Event-free survival of maralixibat-treated patients with Alagille syndrome compared to a real-world cohort from GALA.

BACKGROUND AND AIMS: Alagille syndrome (ALGS) is characterized by chronic cholestasis with associated pruritus and extrahepatic anomalies. Maralixibat, an ileal bile acid transporter inhibitor, is an approved pharmacologic therapy for cholestatic pruritus in ALGS. Since long-term placebo-controlled studies are not feasible or ethical in children with rare diseases, a novel approach was taken comparing 6-year outcomes from maralixibat trials with an aligned and harmonized natural history cohort from the G lobal AL agille A lliance (GALA) study. APPROACH AND RESULTS: Maralixibat trials comprise 84 patients with ALGS with up to 6 years of treatment. GALA contains retrospective data from 1438 participants. GALA was filtered to align with key maralixibat eligibility criteria, yielding 469 participants. Serum bile acids could not be included in the GALA filtering criteria as these are not routinely performed in clinical practice. Index time was determined through maximum likelihood estimation in an effort to align the disease severity between the two cohorts with the initiation of maralixibat. Event-free survival, defined as the time to first event of manifestations of portal hypertension (variceal bleeding, ascites requiring therapy), surgical biliary diversion, liver transplant, or death, was analyzed by Cox proportional hazards methods. Sensitivity analyses and adjustments for covariates were applied. Age, total bilirubin, gamma-glutamyl transferase, and alanine aminotransferase were balanced between groups with no statistical differences. Event-free survival in the maralixibat cohort was significantly better than the GALA cohort (HR, 0.305; 95% CI, 0.189-0.491; p <0.0001). Multiple sensitivity and subgroup analyses (including serum bile acid availability) showed similar findings. CONCLUSIONS: This study demonstrates a novel application of a robust statistical method to evaluate outcomes in long-term intervention studies where placebo comparisons are not feasible, providing wide application for rare diseases. This comparison with real-world natural history data suggests that maralixibat improves event-free survival in patients with ALGS.

Neighborhood-Level Deprivation Impacts Graft and Patient Outcomes Among Pediatric Liver Transplant Recipients.

BACKGROUND: The impact of social determinants of health (SDOH) on pediatric liver transplant outcomes has not been extensively investigated. The purpose of this study was to examine the effect of neighborhood-level deprivation on graft and patient survival among pediatric liver transplant recipients. METHODS: This is a single-center observational study among children who received a primary liver transplant from March 1996 to March 2023. The Childhood Opportunity Index (COI) score was used to measure the degree of neighborhood deprivation. RESULTS: A total of 252 children were included, half (52.8%) were female and the majority were non-Hispanic White (71%). Half (53.6%) of the children had private insurance, and the median COI score was 64. A total of 28 children were transplanted from a very low-to-low COI area. Children in very low-to-low COI areas had worse patient (HR = 4.90, 95% CI = 1.41-17.04, p = 0.01) and graft (HR = 3.41, 95% CI = 1.19-9.75, p = 0.02) survival outcomes compared to children in moderate-to-very high areas when adjusting for clinical characteristics. COI score remained a significant predictor of graft survival after adjusting for race and insurance type (HR = 3.41, 95% CI = 1.12-10.40, p = 0.03). CONCLUSIONS: Pediatric liver transplant recipients from very low-to-low COI score areas have worse graft outcomes when accounting for clinical factors, race/ethnicity, and insurance status. Future research should investigate the impact of multiple, intersecting SDOH factors, rather than solely a collection of isolated variables.

Rapid Genome Sequencing Diagnosis in Pediatric Patients with Liver Dysfunction.

