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Sensitivity to noise is currently an obstacle to the use of quantum imaging techniques in real-world scenarios. Here, exploiting non-local cancellation of dispersion on time-frequency entangled photons, the authors show a 43dB improvement in resilience to noise for imaging protocols towards a quantum LiDAR.

Background: Light chain proximal tubulopathy (LCPT) is a rare form of paraprotein-related disease, occurring in two main histopathological forms: crystalline and non-crystalline. The clinicopathological features, treatment strategies and outcomes, especially of the non-crystalline form, are not well described., Methods: We conducted a single-centre retrospective case series of 12 LCPT patients, 5 crystalline and 7 non-crystalline, between 2005 and 2021., Results: The median age was 69.5 years (range 47-80). Ten patients presented with CKD and significant proteinuria (median estimated glomerular filtration rate of 43.5 ml/min/1.73 m2; urine protein:creatinine ratio 328 mg/mmol). Only six patients had known haematological disease at the time of renal biopsy. Multiple myeloma (MM) was diagnosed in seven patients cases and monoclonal gammopathy of renal significance (MGRS) in five patients. A clone was detected in all cases combining serum/urine electrophoresis and free light chain (LC) assays. Crystalline and non-crystalline variants had similar clinical presentations. For the non-crystalline variant, a diagnosis was reached based on a combination of CKD without another cause, haematological workup, LC restriction on immunofluorescence and abnormalities on electron microscopy (EM). Nine of 12 patients received clone-directed treatment. Patients who achieved haematological response (including all non-crystalline LCPT) had improved renal outcomes over a median follow-up of 79 months., Conclusions: The non-crystalline variant may go unrecognised because of its subtle histopathological features and requires EM to distinguish it from 'excessive LC resorption without tubular injury'. Clone-directed treatment with good haematological response improves renal outcomes in both variants but limited data exist in MGRS. Multicentre prospective studies are needed to better define the clinicopathological characteristics associated with poor outcomes and optimize treatment strategies in patients with MGRS., (© The Author(s) 2023. Published by Oxford University Press on behalf of the ERA.)

Aims: Previous studies in adult congenital heart disease (CHD) have demonstrated a link between renal dysfunction and mortality. However, the prognostic significance of renal dysfunction in CHD and decompensated heart failure (HF) remains unclear. We sought to assess the association between renal dysfunction and outcomes in adults with CHD presenting to our centre with acute HF between 2010 and 2021., Methods and Results: This retrospective analysis focused on the association between renal dysfunction, pre-existing and on admission, and outcomes during and after the index hospitalization. Chronic kidney disease (CKD) was defined as an estimated glomerular filtration rate <60 mL/min/1.73 m2. Cox regression analysis was used to identify the predictors of death post-discharge. In total, 176 HF admissions were included (mean age 47.7 ± 14.5 years, 43.2% females). One-half of patients had a CHD of great complexity, 22.2% had a systemic right ventricle, and 18.8% had Eisenmenger syndrome. Chronic kidney disease was present in one-quarter of patients. The median length of intravenous diuretic therapy was 7 (4-12) days, with a maximum dose of 120 (80-160) mg furosemide equivalents/day, and 15.3% required inotropic support. The in-hospital mortality rate was 4.5%. The 1- and 5-year survival rates free of transplant or ventricular assist device (VAD) post-discharge were 75.4% [95% confidence interval (CI): 69.2-82.3%] and 43.3% (95% CI: 36-52%), respectively. On multivariable Cox analysis, CKD was the strongest predictor of mortality or transplantation/VAD. Highly complex CHD and inpatient requirement of inotropes also remained predictive of an adverse outcome., Conclusion: Adult patients with CHD admitted with acute HF are a high-risk cohort. CKD is common and triples the risk of death/transplantation/VAD. An expert multidisciplinary approach is essential for optimizing outcomes., Competing Interests: Conflict of interest: K.K. and A.C. have received educational grants, personal fees, and non-financial support from Janssen-Cilag Limited. K.D. has received non-financial support from Janssen-Cilag Limited and has been a consultant to, and received grants and personal fees from, Janssen-Cilag Limited, Pfizer, GlaxoSmithKline, and Bayer/MSD. S.V.B.-N. is supported by the British Heart Foundation, London, UK (EX/18/1/34296). All of the remaining authors have no relationships with the industry to declare., (© The Author(s) 2023. Published by Oxford University Press on behalf of the European Society of Cardiology.)

