Reference Network Research Articles

Global research of artificial intelligence in eyelid diseases: A bibliometric analysis

PurposeTo generate an overview of global research on artificial intelligence (AI) in eyelid diseases using a bibliometric approach. MethodsAll publications related to AI in eyelid diseases from 1900 to 2023 were retrieved from the Web of Science (WoS) Core Collection database. After manual screening, 98 publications published between 2000 and 2023 were finally included. We analyzed the annual trend of publication and citation count, productivity and co-authorship of countries/territories and institutions, research domain, source journal, co-occurrence and evolution of the keywords and co-citation and clustering of the references, using the analytic tool of the WoS, VOSviewer, Wordcloud Python package and CiteSpace. ResultsBy analyzing a total of 98 relevant publications, we detected that this field had continuously developed over the past two decades and had entered a phase of rapid development in the last three years. Among these countries/territories and institutions contributing to this field, China was the most productive country and had the most institutions with high productivity, while USA was the most active in collaborating with others. The most popular research domains was Ophthalmology and the most productive journals were Ocular Surface. The co-occurrence network of keywords could be classified into 3 clusters respectively concerned about blepharoptosis, meibomian gland dysfunction and blepharospasm. The evolution of research hotspots is from clinical features to clinical scenarios and from image processing to deep learning. In the clustering analysis of co-cited reference network, cluster “0# deep learning” was the largest and latest, and cluster “#5 meibomian glands visibility assessment” existed for the longest time. ConclusionsAlthough the research of AI in eyelid diseases has rapidly developed in the last three years, there are still gaps in this area. Our findings provide researchers with a better understanding of the development of the field and a reference for future research directions.

Transition from pediatric to adult care in patients with chronic intestinal failure on home parenteral nutrition: How to do it right?

BackgroundLife expectancy of children with chronic intestinal failure (CIF) on home parenteral nutrition has greatly improved. Children are now able to grow into adulthood which requires transfer from pediatric to adult health care. A guideline for structured transition is lacking and the demand for a more standardized care for this patient group is necessary. Therefore, we investigated the perceptions of health care professionals from various disciplines working in this specific field, concerning effective interventions regarding transition to adult health care. AimTo create a standardized protocol which provides practical guidance for health care professionals in order to bridge the gap between pediatric and adult health care and to facilitate successful transition of children with chronic intestinal failure. MethodsA survey consisting of 20 interventions for transition was sent out to members of the Intestinal Failure working group of European Reference Network for Rare Inherited Congenital (gastrointestinal and digestive) Anomalies (ERNICA) and the Network of Intestinal Failure and Intestinal Transplant in Europe (NITE) group - European Society for Paediatric Gastroenterology Hepatology and Nutrition (ESPGHAN) healthcare professionals in 48 medical centers in various countries. Next to 20 interventions, an open-ended question to fill in any other suggestion with respect to most effective intervention was included. Interventions scoring higher than 80% by the participants were included in the protocol. Interventions scoring between 50% and 80% and other own suggestions were discussed during a consensus meeting and included when consensus, defined as unanimous agreement, was reached. Interventions scoring as effective by < 50% of participants were excluded directly. ResultsA total of 80 healthcare professionals from 33 medical centers (participation rate 69%) participated. The protocol consisted of modifiable components expected to be targets of interventions. The most important key outcomes of the survey were: 1) assessment of patient’s transition readiness and provision of knowledge to the patient by the pediatric team, 2) involvement of parents in the transition process, and 3) collaboration between the pediatric and adult chronic intestinal failure team. In addition it is advised that the transition process should start 1 to 2 years before transfer. A nurse specialist working in both services should form a bridge. All interventions must be tailor-made and based on the maturity of the patient. ConclusionThis study provides a protocol describing transition of children with chronic intestinal failure from pediatric to adult care. This international protocol will serve as practical guidance for pediatric chronic intestinal failure which will provide a more structured, optimal transition process. It is advised to use this protocol as a formal checklist that can be placed in the patient’s chart to review and track the transition process by CIF team members. Future research investigating transition readiness of CIF patients is needed.

