bluuebunny/crossref_metadata_2025_split huggingface dataset:
Native-valve Enterococcus hirae endocarditis: a case report and review of the literature
Authors: Pinkes Mary E. , White Catherine , Wong Cynthia S. 
Published: December 2019

Abstract

Background
Enterococcus hirae is rarely identified in humans and may be a commensal pathogen in psittacine birds. We present the fifth known case of E. hirae endocarditis.

Case presentation
A 64-year-old Caucasian female presented with fever, hypotension, atrial fibrillation with rapid ventricular response, and a two-week history of lightheadedness. Her previous medical history included COPD, recurrent DVT, atrial fibrillation (on warfarin), hypertension, hypothyroidism, and Hodgkin’s lymphoma. Physical exam was notable for expiratory wheezes and a 2/6 systolic ejection murmur at the right sternal border. 2D echocardiogram revealed severe aortic stenosis. The patient underwent right and left heart catheterization, where she was found to have severe aortic stenosis and mild pulmonary hypertension. She subsequently underwent minimally invasive aortic valve replacement with a bovine pericardial valve, bilateral atrial cryoablation, and clipping of the left atrial appendage. Her aortic valve was found to have a bicuspid, thickened appearance with calcifications, multiple small vegetations, and a root abscess beneath the right coronary cusp. With a new suspicion of infective endocarditis, the patient was placed on broad-spectrum IV antibiotics. Intra-operative blood cultures were negative. A tissue culture from the aortic valve vegetations identified Enterococcus hirae susceptible to ampicillin through MALDI-TOF. Antibiotic treatment was then switched to IV ampicillin and ceftriaxone; she declined aminoglycoside treatment due to toxicity concerns. The patient had an uncomplicated postoperative course and was discharged with 6 weeks of antibiotics. To date, she continues to be followed with no signs of relapsing disease.

Conclusions
To our knowledge, this case constitutes the fifth known case of E. hirae endocarditis, and the second case to have been identified with MALDI-TOF and treated with ampicillin and ceftriaxone. This case reinforces the efficacy of ampicillin and ceftriaxone for the treatment of E. hirae endocarditis.
Homozygous variants in the HEXB and MBOAT7 genes underlie neurological diseases in consanguineous families
Authors: Khan Shazia , Rawlins Lettie E. , Harlalka Gaurav V. , Umair Muhammad , Ullah Asmat , Shahzad Shaheen , Javed Muhammad , Baple Emma L. , Crosby Andrew H. , Ahmad Wasim , Gul Asma 
Published: December 2019

Abstract
Background
Neurological disorders are a common cause of morbidity and mortality within Pakistani populations. It is one of the most important challenges in healthcare, with significant life-long socio-economic burden.

Methods
We investigated the cause of disease in three Pakistani families in individuals with unexplained autosomal recessive neurological conditions, using both genome-wide SNP mapping and whole exome sequencing (WES) of affected individuals.

Results
We identified a homozygous splice site variant (NM_000521:c.445 + 1G > T) in the hexosaminidase B (HEXB) gene confirming a diagnosis of Sandhoff disease (SD; type II GM2-gangliosidosis), an autosomal recessive lysosomal storage disorder caused by deficiency of hexosaminidases in a single family. In two further unrelated families, we identified a homozygous frameshift variant (NM_024298.3:c.758_778del; p.Glu253_Ala259del) in membrane-bound O-acyltransferase family member 7 (MBOAT7) as the likely cause of disease. MBOAT7 gene variants have recently been identified as a cause of intellectual disability (ID), seizures and autistic features.