Aim: Triage is key to effective management of major incidents, yet there is scarce evidence surrounding the optimal method of paediatric major incident triage (MIT). This study aimed to derive consensus on key components of paediatric MIT among healthcare professionals responsible for triage during paediatric major incidents., Methods: Two-round online Delphi consensus study delivered July 2021-October 2021, including participants from pre-hospital and hospital specialities responsible for triage during paediatric major incidents. A 5-point Likert scale was used to determine consensus, set a priori at 70%., Results: 111 clinicians completed both rounds; 13 of 17 statements reached consensus. Positive consensus was reached on rescue breaths in mechanisms associated with hypoxia or asphyxiation, mobility assessment as a crude discriminator and use of adult physiology for older children. Whilst positive consensus was reached on the benefits of a single MIT tool across all adult and paediatric age ranges, there was negative consensus in relation to clinical implementation., Conclusions: This Delphi study has established consensus among a large group of clinicians involved in the management of major incidents on several key elements of paediatric major incident triage. Further work is required to develop a triage tool that can be implemented based on emerging and ongoing research and which is acceptable to clinicians., (© 2022 Foundation Acta Paediatrica. Published by John Wiley & Sons Ltd.)

Type 1 von Willebrand disease (VWD) is the most common subtype of VWD, comprising 75% of VWD patients. We provide a systematic review of type 1 VWD in pregnancy. Our objective was to evaluate the rate of postpartum hemorrhage (PPH) in patients with known type 1 VWD. The primary outcome was rate of PPH. Primary PPH was defined as a cumulative blood loss ≥1,000 mL, or blood loss accompanied by signs and symptoms of hypovolemia within 24 hours postpartum or requiring blood products. Secondary PPH was defined as significant bleeding 24 hours to 12 weeks postpartum. Relevant articles published in English pertaining to VWD and pregnancy were identified without any time or study limitations. Seven articles ( n  = 144 pregnancies) met inclusion criteria. The rate of primary PPH was 4/144 (2.8%). The secondary PPH rate was reported in four studies, and occurred in 7/48 pregnancies (14.6%), ranging from 2 to 19 days postpartum. In conclusion, according to this systematic review, the frequency of primary PPH in pregnancies with known type 1 VWD is 2.8%. This is similar to the overall PPH rates of 3% reported in the literature. Although the sample size was small, secondary PPH occurred in almost 15% of pregnancies, while in the overall obstetrical population this occurs in approximately 1% of cases. Patients with known type 1 VWD may not be at increased risk of primary PPH, though they appear to bear increased risk of secondary PPH., Competing Interests: None declared., (Thieme. All rights reserved.)

Aviation is an important contributor to the global economy, satisfying society's mobility needs. It contributes to climate change through CO 2 and non-CO 2 effects, including contrail-cirrus and ozone formation. There is currently significant interest in policies, regulations and research aiming to reduce aviation's climate impact. Here we model the effect of these measures on global warming and perform a bottom-up analysis of potential technical improvements, challenging the assumptions of the targets for the sector with a number of scenarios up to 2100. We show that although the emissions targets for aviation are in line with the overall goals of the Paris Agreement, there is a high likelihood that the climate impact of aviation will not meet these goals. Our assessment includes feasible technological advancements and the availability of sustainable aviation fuels. This conclusion is robust for several COVID-19 recovery scenarios, including changes in travel behaviour.

