bluuebunny/crossref_metadata_2025_split huggingface dataset:
Pre natal evaluation of heterotaxy syndrome by fetal echocardiography and correlating with autopsy
Authors: Routhu Madhavilatha , Mohammad Imran Ali 
Published: March 2019

Introduction Heterotaxy syndrome/ isomerism is characterized by an abnormal symmetry of the viscera that are normally dissimilar due to abnormal lateralization of thoracic and abdominal viscera and is frequently associated with complex cardiac anomalies. Isomerism may be of right or left. Materials and methods This article describes the morphological characteristics of heterotaxy and suggests an approach in evaluating the spectrum of abnormalities associated with this syndrome. This study is based on 12 cases diagnosed on antenatal ultrasound as heterotaxy syndrome. Results of the examinations were re-evaluated and compared by fetal autopsy. Result Based on the following echocardiographic criteria, a diagnosis of left isomerism was made if there was viscerocardiac heterotaxy associated with an interruption of inferior vena cava or with bilateral finger-like atrial appendages or if it was associated with heart block. If there was evidence of viscerocardiac heterotaxy with complex cardiac anomalies then it was diagnosed as right atrial isomerism or visceral heterotaxy syndrome. We diagnosed 6/12 as left isomerism and rest of the cases as right isomerism/visceral heterotaxy syndrome. In Autopsy we evaluated visceral situs and morphology of the lungs and the main bronchi, the state of the liver, spleen, bowel, and the precise anatomy of the heart and confirmed 4/12 as left isomerism 4/12 as right isomerism and two cases as visceral heterotaxy syndrome (VHS). Rest of the two cases were included in the study despite missing autopsy data, as the combination of abnormal situs with interrupted inferior vena cava, and cardiac malformation allowed a diagnosis of left isomerism with high probability. Conclusion In this study, we aimed to find common features of heterotaxy syndrome on prenatal ultrasound as well as on fetal autopsy. This syndrome should be accurately diagnosed in the prenatal period in order to allow appropriate counseling of parents.
Anorectal biofeedback: an effective therapy, but can we shorten the course to improve access to treatment?
Authors: Mazor Yoav , Kellow John E. , Prott Gillian M. , Jones Michael P. , Malcolm Allison 
Published: March 2019

Background: Instrumented anorectal biofeedback (BF) improves symptoms and quality of life in patients with faecal incontinence and defecation disorder-associated chronic constipation. However, demand for BF greatly outweighs availability, so refinement of the BF protocol, in terms of the time and resources required, is of importance. Our aim was to evaluate the outcomes of an abbreviated BF protocol in patients with defecation disorder-associated chronic constipation and/or faecal incontinence compared to standard BF. Methods: Data were collected from consecutive patients ( n = 31; age 54 ± 15; 29 females; 61% functional constipation) undergoing an intentionally abbreviated BF protocol, and compared in a 1:2 ratio with 62 age, gender and functional anorectal disorder-matched control patients undergoing a standard BF. Outcomes included change in symptoms, physiology, patient satisfaction and quality of life. Results: On intention to treat, patients in both protocols showed significant improvement in symptom scores and the magnitude did not differ between groups. Impact on quality of life, satisfaction and control over bowel movements improved in both protocols, but satisfaction improved to a greater extent in the standard BF protocol ( p = 0.009). Physiological parameters were unchanged after BF apart from improvement in rectal sensation in the standard BF group compared to abbreviated BF ( p ⩽ 0.002). Conclusions: Abbreviated anorectal BF offered to patients travelling from far away was not different to a standard BF in providing substantial, at least short term, improvements in symptoms of constipation and faecal incontinence, quality of life and feeling of control over bowel movements. Refinement of the standard BF protocol according to individual patient phenotypes and desired outcomes warrants further study in order to maximize efficacy and improve access for patients.
Oligoclonal bands increase the specificity of MRI criteria to predict multiple sclerosis in children with radiologically isolated syndrome
Authors: Makhani Naila , Lebrun Christine , Siva Aksel , Narula Sona , Wassmer Evangeline , Brassat David , Brenton J Nicholas , Cabre Philippe , Carra Dallière Clarisse , de Seze Jérôme , Durand Dubief Francoise , Inglese Matilde , Langille Megan , Mathey Guillaume , Neuteboom Rinze F , Pelletier Jean , Pohl Daniela , Reich Daniel S , Ignacio Rojas Juan , Shabanova Veronika , Shapiro Eugene D , Stone Robert T , Tenembaum Silvia , Tintoré Mar , Uygunoglu Ugur , Vargas Wendy , Venkateswaren Sunita , Vermersch Patrick , Kantarci Orhun , Okuda Darin T , Pelletier Daniel ,   on behalf of Observatoire Francophone de la Sclérose en Plaques (OFSEP), Société Francophone de la Sclérose en Plaques (SFSEP), the Radiologically Isolated Syndrome Consortium (RISC) and the Pediatric Radiologically Isolated Syndrome Consortium (PARIS)
Published: March 2019