Conclusions
We identified two metabolic disorders of lipid biosynthesis within three Pakistani families presenting with undiagnosed neurodevelopmental conditions. These findings enabled an accurate neurological disease diagnosis to be provided for these families, facilitating disease management and genetic counselling within this population. This study consolidates variation within MBOAT7 as a cause of neurodevelopmental disorder, broadens knowledge of the clinical outcomes associated with MBOAT7-related disorder, and confirms the likely presence of a regionally prevalent founder variant (c.758_778del; p.Glu253_Ala259del) in Pakistan.
Broadening the phenotype of the TWNK gene associated Perrault syndrome
Authors: Fekete Bálint , Pentelényi Klára , Rudas Gabor , Gál Anikó , Grosz Zoltán , Illés Anett , Idris Jimoh , Csukly Gabor , Domonkos Andor , Molnar Maria Judit 
Published: December 2019

Abstract
Background
Perrault syndrome is a genetically heterogenous, very rare disease, characterized clinically by sensorineural hearing loss, ovarian dysfunction and neurological symptoms. We present the case of a 33 years old female patient with TWNK-associated Perrault syndrome. The TWNK gene is coding the mitochondrial protein Twinkle and currently there are only two reports characterizing the phenotype of TWNK-associated Perrault syndrome. None of these publications reported about special brain MRI alterations and neuropathological changes in the muscle and peripheral nerves.

Case presentation
Our patients with TWNK-dependent Perrault syndrome had severe bilateral hypoacusis, severe ataxia, polyneuropathy, lower limb spastic paraparesis with pyramidal signs, and gonadal dysgenesis. Psychiatric symptoms such as depression and paranoia were present as well. Brain MRI observed progressive cerebellar hyperintensive signs associated with cerebellar, medulla oblongata and cervical spinal cord atrophy. Light microscopy of the muscle biopsy detected severe neurogenic lesions. COX staining was centrally reduced in many muscle fibers. Both muscle and sural nerve electron microscopy detected slightly enlarged mitochondria with abnormal cristae surrounded by lipid vacuoles. In the sural nerve, dystrophic axons had focally uncompacted myelin lamellae present. Genetic investigation revealed multiple mtDNA deletion and compound heterozygous mutations of the TWNK gene (c.1196 A > G, c.1358 G > A).

Conclusion
This study demonstrates that TWNK associated Perrault syndrome has a much broader phenotype as originally published. The coexistence of severe hypoacusis, spastic limb weakness, ataxia, polyneuropathy, gonadal dysgensia, hyperintense signals in the cerebellum and the presence of the mtDNA multiple deletion could indicate the impairment of the TWNK gene. This is the first report about pyramidal tract involvement and cerebellar MRI alteration associated with TWNK-related Perrault syndrome.
Perinephric urinoma following spontaneous renal rupture in the third trimester of pregnancy: a case report and brief review of the literature
Authors: Chen Ya , Yan Yun Fang , Zhang Ying , Carroll Xianming , Li Hui Rong , Tao Li , Sun Mei Guo , Leeper-Woodford Sandra 
Published: December 2019

Abstract
Background
Spontaneous formation of urinoma is a rare condition, especially for pregnant women. We report a patient in the third trimester of pregnancy with a spontaneous renal rupture who then develops a urinoma from urine leaking into the perinephric space.

Case presentation
A 23-year-old primagravida was diagnosed with a spontaneous renal rupture and acute left loin pain accompanied by hematuria when she was 35 weeks pregnant. A sub-capsular perinephric cyst then developed to a size of 319 × 175 × 253 mm, and because of discomfort to the patient, we performed Cesarean section. After a healthy male newborn was delivered, fluid was suctioned from a large perirenal cyst that had an estimated size of 300 × 200 × 300 mm. A percutaneous nephrostomy tube was left in the cyst until CT showed no remaining fluid. In the six-month follow-up, the patient showed no perirenal extravasation according to an ultrasound scan, and the urine analysis and renal function tests were normal.

