bluuebunny/crossref_metadata_2025_split huggingface dataset:
Impact of drusen and drusenoid retinal pigment epithelium elevation size and structure on the integrity of the retinal pigment epithelium layer
Authors: Schlanitz Ferdinand , Baumann Bernhard , Sacu Stefan , Baumann Lukas , Pircher Michael , Hitzenberger Christoph K , Schmidt-Erfurth Ursula Margarethe 
Published: April 2018

PurposeTo evaluate the impact of drusen size and structure on retinal pigment epithelium (RPE) and photoreceptor layers in eyes with early to intermediate age-related macular degeneration (AMD) using polarisation-sensitive optical coherence tomography (OCT).DesignRetrospective investigation of an observational cross-sectional study.ParticipantsPatients with early to intermediate AMD.MethodsTwenty-five eyes of 25 patients with drusen were imaged with polarisation-sensitive OCT using macular volume scans. Each scan was manually graded for six distinct drusen characteristics and the integrity of both the overlying RPE and photoreceptor layer. The central scan of each single druse, as well as its diameter and location, were selected for statistical calculations.ResultsA total number of 5933 individual drusen including their adjacent RPE and photoreceptor layer were evaluated. 41.3% of all drusen demonstrated an intact overlying RPE; in 28.1% the RPE layer was irregular, but continuous. In 30.6%, the RPE layer signal was discontinuous above the area of drusen. The level of RPE alteration was significantly related to shape (p<0.001), internal reflectivity (p<0.001) and homogeneity (p<0.001) of the drusen and their diameter, with a higher probability for larger drusen to have a discontinuous RPE (OR 3.2, p<0.001). The number of drusen showing overlying foci or an altered photoreceptor layer was too small to be conclusive, but showed a trend towards an altered RPE if present.ConclusionsPolarisation-sensitive OCT reveals a correlation between specific drusen characteristics and the integrity of the overlying RPE layer. Drusen diameter and configuration were significantly associated with RPE loss.
Association between HLA-B*44:03-HLA-C*07:01 haplotype and cold medicine-related Stevens-Johnson syndrome with severe ocular complications in Thailand
Authors: Jongkhajornpong Passara , Lekhanont Kaevalin , Pisuchpen Phattrawan , Chantaren Patchima , Puangsricharern Vilavun , Prabhasawat Pinnita , Suphapeetiporn Kanya , Kinoshita Shigeru , Ueta Mayumi 
Published: April 2018

BackgroundPolymorphisms in human leucocyte antigen (HLA) class I genes have been found to be associated with cold medicine (CM)-related Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) with severe ocular complications (SOC). Because ethnic differences in genetic predisposition to SJS/TEN among different populations have been proposed, we focused on Thai patients and investigated the association between HLA class I genotypes and CM-SJS/TEN with SOC.MethodsThis multicentre case–control study was conducted between September 2014 and August 2017. Seventy-one Thai patients with SJS/TEN with SOC and 159 healthy Thai controls were enrolled. HLA typing was performed. Genetic relationships were analysed using Fisher’s exact test.ResultsOf 71 patients with SJS/TEN with SOC (28 male, 43 female), 49 (69%) had a history of taking cold medications prior to SJS/TEN onset. The mean age of onset was 26.7±17.1 years (range, 2–77 years). HLA-B*44:03 (OR, 7.2, p=5.5×10-6, pc=1.1×10-4) and HLA-C*07:01 (OR, 6.1, p=7.1×10-6, pc=1.1×10-4) showed significant positive associations with Thai patients with CM-SJS/TEN with SOC. Additionally, 17 of 49 patients with CM-SJS/TEN with SOC (34.7%) significantly harboured the HLA-B*44:03 and HLA-C*07:01 haplotype compared with only 11 of 159 healthy controls (6.9%) (OR=7.1, p=5.5×10-6).ConclusionsHLA-B*44:03-HLA-C*07:01 haplotype is a potential risk factor for CM-SJS/TEN with SOC in the Thai population. This study supports that HLA-B*44:03 might be a common marker for CM-SJS/TEN with SOC in Eurasia populations, including European, Indian, Japanese and Thai.
Quantifying vascular density and morphology using different swept-source optical coherence tomography angiographic scan patterns in diabetic retinopathy
Authors: Hirano Takao , Kitahara Jyunya , Toriyama Yuichi , Kasamatsu Hirotsugu , Murata Toshinori , Sadda Srinivas 
Published: April 2018