Background Steps towards the development of diagnostic criteria are needed for children with the radiologically isolated syndrome to identify children at risk of clinical demyelination. Objectives To evaluate the 2005 and 2016 MAGNIMS magnetic resonance imaging criteria for dissemination in space for multiple sclerosis, both alone and with oligoclonal bands in cerebrospinal fluid added, as predictors of a first clinical event consistent with central nervous system demyelination in children with radiologically isolated syndrome. Methods We analysed an international historical cohort of 61 children with radiologically isolated syndrome (≤18 years), defined using the 2010 magnetic resonance imaging dissemination in space criteria (Ped-RIS) who were followed longitudinally (mean 4.2 ± 4.7 years). All index scans also met the 2017 magnetic resonance imaging dissemination in space criteria. Results Diagnostic indices (95% confidence intervals) for the 2005 dissemination in space criteria, with and without oligoclonal bands, were: sensitivity 66.7% (38.4–88.2%) versus 72.7% (49.8–89.3%); specificity 83.3% (58.6–96.4%) versus 53.9% (37.2–69.9%). For the 2016 MAGNIMS dissemination in space criteria diagnostic indices were: sensitivity 76.5% (50.1–93.2%) versus 100% (84.6–100%); specificity 72.7% (49.8–89.3%) versus 25.6% (13.0–42.1%). Conclusions Oligoclonal bands increased the specificity of magnetic resonance imaging criteria in children with Ped-RIS. Clinicians should consider testing cerebrospinal fluid to improve diagnostic certainty. There is rationale to include cerebrospinal fluid analysis for biomarkers including oligoclonal bands in planned prospective studies to develop optimal diagnostic criteria for radiologically isolated syndrome in children.
Historical lead seals and the influence of disinfectants on the lead corrosion rate
Authors: Msallamova Sarka , Kouril Milan , Strachotova Kristyna Charllote , Stoulil Jan , Popova Kateryna , Dvorakova Pavla 
Published: March 2019

Abstract
Lead used to be a common material for setting seal to historical documents. Lead seals formed parts of historical documents as a guarantee of their legal validity. Disinfectants are commonly used during the restoration of historical documents. They successfully remove mould, bacteria and microscopic fungi from the surface of parchment documents. However, some disinfectants could also be a source of corrosion damage to lead seals. This work was carried out to examine possible corrosion damage to lead seals caused by disinfectants [a solution of carbethopendecinium bromide, a solution of glutaraldehyde, butanol (vapours), a solution of 1-propanol, 2-propanol + ethanol called Bacillol AF], which are commonly used during restoration of historical documents. The lead corrosion rates were determined by using lead resistometric probes. The solutions of carbethopendecinium bromide and glutaraldehyde increase the lead corrosion rate and corrosion products based on carbonates, nitrates and organic lead salts are formed on the lead surface. The most appropriate disinfectants for the restoration of historical documents with lead seals are alcohol-based solutions, such as butanol (vapours) and a solution of 1-propanol, 2-propanol and ethanol called Bacillol AF.
Comparison of Population-Based Observational Studies With Randomized Trials in Oncology
Authors: Soni Payal D. , Hartman Holly E. , Dess Robert T. , Abugharib Ahmed , Allen Steven G. , Feng Felix Y. , Zietman Anthony L. , Jagsi Reshma , Schipper Matthew J. , Spratt Daniel E. 
Published: March 2019