Conclusion
Close follow-up should be recommended for the patient who has renal rupture after conservative therapy, especially for pregnant woman. CT or MRI should be considered in addition to utilizing ultrasound in the management of pregnant women who present with urinomas. Percutaneous nephrostomy is suggested as an appropriate treatment for large urinomas.
A scoping review of the literature on the current mental health status of physicians and physicians-in-training in North America
Authors: Mihailescu Mara , Neiterman Elena 
Published: December 2019

AbstractBackgroundThis scoping review summarizes the existing literature regarding the mental health of physicians and physicians-in-training and explores what types of mental health concerns are discussed in the literature, what is their prevalence among physicians, what are the causes of mental health concerns in physicians, what effects mental health concerns have on physicians and their patients, what interventions can be used to address them, and what are the barriers to seeking and providing care for physicians. This review aims to improve the understanding of physicians’ mental health, identify gaps in research, and propose evidence-based solutions.MethodsA scoping review of the literature was conducted using Arksey and O’Malley’s framework, which examined peer-reviewed articles published in English during 2008–2018 with a focus on North America. Data were summarized quantitatively and thematically.ResultsA total of 91 articles meeting eligibility criteria were reviewed. Most of the literature was specific to burnout (n = 69), followed by depression and suicidal ideation (n = 28), psychological harm and distress (n = 9), wellbeing and wellness (n = 8), and general mental health (n = 3). The literature had a strong focus on interventions, but had less to say about barriers for seeking help and the effects of mental health concerns among physicians on patient care.ConclusionsMore research is needed to examine a broader variety of mental health concerns in physicians and to explore barriers to seeking care. The implication of poor physician mental health on patients should also be examined more closely. Finally, the reviewed literature lacks intersectional and longitudinal studies, as well as evaluations of interventions offered to improve mental wellbeing of physicians.
Campbelltown – Changing our Future: study protocol for a whole of system approach to childhood obesity in South Western Sydney
Authors: Maitland Nicola , Williams Mandy , Jalaludin Bin , Allender Steven , Strugnell Claudia , Brown Andrew , Hayward Joshua , Crooks Nicholas , Tredoux Jaimie , Li Vincy , Wardle Karen 
Published: December 2019

Abstract
Background
In Australia, around 67% of adults and 25% of children (5–17 years) are currently overweight or obese (Australian Bureau of Statistics, 4364.0.55.001 - National Health Survey: First Results, 2017–18, 2018). The Campbelltown – Changing our Future study will translate ‘a whole of system’ approach, previously trialed in rural communities in Victoria and the Australian Capital Territoty, to Campbelltown Local Government Area (LGA), a socioeconomically and ethnically diverse urban community in south western Sydney, NSW.

Methods
The study intervention will use a five-step approach; 1 – set up a childhood obesity monitoring system by collecting baseline data from children in primary schools across Campbelltown LGA to give a local context to the community when developing the systems map; 2 - key stakeholders develop systems maps which inform the development of the interventions; 3 - key stakeholders and community groups identify priority areas for action and form working groups; 4 - implementation of the interventions; 5 - evaluation of the interventions. The study will adopt a longitudinal pre/post design with repeated measures at baseline, 2 years and 4 years. Both qualitative and quantitative methods will be used to collect and analyse the data.

Discussion
Addressing childhood overweight and obesity is complex and requires a multifaceted intervention. This approach has the capacity to impact a range of factors that influence childhood overweight and obesity utilising existing capacity of multiple partners with broad community reach. Findings will develop local responses which capture the complexity of obesity at a community level and further our understanding of the interrelationships and relative importance of local factors impacting childhood overweight and obesity. This study aims to provide evidence for systems methods and approaches suitable for adaption and scaling and may provide evidence of successful community intervention elements.
Efficacy of glucocorticoids for the treatment of macrolide refractory mycoplasma pneumonia in children: meta-analysis of randomized controlled trials
Authors: Kim Hwan Soo , Sol In Suk , Li Donghe , Choi Miyoung , Choi Yun Jung , Lee Kyung Suk , Seo Ju Hee , Lee Yong Ju , Yang Hyeon-Jong , Kim Hyun Hee 
Published: December 2019

Abstract
Background
Mycoplasma pneumoniae is one of the most common pathogens causing community acquired pneumonia in children. Although the rate of macrolide-refractory Mycoplasma pneumoniae (MRMP) has increased, systemic glucocorticoids as a treatment option has not been validated yet. The purpose of this study was to assess the efficacy of glucocorticoids add-on in the treatment of MRMP in children through systematic review and meta-analysis.

