bluuebunny/crossref_metadata_2025_split huggingface dataset:
Fertility intentions and the way they change following birth- a prospective longitudinal study
Authors: Preis Heidi , Tovim Selen , Mor Pnina , Grisaru-Granovsky Sorina , Samueloff Arnon , Benyamini Yael 
Published: April 2020

AbstractBackgroundWomen’s fertility intentions, their desired number of children and desired inter-pregnancy interval (IPI) are related to micro (personal) and macro (socio-cultural) level factors. We investigated factors that contribute to changes in women’s fertility intentions in Israel, a developed country with high birth rates.MethodsPregnant women (N = 1163), recruited from prenatal clinics and hospitals in two major metropolitan areas, completed self-report questionnaires prenatally (≥24 weeks gestation) and postpartum (2 months after childbirth). Women reported their socio-demographic background and obstetric history prenatally, their desired number of children and IPI at both time-points, and their objective and subjective birth experiences postpartum.ResultsThe findings indicated that background characteristics were related to prenatal fertility intentions. The strongest contributor to prenatal fertility intentions was women’s degree of religiosity- the more religious they were, the more children they desired and the shorter their intended IPI. Women’s postpartum fertility intentions were mostly consistent with their prenatal reports. In regression models, women who were very-religious, more educated and had previously given birth were less likely to report a lower number of desired of children at postpartum, compared to their prenatal report. Women who reported greater birth satisfaction and gave birth for the first time were less likely to change desired IPI.ConclusionHaving a negative birth experience could adversely affect women’s fertility intentions. Yet, in a pronatalist and medicalized birth culture, social pressures may decrease the effects of birth experiences on fertility intentions.
Busulfan systemic exposure and its relationship with efficacy and safety in hematopoietic stem cell transplantation in children: a meta-analysis
Authors: Feng Xinying , Wu Yunjiao , Zhang Jingru , Li Jiapeng , Zhu Guanghua , Fan Duanfang , Yang Changqing , Zhao Libo 
Published: April 2020

AbstractBackgroundBusulfan (Bu) is a key component of several conditioning regimens used before hematopoietic stem cell transplantation (HSCT). However, the optimum systemic exposure (expressed as the area under the concentration-time curve [AUC]) of Bu for clinical outcome in children is controversial.MethodsResearch on pertinent literature was carried out at PubMed, EMBASE, Web of science, the Cochrane Library andClinicalTrials.gov. Observational studies were included, which compared clinical outcomes above and below the area under the concentration-time curve (AUC) cut-off value, which we set as 800, 900, 1000, 1125, 1350, and 1500 μM × min. The primary efficacy outcome was notable in the rate of graft failure. In the safety outcomes, incidents of veno-occlusive disease (VOD) were recorded, as well as other adverse events.ResultsThirteen studies involving 548 pediatric patients (aged 0.3–18 years) were included. Pooled results showed that, compared with the mean Bu AUC (i.e., the average value of AUC measured multiple times for each patient) of > 900 μM × min, the mean AUC value of < 900 μM × min significantly increased the incidence of graft failure (RR = 3.666, 95% CI: 1.419, 9.467). The incidence of VOD was significantly decreased with the mean AUC < 1350 μM × min (RR = 0.370, 95% CI: 0.205–0.666) and < 1500 μM × min (RR = 0.409, 95% CI: 0182–0.920).ConclusionsIn children, Bu mean AUC above the cut-off value of 900 μM × min (after every 6-h dosing) was associated with decreased rates of graft failure, while the cut-off value of 1350 μM × min were associated with increased risk of VOD, particularly for the patients without VOD prophylaxis therapy. Further well-designed prospective and multi centric randomized controlled trials with larger sample size are necessary before putting our result into clinical practices.
Atypical hemolytic uremic syndrome precipitated by thyrotoxicosis: a case report
Authors: Hou Ling , Du Yue 
Published: April 2020

Abstract
Background
Autoimmune thyroid disease (AITD) has a complex pathogenesis and is associated with the development of autoimmunity against the thyroid. Graves’ disease and Hashimoto’s thyroiditis are the two main types of AITD, and they are characterized by thyrotoxicosis and hypothyroidism, respectively. Atypical hemolytic uremic syndrome (aHUS) is a rare disease, presenting with microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. aHUS is caused by dysregulation of the alternative complement pathway, and its co-existence with AITD is rare.