PURPOSE Comparative efficacy research performed using population registries can be subject to significant bias. There is an absence of objective data demonstrating factors that can sufficiently reduce bias and provide accurate results. METHODS MEDLINE was searched from January 2000 to October 2016 for observational studies comparing two treatment regimens for any diagnosis of cancer, using SEER, SEER-Medicare, or the National Cancer Database. Reporting quality and statistical methods were assessed using components of the STROBE criteria. Randomized trials comparing the same treatment regimens were identified. Primary outcome was correlation between survival hazard ratio (HR) estimates provided by the observational studies and randomized trials. Secondary outcomes included agreement between matched pairs and predictors of agreement. RESULTS Of 3,657 studies reviewed, 350 treatment comparisons met eligibility criteria and were matched to 121 randomized trials. There was no significant correlation between the HR estimates reported by observational studies and randomized trials (concordance correlation coefficient, 0.083; 95% CI, −0.068 to 0.230). Forty percent of matched studies were in agreement regarding treatment effects (κ, 0.037; 95% CI, −0.027 to 0.1), and 62% of the observational study HRs fell within the 95% CIs of the randomized trials. Cancer type, data source, reporting quality, adjustment for age, stage, or comorbidities, use of propensity weighting, instrumental variable or sensitivity analysis, and well-matched study population did not predict agreement. CONCLUSION We were unable to identify any modifiable factor present in population-based observational studies that improved agreement with randomized trials. There was no agreement beyond what is expected by chance, regardless of reporting quality or statistical rigor of the observational study. Future work is needed to identify reliable methods for conducting population-based comparative efficacy research.
Defective Regulatory B Cells Are Associated With Thyroid-Associated Ophthalmopathy
Authors: Chen Guo , Ding Yungang , Li Qian , Li Yanbing , Wen Xiaofeng , Ji Xian , Bi Shaowei , Chen Jingqiao , Xu Jianan , Chen Rongxin , Ye Huijing , Wei Lai , Yang Huasheng 
Published: March 2019

AbstractPurposeTo investigate the change in IL-10–producing regulatory B cells (Breg), which suppress peripheral immune responses, in patients with thyroid-associated ophthalmopathy (TAO).MethodsPeripheral blood mononuclear cells (PBMCs) were isolated from healthy controls (n = 54), patients with Graves disease (n = 26), and patients with TAO (N=125), and stimulated with CpG/CD40L. The frequency of IL-10–producing Bregs and the expression of IL-10 in response to TSH stimulation were measured by flow cytometry. CD4+ T cells were cultured with Breg-depleted PBMCs to elucidate the function of Bregs in patients with TAO. The potential immunoregulatory mechanism was also investigated by Western blot and chromatin immunoprecipitation assays.ResultsPatients with active TAO had higher baseline levels of Bregs in their peripheral blood than both healthy controls and inactive patients. TSH promoted Bregs. Bregs from patients with TAO were defective in suppressing the activation of interferon (IFN)-γ+ and IL-17+ T cells in vitro.ConclusionsRegulatory B cells in patients with TAO are functionally defective, suggesting that the defective Bregs might be responsible for the pathogenesis of TAO.
Novel GC-MS/MS Technique Reveals a Complex Steroid Fingerprint of Subclinical Hypercortisolism in Adrenal Incidentalomas
Authors: Hána Václav , Ježková Jana , Kosák Mikuláš , Kršek Michal , Hána Václav , Hill Martin 
Published: March 2019

AbstractContextImprovement of imaging methods has led to more incidental adrenal tumor findings, especially adenomas. Routine hormonal evaluation uses only a few steroids to evaluate possible hormonal hypersecretion of these adenomas, but a wide spectrum of serum steroid hormone changes has not been published.ObjectiveTo measure the serum levels of 83 steroids from patients with unilateral and bilateral adrenal incidentalomas to uncover full steroid profile changes in patients with subclinical hypercortisolism (SH).DesignCross-sectional study.SettingThe study was conducted at a tertiary inpatient clinic.PatientsFifty-two patients with adrenal incidentalomas (unilateral, n = 29; bilateral, n = 23), including nonfunctioning (n = 11) vs SH (n = 41), and 26 age- and sex-matched controls from the general population were included.Main Outcome MeasuresEighty-three serum steroids were measured by gas chromatography–tandem mass spectrometry (GC-MS/MS) before and after 1 mg dexamethasone, ACTH, midnight serum cortisol, and urinary free cortisol/24 hour.ResultsOf 83 measured steroids, 10 were significantly decreased in patients with SH, including dehydroepiandrosterone sulfate (DHEAS), androsterone sulfate, epiandrosterone sulfate, androstenediol sulfate, conjugated 5α-androstane-3β,17β-diol, and conjugated 5α-androstane-3α,17β-diol. This finding was observed even when unilateral, bilateral, male, and female subgroups were analyzed separately. When we compared routine clinical methods and GC-MS/MS‒measured steroids, the most discriminatory was DHEAS followed by midnight serum cortisol, epiandrosterone sulfate, androsterone sulfate, ACTH, and 16α-hydroxypregnenolone.ConclusionsSH was associated with decreased levels of adrenal androgens, their metabolites, and pregnenolone metabolite. GC-MS/MS is a powerful tool for measuring serum levels of these undescribed changes in steroid metabolism, which are characteristic of SH in adrenal incidentalomas.
Glycated Hemoglobin and All-Cause and Cause-Specific Mortality Among Adults With and Without Diabetes
Authors: Li Fu-Rong , Zhang Xi-Ru , Zhong Wen-Fang , Li Zhi-Hao , Gao Xiang , Kraus Virginia Byers , Lv Yue-Bin , Zou Meng-Chen , Chen Guo-Chong , Chen Pei-Liang , Zhang Min-Yi , Kur Akech Kuol Akech , Shi Xiao-Ming , Wu Xian-Bo , Mao Chen 
Published: March 2019