Methods
Data sources
A systematic literature search was conducted using ten electronic bibliographic databases including English, Korean, Chinese and Japanese languages, up to March 8, 2018.
Study selection
The study was conducted according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses checklist and selected randomized control trials which compared the efficacy of glucocorticoids add-on to macrolide in the treatment of MRMP in children.
Data extraction
Two independent reviewers extracted: primary outcomes as hospital days, fever duration, and change in C-reactive protein (CRP) and main analysis was performed through meta-analysis with random effects model.

Results
Twenty-four unique randomized controlled trials met the inclusion criteria. The mean length of hospital stay in glucocorticoids treatment group was significantly shorter than that in conventional macrolide-treatment group (Weighted mean difference (WMD) = − 4.03 days). The mean length of fever duration was significantly shorter in the glucocorticoid treatment group in comparison with the conventional treatment group (WMD = -3.32 days). Level of CRP after treatment was significantly lower in the glucocorticoid treatment group than that in the conventional treatment group (WMD = -16.03). Sensitivity analysis and subgroup analysis showed no significant improvement in heterogeneity. As limitations of the study, most of the studies included were from a single country and we were unable to control for heterogeneity across interventions, lack of standardized measures, and different time points of assessments across studies.

Conclusions
Glucocorticoid add-on treatment for MRMP can significantly shorten the duration of fever and hospital stay and decrease the level of CRP. These results should be confirmed by adequately powered studies in the future.
Biochemical and nutritional characterization of the medfly gut symbiont Enterobacter sp. AA26 for its use as probiotics in sterile insect technique applications
Authors: Azis Konstantinos , Zerva Ioanna , Melidis Paraschos , Caceres Carlos , Bourtzis Kostas , Ntougias Spyridon 
Published: December 2019

Abstract
Background
Enterobacter sp. AA26 was recently isolated from the midgut of Ceratitis capitata (Wiedemann) and it was shown to have positive effects in rearing efficiency when used as larval probiotics. In this study, biomass production was carried out in bench-scale bioreactors to elucidate the biokinetic properties of Enterobacter sp. AA26 and its nutritional value.

Results
Strain AA26 is a psychrotolerant, halotolerant, facultatively anaerobic bacterium with broad pH range for growth (pH 4 to 10.2), which possessed the typical biochemical profile of Enterobacter spp. The specific oxygen uptake rate (SOUR) was calculated as 63.2 ± 1.26 and 121 ± 1.73 mg O2 g− 1 VSS h− 1, with the yield coefficients in acetate and glucose being equal to 0.62 ± 0.03 and 0.67 ± 0.003 g biomass produced/g substrate consumed, respectively. The maximum specific growth rate (μmax) of strain AA26 grown in fill-and-draw bioreactors at 20 °C and 35 °C was 0.035 and 0.069 h− 1, respectively. Strain AA26 grew effectively in agro-industrial wastewaters, i.e. cheese whey wastewater (CWW), as alternative substrate for replacing yeast-based media. Biomass of strain AA26 could provide all the essential amino acids and vitamins for the artificial rearing of C. capitata. Greater intracellular α- and β-glucosidase activities were observed during growth of strain AA26 in CWW than in yeast-based substrate, although the opposite pattern was observed for the respective extracellular activities (p < 0.01). Low protease activity was exhibited in cells grown in yeast-based medium, while no lipase activities were detected.

