed to identify instruments, analyze their structures, and present the main criteria used in the screening protocols, in order to help the development of guidelines and policies for the allocation of mechanical ventilators in the COVID-19 pandemic. The instruments have a low level of scientific evidence, and, in general, are structured by various clinical, non-clinical, and tiebreaker criteria that contain ethical aspects. Few instruments included public participation in their construction or validation. We believe that the elaboration of these guidelines cannot be restricted to specialists as this question involves ethical considerations which make the participation of the population necessary. Finally, we propose seventeen elements that can support the construction of screening protocols in the COVID-19 pandemic.
Randomised controlled trial of Compensatory Cognitive Training and a Computerised Cognitive Remediation programme
Authors: Dark Frances , Newman Ellie , Gore-Jones Victoria , De Monte Veronica , Garrido Marta I. , Dzafic Ilvana 
Published: September 2020

Abstract
Background
Compensation and adaptation therapies have been developed to improve community functioning via improving neurocognitive abilities in people with schizophrenia. Various modes of delivering compensation and adaptation therapies have been found to be effective. The aim of this trial is to compare two different cognitive interventions, Compensatory Cognitive Training (CCT) and Computerised Interactive Remediation of Cognition–Training for Schizophrenia (CIRCuiTS). The trial also aims to identify if mismatch negativity (MMN) can predict an individual’s response to the compensation and adaptation programmes.

Methods
This study will use a randomised, controlled trial of two cognitive interventions to compare the impact of these programmes on measures of neurocognition and function. One hundred clinically stable patients aged between 18 and 65 years with a diagnosis of a schizophrenia spectrum disorder will be recruited. Participants will be randomised to either the CCT or the CIRCuiTS therapy groups. The outcome measures are neurocognition (BACS), subjective sense of cognitive impairment (SSTICS), social functioning (SFS), and MMN (measured by EEG) in people with schizophrenia spectrum disorders.

Discussion
This trial will determine whether different approaches to addressing the cognitive deficits found in schizophrenia spectrum disorders are of comparable benefit using the outcome measures chosen. This has implications for services where cost and lack of computer technology limit the implementation and dissemination of interventions to address cognitive impairment in routine practice. The trial will contribute to the emerging evidence of MMN as a predictor of response to cognitive interventions.

Trial registration
Australian New Zealand Clinical Trials Registry (ANZCTR) ACTRN12618000161224. Registered on 2 February 2018. Protocol version: 4.0, 18 June 2018.
Comparison of endoscopic ultrasound-guided hepaticogastrostomy and the antegrade technique in the management of unresectable malignant biliary obstruction: study protocol for a prospective, multicentre, randomised controlled trial
Authors: Liao Ye , Giovannini Marc , Zhong Ning , Xiao Tingyue , Sheng Shiyun , Wu Yufan , Zhang Jingjing , Wang Sheng , Liu Xiang , Sun Siyu , Guo Jintao 
Published: September 2020

Abstract
Background
Endoscopic ultrasound-guided biliary drainage (EUS-BD) is used after failed endoscopic retrograde cholangiopancreatography. Based on existing studies, intrahepatic (IH) approaches are preferred in patients with dilated IH bile ducts. Both ultrasound-guided hepaticogastrostomy (EUS-HGS) and ultrasound-guided antegrade treatment (EUS-AG) are appropriate for patients with unreachable papillae. Nevertheless, there have been no direct comparisons between these two approaches. Therefore, we aim to evaluate and compare the safety and efficiency of EUS-HGS and EUS-AG in patients with an unreachable papilla.

Methods
This is a prospective, randomised, controlled, multicentre study with two parallel groups without masking. One hundred forty-eight patients from three hospitals who met the inclusion criteria will be randomly assigned (1:1) to undergo either EUS-HGS or EUS-AG for relief of malignant biliary obstruction. The final study follow-up is scheduled at 1 year postoperatively. The primary endpoint is efficiency, described by technical and clinical success rates of EUS-HGS and EUS-AG in patients with unreachable papillae. The secondary endpoints include stent patency, overall survival rates, complication rates, length of hospital stays, and hospitalisation expenses. The chi-square test, Kaplan–Meier methods, log-rank test, and Cox regression analysis will be used to analyse the data.

