bluuebunny/crossref_metadata_2025_split huggingface dataset:
Advancing play participation for all: The challenge of addressing play diversity and inclusion in community parks and playgrounds
Authors: Lynch Helen , Moore Alice , Edwards Claire , Horgan Linda 
Published: December 2019

IntroductionOutdoor parks and playgrounds are important sites of social inclusion in many urban communities. However, these playspaces are often inaccessible and unusable for many children with disabilities. This paper presents findings from a case study of one urban municipality in Ireland. The study aimed to understand play participation in five local playgrounds by exploring the perspectives of play providers and families with diverse abilities, through the lens of universal design.MethodsMultiple qualitative methods were used, including playground audits, walk-and-talk observations, and semi-structured interviews. Four play providers, 12 children, and 10 adult users took part. Inductive analysis was conducted to understand the usability and accessibility of playgrounds from a universal design perspective.FindingsThese playgrounds provided high play value for younger children, but low play value for older children and those with disabilities, due to lack of accessibility or usability. While local authorities aimed to provide inclusive playgrounds, they lacked knowledge on universal design for playspaces.ConclusionChildren with disabilities continue to experience exclusion in community playspaces, despite a commitment to inclusion in local authorities. Play providers need support to tailor principles of universal design to playground design. Occupational therapists are ideally situated to collaborate with local authorities on universal design for enhancing children’s play participation in community settings.
Incidence of Intra-Abdominal Hypertension and Abdominal Compartment Syndrome: A Systematic Review
Authors: Khot Zaid , Murphy Patrick B. , Sela Nathalie , Parry Neil G. , Vogt Kelly , Ball Ian M. 
Published: December 2019

Objective: To determine the contemporary prevalence of intra-abdominal hypertension (IAH) and abdominal compartment syndrome in critically ill patients. Data Sources: Medline, Embase, and Central databases. Study Selection: Studies reporting on the prevalence of IAH in consecutively admitted critically ill patients using the World Society of Abdominal Compartment Syndrome (WSACS) consensus guidelines for intra-abdominal pressure (IAP) measurement. Data Extraction: Duplicate independent review and data abstraction. Data Synthesis: The search identified 2428 titles with 6 eligible studies (n = 1965). Reported prevalence ranged from 30% to 49%. Despite abiding by the WSACS guidelines for IAP measurement, studies varied in their definition of IAH, frequency and duration of IAP measurement, and reporting of outcomes. Three of 6 studies reported that IAH, especially at higher grades, was an independent predictor of mortality. Conclusions: Intra-abdominal hypertension is a common finding in critically ill patients and may be associated with increased mortality, especially at higher grades. Further prospective research is required to examine the effect of screening and treatment of IAH on patient outcomes.
Proximal Medial Gastrocnemius Release Versus Open Plantar Fasciotomy for the Surgical Treatment in Recalcitrant Plantar Fasciitis
Authors: Gamba Carlo , Serrano-Chinchilla Paula , Ares-Vidal Jesus , Solano-Lopez Alberto , Gonzalez-Lucena Gemma , Ginés-Cespedosa Alberto 
Published: December 2019