OBJECTIVE: To describe the usefulness of rapid whole genome sequencing (rWGS) in a cohort of children presenting with acute liver dysfunction. STUDY DESIGN: This was a retrospective, population-based cohort study conducted at Primary Children's Hospital in Salt Lake City, Utah. Children meeting criteria for acute liver dysfunction who received rWGS between August 2019 and December 2021 were included. rWGS was performed on blood samples from the patient and parents (1 or both depending on availability). The clinical characteristics of patients with positive rWGS results were compared with those with negative results. RESULTS: Eighteen patients with pediatric acute liver dysfunction who had rWGS were identified. The median turnaround time from the date rWGS testing was ordered to the date an initial report was received was 8 days with a shorter turnaround time in patients with a diagnostic rWGS (4 days vs 10 days; P = .03). A diagnostic result was identified in 7 of 18 patients (39%). Subsequently, 4 patients in this cohort, who had negative rWGS results, were found to have a toxic exposure accounting for their liver dysfunction. With removal of these patients, the diagnostic rate of rWGS was 7 of 14 (50%). The use of rWGS led to a change in management for 6 of 18 patients (33%). CONCLUSIONS: We found that rWGS provided a diagnosis in up to 50% of pediatric acute liver dysfunction. rWGS allows for higher diagnostic rates in an expedited fashion that affects clinical management. These data support the routine use of rWGS for life-threatening disorders in children, specifically acute liver dysfunction.

Fractionated Bilirubin Among 252 892 Utah Newborns with and Without Biliary Atresia: A 15-year Historical Birth Cohort Study.

OBJECTIVES: To determine whether neonatal conjugated or direct bilirubin levels were elevated in infants with biliary atresia (BA) and to estimate the number of newborns who would have positive screens in the nursery necessitating repeat testing after discharge. STUDY DESIGN: We used administrative data from a large integrated healthcare network in Utah to identify newborns who had a fractionated bilirubin recorded during birth admission from 2005 through 2019. Elevated conjugated bilirubin was defined as greater than 0.2 mg/dL and direct bilirubin was defined as greater than 0.5 mg/dL (>97.5th percentile for the assays). We performed simulations to estimate the anticipated number of false-positive screens. RESULTS: There were 32 cases of BA and 468 161 live births during the study period (1/14 700). There were 252 892 newborns with fractionated bilirubin assessed, including 26 of those subsequently confirmed to have BA. Conjugated or direct bilirubin was elevated in all 26 infants with BA and an additional 3246 newborns (1.3%) without BA. Simulated data suggest 9-21 per 1000 screened newborns will have an elevated conjugated or direct bilirubin using laboratory-based thresholds for a positive screen. Screening characteristics improved with higher thresholds without increasing false-negative tests. CONCLUSIONS: This study validates the previous findings that conjugated or direct bilirubin are elevated in the newborn period in patients with BA. A higher threshold for conjugated bilirubin improved screening performance. Future studies are warranted to determine the optimal screening test for BA and to assess the effectiveness and cost-effectiveness of implementing such a program.

CD137 deficiency causes immune dysregulation with predisposition to lymphomagenesis.

Dysregulated immune responses are essential underlying causes of a plethora of pathologies including cancer, autoimmunity, and immunodeficiency. We here investigated 4 patients from unrelated families presenting with immunodeficiency, autoimmunity, and malignancy. We identified 4 distinct homozygous mutations in TNFRSF9 encoding the tumor necrosis factor receptor superfamily member CD137/4-1BB, leading to reduced, or loss of, protein expression. Lymphocytic responses crucial for immune surveillance, including activation, proliferation, and differentiation, were impaired. Genetic reconstitution of CD137 reversed these defects. CD137 deficiency is a novel inborn error of human immunity characterized by lymphocytic defects with early-onset Epstein-Barr virus (EBV)-associated lymphoma. Our findings elucidate a functional role and relevance of CD137 in human immune homeostasis and antitumor responses.

Quantitative Liver Fibrosis Using Collagen Hybridizing Peptide to Predict Native Liver Survival in Biliary Atresia: A Pilot Study.