Study of high-precision time transfer method enhanced by PPP-AR/PPP-RTK

Abstract With the continuous development and improvement of Global Navigation Satellite Systems (GNSS), the use of GNSS for high-precision time transfer has become increasingly mature. To delve into the contribution of information-enhanced GNSS PPP to time transfer performance, this paper focuses on the comprehensive evaluation and analysis of the time transfer performance of PPP-AR and PPP-RTK. Using GPS as a case study, experimental results demonstrate that the average success fixing rate of PPP integer ambiguity resolution across five stations is 93.57%, with initial fixing times consistently below 20 minutes. The stability of the station clock offset sequence of PPP-AR is better than that of PPP floating solution. Compared with the PPP floating solution, the average improvement of PPP-AR stability is 16.54%. Furthermore, using PPP-AR for time transfer not only enhances the stability of the time transfer link clock offset sequence, but also improves its noise level. The stability is increased by 14.41% on average, and the noise level is improved by 9.11% on average. PPP-RTK also improves the stability and noise level of time transfer even better than PPP-AR. However, as the reference network scale increases, the accuracy of the interpolated atmospheric delay correction decreases, impacting the performance of PPP-RTK.&amp;#xD;

Proposal for standardized prenatal assessment of fetal open dysraphisms by the European reference network for Intellectual disability, TeleHealth, Autism and Congenital Anomalies (ITHACA) and eUROGEN.

Open dysraphisms, that is, myelomeningocele and myeloschisis, are rare diseases associated with a risk of severe disability, including lower limb motor and sensory deficiency, sphincter deficiency, and potential intellectual deficiency. Open dysraphism is diagnosed in Europe in 93.5% of cases. In case of suspicion of fetal open dysraphism, a detailed fetal morphologic assessment is required to confirm the diagnosis and exclude associated structural anomalies, as well as genetic assessment. In case of isolated fetal open dysraphism, assessment of prognosis is based on fetal imaging including the level of the lesion, the presence or not of a sac, the presence and nature of intra cranial anomalies, and the anatomical and functional evaluation of the lower extremities. Based on these biomarkers, a personalized prognosis as well as comprehensive information about prenatal management alternatives will allow parents to decide on further management options. Standardization of prenatal assessment is essential to compare outcomes with benchmark data and make assessment of surgical innovation possible. Herein, we propose a protocol for the standardized ultrasound assessment of fetuses with isolated open dysraphism.

OTHR-02. ONE-YEAR FOLLOW-UP OF THE NEW ERN PAEDCAN CNS TUMOR BOARD: LESSONS LEARNT AND FUTURE PROSPECTS

Abstract BACKGROUND European Reference Networks (ERN) are collaborative networks connecting healthcare professionals across Europe. A virtual tumor board for pediatric patients with central nervous system (CNS) tumors was established within the ERN for pediatric oncology (ERN PaedCan) in September 2022. We report the feasibility, the implementation of recommendations and satisfaction of the participants. METHODS A web-based questionnaire was distributed to physicians presenting cases between November 2022 and November 2023. Questions addressed the implementation of recommendations, satisfaction and basic information about the local institution. Basic data of the presented cases were taken from anonymized tumor board protocols. RESULTS In the first year, 19 patients from 11 institutions located in nine European countries were discussed in 21 tumor boards. Median age at diagnosis was 10 (1-17) years. Demonstration of MRI findings by the German national reference center for neuroradiology was conducted in 19/21 conferences. 18/21 questionnaires (86%) were answered by physicians from eight countries. Main reason to request the discussion were questions about therapy (78%, n=14/18). Inquiring institutions treated a median of 10 (5-150) neuro-oncological pediatric patients per year. All hospitals conducted own institutional tumor boards. National central review was available in 3/8 countries (38%). Recommendations were followed, at least partly, in all cases except for one patient experiencing unexpected clinical deterioration. Recommendations were deemed helpful in 89%. All participants would recommend the tumor board to colleagues. Technical issues regarding data provision were reported as the main obstacle in 50 %. CONCLUSIONS A European virtual tumor board for pediatric patients with CNS tumors is feasible and perceived as useful by the participating institutions. Recommendations were followed frequently. Optimization of privacy-compliant data exchange is crucial for continuance of the format. This project has been supported by ERN PaedCan, which is funded by the European Union within the EU4Health Program 2021-2027. Funded by EU-grant 101085543.

Development of the CDISC Pediatrics User Guide: a CDISC and conect4children collaboration.