Objective: The aims of this study were to determine the percentage of patients who opt to undergo pessary self-care versus those who return to the office for care and to identify any differences in the characteristics between the 2 groups., Methods: This study is a retrospective chart review conducted at a tertiary care center. Demographic data; details regarding pessary use; body weight; pelvic organ prolapse stage; and medical, surgical, and obstetrical histories were evaluated. Variables were compared between groups of patients using the t test, Wilcoxon rank sum test, χ2 test, and Fisher exact test., Results: The rate of patients choosing to perform pessary self-care was 31%. The patients in the self-care group were significantly younger (65.0 vs 75.0 years, P < 0.001). Those who were performing self-care were more likely to be premenopausal (6.9% vs 1.5%, P = 0.003), have a lower stage of pelvic organ prolapse (61.3% vs 42.1%, P < 0.001), and more likely to be sexually active (40.5% vs 9.6%, P < 0.001). Those not performing self-care had higher rates of vaginal bleeding (26.0% vs 16.4%, P = 0.012) and erosion (23.5% vs 9.9%, P < 0.001). Self-care patients predominantly used the ring with support pessary, whereas those in the non-self-care group were mostly using the Gellhorn., Conclusions: Only one third of patients who use a pessary chose to perform self-care. Patients who use self-care are younger, premenopausal, sexually active, and have lower degrees of prolapse. These data can help educate patients on the characteristics more often seen in those choosing self-care. This study highlights the importance of further exploring pessary care preferences and using this information for counseling., Competing Interests: D.O.S. has received grant funding from Tepha. Other authors have declared they have no conflicts of interest., (Copyright © 2021 American Urogynecologic Society. All rights reserved.)

The purpose of this study was to determine which patient- or surgery-related factors are predictive of need for perioperative transfusion to avoid obtaining unnecessary pre-operative type and screens (T&S). We conducted an observational retrospective cohort study of 1200 women ≥ 18 years old undergoing gynecologic surgery for benign, possibly benign, or malignant indications on a gynecologic oncology service at a university medical center from 2009-2016. A logistic regression model was used to examine patient-related and surgery-related variables predictive of outcome of transfusion. Independent variables included patient demographics, comorbidities, and surgical indication surgical route, and surgical type. Dependent variable was transfusion outcome (T&S only, conversion to type and cross (T&C), or transfusion). Eight hundred ninety-nine (74.9%) women underwent pre-operative T&S, of which 118 (9.8%) were converted to T&C, and 80 (6.7%) received a transfusion of blood or blood products. Cancer indication, major surgery, and preoperative hematocrit less than 36% were significantly associated with need for transfusion ( P = 0.002, P < 0.0001, P < 0.0001, respectively). Patients with a benign indication undergoing minor procedures and with normal preoperative hematocrit are least likely to require transfusion., Competing Interests: Conflict of interest The authors have no conflicts of interest to report. The authors have no relevant financial disclosures to report. Data from this work were presented at the 2019 ACOG Annual Clinical and Scientific Meeting in Nashville, TN.

Background: During the coronavirus disease 2019 (COVID-19) epidemic, many countries have instituted population-wide measures for social distancing. The requirement of patients on dialysis for regular treatment in settings typically not conducive to social distancing may increase their vulnerability to COVID-19., Methods: Over a 6-week period, we recorded new COVID-19 infections and outcomes for all adult patients receiving dialysis in a large dialysis center. Rapidly introduced control measures included a two-stage routine screening process at dialysis entry (temperature and symptom check, with possible cases segregated within the unit and tested for SARS-CoV-2), isolated dialysis in a separate unit for patients with infection, and universal precautions that included masks for dialysis nursing staff., Results: Of 1530 patients (median age 66 years; 58.2% men) receiving dialysis, 300 (19.6%) developed COVID-19 infection, creating a large demand for isolated outpatient dialysis and inpatient beds. An analysis that included 1219 patients attending satellite dialysis clinics found that older age was a risk factor for infection. COVID-19 infection was substantially more likely to occur among patients on in-center dialysis compared with those dialyzing at home. We observed clustering in specific units and on specific shifts, with possible implications for aspects of service design, and high rates of nursing staff illness. A predictive epidemic model estimated a reproduction number of 2.2; cumulative cases deviated favorably from the model from the fourth week, suggesting that the implemented measures controlled transmission., Conclusions: The COVID-19 epidemic affected a large proportion of patients at this dialysis center, creating service pressures exacerbated by nursing staff illness. Details of the control strategy and characteristics of this epidemic may be useful for dialysis providers and other institutions providing patient care., (Copyright © 2020 by the American Society of Nephrology.)