AimsTo evaluate quantitative metrics of the retinal microvasculature in eyes with diabetic retinopathy (DR) using various en face swept-source optical coherence tomography angiography (SS-OCTA) image sizes.MethodsNon-segmented and segmented images were acquired using an SS-OCTA device (PLEX Elite 9000; Carl Zeiss Meditec, Dublin, California, USA). The scanning protocols included the 3×3 mm, 6×6 mm and 12×12 mm fields of view. Quantitative analysis of the perfusion density (PD), vessel length density (VLD) and fractal dimension (FD) was performed. The area under the receiver operating characteristic curve was estimated to assess the ability of each image size to predict DR.ResultsThis prospective, cross-sectional study included 60 eyes, (non-DR, 13 eyes; non-proliferative DR (NPDR), 24 eyes; proliferative DR (PDR), 23 eyes) of 46 patients with diabetes and 21 eyes of 16 healthy individuals. In the 12×12 mm images, the PD of healthy individuals was significantly greater than that of patients with NPDR or PDR for all layers (NPDR, p<0.05; PDR, p<0.001 0.001; FD, p<0.001) or PDR (VLD, p<0.001; FD, p<0.001 for all layers). The results were similar for the 3×3 mm and 6×6 mm images. Overall, PD, VLD and FD progressively decreased with worsening DR severity in segmented and non-segmented layers for all SS-OCTA scan sizes. For detecting DR, 3×3 mm images best predicted DR for all evaluated quantitative parameters.ConclusionsVascular changes in DR can be monitored in detail through quantitative evaluations that combine different SS-OCTA scan sizes and parameters.
Variations in outcomes by residential location for women with breast cancer: a systematic review
Authors: Dasgupta Paramita , Baade Peter D , Youlden Danny R , Garvey Gail , Aitken Joanne F , Wallington Isabella , Chynoweth Jennifer , Zorbas Helen , Youl Philippa H 
Published: April 2018

ObjectivesTo systematically assess the evidence for variations in outcomes at each step along the breast cancer continuum of care for Australian women by residential location.DesignSystematic review.MethodsSystematic searches of peer-reviewed articles in English published from 1 January 1990 to 24 November 2017 using PubMed, EMBASE, CINAHL and Informit databases. Inclusion criteria were: population was adult female patients with breast cancer; Australian setting; outcome measure was survival, patient or tumour characteristics, screening rates or frequencies, clinical management, patterns of initial care or post-treatment follow-up with analysis by residential location or studies involving non-metropolitan women only. Included studies were critically appraised using a modified Newcastle–Ottawa Scale.ResultsSeventy-four quantitative studies met the inclusion criteria. Around 59% were considered high quality, 34% moderate and 7% low. No eligible studies examining treatment choices or post-treatment follow-up were identified. Non-metropolitan women consistently had poorer survival, with most of this differential being attributed to more advanced disease at diagnosis, treatment-related factors and socioeconomic disadvantage. Compared with metropolitan women, non-metropolitan women were more likely to live in disadvantaged areas and had differing clinical management and patterns of care. However, findings regarding geographical variations in tumour characteristics or diagnostic outcomes were inconsistent.ConclusionsA general pattern of poorer survival and variations in clinical management for Australian female patients with breast cancer from non-metropolitan areas was evident. However, the wide variability in data sources, measures, study quality, time periods and geographical classification made direct comparisons across studies challenging. The review highlighted the need to promote standardisation of geographical classifications and increased comparability of data systems. It also identified key gaps in the existing literature including a lack of studies on advanced breast cancer, geographical variations in treatment choices from the perspective of patients and post-treatment follow-up.
Patients’ perspectives on how to decrease the burden of treatment: a qualitative study of HIV care in sub-Saharan Africa
Authors: Tran Viet-Thi , Messou Eugene , Mama Djima Mariam , Ravaud Philippe , Ekouevi Didier K 
Published: April 2018

ObjectivePatients living with HIV infection (PLWH) in sub-Saharan Africa face an important burden of treatment related to everything they do to take care of their health: doctor visits, tests, regular refills, travels, and so on. In this study, we involved PLWH in proposing ideas on how to decrease their burden of treatment and assessed to what extent these propositions could be implemented in care.MethodsAdult PLWH recruited in three HIV care centres in Côte d’Ivoire participated in qualitative interviews starting with ‘What do you believe are the most important things to change in your care to improve your burden of treatment?’ Two independent investigators conducted a thematic analysis to identify and classify patients' propositions to decrease their burden of treatment. A group of experts involving patients, health professionals, hospital leaders and policymakers evaluated each patient proposition to assess its feasibility.ResultsBetween February and April 2017, 326 participants shared 748 ideas to decrease their burden of treatment. These ideas were grouped into 59 unique patient propositions to improve their personal care and the organisation of their hospital or clinic and/or the health system. Experts considered that 27 (46%), 19 (32%) and 13 (22%) of patients' propositions were easy, moderate and difficult, respectively, to implement. A total of 118 (36%) participants offered at least one proposition considered easily implementable by our experts.ConclusionAsking PLWH in sub-Saharan Africa about how their care could be improved led to identifying meaningful propositions. According to experts, half of the ideas identified could be implemented easily at low cost for minimally disruptive HIV care.
Faecal microbiota transplantation as a treatment for inflammatory bowel disease: a national survey of adult and paediatric gastroenterologists in the UK
Authors: Mcilroy James Roger , Nalagatla Niharika , Hansen Richard , Hart Ailsa , Hold Georgina Louise 
Published: April 2018