BACKGROUND/RATIONALE: Biliary atresia (BA) is a cholangiopathy characterized by bile flow obstruction due to destruction of the biliary tree. Without surgical correction with Kasai portoenterostomy (KPE), BA leads to death or liver transplant (LTx). Early-onset, progressive liver fibrosis is a defining characteristic of BA. Collagen hybridizing peptide (CHP) is a synthetic peptide which binds to denatured collagen strands allowing quantification of fibrosis. This technique has not been used on human liver tissue. The aim of this pilot study was to evaluate the utility of CHP as a measurement of quantitative fibrosis to allow earlier survival with native liver prognostication. RESULTS: We identified 21 patients with wedge liver biopsies available, of which 14 required LTx. No deaths occurred. Patients requiring LTx tended to be girls with a significantly different mean bilirubin (P = 0.002), albumin (P = 0.001), and alanine aminotransferase (P = 0.03) at 3 months post-KPE. By 1 year post-KPE, 50% of patients in the high CHP intensity group required LTx versus 27% in the low CHP. Overall, fibrosis as quantified by CHP at time of KPE was associated with more than 3 times the risk of requiring LTx by 4 years of age (hazard ratio 3.6, 95% confidence interval 1.15-10.93, P = 0.03). When controlling for sex and total bilirubin >2 mg/dL and albumin at 3 months post-KPE, it predicted nearly 7 times the risk of LTx (hazard ratio 6.89, 95% confidence interval 1.38-34.32, P = 0.02). CONCLUSION: Our results suggest that quantitative assessment of fibrosis at the time of KPE holds promise as an earlier predictor of LTx requirement in BA. A larger study is justified to assess quantitative fibrosis as a BA prognostic tool.

Dissecting the localization of lipopolysaccharide-responsive and beige-like anchor protein (LRBA) in the endomembrane system.

INTRODUCTION AND OBJECTIVES: LRBA deficiency is caused by loss of LRBA protein expression, due to either homozygous or compounds heterozygous mutations in LRBA. LRBA deficiency has been shown to affect vesicular trafficking and autophagy. To date, LRBA has been observed in the cytosol, Golgi apparatus and some lysosomes in LPS-stimulated murine macrophages. The objectives of the present study were to study the LRBA localization in organelles involved in vesicular traffic, phagocytosis, and autophagy in mononuclear phagocytes (MP). MATERIALS AND METHODS: We analyzed LRBA colocalization with different endosomes markets using confocal microscopy in MP. We used the autophagy inhibitors to determine the role of LRBA in formation, maturation or degradation of the autophagosome. RESULTS: LRBA intracellular trafficking depends on the activity of the GTPase ADP ribosylation factor-1 (ARF) in MP. LRBA was identified in early, late endosomes but did not colocalize strongly with lysosomal markers. Although LRBA appears not to be recruited during the phagocytic cargo uptake, it greatly colocalized with the microtubule-associated protein 1A/1B-light chain 3 (LC3) under a steady state and this decreased after the induction of autophagy flux. Although the use of inhibitors of lysosome fusion did not restore the LRBA/LC3 colocalization, inhibitors of either early to late endosomes trafficking or PI3K pathway did. CONCLUSIONS: Taken together, our results show that LRBA is located in endomembrane system vesicles, mainly in the early and late endosomes. Although LRBA appears not to be involved in the phagocytic uptake, it is recruited in the early steps of the autophagy flux.

Acute cor pulmonale following uterine artery embolization.

Uterine artery embolization (UAE) is a common procedure for controlling gynecological and obstetric bleeding. We report a case of a 38-year-old female with placenta increta who underwent UAE following a cesarean section and subsequently developed pulmonary embolism from nontarget embolization. Excellent CTPA images demonstrate embolization agents used in the procedure in the pulmonary vasculature. The patient developed acute cor pulmonale, an infrequent complication from the procedure. This case presents a rare and clear demonstration of a complication of uterine artery embolization with clear imaging evidence.

High-density pulmonary lesions: Review in chest imaging.

