Absent ileocecal valve predicts the need for repeated step in children

Background. Serial transverse enteroplasty facilitates weaning from parenteral support in selected patients with short bowel syndrome, although repeated procedure is frequently required. Our aim was to evaluate the outcome of a series of patients after serial transverse enteroplasty and define predictors of repeated serial transverse enteroplasty and weaning off parenteral support. Methods. All children who underwent serial transverse enteroplasty at 4 Nordic pediatric surgery centers from 2004-2015 were included in this observational study. Data were collected from the patient records. The study was approved by the local ethics review boards. Results. Twenty-seven children with short bowel with initial median small bowel length of 26 cm (range, 10-100 cm) were included. Eleven patients had the ileocecal valve remaining. Serial transverse enteroplasty was performed at median age of 7.5 months (range, 0.9-224 months). Serial transverse enteroplasty made the small bowel 46% (0-233%) longer. Eleven patients (41 %) underwent a repeated serial transverse enteroplasty 12 months (1.0-72 months) later; 7 patients required additional operative procedures, but none were transplanted. At follow-up, 45.1 months (1.8-126 months) after the first serial transverse enteroplasty, 11 (41 %) patients needed parenteral support. The remaining 16 patients had been weaned off parenteral support. One patient had died. Absence of the ileocecal valve was the only factor, which predicted the need for a repeated serial transverse enteroplasty (odds ratio 16.7, 95 % confidence interval, 1.7-164.8, P =.007). No factor was identified predicting need for parenteral support at follow-up. Conclusion. A majority of children with short bowel syndrome can be weaned from parenteral support after serial transverse enteroplasty. The absence of the ileocecal valve predicts the need for a repeated serial transverse enteroplasty, which was required by 40% of the patients.Peer reviewe

Fostering continuous quality improvement in a European rare disease network

Background: The European Reference Network for rare Inherited Congenital Anomalies (ERNICA) is a clinical network dedicated to improving the quality of care for patients with rare and complex digestive and gastrointestinal diseases, many of whom require surgery in early life. The network brings together clinicians, researchers and patient representatives from 22 countries in Europe. By pooling expertise, ERNICA is able to facilitate improvement initiatives that may not otherwise be possible. However, describing the desired quality of care and transferring it to local practice remains a challenge, complicated by our low-prevalence patient population, multidisciplinary clinical involvement and heterogeneous European context. In an attempt to mitigate these challenges, and foster a system of continuous quality improvement, we present the “ERNICA quality cycle”. Main body: The ERNICA quality cycle is comprised of five steps: (1) Describing the desired quality of care (2) Promoting guideline implementation (3) Measuring quality of care (4) Evaluating clinical practice (5) Conducting research. It offers a structured, continuous and collaborative approach to the improvement of care for patients with rare and complex digestive and gastrointestinal diseases. Evaluating the approach, through qualitative process evaluation, will be critical to capturing learning points. Conclusions: The ERNICA quality cycle holds tremendous potential for improving the quality of care for patients with rare and complex conditions, both within ERNICA and for other European Reference Networks.</p

Bridging the Gap:Reporting Baseline Characteristics, Process and Outcome Parameters in Hirschsprung's Disease. A Systematic Review

Introduction: The variation in standardized, well-defined parameters in Hirschsprung's disease (HSCR) research hinders overarching comparisons and complicates evaluations of care quality across healthcare settings. This review addresses the significant variability observed in these parameters as reported in recent publications. The goal is to compile a list of commonly described baseline characteristics, process and outcome measures, and to investigate disparities in their utilization and definitions. Materials and Methods: A systematic review of literature on the primary care process for HSCR was performed according to PRISMA guidelines. Relevant literature published between 2015 and 2021 was obtained by combining the search term Hirschsprung's disease with treatment outcome, complications, mortality, morbidity, survival in Medline, Embase and the Cochrane Library. We extracted study characteristics, reported process and outcome parameters, and patient and disease characteristics. Results: We extracted 1026 parameters from 200 publications and categorized these into patient characteristics (n=226), treatment and care process characteristics (n=199), and outcomes (n=601). 116 parameters were reported in more than 5% of publications. The most frequently reported characteristics were sex (88%), age at surgery (66%), postoperative Hirschsprung-associated enterocolitis (64%), type of repair (57%), fecal incontinence (54%), and extent of aganglionosis (51%). Conclusion: This review underscores the pronounced variation in reported parameters within HSCR studies, highlighting the necessity for consistent, well-defined measures and reporting systems in order to foster improved data interpretability. Moreover, it advocates for the use of these findings in the development of a Core Indicator Set, complementing the recently developed Core Outcome Set. This will facilitate quality assessments across pediatric surgical centers throughout Europe.</p

The radical-binding lipocalin A1M binds to a Complex I subunit and protects mitochondrial structure and function.