Background: Plantar fasciitis is a common cause of foot pain. If conservative treatment fails, there is no consensus as to the best surgical management for recalcitrant plantar fasciitis (RPF). The aim of this study was to compare the results obtained from proximal medial gastrocnemius release (PMGR) with those obtained from open plantar fasciotomy (OPF) in terms of pain, satisfaction, health-related quality of life, and American Orthopaedic Foot & Ankle Society (AOFAS) score. Methods: This is a prospective randomized trial conducted between 2012 and 2016. Patients with RPF for at least 9 months were included. Diagnosis was clinically made. The exclusion criteria were neuropathic heel pain; a history of previous foot fracture, surgery, or deformity; rheumatoid arthritis; or the need for long-term analgesic administration. After ruling out other conditions with magnetic resonance or ultrasound imaging, patients were randomized to be operated on with OPF or PMGR independently of the Silfverskjold test. Follow-up was carried out for up to 1 year. The AOFAS, visual analog scale (VAS) for pain, SF-36, and Likert scale for satisfaction were used to evaluate the results obtained. The analysis was done with 21 patients in the OPF group and 15 in the PMGR group. The demographic data (age, sex, body mass index, duration of symptoms, and positivity to the Silfverskjold test) of the groups were comparable. Results: No differences were found in terms of the AOFAS ( P = .24), VAS ( P = .14), or any item of the SF-36. Satisfaction was very good in 85.8% of the PMGR group and 89.5% of the OPF group ( P = .27). Faster recovery was observed in the PMGR group. Conclusion: OPF and PMGR provided good results for patients with RPF. Neither was superior to the other relative to pain, AOFAS score, satisfaction, or the SF-36. We recommend PMGR as the first option in RPF surgical management in order to avoid potential biomechanical complications related to OPF. Level of Evidence: Level I, therapeutic randomized controlled trial.
Bioinformatics Analysis of Expression and Alterations of BARD1 in Breast Cancer
Authors: Chen Yong-Zi , Zuo Duo , Ren Hai-Ling , Fan Sai-Jun , Ying Guoguang 
Published: December 2019

Background: Breast cancer is one of the most common malignant tumor type in women worldwide. BARD1 could impact function of BRCA1 as its interaction partner. In the current study, we aimed to investigate the prognostic role of BARD1 expression as well as its alterations in breast cancer using different online tools. Methods: We performed a bioinformatics analysis for BARD1 in patients with breast cancer using several online databases, including Oncomine, bc-GenExMiner, PrognoScan, Search Tool for the Retrieval of Interacting Genes, Cytoscape, and cBioPortal. Results: We found that BARD1 was highly expressed in basal-like, HER2-E, and luminal B compared with normal-like subtype. Forest plot showed that BARD1 overexpression was correlated with worse distant metastasis-free survival (hazard ratio: 2.72, 95% confidence interval: 1.02-2.21; P = .0448), disease-specific survival (hazard ratio: 2.65, 95% confidence interval: 1.37-5.12; P = .0037), and disease-free survival (hazard ratio: 1.98, 95% confidence interval: 1.22-3.24; P = .0062) but positively correlated with overall survival (hazard ratio: 0.66, 95% confidence interval: 0.50-0.85; P = .0017). Multivariate analysis indicated that BARD1 expression was significantly associated with distant metastasis-free survival (hazard ratio: 4.60, 95% confidence interval: 1.22-17.28; P = .0239) whereas marginally significant for disease-free survival (hazard ratio: 1.00, 95% confidence interval: 1.00-1.01, P = .0630) and disease-specific survival (hazard ratio: 1.96, 95% confidence interval: 0.97-3.96; P = .0602). Meanwhile, alterations in BARD1 interaction network were associated with worse overall survival instead of BARD1 alteration alone. Conclusions: Bioinformatics analysis revealed that BARD1 may be a predictive biomarker for prognosis of breast cancer. However, future research is required to validate our findings.
High serum concentration of vitamin D may protect against multiple sclerosis
Authors: Biström Martin , Alonso-Magdalena Lucia , Andersen Oluf , Jons Daniel , Gunnarsson Martin , Vrethem Magnus , Hultdin Johan , Sundström Peter 
Published: December 2019

Background High 25-hydroxyvitamin D concentrations have been associated with a reduced risk of multiple sclerosis, with indications of a stronger effect among young individuals. Objective Investigate the 25-hydroxyvitamin D association with multiple sclerosis and test if this association is age dependent. Methods Prospectively drawn blood samples from individuals later developing relapsing–remitting multiple sclerosis and controls matched for biobank, sex, age and date of sampling, were analysed with liquid chromatography tandem mass spectrometry. Results High levels of 25-hydroxyvitamin D (top quintile) were associated with a reduced multiple sclerosis risk (odds ratio 0.68, 95% confidence interval 0.50–0.93). Conclusion These findings further support a role for vitamin D in MS aetiology.
Comparison of Gastrocnemius Turn Flap and Hamstring Graft for the Treatment of Kuwada Type 3 Chronic Ruptures of the Achilles Tendon: A Retrospective Study
Authors: Bai Lu , Guan Siyao , You Tian , Zhang Wentao , Chen Peng 
Published: December 2019