The conect4children (c4c) project aims to facilitate efficient planning and delivery of paediatric clinical trials. One objective of c4c is data standardization and reuse. Interoperability and reusability of paediatric clinical trial data is challenging due to a lack of standardization. The Clinical Data Interchange Standards Consortium (CDISC) standards that are required or recommended for regulatory submissions in several countries lack paediatric specificity with limited awareness within academic institutions. To address this, c4c and CDISC collaborated to develop the Pediatrics User Guide (PUG) consisting of cross-cutting data items that are routinely collected in paediatric clinical trials, factoring in all paediatric age ranges. The development of the PUG consisted of six stages. During the scoping phase, subtopics (each containing several clinically relevant concepts) were suggested and debated for inclusion in the PUG. Ninety concepts were selected for the modelling phase. Concept maps describing the Research Topic and representation procedure were developed for the 19 concepts that had no (or partial) previous modelling in CDISC. Next, metadata and implementation examples were developed for concepts. This was followed by a CDISC internal review and a public review. For both these review stages, the feedback comments were either implemented or rejected based on budget, timelines, expert review, and scope. The PUG was published on the CDISC website on February 23, 2023. The PUG is a first step in bridging the lack of child specific CDISC standards, particularly within academia. Several academic and industrial partners were involved in the development of the PUG, and c4c has undertaken multiple steps to publicize the PUG within its academic partner organizations - in particular, the European Reference Networks (ERNs) that are developing registries and dictionaries in 24 disease areas. In the long term, continued use of the PUG in paediatric clinical trials will enable the pooling of data from multiple trials, which is particularly important for medical domains with small populations.

Analyses of 1236 genotyped primary ciliary dyskinesia individuals identify regional clusters of distinct DNA variants and significant genotype-phenotype correlations.

Primary ciliary dyskinesia (PCD) represents a group of rare hereditary disorders characterised by deficient ciliary airway clearance that can be associated with laterality defects. We aimed to describe the underlying gene defects, geographical differences in genotypes and their relationship to diagnostic findings and clinical phenotypes. Genetic variants and clinical findings (age, sex, body mass index, laterality defects, forced expiratory volume in 1 s (FEV1)) were collected from 19 countries using the European Reference Network's ERN-LUNG international PCD Registry. Genetic data were evaluated according to American College of Medical Genetics and Genomics guidelines. We assessed regional distribution of implicated genes and genetic variants as well as genotype correlations with laterality defects and FEV1. The study included 1236 individuals carrying 908 distinct pathogenic DNA variants in 46 PCD genes. We found considerable variation in the distribution of PCD genotypes across countries due to the presence of distinct founder variants. The prevalence of PCD genotypes associated with pathognomonic ultrastructural defects (mean 72%, range 47-100%) and laterality defects (mean 42%, range 28-69%) varied widely among countries. The prevalence of laterality defects was significantly lower in PCD individuals without pathognomonic ciliary ultrastructure defects (18%). The PCD cohort had a reduced median FEV1 z-score (-1.66). Median FEV1 z-scores were significantly lower in CCNO (-3.26), CCDC39 (-2.49) and CCDC40 (-2.96) variant groups, while the FEV1 z-score reductions were significantly milder in DNAH11 (-0.83) and ODAD1 (-0.85) variant groups compared to the whole PCD cohort. This unprecedented multinational dataset of DNA variants and information on their distribution across countries facilitates interpretation of the genetic epidemiology of PCD and indicates that the genetic variant can predict diagnostic and phenotypic features such as the course of lung function.

SAILoR: Structure-Aware Inference of Logic Rules.

Boolean networks provide an effective mechanism for describing interactions and dynamics of gene regulatory networks (GRNs). Deriving accurate Boolean descriptions of GRNs is a challenging task. The number of experiments is usually much smaller than the number of genes. In addition, binarization leads to a loss of information and inconsistencies arise in binarized time-series data. The inference of Boolean networks from binarized time-series data alone often leads to complex and overfitted models. To obtain relevant Boolean models of gene regulatory networks, inference methods could incorporate data from multiple sources and prior knowledge in terms of general network structure and/or exact interactions. We propose the Boolean network inference method SAILoR (Structure-Aware Inference of Logic Rules). SAILoR incorporates time-series gene expression data in combination with provided reference networks to infer accurate Boolean models. SAILoR automatically extracts topological properties from reference networks. These can describe a more general structure of the GRN or can be more precise and describe specific interactions. SAILoR infers a Boolean network by learning from both continuous and binarized time-series data. It navigates between two main objectives, topological similarity to reference networks and correspondence with gene expression data. By incorporating the NSGA-II multi-objective genetic algorithm, SAILoR relies on the wisdom of crowds. Our results indicate that SAILoR can infer accurate and biologically relevant Boolean descriptions of GRNs from both a static and a dynamic perspective. We show that SAILoR improves the static accuracy of the inferred network compared to the network inference method dynGENIE3. Furthermore, we compared the performance of SAILoR with other Boolean network inference approaches including Best-Fit, REVEAL, MIBNI, GABNI, ATEN, and LogBTF. We have shown that by incorporating prior knowledge about the overall network structure, SAILoR can improve the structural correctness of the inferred Boolean networks while maintaining dynamic accuracy. To demonstrate the applicability of SAILoR, we inferred context-specific Boolean subnetworks of female Drosophila melanogaster before and after mating.