Discussion
To our knowledge, this is the first study to compare these two EUS-BD approaches directly using a multicentre, randomised, controlled trial design. The clinical economic indexes will also be compared, as they may also affect the patient’s choice. The result may contribute to establishing a strategic guideline for choosing IH EUS-BD approaches.

Trial registration
Chinese Clinical Trial Registry (ChiCTR) ChiCTR1900020737. Registered on 15 January 2019
Variation in hatching responses of Nematodirus battus eggs to temperature experiences
Authors: Melville Lynsey A. , Van Dijk Jan , Mitchell Sian , Innocent Giles , Bartley David J. 
Published: September 2020

Abstract
Background
Nematodirus battus, unlike most other gastrointestinal nematodes, undergoes maturation to an infective larva within the egg. Historically, eggs were considered to require a period of chilling over winter followed by a period of temperature above 10 °C for synchronous hatching to occur (generally in spring). Anecdotal reports of Nematodirus infection out-with spring in veterinary journals and the farming press suggest that the concentrated pasture abundance of N. battus infective larvae may be changing. In order for control practices to be adapted, and unexpected disease outbreaks to be avoided, it is important to quantify how parasite epidemiology is changing and research the drivers behind it.

Method
The present study investigated the in vitro hatching response to temperature experiences (with and without a period of chilling) for egg samples of 90 N. battus populations obtained from 73 commercial sheep farms. Six aliquots of larvated eggs were prepared per population, three aliquots were placed at 4 °C for 6 weeks to provide a chill stimulus then incubated at the optimal hatching temperature for the species. The remaining three aliquots of eggs were incubated at the hatching temperature without a prior chill stimulus and the number of hatched larvae was compared between treatments.

Results
Median hatch rate across all populations with chilling was 45% (95% CI: 42–48%) and without chilling was 4% (95% CI: 2–6%). Inter-population variation in hatching ranged from 0 to 87% of eggs able to hatch in the absence of a chill stimulus, mean non-chill hatching was 13 ± 2% of eggs (mean ± SE). Non-chill hatching rates were greater than chilled hatching rates in seven of the 90 populations tested.

Conclusions
Clearly, the variation in hatching responses to temperature experience is very large and therefore the seasonality of the parasite may vary not only between regions but also at farm level. In contrast to what previous work has suggested, there was a geographical trend towards higher non-chill hatching in the Northern parts of the UK.
Increased high molecular weight adiponectin and lean mass during tocilizumab treatment in patients with rheumatoid arthritis: a 12-month multicentre study
Authors: Toussirot Eric , Marotte Hubert , Mulleman Denis , Cormier Grégoire , Coury Fabienne , Gaudin Philippe , Dernis Emmanuelle , Bonnet Christine , Damade Richard , Grauer Jean-Luc , Abdesselam Tassadit Ait , Guillibert-Karras Caroline , Lioté Frédéric , Hilliquin Pascal , Sacchi Antoinette , Wendling Daniel , Le Goff Benoît , Puyraveau Marc , Dumoulin Gilles 
Published: September 2020

Abstract
Background
Patients with rheumatoid arthritis (RA) have an increased risk of cardiovascular (CV) disease. Adiponectin is involved in the metabolism of glucose and lipids with favourable effects on CV disease, especially its high molecular weight (HMW) isoform. Body composition changes are described in RA with various phenotypes including obesity. The effects of tocilizumab on serum adiponectin and body composition, especially fat mass, in patients with RA are not well determined.

Methods
Patients with active RA despite previous csDMARDs and/or bDMARDs and who were tocilizumab naïve were enrolled in a multicentre open-label study. They were evaluated at baseline, 1, 3, 6 and 12 months. Clinical assessment included body mass index (BMI) and anthropometric measurements. Lipid and metabolic parameters, serum adiponectin (total and HMW), leptin, resistin and ghrelin were measured at each time point. Body composition (lean mass, fat mass, % fat, fat in the android and gynoid regions) was evaluated at baseline, 6 and 12 months.