Case presentation
We report the case of a 12-year-old girl with recent onset thyrotoxicosis. She was first treated with propylthiouracil for 2 months and then developed AITD presenting as abrupt-onset thrombocytopenia, acute kidney injury, and microangiopathic hemolytic anemia. Thyroid function tests favored hyperthyroidism, with increased free T4 and free T3 levels and a very low level of thyroid-stimulating hormone (TSH). We suspected aHUS, and the patient’s condition responded dramatically to therapeutic plasma exchange (TPE) with disease remission. She experienced recurrent aHUS after subsequently receiving methimazole for 1 month, and in the recurrent episode, her condition responded again to TPE and concomitant glucocorticoids. She achieved euthyroidism with thiamazole ointment treatment, without aHUS recurrence during the 6-month follow-up. Mycophenolate mofetil was administered to manage proteinuria after 3 months of treatment with the steroid and angiotensin-converting enzyme inhibitor.

Conclusions
The coexistence of aHUS and AITD in this case is likely more than coincidence, because both are autoimmune in origin. aHUS is associated with a high mortality without appropriate therapy, and treatment with TPE and adjunct immunosuppressants can be helpful.
Autosomal dominant transmission of transient neonatal lactic acidosis: a case report
Authors:   Care4Rare Consortium, Mardian Emily B. , Lines Matthew A. , Moore Gregory P. 
Published: April 2020

Abstract
Background
Lactic acidosis is a common finding in neonates, in whom mitochondrial dysfunction is often secondary to tissue hypoperfusion, respiratory failure, and/or sepsis. Primary (non-physiological) lactic acidosis is comparatively rare, and suggests the presence of an inborn error of mitochondrial energy metabolism. Optimal medical management and accurate prognostication requires the correct determination of the etiology of lactic acidosis in a given patient. Unfortunately, genetic diagnoses are rare and highly variable for neonates presenting with primary lactic acidosis; individual case reports may offer the most promise for treatment considerations.
The mitochondrion is a complex molecular machine incorporating the products of > 1000 distinct nuclear genes. Primary lactic acidoses are therefore characterized by high genetic heterogeneity and a specific genetic diagnosis currently remains out of reach in most cases. Most mitochondriopathies with neonatal onset follow autosomal recessive inheritance and carry a poor prognosis. Here we detail the case of a father and daughter with dominantly-inherited, resolving (i.e. transient) neonatal hyperlactatemia due to complex IV deficiency. We found no other published descriptions of benign transient complex IV deficiency with autosomal dominant inheritance.

Case presentation
Both individuals presented as neonates with unexplained, marked lactic acidosis suggesting a primary mitochondrial disorder. Within the first weeks of life, elevated blood lactate levels normalized. Their clinical and developmental outcomes were normal. Biochemical studies in the proband showed multiple abnormalities consistent with a complex IV respiratory chain defect. Cultured skin fibroblasts showed an elevated lactate-to-pyruvate ratio, deficient complex IV activity, and normal pyruvate dehydrogenase and pyruvate carboxylase activities. Whole-exome sequencing of the proband and both parents did not identify a causative mutation.

Conclusion
We conclude that the proband and her father appear to have a dominant form of transient neonatal hyperlactatemia due to heterozygous changes in an as-yet unidentified gene. This transient neonatal complex IV deficiency should be considered in the differential diagnosis of primary neonatal hyperlactatemia; notable clinical features include autosomal-dominant inheritance and an apparently benign postnatal course. This report exemplifies the growing differential diagnosis for neonatal lactic acidosis and highlights the importance of both physician counselling and the use of family history in communicating with parents.
Towards improving the identification of anterior cruciate ligament tears in primary point-of-care settings
Authors: Whittaker Jackie L. , Chan Michelle , Pan Bo , Hassan Imran , Defreitas Terry , Hui Catherine , Macedo Luciana , Otto David 
Published: April 2020

Abstract
Background
Only a small proportion of anterior cruciate ligament (ACL) tears are diagnosed on initial healthcare consultation. Current clinical guidelines do not acknowledge that primary point-of-care practitioners rely more heavily on a clinical history than special clinical tests for diagnosis of an ACL tear. This research will assess the accuracy of combinations of patient-reported variables alone, and in combination with clinician-generated variables to identify an ACL tear as a preliminary step to designing a primary point-of-care clinical decision support tool.