Conclusions
The ability of strain AA26 to grow in agro-industrial wastes and to provide all the essential nutrients can minimize the cost of commercial media used for mass rearing and large scale sterile insect technique applications.
“How do we use the time?” – an observational study measuring the task time distribution of nurses in psychiatric care
Authors: Glantz Andreas , Örmon Karin , Sandström Boel 
Published: December 2019

Abstract
Background
The nurse’s primary task in psychiatric care should be to plan for the patient’s care in cooperation with the patient and spend the time needed to build a relationship. Psychiatric care nurses however claim that they lack the necessary time to communicate with patients. To investigate the validity of such claims, this time-motion study aimed at identifying how nurses working at inpatient psychiatric wards distribute their time between a variety of tasks during a working day.

Methods
During the period of December 2015 and February 2016, a total of 129 h and 23 min of structured observations of 12 nurses were carried out at six inpatient wards at one psychiatric clinic in southern Sweden. Time, frequency of tasks and number of interruptions were recorded and analysed using descriptive statistics.

Results
Administering drugs or medications accounted for the largest part of the measured time (17.5%) followed by indirect care (16%). Relatively little time was spent on direct care, the third largest category in the study (15.3%), while an unexpectedly high proportion of time (11.3%) was spent on ward related tasks. Nurses were also interrupted in 75% of all medication administering tasks.

Conclusions
Nurses working in inpatient psychiatric care spend little time in direct contact with the patients and medication administration is interrupted very often. As a result, it is difficult to establish therapeutic relationships with patients. This is an area of concern for both patient safety and nurses’ job satisfaction.
Correction to: Potential cost-savings from the use of the biosimilars filgrastim, infliximab and insulin glargine in Canada: a retrospective analysis
Authors: Mansell Kerry , Bhimji Hishaam , Eurich Dean , Mansell Holly 
Published: December 2019

In the original publication of this article [1], there is a mistake in the Fig. 2a, 2b, and 2c.
Maternal milk and fecal microbes guide the spatiotemporal development of mucosa-associated microbiota and barrier function in the porcine neonatal gut
Authors: Liu Hongbin , Zeng Xiangfang , Zhang Guolong , Hou Chengli , Li Ning , Yu Haitao , Shang Lijun , Zhang Xiaoya , Trevisi Paolo , Yang Feiyun , Liu Zuohua , Qiao Shiyan 
Published: December 2019

Abstract
Background
The early-life microbiota exerts a profound and lifelong impact on host health. Longitudinal studies in humans have been informative but are mostly based on the analysis of fecal samples and cannot shed direct light on the early development of mucosa-associated intestinal microbiota and its impact on GI function. Using piglets as a model for human infants, we assess here the succession of mucosa-associated microbiota across the intestinal tract in the first 35 days after birth.

Results
Although sharing a similar composition and predicted functional profile at birth, the mucosa-associated microbiome in the small intestine (jejunum and ileum) remained relatively stable, while that of the large intestine (cecum and colon) quickly expanded and diversified by day 35. Among detected microbial sources (milk, vagina, areolar skin, and feces of sows, farrowing crate, and incubator), maternal milk microbes were primarily responsible for the colonization of the small intestine, contributing approximately 90% bacteria throughout the first 35 days of the neonatal life. Although maternal milk microbes contributed greater than 90% bacteria to the large intestinal microbiota of neonates upon birth, their presence gradually diminished, and they were replaced by maternal fecal microbes by day 35. We found strong correlations between the relative abundance of specific mucosa-associated microbes, particularly those vertically transmitted from the mother, and the expression levels of multiple intestinal immune and barrier function genes in different segments of the intestinal tract.

Conclusion
We revealed spatially specific trajectories of microbial colonization of the intestinal mucosa in the small and large intestines, which can be primarily attributed to the colonization by vertically transmitted maternal milk and intestinal microbes. Additionally, these maternal microbes may be involved in the establishment of intestinal immune and barrier functions in neonates. Our findings strengthen the notion that studying fecal samples alone is insufficient to fully understand the co-development of the intestinal microbiota and immune system and suggest the possibility of improving neonatal health through the manipulation of maternal microbiota.