Objective: Our objective was to review the phenotypic and genetic characteristics of tetrasomy 5p from the fetal period until adulthood including prenatal diagnostic evaluations., Background: Tetrasomy 5p is a rare chromosomal abnormality. Of the 14 reports, most document mosaic tetrasomy 5p resulting from a supernumerary marker chromosome or isochromosome. There is a wide range of phenotypic manifestations with severity related to more proximal breakpoints and the degree of mosaicism., Design: We conducted a systematic review using Scopus, PubMed Central® and Ovid MEDLINE® from inception through July 1, 2018 for all articles describing tetrasomy 5p. All articles describing the syndrome of tetrasomy 5p were included., Results: Of the 15 included cases, 13 exhibited mosaic tetrasomy and two had complete tetrasomy identified by amniocentesis. The most common features include seizures (8/11 live births, 73%), hypotonia (7/11 live births, 64%), developmental delay (7/9 cases that reached childhood, 78%), abnormal external ears (6/11 live births, 55%), short stature (6/11 live births, 55%), ventriculomegaly (5/11 live births, 45.5%) and congenital heart defect (6/15 cases, 40%). The clinical phenotype ranged in severity from mild with no defining characteristics to severe with seizures, developmental delay, and multiple congenital anomalies, resulting in early death. Of these 15 cases, only 6 were diagnosed prenatally by prenatal genetic testing (40%) with prenatal ultrasound identifying abnormalities in 4/6 (67%). Confined placental mosaicism (CPM) was diagnosed in six additional cases due to discordance between CVS and amniocentesis results. Four of the five live births returned for evaluation and each showed normal development., Conclusions: Fourteen out of 15 (93%) cases of tetrasomy 5p were associated with an abnormal phenotype. Once a diagnosis is made prenatally, a detailed anatomy ultrasound and fetal echocardiogram must be performed to further characterize any structural abnormalities of the fetus and potentially estimate the clinical severity. Caution should be exercised when prenatal diagnosis of mosaic tetrasomy 5p is found by chorionic villus sampling. CVS alone is insufficient to diagnose tetrasomy 5p and needs to be confirmed with amniocentesis. Our review seeks to inform clinicians on the current literature regarding tetrasomy 5p so that they may better counsel patients when this syndrome is diagnosed., (Copyright © 2019 Elsevier Masson SAS. All rights reserved.)

Background and Objectives: Central venous catheters have traditionally provided access for urgent hemodialysis, but are also sometimes advocated as an option for older or more comorbid patients. Adverse effects of this type of dialysis access include central venous stenosis, for which the risk factors and consequences are incompletely understood., Design, Setting, Participants, & Measurements: We conducted two studies within the same population cohort, comprising all patients starting hemodialysis in a single center from January 2006 to December 2013. First, patients were retrospectively analyzed for the presence of central venous stenosis; their access outcomes are described and survival compared with matched controls drawn from the same population. Second, a subset of patients with a history of catheter access within this cohort was analyzed to determine risk factors for central venous stenosis., Results: Among 2811 patients, central venous stenosis was diagnosed in 120 (4.3%), at a median dialysis vintage of 2.9 (interquartile range, 1.8-4.6) years. Compared with matched controls, patients with central venous stenosis had similar survival (median 5.1 versus 5.2 years; P =0.54). Among a subset of 500 patients, all with a history of catheter use, 34 (6.8%) developed central venous stenosis, at a rate of 2.2 per 100 patient-years. The incidence of central venous stenosis was higher with larger number of previous catheters (relative risk [RR], 2.2; 95% confidence interval [95% CI]. 1.6 to 2.9), pacemaker insertion (RR, 3.9; 95% CI, 1.7 to 8.9), and was lower with older age (RR, 0.7 per decade; 95% CI, 0.6 to 0.8). In a Cox proportional hazards model, the catheter number, pacemaker, and younger age at dialysis initiation were all significant independent risk factors for central venous stenosis., Conclusions: Central venous stenosis occurred in a minority of patients on hemodialysis, and was associated with compromised future access, but unchanged survival. Among patients with a history of catheter use, risk related to both the number of catheters and the total catheter duration, although nondialysis factors such as pacemakers were also important. Central venous stenosis risk was lower in older patients, supporting the selective use of tunneled catheters in this group., (Copyright © 2019 by the American Society of Nephrology.)