Methods
Electronic medical records (EMRs) of individuals aged 15–45 years, with ICD-9 codes corresponding to a knee condition, and confirmed (ACL+) or denied (ACL−) first-time ACL tear seen at a University-based Clinic between 2014 and 2016 were eligible for inclusion. Demographics, relevant diagnostic indicators and ACL status based on orthopaedic surgeon assessment and/or MRI reports were manually extracted. Descriptive statistics calculated for all variables by ACL status. Univariate between group comparisons, clinician surveys (n = 17), availability of data and univariable logistic regression (95%CI) were used to select variables for inclusion into multivariable logistic regression models that assessed the odds (95%CI) of an ACL-tear based on patient-reported variables alone (consistent with primary point-of-care practice), or in combination with clinician-generated variables. Model performance was assessed by accuracy, sensitivity, specificity, positive and negative predictive values, and positive and negative likelihood ratios (95%CI).

Results
Of 1512 potentially relevant EMRs, 725 were included. Participant median age was 26 years (range 15–45), 48% were female and 60% had an ACL tear. A combination of patient-reported (age, sport-related injury, immediate swelling, family history of ACL tear) and clinician-generated (Lachman test result) variables were superior for ACL tear diagnosis [accuracy; 0.95 (90,98), sensitivity; 0.97 (0.88,0.98), specificity; 0.95 (0.82,0.99)] compared to the patient-reported variables alone [accuracy; 84% (77,89), sensitivity; 0.60 (0.44,0.74), specificity; 0.95 (0.89,0.98)].

Conclusions
A high proportion of individuals without an ACL tear can be accurately identified by considering patient-reported age, injury setting, immediate swelling and family history of ACL tear. These findings directly inform the development of a clinical decision support tool to facilitate timely and accurate ACL tear diagnosis in primary care settings.
Associations between learning environment variables and students’ approaches to studying: a cross-sectional study
Authors: Mørk Gry , Magne Trine A. , Carstensen Tove , Stigen Linda , Åsli Lene A. , Gramstad Astrid , Johnson Susanne G. , Bonsaksen Tore 
Published: April 2020

Abstract
Background
Aspects of the learning environment may be related to students` approaches to studying, but few studies have investigated these relationships in the context of occupational therapy education.

Objective
To examine associations between occupational therapy students’ perceptions of the learning environment and their approaches to studying.

Method
One hundred eighty-seven first-year occupational therapy students in Norway (response rate 61.3%) participated in this study. Aside from sociodemographic information, the students completed the Course Experience Questionnaire and the Approaches and Study Skills Inventory for Students. Associations between learning environment variables and study approaches were investigated with hierarchical linear regression analyses.

Results
Higher scores on Generic skills were associated with higher scores on the deep and strategic approach scales (β ranging 0.18–0.51), while lower scores were associated with higher surface approach scale scores (β = − 0.24). Lower scores on Clear goals and standards and Appropriate workload were associated with higher surface approach scores (β ranging − 0.16 - -0.42).

Conclusion
By improving aspects of the learning environment, there may be a potential for influencing occupational therapy students’ approaches to studying. Based on this study, emphasizing how generic skills developed in the study program may become useful in practising a profession, ensuring clarity of goals and standards, and maintaining an appropriate workload on students appear to be important.
Resilient Health Care: a systematic review of conceptualisations, study methods and factors that develop resilience
Authors: Iflaifel Mais , Lim Rosemary H. , Ryan Kath , Crowley Clare 
Published: April 2020