Results
One hundred seven patients were included. Both total and HMW adiponectin significantly increased from baseline to month 3, peaking respectively at month 3 (p = 0.0105) and month 1 (p < 0.0001), then declining progressively until month 6 to 12 and returning to baseline values. Significant elevation in HMW adiponectin persisted at month 6 (p = 0.001). BMI and waist circumference significantly increased at month 6 and 12, as well as lean mass at month 6 (p = 0.0097). Fat mass, percentage fat and android fat did not change over the study period. Lipid parameters (total cholesterol and LDL cholesterol) increased while glycaemia, insulin and HOMA-IR remained stable. Serum leptin, resistin and ghrelin did not change during follow-up.

Conclusions
Tocilizumab treatment in RA patients was associated with a significant increase in total and HMW adiponectin, especially at the onset of the treatment. Tocilizumab also induced a significant gain in lean mass, while fat mass did not change. These variations in adiponectin levels during tocilizumab treatment could have positive effects on the CV risk of RA patients. In addition, tocilizumab may have an anabolic impact on lean mass/skeletal muscle.

Trial registration
The ADIPRAT study was a phase IV open-label multicentre study retrospectively registered on ClinicalTrials.gov under the number NCT02843789 (date of registration: July 26, 2016).
Rheumatoid arthritis patients on persistent moderate disease activity on biologics have adverse 5-year outcome compared to persistent low-remission status and represent a heterogeneous group
Authors: Genitsaridi Irini , Flouri Irini , Plexousakis Dimitris , Marias Konstantinos , Boki Kyriaki , Skopouli Fotini , Drosos Alexandros , Bertsias George , Boumpas Dimitrios , Sidiropoulos Prodromos 
Published: September 2020

Abstract
Background
The long-term outcome of rheumatoid arthritis (RA) patients who in clinical practice exhibit persistent moderate disease activity (pMDA) despite treatment with biologics has not been adequately studied. Herein, we analyzed the 5-year outcome of the pMDA group and assessed for within-group heterogeneity.

Methods
We included longitudinally monitored RA patients from the Hellenic Registry of Biologic Therapies with persistent (cumulative time ≥ 50% of a 5-year period) moderate (pMDA, 3.2 < DAS28 ≤ 5.1) or remission/low (pRLDA, DAS28 ≤ 3.2) disease activity. The former was further classified into persistent lower-moderate (plMDA, DAS28 < 4.2) and higher-moderate (phMDA, DAS28 ≥ 4.2) subgroups. Five-year trajectories of functionality (HAQ) were the primary outcome in comparing pRLDA versus pMDA and assessing heterogeneity within the pMDA subgroups through multivariable mixed-effect regression. We further compared serious adverse events (SAEs) occurrence between the two groups.

Results
We identified 295 patients with pMDA and 90 patients with pRLDA, the former group comprising of plMDA (n = 133, 45%) and phMDA (n = 162, 55%). pMDA was associated with worse 5-year functionality trajectory than pRLDA (+ 0.27 HAQ units, CI 95% + 0.22 to + 0.33; p < 0.0001), while the phMDA subgroup had worse 5-year functionality than plMDA (+ 0.26 HAQ units, CI 95% 0.18 to 0.36; p < 0.0001). Importantly, higher persistent disease activity was associated with more SAEs [pRLDA: 0.2 ± 0.48 vs pMDA: 0.5 ± 0.96, p = 0.006; plMDA: 0.32 ± 0.6 vs phMDA: 0.64 ± 1.16, p = 0.038]. Male gender (p = 0.017), lower baseline DAS28 (p < 0.001), HAQ improvement > 0.22 (p = 0.029), and lower average DAS28 during the first trimester since treatment initiation (p = 0.001) independently predicted grouping into pRLDA.

Conclusions
In clinical practice, RA patients with pMDA while on bDMARDs have adverse long-term outcomes compared to lower disease activity status, while heterogeneity exists within the pMDA group in terms of 5-year functionality and SAEs. Targeted studies to better characterize pMDA subgroups are needed, in order to assist clinicians in tailoring treatments.