European standard clinical practice recommendations for primary pediatric low-grade gliomas

Pediatric low-grade gliomas are the most common brain tumours in childhood and adolescence. Despite the excellent prognosis, pediatric low-grade glioma survivors may suffer from variable long-term complications and may require repeated therapies, implying that this is a chronic disease. The current review describes the European Standard Clinical Practice recommendations for low-grade gliomas at primary diagnosis, that were developed on behalf of SIOPe BTG LGG Working Group within the framework of European Reference Network PaedCan. The manuscript describes the diverse spectrum of pediatric low-grade gliomas in terms of location, age, underlying cancer predisposition syndromes, and special circumstances, such as infantile chiasmatic hypothalamic glioma and diencephalic syndrome, as well as current diagnostic criteria and indications for treatment. Furthermore, it provides current knowledge in histopathology and molecular pathology. Finally, the review focuses on the need for a multidisciplinary approach and treatment indications providing a guide on current treatment modalities, used as first-line therapy in Europe along with information on adverse effects, and follow-up.

THU-106-YI Prospective european reference network registry (R-LIVER) supports improvement in bone disease care delivery at local level for patients with autoimmune liver diseases and enables detailed description of risk elements

THU-106-YI Prospective european reference network registry (R-LIVER) supports improvement in bone disease care delivery at local level for patients with autoimmune liver diseases and enables detailed description of risk elements

Quality assurance for next-generation sequencing diagnostics of rare neurological diseases in the European Reference Network.

In the past decade, next-generation sequencing (NGS) has revolutionised genetic diagnostics for rare neurological disorders (RND). However, the lack of standardised technical, interpretative, and reporting standards poses a challenge for ensuring consistent and high-quality diagnostics globally. To address this, the European Reference Network for Rare Neurological Diseases (ERN-RND) collaborated with the European Molecular Genetics Quality Network (EMQN) to establish an external quality assessment scheme for NGS-based diagnostics in RNDs. The scheme, initiated in 2021 with a pilot involving 29 labs and followed by a second round in 2022 with 42 labs, aimed to evaluate the performance of laboratories in genetic testing for RNDs. Each participating lab analysed genetic data from three hypothetical cases, assessing genotyping, interpretation, and clerical accuracy. Despite a majority of labs using exome or genome sequencing, there was considerable variability in gene content, sequencing quality, adherence to standards, and clinical guidance provision. Results showed that while most labs provided correct molecular diagnoses, there was significant variability in reporting technical quality, adherence to interpretation standards, reporting strategies, and clinical commentary. Notably, some labs returned results with the potential for adverse medical outcomes. This underscores the need for further harmonisation, guideline development, and external quality assessment in the evolving landscape of genomic diagnostics for RNDs. Overall, the experience with the scheme highlighted the generally good quality of participating labs but emphasised the imperative for ongoing improvement in data analysis, interpretation, and reporting to enhance patient safety.