AbstractBackgroundTraditional approaches to safety management in health care have focused primarily on counting errors and understanding how things go wrong. Resilient Health Care (RHC) provides an alternative complementary perspective of learning from incidents and understanding how, most of the time, work is safe. The aim of this review was to identify how RHC is conceptualised, described and interpreted in the published literature, to describe the methods used to study RHC, and to identify factors that develop RHC.MethodsElectronic searches of PubMed, Scopus and Cochrane databases were performed to identify relevant peer-reviewed studies, and a hand search undertaken for studies published in books that explained how RHC as a concept has been interpreted, what methods have been used to study it, and what factors have been important to its development. Studies were evaluated independently by two researchers. Data was synthesised using a thematic approach.ResultsThirty-six studies were included; they shared similar descriptions of RHC which was the ability to adjust its functioning prior to, during, or following events and thereby sustain required operations under both expected and unexpected conditions. Qualitative methods were mainly used to study RHC. Two types of data sources have been used: direct (e.g. focus groups and surveys) and indirect (e.g. observations and simulations) data sources. Most of the tools for studying RHC were developed based on predefined resilient constructs and have been categorised into three categories: performance variability and Work As Done, cornerstone capabilities for resilience, and integration with other safety management paradigms. Tools for studying RHC currently exist but have yet to be fully implemented. Effective team relationships, trade-offs and health care ‘resilience’ training of health care professionals were factors used to develop RHC.ConclusionsAlthough there was consistency in the conceptualisation of RHC, methods used to study and the factors used to develop it, several questions remain to be answered before a gold standard strategy for studying RHC can confidently be identified. These include operationalising RHC assessment methods in multi-level and diverse settings and developing, testing and evaluating interventions to address the wider safety implications of RHC amidst organisational and institutional change.
Comparison of ELISA with electro-chemiluminescence technology for the qualitative and quantitative assessment of serological responses to vaccination
Authors: Bolton Jessica S. , Chaudhury Sidhartha , Dutta Sheetij , Gregory Scott , Locke Emily , Pierson Tony , Bergmann-Leitner Elke S. 
Published: April 2020

Abstract
Background
Profiling immune responses induced by either infection or vaccination can provide insight into identification of correlates of protection. Furthermore, profiling of serological responses can be used to identify biomarkers indicative of exposure to pathogens. Conducting such immune surveillance requires readout methods that are high-throughput, robust, and require small sample volumes. While the enzyme-linked immunosorbent assay (ELISA) is the classical readout method for assessing serological responses, the advent of multiplex assays has significantly increased the throughput and capacity for immunoprofiling. This report describes the development and assay performance (sensitivity, linearity of detection, requirement for multiple dilutions for each sample, intra- and inter-assay variability) of an electro-chemiluminescence (ECLIA)-based multiplex assay.

Methods
The current study describes the development of a multiplex ECLIA-based assay and characterizes the sensitivity, linear range, and inter- and intra-assay variability of the ECLIA platform and its agreement with the traditional ELISA. Special emphasis was placed on potential antigenic competition when testing closely related antigens in the multiplex format.

Results
Multiplexing of antigens in ECLIA provides significant practical benefits in terms of reducing sample volume requirements and experimental time. Beyond the practical advantages of multiplexing, the ECLIA provides superior assay performance when compared to the ELISA. Not only does ECLIA show good agreement with the ELISA assay, but the linear range of ECLIA is also sufficiently wide to permit single-dilution measurements of concentration without the need to do serial dilutions. The lack of antigenic competition allows the simultaneous testing of closely related antigens, such as plate antigens representing different alleles of the same protein, which can inform about cross-reactivities—or lack thereof—of serological responses.

Conclusion
The advantages of the newly developed tool for assessing the antigen profiles of serological responses may ultimately lead to the identification of biomarkers associated with various disease stages and or protection against disease.
Health-related quality of life and scar satisfaction in a cohort of children operated on for sacrococcygeal teratoma
Authors: Hambraeus Mette , Hagander Lars , Arnbjörnsson Einar , Börjesson Anna , Stenström Pernilla 
Published: April 2020

Abstract
Aim
The aims of this study were to evaluate health-related quality of life (HRQoL) in children with sacrococcygeal teratoma and to explore the effect of the scar on physical, emotional and behavioral aspects.

Methods
A cohort of children operated on for sacrococcygeal teratoma between 2000 and 2013 at Lund University Hospital, Sweden, and their parents were interviewed. HRQoL was evaluated with PedsQL, and scar satisfaction was estimated through Patient Observer Scar Assessment Score (POSA).

Results
All eligible children (n = 17) were included (100% response rate). Median age was 7.3 years (range 3.5–16.0). Mean total PedsQL score was 92.3 (range 72.0 to 99.0). Patients with comorbidity scored lower (87.5) than those without (95.0) (p < 0.05). Pain during sitting down was reported by two (20%) patients, and itching was reported by another two patients (20%) aged > 8 years. No children reported that they avoided situations due to the scar, and most (80% of children and 90% of parents) reported absent or only mild negative emotions when considering the scar.