High-density pulmonary lesions are frequently seen in chest imaging, and it is important to identify their different causes. Radiologists must be able to distinguish between common and rare conditions in order to provide the best diagnosis and treatment. This article provides an overview of the various causes and imaging features of high-density lesions in the lungs. The lesions are classified into various categories, such as pulmonary nodules, inflammatory conditions, deposition diseases, contrast-related lesions, and thoracic devices. A clear understanding of these categories can help radiologists accurately diagnose and manage high-density pulmonary lesions encountered in practice.

Multifocal Mycotic Aneurysms and Pseudoaneurysms.

Mycotic aneurysms and pseudoaneurysms, though rare, present significant diagnostic and therapeutic challenges. The case follows a 74-year-old male with a history of bladder cancer who developed multifocal mycotic aneurysms and pseudoaneurysms following sepsis. Initially misdiagnosed as a Pancoast tumor, imaging revealed an extensive disease involving the right subclavian artery, proximal descending thoracic aorta, infrarenal abdominal aorta, and right common iliac artery. This case highlights the importance of considering mycotic aneurysms in the differential diagnosis of patients with a history of infection and highlights the role of Computed Tomography Angiography in early diagnosis.

The Impact of Signaling in Diagnostic Radiology Residency.

Signaling in the US Residency MATCH process allows medical students to indicate preference for specific programs. In diagnostic radiology, "Gold" and "Silver" signals significantly increase the chances of securing interviews from targeted programs. Specialty advisors should strategically evaluate applicant competitiveness and program desirability to optimize signal placement. METHODS: This study included fourth-year medical students and US or Canadian medical school graduates applying for the 2024 Match. Participants completed a survey on signaling use, interview invitations, and the resources and advice received. RESULTS: Interview invitations for programs with gold, silver, and non-signaled programs were analyzed using the Wilcoxon signed-rank test and generalized estimating equation models. CONCLUSION: Signaling significantly increases the likelihood of receiving interviews in diagnostic radiology, emphasizing the importance of strategic signal placement. Further research is needed to assess its overall impact on the MATCH process.

Treatment Options for Failed Back Surgery Syndrome: An Umbrella Systematic Review of Systematic Reviews on the Effectiveness of Therapeutic Interventions.

Background: Failed back surgery syndrome (FBSS) is a common and incapacitating condition affecting patients with previous spine surgery in whom treatment approach can be challenging. This study aimed to summarize existing secondary studies and up-to-date randomized clinical trials (RCTs) that assess the effectiveness of available treatment options for FBSS. Methods: Systematic searches were carried out in five databases (PubMed, Cochrane, Scielo, Epistemonikos, and Google scholar) for all systematic reviews on the effectiveness of treatment options for FBSS published after 2012. Outcomes of interest were pain levels measured through visual analog scale or numeric rating scale, Oswestry Disability Index, and quality of life. Methodological and risk of bias assessments were performed with the AMSTAR-2 tool for systematic reviews and the Joanna Briggs Institute checklist for RCT. Prospective PROSPERO registration: CRD42022307609. Results: Fifteen studies, seven systematic reviews, and eight RCTs met the inclusion criteria and fulfilled the methodological quality assessment. Of the 15 included studies, 8 were on neurostimulation, 4 on adhesiolysis, 4 on epidural or intrathecal injections, and 3 on other treatment modalities. The risk of bias was low in seven studies, moderate in five, and high in three. Conclusions: Based on this systematic overview and the considerable heterogeneity among studies, the FBSS therapeutic approach must be individualized. FBSS treatment should start with conservative management, considering the implementation of neurostimulation, a technique with the most robust evidence of effective results, in cases of refractory axial or neuropathic pain. As the last resource, in light of the evidence found, more invasive procedures or new surgical interventions are indicated.

Flexible Sigmoidoscopy Utility in the Diagnosis of Pediatric Gastrointestinal Disorders.