Розробка цифрових карт модуля індукції геомагнітного поля для території України

Knowledge of Ukraine`s geomagnetic field is based on magnetic surveys of the geomagnetic field induction module B and its power and angular components, as well as on observations or calculations of their anomalous values. The article describes the methods for constructing a digital map of the induction module of the geomagnetic field B. The map of module B for the epoch of 1969.5 is developed, which is the ground to create the maps of module B and its anomalies ΔB for an arbitrary epoch. The results of the ground absolute magnetic survey of 1969-1972, the analytical model of the main geomagnetic field BIGRF, as well as the map of the anomalous magnetic field on a scale of 1: 1,000,000 were used to construct the map of the geomagnetic field induction module B. The digital map of the module and anomalies of the geomagnetic field induction module B for the 1969.5 epoch was obtained by calculating the correction based on their difference values at the points of the absolute reference network. It was spread by interpolation according to the 1x1km matrix to the entire data array for the territory of Ukraine. The geomagnetic field induction module B for the 2005.5 epoch was calculated by adding to its values in the 1969.5 epoch the calculated values of the field dynamics ВIGRF and the "magnetizing" effect of anomalies of the induction module ΔΔB. The developed map is an approximate representation of the magnetic field modulus, since the amount of remanent magnetization was not taken into account during the calculation, which may cause minor deviations of the calculated and observed field values. The induction module of the geomagnetic field B in the epoch of 1969.5 varies in the range of 46,500÷62,000 nT, and in the epoch of 2005.5 ‒ 47,000÷63000 nT, increasing from the southwest to the northeast of the territory of Ukraine. Its maximum values are observed in the areas of regional and local magnetic anomalies, in particular in the area of the Kryvyi Rih-Kremenchuk anomalous strip and the southeastern edge of the Kursk anomaly. The dynamics of the BIGRF field for the period of 1969.5÷2005.5 is characterized by values from 700 nT in the east of the territory of Ukraine to 950 nT in the west, and the magnitude of the magnetizing effect ΔΔB varies within ±10 nT, reaching maximum values (up to 100-190 nT) in the regions of intense magnetic anomalies. The digital map of the geomagnetic field induction module B, developed in 2005.5, has been verified. This was done by measuring the values of module B in the magnetic observatories of Kyiv, L’viv, and Odesa, as well as in the Repeat Stations of the Ist and IInd classes. The results showed that there was only a small difference (-20 ÷ 40 nT) between the calculated and measured values of modulus B. Their most significant differences are spatially correlated with areas of modern activation of the Earth's crust on the territory of Ukraine and electrical conductivity anomalies.

THU-167 Diagnostic and therapeutic trends in primary biliary cholangitis: insights from the european reference network registry (R-LIVER)

THU-167 Diagnostic and therapeutic trends in primary biliary cholangitis: insights from the european reference network registry (R-LIVER)

#276 ADTKD genetic diagnostics in Europe: where do we stand and what is needed?

Abstract Background and Aims We are the first European patient organization to pursue the goal of raising awareness of the monogenic kidney disease ADTKD (autosomal dominant tubulointerstitial kidney disease) and supporting its research (www.adtkd.de/en-gb). To date, causative mutations are known in at least five disease genes (UMOD, MUC1, REN, HNF1B, SEC61A1) [1] and one can expect that even more disease genes exist. There are already strong international collaborations to define the clinical course of ADTKD [2] and to evaluate a targeted therapy in clinical trials [3]. Clinical registries form the basis for the inclusion of patients, preferably at an early stage of the disease. Such a registry already exists in the USA and is currently being established in Europe (ADTKD-Net) as part of the European Joint Program on Rare Diseases (EJP RD). Due to its non-specific and variable clinical characteristics, ADTKD is often misdiagnosed or not diagnosed at all. Experts therefore suspect a high number of unreported cases. There are also challenges in genetic diagnostics. While next generation sequencing (NGS) panels have been established for ADTKD, they are usually unable to detect pathogenic MUC1 variants due to their specific location in the genome. This is the insertion of an additional cytosine (dupC) in a VNTR (Variable Number of Tandem Repeats) domain of the MUC1 gene, which cannot be detected by NGS and therefore requires special procedures such as SnaPshot Minisequencing [4] or VNtyper [5]. For instance, there are only two laboratories that can perform this specific MUC1 testing in Germany to date. The aim of this study is to assess the status quo of ADTKD diagnostics in Europe. We hypothesize that there is a lack of appropriate structures to adequately and reliably diagnose ADTKD, notably ADTKD-MUC1. The study is intended to help identify deficits and obstacles in order to address these in a targeted manner. Methods We used Survey Monkey to create a digital survey, which we sent by email to around 50 European ADTKD experts. They were asked to answer 7 key questions (Fig. 1). For the selection of suitable ADTKD experts, we use various sources such as publications on ADTKD in PubMed, the Expert Finder and the European Patient Advocacy Groups (ePAG) in the European Reference Network for Rare Kidney Diseases (ERKNet) and our own network. Our aim is to cover at least the 30 largest countries in Europe in terms of population. Results Results will be presented at the ERA conference. Conclusion With our survey we would like to substantiate the hypothesis that the possibilities of molecular diagnostics of ADTKD in Europe are currently insufficient. Based on our results, short and long-term measures should be taken to remedy potential deficits so that as many patients as possible can be identified and have access to therapeutic options in the future. Another interesting aspect is the awareness of ADTKD among European nephrologists. This could be a future activity of our patient organization.