Abstract

Context
The patterns of associations between glycated Hb (HbA1c) and mortality are still unclear.

Objective
To explore the extent to which ranges of HbA1c levels are associated with the risk of mortality among participants with and without diabetes.

Design, Setting, and Patients
This was a nationwide, community-based prospective cohort study. Included were 15,869 participants (median age 64 years) of the Health and Retirement Study, with available HbA1c data and without a history of cancer. Cox proportional hazards regression models were used to estimate hazard ratios with 95% CIs for mortality.

Results
A total of 2133 participants died during a median follow-up of 5.8 years. In participants with diabetes, those with an HbA1c level of 6.5% were at the lowest risk of all-cause mortality. When HbA1c level was &lt;5.6% or &gt;7.4%, the increased all-cause mortality risk became statistically significant as compared with an HbA1c level of 6.5%. As for participants without diabetes, those with an HbA1c level of 5.4% were at the lowest risk of all-cause mortality. When the HbA1c level was &lt;5.0%, the increased all-cause mortality risk became statistically significant as compared with an HbA1c level of 5.4%. However, we did not observe a statistically significant elevated risk of all-cause mortality above an HbA1c level of 5.4%.

Conclusions
A U-shaped and reverse J-shaped association for all-cause mortality was found among participants with and without diabetes. The corresponding optimal ranges for overall survival are predicted to be 5.6% and 7.4% and 5.0% and 6.5%, respectively.
Synthesis of Pyrrole-Containing Chiral Spiro Molecules and Their Optical and Chiroptical Properties
Authors: Takase Ko , Noguchi Keiichi , Nakano Koji 
Published: March 2019

Abstract
The pyrrole-containing chiral spiro π-conjugated compounds were successfully synthesized from 10,10′-spirobi[indeno[1,2-b][1]benzothiophene] 5,5-dioxide and 5,5,5′,5′-tetraoxide via a sequential inter/intramolecular nucleophilic aromatic substitution with arylamines. The enantiopure ones were also prepared from the enantiopure substrates. UV-vis absorption and photoluminescence spectroscopies and theoretical calculations revealed that the replacement of thiophene unit(s) of 10,10′-spirobi[indeno[1,2-b][1]benzothiophene] with pyrrole unit(s) has a great impact on photophysical properties. The pyrrole-containing chiral spiro π-conjugated compounds exhibited circularly polarized luminescence with a relatively large dissymmetry factor.
Using pens as an incentive for trial recruitment of older adults: An embedded randomised controlled trial
Authors: Whiteside Katie , Flett Lydia , Mitchell Alex S. , Fairhurst Caroline , Cockayne Sarah , Rodgers Sara , Torgerson David J. ,   OTIS Study Group
Published: March 2019

Background: Meeting recruitment targets for randomised controlled trials is challenging. This trial evaluated the effectiveness of including a pen within the trial invitation pack on the recruitment of older adults into a randomised controlled trial. Methods: This trial was embedded within the Occupational Therapist Intervention Study, a falls-prevention randomised controlled trial. Potential participants (n = 1862), who were posted an invitation pack from two General Practitioner practices, were randomised to either not receive a pen (n = 1295) or receive a pen (n = 648) with their invitation pack, using a 2:1 ratio. The primary outcome was the likelihood of being randomised, and therefore fully recruited, to the host trial. To be randomised to the host trial, participants had to: return a consent form and screening form; be eligible on their screening form; and return a baseline questionnaire and a monthly falls calendar. Secondary outcomes were: the likelihood of returning (and time to return) a screening form; being eligible for the host trial; and remaining in the trial for at least 3 months. Results: The likelihood of being randomised to the host trial did not differ between the pen group (4.5%) and no pen group (4.3%; odds ratio 1.04; 95% confidence interval: 0.65 to 1.67; p = 0.86). There were marginal differences in secondary outcomes in favour of the pen group, particularly in screening form return rates, though these differences were not statistically significant. Conclusion: Pens may not be an effective incentive for the recruitment of older adults into randomised controlled trials, though future trials are required. Registration: ISRCTN22202133; SWAT 37.
The nurse-led model of hospice care
Authors: Hickish Dinah , Roberts Debbie 
Published: March 2019