We show real-time measurement of error-free superchannel transmission over more than 10,500 km of large-area fiber at a spectral efficiency of 4.66 b/s/Hz, utilizing subcarrier based signal processing optimized at 5.4 GBd.

Cognitive-behavioral models of obsessions, although widely accepted, do not entirely explain obsessive-compulsive (OC) symptoms. Constructs from relational frame theory (RFT; e.g., cognitive fusion-the tendency for behavior to be overly regulated and influenced by cognition) may improve our understanding of OC symptoms above and beyond cognitive-behavioral constructs (e.g., intolerance of uncertainty [IU]). This study examined the extent to which cognitive fusion accounts for unique variability in four OC symptom dimensions: contamination, responsibility for harm, unacceptable thoughts, and order/symmetry. Participants completed measures of cognitive fusion, general distress, thought action fusion, IU, and OC symptoms. Regression analyses showed that IU and thought-action fusion (TAF) were significant predictors across the OC symptom dimensions; however, cognitive fusion was only a unique predictor of the unacceptable thoughts symptom dimension. Results support the notion that RFT may best relate to the unacceptable thoughts domain of OCD. Study findings, limitations, and future directions are discussed.

Bacterial conjugate vaccines have dramatically changed the epidemiology of childhood meningitis; viral causes are increasingly predominant, but the current UK epidemiology is unknown. This prospective study recruited children under 16 years of age admitted to 3 UK hospitals with suspected meningitis. 70/388 children had meningitis-13 bacterial, 26 viral and 29 with no pathogen identified. Group B Streptococcus was the most common bacterial pathogen. Infants under 3 months of age with bacterial meningitis were more likely to have a reduced Glasgow Coma Score and respiratory distress than those with viral meningitis or other infections. There were no discriminatory clinical features in older children. Cerebrospinal fluid (CSF) white blood cell count and plasma C-reactive protein at all ages, and CSF protein in infants <3 months of age, distinguished between bacterial meningitis and viral meningitis or other infections. Improved diagnosis of non-bacterial meningitis is urgently needed to reduce antibiotic use and hospital stay., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.)

Growing concern over emissions from increased airport operations has resulted in a need to assess the impact of aviation related activities on local air quality in and around airports, and to develop strategies to mitigate these effects. One such strategy being investigated is the use of alternative fuels in aircraft engines and auxiliary power units (APUs) as a means to diversify fuel supplies and reduce emissions. This paper summarizes the results of a study to characterize the emissions of an APU, a small gas turbine engine, burning conventional Jet A-1, a fully synthetic jet fuel, and other alternative fuels with varying compositions. Gas phase emissions were measured at the engine exit plane while PM emissions were recorded at the exit plane as well as 10 m downstream of the engine. Five percent reduction in NO(x) emissions and 5-10% reduction in CO emissions were observed for the alternative fuels. Significant reductions in PM emissions at the engine exit plane were achieved with the alternative fuels. However, as the exhaust plume expanded and cooled, organic species were found to condense on the PM. This increase in organic PM elevated the PM mass but had little impact on PM number.

We report on the particulate-bound polycyclic aromatic hydrocarbons (PAH) in the exhaust of a test-bed gas turbine engine when powered by Jet A-1 aviation fuel and a number of alternative fuels: Sasol fully synthetic jet fuel (FSJF), Shell gas-to-liquid (GTL) kerosene, and Jet A-1/GTL 50:50 blended kerosene. The concentration of PAH compounds in the exhaust emissions vary greatly between fuels. Combustion of FSJF produces the greatest total concentration of PAH compounds while combustion of GTL produces the least. However, when PAHs in the exhaust sample are measured in terms of the regulatory marker compound benzo[a]pyrene, then all of the alternative fuels emit a lower concentration of PAH in comparison to Jet A-1. Emissions from the combustion of Jet A-1/GTL blended kerosene were found to have a disproportionately low concentration of PAHs and appear to inherit a greater proportion of the GTL emission characteristics than would be expected from volume fraction alone. The data imply the presence of a nonlinear relation between fuel blend composition and the emission of PAH compounds. For each of the fuels, the speciation of PAH compounds present in the exhaust emissions were found to be remarkably similar (R(2) = 0.94-0.62), and the results do provide evidence to support the premise that PAH speciation is to some extent indicative of the emission source. In contrast, no correlation was found between the PAH species present in the fuel with those subsequently emitted in the exhaust. The results strongly suggests that local air quality measured in terms of the particulate-bound PAH burden could be significantly improved by the use of GTL kerosene either blended with or in place of Jet A-1 kerosene.