AIM:  Although flexible sigmoidoscopy (FS) is utilized in children for the diagnosis of pediatric gastrointestinal conditions, such as inflammatory bowel disease and juvenile polyp disorders, the diagnostic yield of FS in pediatric patients is unknown. MATERIALS AND METHODS:  We retrospectively reviewed FS cases in children under 18 years of age over a five-year period at our institution. Indications for the procedure, endoscopic visual findings, histologic findings, final diagnosis, and any management changes based on FS findings were included. RESULTS:  A total of 354 cases were included in the analysis for which 40 cases (11.3%) had abnormal visual findings, 48 cases (13.6%) had abnormal histologic findings, and 13 cases (3.7%) had both abnormal endoscopic visual and histologic findings. Of the 88 cases with abnormal visual and/or histologic abnormalities, only the results of 34 of these FS cases led to a change in management based on endoscopic findings (9.6%). Most patients with a non-diagnostic FS had a final diagnosis of functional abdominal pain; most patients with a diagnostic FS had a final diagnosis of colitis, not otherwise specified. CONCLUSION:  Our findings suggest that FS is not a helpful diagnostic endoscopic intervention in pediatric patients, especially in children with reassuring history and physical exam findings.

Education and Training Adaptations for Health Workers during the COVID-19 Pandemic: A Scoping Review of Lessons Learned and Innovations.

Introduction: The COVID-19 pandemic has considerably impacted the clinical education and training of health workers globally, causing severe disruptions to learning environments in healthcare facilities and limiting the acquisition of new clinical skills. Consequently, urgent adaptation measures, including simulation training and e-learning, have been implemented to mitigate the adverse effects of clinical education. This scoping review aims to assess the impact of COVID-19 on medical education and training, examine the implemented adaptation measures, and evaluate their effectiveness in improving health workers' education and training during the pandemic. Methods: Employing the PRISMA-ScR framework and Arksey and O'Malley's methodological guidance, we conducted a scoping review, systematically searching PubMed, medRxiv, Google, and DuckDuckGo databases to account for the grey literature. The search included studies published between 1 December 2019 and 13 October 2021, yielding 10,323 results. Of these, 88 studies focused on health worker education and training during the pandemic. Results: Our review incorporated 31,268 participants, including physicians, medical trainees, nurses, paramedics, students, and health educators. Most studies (71/88, 81%) were conducted in high-income and lower-middle-income countries. The pandemic's effects on health workers' clinical skills and abilities have necessitated training period extensions in some cases. We identified several positive outcomes from the implementation of simulation training and e-learning as adaptation strategies, such as enhanced technical and clinical performance, increased confidence and comfort, and an expanded global educational outreach. Conclusions: Despite challenges like insufficient practical experience, limited interpersonal interaction opportunities, and internet connectivity issues, simulation training, e-learning, and virtual training have proven effective in improving clinical education and training during the COVID-19 pandemic. Further research is required to bolster preparedness for future pandemics or similar situations.

Strengthening national public health institutes: a systematic review on institution building in the public sector.

Introduction: Strong and efficient institutions are vital to the development of well-functioning governments and strong societies. The term "institution building" encompasses the creation, support, development, and strengthening of organizations and institutions. Still, there is little aggregated evidence on "institution building" considering a wider system-thinking approach, best practices, or development cooperation specifically in the field of public health. In 2007, the International Association of National Public Health Institutes (IANPHI) created a guiding Framework that countries may use for developing National Public Health Institutes (NPHIs). This Framework is currently being revised. Methods: In this context, we conducted a systematic review to facilitate this revision with recent evidence on institution building and its potential contribution to NPHI. We followed the PRISMA guidelines for systematic reviews, searching for relevant publications in seven scientific databases (Pubmed, VHL/LILACS, EconLit, Google Scholar, Web of Science, World Affairs Online, ECONBIZ) and four libraries (World Bank; European Health for All database of the World Health Organization European Region, WHO; Organization for Economic Cooperation and Development, OECD; and the African Union Common Repository). The search was carried out in October 2021. We used the "framework analysis" tool for systematically processing documents according to key themes. Results: As a result, we identified 3,015 records, of which we included 62 documents in the final review. This systematic review fills a major gap of aggregated information on institution building in the field of public health and National Public Health Institutes. It is to our knowledge the first systematic review of this kind. The overriding result is the identification and definition of six domains of institution building in the health sector: "governance," "knowledge and innovation," "inter-institutional cooperation," "monitoring and control," "participation," and "sustainability and context-specific adaptability." Discussion: Our results show that the described domains are highly relevant to the public health sector, and that managers and the scientific community recognize their importance. Still, they are often not applied consistently when creating or developing NPHIs. We conclude that organizations engaged in institution building of NPHIs, including IANPHI, may greatly benefit from state-of-the-art research on institution building as presented in this study.