BackgroundInterest in the use of faecal microbiota transplantation (FMT) in inflammatory bowel disease (IBD) has increased following outcomes in patients withClostridioides difficileinfection (CDI). While research exploring clinician awareness and attitude towards the use of FMT in CDI has been carried out, data for IBD are currently lacking.ObjectiveTo assess the perceptions of gastroenterologists and current practice relating to FMT as a treatment for IBD in the UK.DesignA web-based survey (Snap Survey software) was distributed through the British Society of Gastroenterology (BSG) and British Society of Paediatric Gastroenterology, Hepatology and Nutrition e-newsletters, and at the BSG Conference in June 2017.Results61 respondents completed the survey including presubspecialty trainees, gastroenterology specialists, associate specialists and consultants. Most (95%; n=58) respondents stated that they had heard of FMT being used as a treatment for IBD prior to participating in the survey. Based on current evidence, 34% (n=21) of respondents would consider using FMT in patients with IBD, 26% (n=16) would not and 39% (n=24) were undecided. When asked to rank routes of delivery in terms of preference, nasogastric tube was the least preferred route (39%; n=24) and oral capsule was the most preferred route (34%; n=21).ConclusionsA clear majority of UK gastroenterologists recognise FMT as a potential treatment for IBD; however, uptake is limited. A proportion of clinicians would consider FMT in IBD and the majority would consider entering patients into clinical trials. Future work should explore the utility and efficacy of oral FMT capsules in IBD.
Primary repair versus surgical and transcatheter palliation in infants with tetralogy of Fallot
Authors: Dorobantu Dan M , Mahani Alireza S , Sharabiani Mansour T A , Pandey Ragini , Angelini Gianni D , Parry Andrew J , Tulloh Robert M R , Martin Robin P , Stoica Serban C 
Published: May 2018

ObjectivesTreatment of infants with tetralogy of Fallot (ToF) has evolved in the last two decades with increasing use of primary surgical repair (PrR) and transcatheter right ventricular outflow tract palliation (RVOTd), and fewer systemic-to-pulmonary shunts (SPS). We aim to report contemporary results using these treatment options in a comparative study.MethodsThis a retrospective study using data from the UK National Congenital Heart Disease Audit. All infants (n=1662, median age 181 days) with ToF and no other complex defects undergoing repair or palliation between 2000 and 2013 were considered. Matching algorithms were used to minimise confounding due to lower age and weight in those palliated.ResultsPatients underwent PrR (n=1244), SPS (n=311) or RVOTd (n=107). Mortality at 12 years was higher when repair or palliation was performed before the age of 60 days rather than after, most significantly for primary repair (18.7% vs 2.2%, P<0.001), less so for RVOTd (10.8% vs 0%, P=0.06) or SPS (12.4% vs 8.3%, P=0.2). In the matched groups of patients, RVOTd was associated with more right ventricular outflow tract (RVOT) reinterventions (HR=2.3, P=0.05 vs PrR, HR=7.2, P=0.001 vs SPS) and fewer pulmonary valve replacements (PVR) (HR=0.3 vs PrR, P=0.05) at 12 years, with lower mortality after complete repair (HR=0.2 versus PrR, P=0.09).ConclusionsWe found that RVOTd was associated with more RVOT reinterventions, fewer PVR and fewer deaths when compared with PrR in comparable, young infants, especially so in those under 60 days at the time of the first procedure.
Diagnostic potential of stored dried blood spots for inborn errors of metabolism: a metabolic autopsy of medium-chain acyl-CoA dehydrogenase deficiency
Authors: Kaku Noriyuki , Ihara Kenji , Hirata Yuichiro , Yamada Kenji , Lee Sooyoung , Kanemasa Hikaru , Motomura Yoshitomo , Baba Haruhisa , Tanaka Tamami , Sakai Yasunari , Maehara Yoshihiko , Ohga Shouichi 
Published: May 2018