Background: Chronic Achilles tendon rupture is challenging to repair, and many procedures have been suggested to fill the gap that separates the distal and proximal ends of the ruptured tendon. Purpose: To compare clinical outcomes between the free hamstring graft (HG) and gastrocnemius turn flap (GTF) procedures in the treatment of chronic Achilles tendon rupture. Study Design: Cohort study; Level of evidence, 3. Methods: This retrospective study included 26 patients (25 males, 1 female; mean age, 36.7 years; range, 22-53 years) with Kuwada type 3 chronic rupture of the Achilles tendon. A total of 11 patients underwent GTF surgery, whereas 15 patients underwent HG surgery. Follow-up assessments were conducted at 3, 6, and 12 months postoperatively. Results: The complication rate was significantly higher in the GTF group compared with the HG group (27.2% vs 6.6%, respectively; χ2 = 12.462; P = .001). At the 3-month follow-up, the degree of ankle dorsiflexion was significantly higher in the HG group than in the GTF group ( t = 3.144; P = .004). At 6-month and 1-year follow-up, no significant differences in ankle function were seen between the 2 groups. Conclusion: Hamstring tendon graft is associated with better early recovery of dorsiflexion compared with GTF. The long-term clinical outcomes of these 2 procedures are similar.
There Is No Place Like Home: A Survey on Satisfaction and Reported Outcomes of a Home-Based Rehabilitation Program Among Orthopedic Surgery Patients
Authors: Gagin Roni , HaGani Neta , Levy Itay , Norman Doron 
Published: December 2019

Background: Home-based rehabilitation (HBR) was previously found to be associated with positive outcomes that are equal to inpatient rehabilitation. Few studies have examined the challenges patients are facing during rehabilitation and recovery and their satisfaction from HBR. Objective: The purpose of this study was to examine the overall satisfaction and reported outcomes of HBR. Methods: A telephone survey was conducted among 146 orthopedic surgery patients who participated in an HBR program, at the end of the HBR and 6 months after. The questionnaire included information about level of independence, satisfaction from HBR, and difficulties during HBR. Findings: The overall satisfaction was high (73.1%). Patients reported on improvements in level of independence between the time of hospital discharge, the end of the program ( P = .0001), and the 6 months follow-up ( P = .004). Long wait for beginning of HBR, being a widower, and residing in a facility or with a nonfamilial caregiver were associated significantly with less general well-being and independence. The repeated measures analysis showed age lower than 71 and private health insurance ownership were associated with a better recovery. The most common difficulties reported were physical difficulties, lack of function, and caregivers’ burden. Conclusions: Patients and families need more emotional, social, and physical support during HBR. The increase in health services delivered in community settings requires a more clear-cut policy and supervision for HBR and the follow-up services.
Gene therapy for hemophilia
Authors: Nathwani Amit C. 
Published: December 2019

Abstract
Gene therapy offers the potential for a cure for patients with hemophilia by establishing continuous endogenous expression of factor VIII or factor IX (FIX) following transfer of a functional gene to replace the hemophilic patient’s own defective gene. The hemophilias are ideally suited for gene therapy because a small increment in blood factor levels (≥5% of normal) is associated with significant amelioration of bleeding phenotype in severely affected patients. In 2011, the St. Jude/UCL phase 1/2 trial was the first to provide clear evidence of a stable dose-dependent increase in FIX levels in patients with severe hemophilia B following a single administration of adeno-associated viral (AAV) vectors. Transgenic FIX expression has remained stable at ∼5% of normal in the high-dose cohort over a 7-year follow-up period, resulting in a substantial reduction in spontaneous bleeding and FIX protein usage without toxicity. This study has been followed by unparalleled advances in gene therapy for hemophilia A and B, leading to clotting factor activity approaching normal or near-normal levels associated with a “zero bleed rates” in previously severely affected patients following a single administration of AAV vectors. Thus, AAV gene therapies are likely to alter the treatment paradigm for hemophilia A and B. This review explores recent progress and the remaining limitations that need to be overcome for wider availability of this novel treatment of inherited bleeding disorders.
Monitoring and treatment of MDS in genetically susceptible persons
Authors: Davies Stella M. 
Published: December 2019