Prevalence, management and efficacy of treatment in portal vein obstruction after paediatric liver transplantation: protocol of the retrospective international multicentre PORTAL registry.

INTRODUCTION: Portal vein obstruction (PVO) consists of anastomotic stenosis and thrombosis, which occurs due to a progression of the former. The aim of this large-scale international study is to assess the prevalence, current management practices and efficacy of treatment in patients with PVO. METHODS AND ANALYSIS: The Portal vein Obstruction Revascularisation Therapy After Liver transplantation registry will facilitate an international, retrospective, multicentre, observational study, with 25 centres around the world already actively involved. Paediatric patients (aged <18 years) with a diagnosed PVO between 1 January 2001 and 1 January 2021 after liver transplantation will be eligible for inclusion. The primary endpoints are the prevalence of PVO, primary and secondary patency after PVO intervention and current management practices. Secondary endpoints are patient and graft survival, severe complications of PVO and technical success of revascularisation techniques. ETHICS AND DISSEMINATION: Medical Ethics Review Board of the University Medical Center Groningen has approved the study (METc 2021/072). The results of this study will be disseminated via peer-reviewed publications and scientific presentations at national and international conferences. TRIAL REGISTRATION NUMBER: Netherlands Trial Register (NL9261).

Safety and Immunogenicity of Live Viral Vaccines in a Multicenter Cohort of Pediatric Transplant Recipients.

Importance: Live vaccines (measles-mumps-rubella [MMR] and varicella-zoster virus [VZV]) have not been recommended after solid organ transplant due to concern for inciting vaccine strain infection in an immunocompromised host. However, the rates of measles, mumps, and varicella are rising nationally and internationally, leaving susceptible immunocompromised children at risk for life-threating conditions. Objective: To determine the safety and immunogenicity of live vaccines in pediatric liver and kidney transplant recipients. Design, Setting, and Participants: This cohort study included select pediatric liver and kidney transplant recipients who had not completed their primary MMR and VZV vaccine series and/or who displayed nonprotective serum antibody levels at enrollment between January 1, 2002, and February 28, 2023. Eligibility for live vaccine was determined by individual US pediatric solid organ transplant center protocols. Exposures: Exposure was defined as receipt of a posttransplant live vaccine. Transplant recipients received 1 to 3 doses of MMR vaccine and/or 1 to 3 doses of VZV vaccine. Main Outcome and Measure: Safety data were collected following each vaccination, and antibody levels were obtained at 0 to 3 months and 1 year following vaccination. Comparisons were performed using Mann-Whitney U test, and factors associated with development of postvaccination protective antibodies were explored using univariate analysis. Results: The cohort included 281 children (270 [96%] liver, 9 [3%] kidney, 2 [1%] liver-kidney recipients) from 18 centers. The median time from transplant to enrollment was 6.3 years (IQR, 3.4-11.1 years). The median age at first posttransplant vaccine was 8.9 years (IQR, 4.7-13.8 years). A total of 202 of 275 (73%) children were receiving low-level monotherapy immunosuppression at the time of vaccination. The majority of children developed protective antibodies following vaccination (107 of 149 [72%] varicella, 130 of 152 [86%] measles, 100 of 120 [83%] mumps, and 124 of 125 [99%] rubella). One year post vaccination, the majority of children who initially mounted protective antibodies maintained this protection (34 of 44 [77%] varicella, 45 of 49 [92%] measles, 35 of 42 [83%] mumps, 51 of 54 [94%] rubella). Five children developed clinical varicella, all of which resolved within 1 week. There were no cases of measles or rubella and no episodes of graft rejection within 1 month of vaccination. There was no association between antibody response and immunosuppression level at the time of vaccination. Conclusions and Relevance: The findings suggest that live vaccinations may be safe and immunogenic after solid organ transplant in select pediatric recipients and can offer protection against circulating measles, mumps, and varicella.