In the U.K., cases of sudden unexpected death in infancy are under the jurisdiction of the Coroner and consent for a post-mortem is not required. Prior to the Human Tissue Act 2006 (HTA) there was also no requirement to request retention of tissue (blocks and slides). The HTA stipulates that parental/ guardian consent is mandatory to retain or dispose of all tissues after the Coroners' purposes have been fulfilled. In 2007, in order to avoid confusion with the consent needed for hospital post-mortems, a new form was introduced by Sheffield Children's Hospital NHS Foundation Trust (SCH) called Record of parents'/guardians'wishes regarding samples taken at a Coroner's post mortem. This version specifically asks if blocks and slides may be retained as part of the medical record, or are to be disposed of, and for parental agreement (or not) for the frozen tissue, blocks and slides to be used for education, audit, quality control and medical research. One hundred and nineteen Coroners' postmortems covering the years 2006-2007 were reviewed. All parents/guardians (P/G) were contacted and the outcomes of P/G wishes recorded by SCH staff, Coroners' Officers (CO) and Police Family Liaison Officers (PFLO) were analysed and compared (44% from CO were outstanding at the time of audit). Any delay in recording P/G wishes by these three groups was also compared. In 2006, parental agreement to the use of blocks and slides for education, audit, quality control and medical research was 94%, 77% and 75% for SCH, CO and PFLO, respectively. In 2007 it was 84%, 37% and 100% for the same groups. Permission for the retention of frozen tissue given to SCH, CO and PFLO was 90%, 62% and 100% in 2006 and 90%, 44% and 100% in 2007, respectively. Cases where parents did not wish for the retention or use of tissue (including blocks and slides) were 3%, 15% and 0% in 2006 for SCH, CO and PFLO respectively, and 0% for all groups in 2007. Training of staff in all aspects of post-mortem and bereavement care is essential for ascertaining parental wishes. Families should be provided with the knowledge that allows them to make informed choices. The analysis of the results of the audit supports this view.

Chromosome 6 is a metacentric chromosome that constitutes about 6% of the human genome. The finished sequence comprises 166,880,988 base pairs, representing the largest chromosome sequenced so far. The entire sequence has been subjected to high-quality manual annotation, resulting in the evidence-supported identification of 1,557 genes and 633 pseudogenes. Here we report that at least 96% of the protein-coding genes have been identified, as assessed by multi-species comparative sequence analysis, and provide evidence for the presence of further, otherwise unsupported exons/genes. Among these are genes directly implicated in cancer, schizophrenia, autoimmunity and many other diseases. Chromosome 6 harbours the largest transfer RNA gene cluster in the genome; we show that this cluster co-localizes with a region of high transcriptional activity. Within the essential immune loci of the major histocompatibility complex, we find HLA-B to be the most polymorphic gene on chromosome 6 and in the human genome.

Knowledge of the complete genomic DNA sequence of an organism allows a systematic approach to defining its genetic components. The genomic sequence provides access to the complete structures of all genes, including those without known function, their control elements, and, by inference, the proteins they encode, as well as all other biologically important sequences. Furthermore, the sequence is a rich and permanent source of information for the design of further biological studies of the organism and for the study of evolution through cross-species sequence comparison. The power of this approach has been amply demonstrated by the determination of the sequences of a number of microbial and model organisms. The next step is to obtain the complete sequence of the entire human genome. Here we report the sequence of the euchromatic part of human chromosome 22. The sequence obtained consists of 12 contiguous segments spanning 33.4 megabases, contains at least 545 genes and 134 pseudogenes, and provides the first view of the complex chromosomal landscapes that will be found in the rest of the genome.