AbstractGenetic susceptibility to myelodysplastic syndrome (MDS) occurs in children with inherited bone marrow failure syndromes, including Fanconi anemia, Shwachman Diamond syndrome, and dyskeratosis congenita. Available evidence (although not perfect) supports annual surveillance of the blood count and bone marrow in affected persons. Optimal treatment of MDS in these persons is most commonly transplantation. Careful consideration must be given to host susceptibility to DNA damage when selecting a transplant strategy, because significant dose reductions and avoidance of radiation are necessary. Transplantation before evolution to acute myeloid leukemia (AML) is optimal, because outcomes of AML are extremely poor. Children and adults can present with germline mutations in GATA2 and RUNX1, both of which are associated with a 30% to 40% chance of evolution to MDS. GATA2 deficiency may be associated with a clinically important degree of immune suppression, which can cause severe infections that can complicate transplant strategies. GATA2 and RUNX1 deficiency is not associated with host susceptibility to DNA damage, and therefore, conventional treatment strategies for MDS and AML can be used. RUNX1 deficiency has a highly variable phenotype, and MDS can occur in childhood and later in adulthood within the same families, making annual surveillance with marrow examination burdensome; however, such strategies should be discussed with affected persons, allowing an informed choice.
Update from the clinic: what’s new in the diagnosis of cancer-associated thrombosis?
Authors: Peterson Erica A. , Lee Agnes Y. Y. 
Published: December 2019

AbstractMalignancy is associated with a high risk of venous thromboembolism (VTE), and treatment with anticoagulant therapy is associated with a high risk of bleeding. Thus, accurate and timely VTE diagnosis in cancer patients is essential for identifying individuals who would benefit from anticoagulant therapy and for avoiding unnecessary treatment that can cause anticoagulant-related bleeding. The approach to the diagnosis of VTE in non-cancer patients involves a stepwise process beginning with an assessment of the pretest probability (PTP) of VTE using a validated clinical prediction rule (CPR) followed by D-dimer testing and/or diagnostic imaging. In patients with a low PTP and a negative D-dimer result, VTE can be excluded without additional imaging. However, published data suggest that CPRs and D-dimer testing may not be as accurate or as useful in patients with cancer. Studies have shown that the combination of a low PTP and negative D-dimer result is not efficient for exclusion of deep vein thrombosis (DVT) or pulmonary embolism (PE) in the cancer patient population because the vast majority of patients still require radiologic imaging. We propose that cancer patients with suspected VTE should proceed directly to radiologic imaging to confirm or exclude a diagnosis of DVT or PE.
Beyond JAK-STAT: novel therapeutic targets in Ph-negative MPN
Authors: Gerds Aaron T. 
Published: December 2019

AbstractThe Philadelphia chromosome-negative myeloproliferative neoplasms (MPNs) share a common pathobiology of constitutive activation of the JAK and STAT pathway, despite having the 3 distinct phenotypes of essential thrombocythemia, polycythemia vera, and primary myelofibrosis. Targeting the JAK-STAT pathway has led to remarkable clinical benefit, including reduction in splenomegaly, amelioration of cytokine-driven symptoms, improvement in quality of life, and even some improvement in survival. However, targeting this pathway has not resulted in consistent disease modification by current metrics, including a reduction in mutant allele burden or reversal of fibrosis. Moreover, targeting JAK-STAT can lead to limiting treatment-emergent side effects, such as anemia and thrombocytopenia. Continued discovery points to a complex system of pathogenesis beyond JAK-STAT driving the formation and evolution of MPNs. This article reviews the successes and limitations of JAK-STAT inhibition, surveys the strategies behind emerging therapies, and discusses the challenges that are present in moving beyond JAK-STAT.
Meeting the needs of CML patients in resource-poor countries
Authors: Malhotra Hemant , Radich Jerald , Garcia-Gonzalez Pat 
Published: December 2019