The Development of a European Multidisciplinary Cleft Lip and Palate Registry by the European Reference Network CRANIO: Experiences, Barriers, And Facilitators.

The European Reference Network for Rare Craniofacial Aanomalies and Ear-Nose-Throat disorders aims to improve care for patients with such afflictions, including cleft lip and palate (CL/P) across Europe. Cleft treatment remains varied throughout European centers, inhibiting meaningful comparison of treatment outcomes. To overcome these issues, a European-wide common CL/P dataset and registry was developed, facilitating standardized treatment endpoints and outcome measures for international comparison and benchmarking of CL/P centers. Questionnaires and semi-structured interviews were used to determine the set-up of the registry. Previous CL/P initiatives were analyzed to create an initial dataset, refined through consensus meetings. In total, 87 cleft specialists working in specialized CL/P centers from 16 European nations participated. Consensus on a common dataset was reached. A "Level 1" dataset, with mandatory clinical and patient-reported outcome measures, and "Level 2" dataset with additional outcome measures. Finally, 2 dashboards were developed for data dissemination. The development of the European CL/P common dataset and registry tackled challenges with resource disparities, variations in specialists within CL/P teams, regulatory differences in patient data usage, patient-reported outcome measures availability in European languages, and use of assessment tools. This study described the successful development of the European Reference Network for Rare Craniofacial Aanomalies and Ear-Nose-Throat disorders CL/P common dataset and registry. This achievement will help improve patient care and outcomes for patients with CL/P in Europe. Furthermore, this study provides useful information for initiatives with similar aims.

Group Formation in Science between Homogenization and Differentiation: Modeling the Development of U.S. and German Sociology

We assess the empirical development of scientific homogenization and differentiation processes in terms of group development comparing U.S. and German sociology from 2004 to 2019. We introduce hierarchical multi-layered Bayesian stochastic block models to the issue of scientific group development. Based on publication data from Scopus, we analyze group formation simultaneously as collaboration networks, reference networks, and topical networks. Employing this method allows us to trace back the temporal dynamics of multidimensional group development and formation for each national scientific field. Overall, the findings indicate clear path dependencies in both fields that have been shaping processes of differentiation and homogenization in U.S. and German sociology and that continue to impact on the formation, stabilization, and dissolution of scientific groups.

Correcting an Off-Nadir to a Nadir Land Surface Temperature Using a Multitemporal Thermal Infrared Kernel-Driven Model during Daytime

Land surface temperature (LST) is a fundamental parameter in global climate, environmental, and geophysical studies. Remote sensing is an essential approach for obtaining large-scale and frequently updated LST data. However, due to the wide field of view of remote sensing sensors, the observed LST with diverse view geometries suffers from inconsistency caused by the thermal radiation directionality (TRD) effect, which results in LST products being incomparable, especially during daytime. To address this issue and correct current off-nadir LSTs to nadir LSTs, a semi-physical time-evolved kernel-driven model (TEKDM) is proposed, which depicts multitemporal TRD patterns during the daytime. In addition, we employ a Bayesian optimization method to calibrate seven unknown parameters in the TEKDM. Validation results using the U.S. Climate Reference Network (USCRN) sites show that the RMSE (MBE) for GOES-16 and MODIS off-nadir LST products is reduced from 3.29 K (−2.0 K) to 2.34 K (−0.02 K), with an RMSE reduction of 0.95 K (29%) and a significant reduction in systematic bias. Moreover, the proposed method successfully eliminates the angular and temporal dependence of the LST difference between the satellite off-nadir LST and in situ nadir LST. In summary, this study presents a feasible approach for estimating the high-accuracy nadir LST, which can enhance the applicability of LST products in various domains.

Clinical practice guidelines for the care of girls and women with Turner syndrome.