Background: Palliative care in the UK has become more medicalised over the last 50 years, and it was recognised as a medical speciality in 2004. However, out of financial expediency, an inpatient hospice in North Wales has become nurse-led. Aims: This article considers the history of care of the dying and discusses how nurses are best placed to be masters of care of the dying. Methods: The model of nurse-led care is described in terms of how this innovation in practice has developed, allowing the advanced nurse practitioner role to be extended to an autonomous level of hospice care not practised anywhere else in the UK. Conclusion: The innovation of nurse-led hospice care has secured the future of the hospice, making it fiscally robust and ensuring a sustainable service for the community that it serves.
Impact of Caregiver Burden on Quality of Life for Parents of Adult Children With Autism Spectrum Disorder
Authors: Marsack-Topolewski Christina N. , Church Heather L. 
Published: March 2019

Abstract
This study sought to examine the impact of time, developmental, emotional, and financial burdens on the quality of life (QOL) for parents (aged 50+) of an adult child with autism spectrum disorder (ASD). Participants (N = 320) completed a web-based survey and could indicate interest in participating in one-on-one follow up interviews. Multiple linear regression analysis indicated that two variables, developmental burden and impact of caregiving on finances, were statistically significant predictors of parents' QOL. Developmental burden was the strongest predictor of parental QOL, demonstrating an inverse relationship. Qualitatively, reported findings indicated that many parents were experiencing all four burdens. Findings highlighted the need to provide services and supports to alleviate burden among aging parents of adult children with ASD.
Hubungan Profil Lipid dan Derajat Keparahan Penyakit berdasarkan Skor Pediatric Logistic Organ Dysfunction-2 pada Anak dengan Sepsis
Authors: Restiviona Rince , Yoel Chairul , Sianturi Pertin 
Published: March 2019

Latar belakang. Sepsis adalah penyebab kematian terbanyak pada bayi dan anak di rumah sakit. Sepsis dan syok septik menyebabkan perubahan neuroendokrin dan metabolik termasuk konsentrasi dan komposisi lipid plasma serta lipoprotein. Namun hubungan profil lipid dan derajat keparahan penyakit pada anak dengan sepsis masih belum jelas.Tujuan. Mengetahui cut-off profil lipid pada pasien anak dengan sepsis dan hubungan profil lipid terhadap derajat keparahan penyakit pada anak dengan sepsis.Metode. Penelitian potong-lintang bulan Juli sampai Oktober 2017 di PICU, RSUP Haji Adam Malik. Sampel adalah anak usia 1 bulan sampai <18 tahun yang didiagnosis sepsis. Pasien yang menderita diabetes melitus, sindrom nefrotik, gizi lebih, gizi buruk, mendapatkan terapi statin dan insulin diekslusikan. Didapatkan 30 orang anak yang memenuhi kriteria. Derajat keparahan penyakit dinilai berdasarkan skor PELOD-2. Nilai cut-off masing-masing profil lipid berdasarkan kurva ROC. Hubungan antara profil lipid dan derajat keparahan penyakit dianalisis dengan uji chi-square atau Fisher exact. Nilai p < 0,05 dianggap bermakna secara statistik .Hasil. Dari kurva ROC didapatkan nilai cut-off kadar kolesterol total dan trigliserida 93,5 mg/dL dan 199 mg/dL, dan nilai cut-off untuk kadar HDL dan LDL adalah 20,5 mg/dL dan 48,5 mg/dL. Didapatkan hubungan profil lipid dan derajat keparahan penyakit (PELOD-2) dengan nilai p: kolesterol total 0,007, trigliserida 0,005, HDL 0,063 dan LDL 0,279.Kesimpulan. Terdapat hubungan bermakna antara kadar kolesterol total dan trigliserida dengan derajat keparahan penyakit berdasarkan skor PELOD-2. Tidak terdapat hubungan bermakna antara HDL dan LDL dengan derajat keparahan penyakit berdasarkan skor PELOD-2.