AimIt is estimated that 1–5% of sudden infant death syndrome (SIDS) cases might be caused by undiagnosed inborn errors of metabolism (IEMs); however, the postmortem identification of IEMs remains difficult. This study aimed to evaluate the usefulness of dried blood spots (DBSs) stored after newborn screening tests as a metabolic autopsy to determine the causes of death in infants and children who died suddenly and unexpectedly.MethodsInfants or toddlers who had suddenly died without a definite diagnosis between July 2008 and December 2012 at Kyushu University Hospital in Japan were enrolled in this study. Their Guthrie cards, which had been stored for several years at 4–8°C, were used for an acylcarnitine analysis by tandem mass spectrometry to identify inborn errors of metabolism.ResultsFifteen infants and children who died at less than 2 years of age and for whom the cause of death was unknown were enrolled for the study. After correcting the C0 and C8 values assuming the hydrolysation of acylcarnitine in the stored DBSs, the corrected C8 value of one case just exceeded the cut-off level for medium-chain acyl-CoA dehydrogenase (MCAD) deficiency screening. Genetic and biochemical analyses confirmed this patient to have MCAD deficiency.ConclusionDBSs stored after newborn screening tests are a promising tool for metabolic autopsy. The appropriate compensation of acylcarnitine data and subsequent genetic and biochemical analyses are essential for the postmortem diagnosis of inborn errors of metabolism.
Work history and radioprotection practices in relation to cancer incidence and mortality in US radiologic technologists performing nuclear medicine procedures
Authors: Bernier Marie Odile , Doody Michele M , Van Dyke Miriam E , Villoing Daphné , Alexander Bruce H , Linet Martha S , Kitahara Cari M 
Published: May 2018

IntroductionTechnologists working in nuclear medicine (NM) are exposed to higher radiation doses than most other occupationally exposed populations. The aim of this study was to estimate the risk of cancer in NM technologists in relation to work history, procedures performed and radioprotection practices.MethodsFrom the US Radiologic Technologists cohort study, 72 755 radiologic technologists who completed a 2003–2005 questionnaire were followed for cancer mortality through 31 December 2012 and for cancer incidence through completion of a questionnaire in 2012–2013. Multivariable-adjusted models were used to estimate HRs for total cancer incidence and mortality by history of ever performing NM procedures and frequency of performing specific diagnostic or therapeutic NM procedures and associated radiation protection measures by decade.ResultsDuring follow-up (mean=7.5 years), 960 incident cancers and 425 cancer deaths were reported among the 22 360 technologists who worked with NM procedures. We observed no increased risk of cancer incidence (HR 0.96, 95% CI 0.89 to 1.04) or death (HR 1.05, 95% CI 0.93 to 1.19) among workers who ever performed NM procedures. HRs for cancer incidence but not mortality were higher for technologists who began performing therapeutic procedures in 1960 and later compared with the 1950s. Frequency of performing diagnostic or therapeutic NM procedures and use of radioprotection measures were not consistently associated with cancer risk. No clear associations were observed for specific cancers, but results were based on small numbers.ConclusionCancer incidence and mortality were not associated with NM work history practices, including greater frequency of procedures performed.
Airway responsiveness to methacholine and incidence of COPD: an international prospective cohort study
Authors: Marcon Alessandro , Locatelli Francesca , Keidel Dirk , Beckmeyer-Borowko Anna B , Cerveri Isa , Dharmage Shyamali C , Fuertes Elaine , Garcia-Aymerich Judith , Heinrich Joachim , Imboden Medea , Janson Christer , Johannessen Ane , Leynaert Bénédicte , Pascual Erquicia Silvia , Pesce Giancarlo , Schaffner Emmanuel , Svanes Cecilie , Urrutia Isabel , Jarvis Deborah , Probst-Hensch Nicole M , Accordini Simone 
Published: May 2018