Aims: During cell death, energy-consuming cell degradation and recycling programs are performed. Maintenance of energy-delivery during cell death is therefore crucial but the mechanisms to keep the mitochondrial functions intact during these processes are poorly understood. We have investigated the hypothesis that the heme- and radical-binding ubiquitous protein A1M (α1-microglobulin) is involved in protection of the mitochondria against oxidative insult during cell death. Results: Using blood cells, keratinocytes and liver cells, we show that A1M binds with high affinity to apoptosis-induced cells and is localized to mitochondria. The mitochondrial Complex I subunit NDUFAB1 was identified as a major molecular target of the A1M-binding. Furthermore, A1M was shown to inhibit the swelling of mitochondria, and to reverse the severely abrogated ATP-production of mitochondria when exposed to heme and ROS. Innovation: Import of the radical- and heme-binding protein A1M from the extracellular compartment confers protection of mitochondrial structure and function during cellular insult. Conclusion: A1M binds to a subunit of Complex I and has a role in assisting the mitochondria to maintain its energy delivery during cell death. A1M may also, at the same time, counteract and eliminate the ROS generated by the mitochondrial respiration to prevent oxidative damage to surrounding healthy tissue

Conceptual design of the International Axion Observatory (IAXO)

The International Axion Observatory (IAXO) will be a forth generation axion helioscope. As its primary physics goal, IAXO will look for axions or axion-like particles (ALPs) originating in the Sun via the Primakoff conversion of the solar plasma photons. In terms of signal-to-noise ratio, IAXO will be about 4-5 orders of magnitude more sensitive than CAST, currently the most powerful axion helioscope, reaching sensitivity to axion-photon couplings down to a few $\times 10^{-12}$ GeV$^{-1}$ and thus probing a large fraction of the currently unexplored axion and ALP parameter space. IAXO will also be sensitive to solar axions produced by mechanisms mediated by the axion-electron coupling $g_{ae}$ with sensitivity $-$for the first time$-$ to values of $g_{ae}$ not previously excluded by astrophysics. With several other possible physics cases, IAXO has the potential to serve as a multi-purpose facility for generic axion and ALP research in the next decade. In this paper we present the conceptual design of IAXO, which follows the layout of an enhanced axion helioscope, based on a purpose-built 20m-long 8-coils toroidal superconducting magnet. All the eight 60cm-diameter magnet bores are equipped with focusing x-ray optics, able to focus the signal photons into $\sim 0.2$ cm$^2$ spots that are imaged by ultra-low-background Micromegas x-ray detectors. The magnet is built into a structure with elevation and azimuth drives that will allow for solar tracking for $\sim$12 h each day.Comment: 47 pages, submitted to JINS

Future axion searches with the International Axion Observatory (IAXO)

The International Axion Observatory (IAXO) is a new generation axion helioscope aiming at a sensitivity to the axion-photon coupling of ga\u3b3 3c few 7 10-12 GeV-1, i.e. 1-1.5 orders of magnitude beyond the one achieved by CAST, currently the most sensitive axion helioscope. The main elements of IAXO are an increased magnetic field volume together with extensive use of x-ray focusing optics and low background detectors, innovations already successfully tested in CAST. Additional physics cases of IAXO could include the detection of electron-coupled axions invoked to explain the white dwarf cooling, relic axions, and a large variety of more generic axion-like particles (ALPs) and other novel excitations at the low-energy frontier of elementary particle physics

Paediatric appendicitis : International study of management in the COVID-19 pandemic

Funding Information: T.W. received a grant from the Swedish Medical Research Council. The Swedish Medical Research Council had no role in any part of the study.Peer reviewe

ERNICA guidelines for the management of rectosigmoid Hirschsprung's disease

Background Hirschsprung's disease (HSCR) is a serious congenital bowel disorder with a prevalence of 1/5000. Currently, there is a lack of systematically developed guidelines to assist clinical decision-making regarding diagnostics and management. Aims This guideline aims to cover the diagnostics and management of rectosigmoid HSCR up to adulthood. It aims to describe the preferred approach of ERNICA, the European Reference Network for rare inherited and congenital digestive disorders. Methods Recommendations within key topics covering the care pathway for rectosigmoid HSCR were developed by an international workgroup of experts from 8 European countries within ERNICA European Reference Network from the disciplines of surgery, medicine, histopathology, microbiology, genetics, and patient organization representatives. Recommendation statements were based on a comprehensive review of the available literature and expert consensus. AGREE II and GRADE approaches were used during development. Evidence levels and levels of agreement are noted. Results Thirty-three statements within 9 key areas were generated. Most recommendations were based on expert opinion. Conclusion In rare or low-prevalence diseases such as HSCR, there remains limited availability of high-quality clinical evidence. Consensus-based guidelines for care are presented.Peer reviewe

ERNICA guidelines for the management of rectosigmoid Hirschsprung's disease

Background Hirschsprung's disease (HSCR) is a serious congenital bowel disorder with a prevalence of 1/5000. Currently, there is a lack of systematically developed guidelines to assist clinical decision-making regarding diagnostics and management. Aims This guideline aims to cover the diagnostics and management of rectosigmoid HSCR up to adulthood. It aims to describe the preferred approach of ERNICA, the European Reference Network for rare inherited and congenital digestive disorders. Methods Recommendations within key topics covering the care pathway for rectosigmoid HSCR were developed by an international workgroup of experts from 8 European countries within ERNICA European Reference Network from the disciplines of surgery, medicine, histopathology, microbiology, genetics, and patient organization representatives. Recommendation statements were based on a comprehensive review of the available literature and expert consensus. AGREE II and GRADE approaches were used during development. Evidence levels and levels of agreement are noted. Results Thirty-three statements within 9 key areas were generated. Most recommendations were based on expert opinion. Conclusion In rare or low-prevalence diseases such as HSCR, there remains limited availability of high-quality clinical evidence. Consensus-based guidelines for care are presented.Peer reviewe