Conclusion
Children with sacrococcygeal teratoma had a good overall HRQoL, but comorbidity reduced the outcome. A few children reported scar-related impact on physical, behavioral and emotional aspects.
Low DLG2 gene expression, a link between 11q-deleted and MYCN-amplified neuroblastoma, causes forced cell cycle progression, and predicts poor patient survival
Authors: Keane Simon , Améen Sophie , Lindlöf Angelica , Ejeskär Katarina 
Published: April 2020

Abstract
Background
Neuroblastoma (NB) is a childhood neural crest tumor. There are two groups of aggressive NBs, one with MYCN amplification, and another with 11q chromosomal deletion; these chromosomal aberrations are generally mutually exclusive. The DLG2 gene resides in the 11q-deleted region, thus makes it an interesting NB candidate tumor suppressor gene.

Methods
We evaluated the association of DLG2 gene expression in NB with patient outcomes, stage and MYCN status, using online microarray data combining independent NB patient data sets. Functional studies were also conducted using NB cell models and the fruit fly.

Results
Using the array data we concluded that higher DLG2 expression was positively correlated to patient survival. We could also see that expression of DLG2 was inversely correlated with MYCN status and tumor stage. Cell proliferation was lowered in both 11q-normal and 11q-deleted NB cells after DLG2 over expression, and increased in 11q-normal NB cells after DLG2 silencing. Higher level of DLG2 increased the percentage of cells in the G2/M phase and decreased the percentage of cells in the G1 phase. We detected increased protein levels of Cyclin A and Cyclin B in fruit fly models either over expressing dMyc or with RNAi-silenced dmDLG, indicating that both events resulted in enhanced cell cycling. Induced MYCN expression in NB cells lowered DLG2 gene expression, which was confirmed in the fly; when dMyc was over expressed, the dmDLG protein level was lowered, indicating a link between Myc over expression and low dmDLG level.

Conclusion
We conclude that low DLG2 expression level forces cell cycle progression, and that it predicts poor NB patient survival. The low DLG2 expression level could be caused by either MYCN-amplification or 11q-deletion.

Graphical abstract
Salvage use of tissue plasminogen activator (tPA) in the setting of acute respiratory distress syndrome (ARDS) due to COVID-19 in the USA: a Markov decision analysis
Authors: Choudhury Rashikh , Barrett Christopher D. , Moore Hunter B. , Moore Ernest E. , McIntyre Robert C. , Moore Peter K. , Talmor Daniel S. , Nydam Trevor L. , Yaffe Michael B. 
Published: April 2020

AbstractBackgroundCOVID-19 threatens to quickly overwhelm our existing critical care infrastructure in the USA. Systemic tissue plasminogen activator (tPA) has been previously demonstrated to improve PaO2/FiO2(mmHg) when given to critically ill patients with acute respiratory distress syndrome (ARDS). It is unclear to what extent tPA may impact population-based survival during the current US COVID-19 pandemic.MethodsA decision analytic Markov state transition model was created to simulate the life critically ill COVID-19 patients as they transitioned to either recovery or death. Two patient groups were simulated (50,000 patients in each group); (1) Patients received tPA immediately upon diagnosis of ARDS and (2) patients received standard therapy for ARDS. Base case critically ill COVID-19 patients were defined as having a refractory PaO2/FiO2of < 60 mmHg (salvage use criteria). Transition from severe to moderate to mild ARDS, recovery, and death were estimated. Markov model parameters were extracted from existing ARDS/COVID-19 literature.ResultsThe use of tPA was associated with reduced mortality (47.6% [tTPA] vs. 71.0% [no tPA]) for base case patients. When extrapolated to the projected COVID-19 eligible for salvage use tPA in the USA, peak mortality (deaths/100,000 patients) was reduced for both optimal social distancing (70.5 [tPA] vs. 75.0 [no tPA]) and no social distancing (158.7 [tPA] vs. 168.8 [no tPA]) scenarios.ConclusionsSalvage use of tPA may improve recovery of ARDS patients, thereby reducing COVID-19-related mortality and ensuring sufficient resources to manage this pandemic.