BackgroundIt has been debated, but not yet established, whether increased airway responsiveness can predict COPD. Recognising this link may help in identifying subjects at risk.ObjectiveWe studied prospectively whether airway responsiveness is associated with the risk of developing COPD.MethodsWe pooled data from two multicentre cohort studies that collected data from three time points using similar methods (European Community Respiratory Health Survey and Swiss Cohort Study on Air Pollution and Lung and Heart Diseases in Adults). We classified subjects (median age 37 years, 1st–3rd quartiles: 29–44) by their level of airway responsiveness using quintiles of methacholine dose–response slope at the first examination (1991–1994). Then, we excluded subjects with airflow obstruction at the second examination (1999–2003) and analysed incidence of COPD (postbronchodilator FEV1/FVC below the lower limit of normal) at the third examination (2010–2014) as a function of responsiveness, adjusting for sex, age, education, body mass index, history of asthma, smoking, occupational exposures and indicators of airway calibre.ResultsWe observed 108 new cases of COPD among 4205 subjects during a median time of 9 years. Compared with the least responsive group (incidence rate 0.6 per 1000/year), adjusted incidence rate ratios for COPD ranged from 1.79 (95% CI 0.52 to 6.13) to 8.91 (95% CI 3.67 to 21.66) for increasing airway responsiveness. Similar dose–response associations were observed between smokers and non-smokers, and stronger associations were found among subjects without a history of asthma or asthma-like symptoms.ConclusionsOur study suggests that increased airway responsiveness is an independent risk factor for COPD. Further research should clarify whether early treatment in patients with high responsiveness can slow down disease progression.
Whole Exome and Transcriptome Analyses Integrated with Microenvironmental Immune Signatures of Lung Squamous Cell Carcinoma
Authors: Seo Jeong-Sun , Lee Ji Won , Kim Ahreum , Shin Jong-Yeon , Jung Yoo Jin , Lee Sae Bom , Kim Yoon Ho , Park Samina , Lee Hyun Joo , Park In-Kyu , Kang Chang-Hyun , Yun Ji-Young , Kim Jihye , Kim Young Tae 
Published: May 2018

Abstract
The immune microenvironment in lung squamous cell carcinoma (LUSC) is not well understood, with interactions between the host immune system and the tumor, as well as the molecular pathogenesis of LUSC, awaiting better characterization. To date, no molecularly targeted agents have been developed for LUSC treatment. Identification of predictive and prognostic biomarkers for LUSC could help optimize therapy decisions. We sequenced whole exomes and RNA from 101 tumors and matched noncancer control Korean samples. We used the information to predict subtype-specific interactions within the LUSC microenvironment and to connect genomic alterations with immune signatures. Hierarchical clustering based on gene expression and mutational profiling revealed subtypes that were either immune defective or immune competent. We analyzed infiltrating stromal and immune cells to further characterize the tumor microenvironment. Elevated expression of macrophage 2 signature genes in the immune competent subtype confirmed that tumor-associated macrophages (TAM) linked inflammation and mutation-driven cancer. A negative correlation was evident between the immune score and the amount of somatic copy-number variation (SCNV) of immune genes (r = −0.58). The SCNVs showed a potential detrimental effect on immunity in the immune-deficient subtype. Knowledge of the genomic alterations in the tumor microenvironment could be used to guide design of immunotherapy options that are appropriate for patients with certain cancer subtypes. Cancer Immunol Res; 6(7); 848–59. ©2018 AACR.
Time to Decision and Treatment With tPA (Tissue-Type Plasminogen Activator) Using Telemedicine Versus an Onboard Neurologist on a Mobile Stroke Unit
Authors: Bowry Ritvij , Parker Stephanie A. , Yamal Jose-Miguel , Hwang Hyunsoo , Appana Savitri , Rangel-Gutierrez Nicole , Wu Tzu-Ching , Rajan Suja S. , Grotta James C. 
Published: May 2018

Background and Purpose—
Mobile stroke units (MSUs) can speed treatment with intravenous tPA (tissue-type plasminogen activator). We previously showed substantial agreement between a telemedicine-based vascular neurologist (TM-VN) and an onboard vascular neurologist (OB-VN) for the evaluation of patients with stroke for tPA eligibility on an MSU. However, the time efficiency of the telemedicine-based evaluation remained uncertain. In this study, we examined the speed of decision and treatment from MSU arrival for the TM-VN compared with an OB-VN.

Methods—
In 50 consecutive situations, the TM-VN served as the primary decision maker. Times from MSU arrival to tPA decision and tPA bolus were compared with the same metrics for when the OB-VN served as the primary decision maker.

Results—

Time to tPA decision for the TM-VN was 21 minutes (interquartile range, 16.25–26) versus 18 minutes (interquartile range, 14–22) for the OB-VN (
P
=0.01). Initiation of tPA bolus was 24 minutes (interquartile range, 19.75–30) for the TM-VN versus 24 minutes (interquartile range, 19–27.75) for the OB-VN (
P
=0.5).

Conclusions—
Assessment by a TM-VN is comparable with an OB-VN in making decisions about tPA administration on an MSU and does not lead to treatment delays.

Clinical Trial Registration—

URL:
https://www.clinicaltrials.gov
. Unique identifier: NCT02190500.