Abstract
Subsequent to the development and global availability of BCR/ABL–targeted tyrosine kinase inhibitors (TKIs), the prognosis of patients with chronic myeloid leukemia (CML), at least those in the chronic phase, has markedly improved, and in the developed world, the average lifespan of these patients is now close to that of age- and sex-matched subjects without the disease. However, the situation in low- and middle-income countries (LMICs) may not be so rosy. Many important differences in hematological cancers, including CML, have been highlighted in various publications in LMICs vs developed countries. These include differences in incidence and prevalence rates, age and stage of disease at diagnosis, response rates, and survival. Some of the possible reasons proposed for these are varying socioeconomic milieu (impacting availability of effective drugs and essential monitoring), environmental factors (mainly exposure to viral infections and pesticides), nutritional factors with interplay of malnutrition and diet on drug absorption and blood levels, and possible unknown genetic factors. Although generic first-generation TKIs (imatinib) are available in many parts of the world, several challenges remain in providing optimal treatment to patients with CML in resource-poor countries. Some of these include availability of optimal and high-quality BCR/ABL testing, availability and expense related to use of second- and third-generation TKIs (nilotinib, dasatinib, bosutinib, and ponatinib) and hematopoietic stem cell transplantation, issues with compliance and toxicities of drugs, and ensuring a minimal standard-of-care treatment and monitoring for every patient diagnosed with CML. For the purpose of this article, the more objective country label—LMIC—coined by the World Bank will be used (gross national income per capita between $1026 and $3995; World Bank, June 2019). Some of these issues will be discussed in this article in greater detail by experts in the field in 3 different but interconnected sections.
Primary immunodeficiencies: novel genes and unusual presentations
Authors: Notarangelo Luigi D. , Uzel Gulbu , Rao V. Koneti 
Published: December 2019

Abstract
Recent advances in genomics have greatly expanded the spectrum of primary immune deficiencies (PIDs). Along with the identification of pathogenic variants in novel genes, distinct phenotypes have been associated with different variants in the same gene. Although PIDs have been historically defined based on increased susceptibility to infections, immune dysregulation has emerged as a frequent and in some cases, predominant phenotype. Autoimmune cytopenias with onset in childhood, lasting longer than 12 months, and affecting multiple lineages should raise the suspicion of a possible PID with monogenic origin. Characterization of the various molecular and cellular mechanisms responsible for these unusual manifestations of PIDs, although at times resource intensive, may allow for targeted intervention in many of them.
A program of transition to adult care for sickle cell disease
Authors: Saulsberry Anjelica C. , Porter Jerlym S. , Hankins Jane S. 
Published: December 2019

Abstract
Most children with sickle cell disease (SCD) today survive into adulthood. Among emerging adults, there is a marked increase in acute care utilization and a rise in mortality, which can be exacerbated by not establishing or remaining in adult care. Health care transition programs are therefore essential to prepare, transfer, and integrate emerging adults in the adult care setting. The Six Core Elements of Health Care Transition, created by the Center for Health Care Transition Improvement, define the basic components of health care transition support as follows: (1) transition policy, (2) tracking and monitoring progress, (3) assessing transition readiness, (4) planning for adult care, (5) transferring to adult care, and (6) integrating into adult care. Programs that implement the Six Core Elements have experienced significant declines in care abandonment during adolescence and young adulthood and higher early adult care engagement. Most of the core transition activities are not currently reimbursable, however, posing a challenge to sustain transition programs. Ongoing studies are investigating interventions in comparative effectiveness trials to improve health-related quality of life and reduce acute care utilization among emerging adults with SCD. Although these studies will identify best practices for health care transition, it is also important to define how the transition outcomes will be measured, as no consensus definition exists for successful health care transition in SCD. Future research is needed to define best practices for health care transition, systematically assess transition outcomes, and revise payment models to promote sustainability of health care transition programs.