Telemedicine in Low- and Middle-Income Countries During the COVID-19 Pandemic: A Scoping Review.

Background: COVID-19 has impacted the capacity of healthcare systems worldwide, particularly in low- and middle-income countries (LMICs), which are already under strain due to population growth and insufficient resources. Since the COVID-19 pandemic's emergence, there has been an urgent need for a rapid and adequate reaction to the pandemic's disruption of healthcare systems. To this end, telemedicine has been shown in prior research to be a feasible approach. The overarching objective of this scoping review was to determine the extent and acceptance of telemedicine in healthcare in low- and middle-income countries (LMICs) during the COVID-19 pandemic. Methods: This scoping review followed PRISMA guidelines and Arksey and O'Malley's five-stage framework to identify available evidence. We systematically searched four academic databases for peer-reviewed literature published between January 2020 and April 2021: Medline, PubMed, Web of Science, and Scopus, as well as Google Scholar as a source for grey literature. Results: The search identified 54 articles with 45,843 participants, including 6,966 healthcare professionals and 36,877 healthcare users. We identified a range of reasons for introducing telemedicine in LMICs during COVID-19, most notably to maintain non-emergency healthcare, enhance access to healthcare providers, and reduce the risk of infection among health users and providers. Overall, healthcare providers and users have shown a high level of acceptance for telemedicine services. During the COVID-19 pandemic, telemedicine provided access to healthcare in the majority of included articles. Nonetheless, some challenges to accepting telemedicine as a method of healthcare delivery have been reported, including technological, regulatory, and economical challenges. Conclusion: Telemedicine was found to improve access to high-quality healthcare and decrease infection risk in LMICs during COVID-19. In general, infrastructure and regulatory barriers found to be the most significant barriers to wider telemedicine use, and should be considered when implementing telemedicine more broadly. There appears to be a need to prioritize patient data safety, as many healthcare practitioners utilized commercial apps and services as telemedicine systems. Additionally, it appears as though there is a need to increase capacity, skill, and transparency, as well as to educate patients about telemedicine.

Clinical diagnostic predictive score for Meckel diverticulum.

BACKGROUND/PURPOSE: Meckel diverticulum (MD) is present in 2% of the population. Many practitioner feel the diagnosis relies upon technetium-99 m pertechnetate scintigraphy. When negative, patients undergo additional invasive procedures delaying definitive therapy. This study aims to identify features of bleeding MD and generate a risk score, which could preclude unnecessary testing and facilitate earlier operation. METHODS: All patients <18-years-old who presented with hematochezia from 2005 to 2015 were identified. MD diagnosis was based on histopathology of operative tissue. Controls were patients with hematochezia undergoing colonoscopy. A points system was used generate a risk score. RESULTS: A total of 215 patients presented with hematochezia out of which 42 patients with MD were identified. Predictive variables included infant (OR 7, 95%CI 2-29) and toddler (OR 20, 95%CI 8-50) age groups, duration <6 days (OR 18, 95%CI 8-43), presence of large blood volume (OR 16, 95% CI 7-36), hemoglobin <7 g/dL (OR 6, 95% CI 3-15) and transfusion requirement (OR 16, 95% CI 7-38). A score of 6 or higher is highly suggestive of MD. CONCLUSIONS: This scoring system identifies children with bleeding MD who may benefit from exploratory surgery without undergoing endoscopy. This novel scoring system can be applied to provide accurate clinical diagnosis, reduce unnecessary tests and allow prompt surgical management.