Turner syndrome (TS) affects 50 per 100 000 females. TS affects multiple organs through all stages of life, necessitating multidisciplinary care. This guideline extends previous ones and includes important new advances, within diagnostics and genetics, estrogen treatment, fertility, co-morbidities, and neurocognition and neuropsychology. Exploratory meetings were held in 2021 in Europe and United States culminating with a consensus meeting in Aarhus, Denmark in June 2023. Prior to this, eight groups addressed important areas in TS care: (1) diagnosis and genetics, (2) growth, (3) puberty and estrogen treatment, (4) cardiovascular health, (5) transition, (6) fertility assessment, monitoring, and counselling, (7) health surveillance for comorbidities throughout the lifespan, and (8) neurocognition and its implications for mental health and well-being. Each group produced proposals for the present guidelines, which were meticulously discussed by the entire group. Four pertinent questions were submitted for formal GRADE (Grading of Recommendations, Assessment, Development and Evaluation) evaluation with systematic review of the literature. The guidelines project was initiated by the European Society for Endocrinology and the Pediatric Endocrine Society, in collaboration with members from the European Society for Pediatric Endocrinology, the European Society of Human Reproduction and Embryology, the European Reference Network on Rare Endocrine Conditions, the Society for Endocrinology, and the European Society of Cardiology, Japanese Society for Pediatric Endocrinology, Australia and New Zealand Society for Pediatric Endocrinology and Diabetes, Latin American Society for Pediatric Endocrinology, Arab Society for Pediatric Endocrinology and Diabetes, and the Asia Pacific Pediatric Endocrine Society. Advocacy groups appointed representatives for pre-meeting discussions and the consensus meeting.

Treatment of Hirschsprung's Disease in Germany: Analysis of National Hospital Discharge Data From 2016 to 2022

BackgroundHirschsprung's disease (HD) is a rare and complex malformation. The corrective operation is challenging and schedulable. The complete care situation for the corrective surgery for HD in Germany is uninvestigated. MethodsFor the years 2016–2022, the microdata of the diagnosis-related groups (DRG) -statistics provided by the Research Data Center of the German Federal Statistical Office were accessed. All hospital stays for corrective surgery of HD in patients aged 0–17 were analyzed for patient's comorbidities, treatment characteristics and hospital structures. The occurrence of severe early postoperative complications during the hospital stay were documented. ResultsThe care structure for HD in Germany is decentralized with 109 hospitals performing 1199 corrective surgeries in 7 years. 75% of the participating hospitals performed three or less cases per year and 55 participating hospitals did not perform corrective surgery for HD each year. Early postoperative complications were common with at least one severe early complication in 18.6% of the cases. With an overall low case load per hospital, a volume outcome relationship cannot be established within Germany. Compared to international high volume centers the quality of outcomes for some of the investigated parameters was reduced. Despite the establishing of centers of expertise by the European reference network ERNICA for the treatment of HD no trend towards centralization occurred in Germany. ConclusionsThe corrective surgery for HD in Germany is decentralized and results in an overall high rate of early complications. The comparison with international studies from high-volume centers indicates potential for improvement for the corrective surgery of HD. Centralization remains essential for the improvement of care for patients with HD.

Bayesian Analysis of the Detection Performance of the Lightning Imaging Sensors

Abstract Identical Lightning Imaging Sensors (LIS) aboard the Tropical Rainfall Measuring Mission satellite (TRMM LIS, 1998–2015) and International Space Station (ISS LIS, 2017–23) have provided over two decades of lightning observations over the global tropics, with ISS LIS extending coverage into the midlatitudes. Quantifying the detection performance of both LIS sensors is a necessary step toward generating a combined LIS climatological record and accurately combining LIS data with lightning detections from other sensors and networks. We compare lightning observations from both LIS sensors with reference sources including the Geostationary Lightning Mapper (GLM) and ground-based Earth Networks Total Lightning Network (ENTLN), Earth Networks Global Lightning Network (ENGLN), National Lightning Detection Network (NLDN), and Global Lightning Dataset (GLD360). Instead of a relative detection efficiency (DE) approach that assumes the perfect performance of the reference sensor, we employ a Bayesian approach to estimate the upper limit of the absolute DE (ADE) of each system being analyzed. The results of this analysis illustrate the geographical pattern of ADE as well as its diurnal cycle and yearly evolution. Reference network ADE increased by ∼15%–30% during the TRMM era, leading to a decline in TRMM LIS ADE. ISS LIS flash ADE has been relatively consistent at 61%–65%, about 4%–5% lower than TRMM LIS at the end of its lifetime.