Hematopoietic Cell Transplantation in Patients With Primary Immune Regulatory Disorders (PIRD): A Primary Immune Deficiency Treatment Consortium (PIDTC) Survey.

Primary Immune Regulatory Disorders (PIRD) are an expanding group of diseases caused by gene defects in several different immune pathways, such as regulatory T cell function. Patients with PIRD develop clinical manifestations associated with diminished and exaggerated immune responses. Management of these patients is complicated; oftentimes immunosuppressive therapies are insufficient, and patients may require hematopoietic cell transplant (HCT) for treatment. Analysis of HCT data in PIRD patients have previously focused on a single gene defect. This study surveyed transplanted patients with a phenotypic clinical picture consistent with PIRD treated in 33 Primary Immune Deficiency Treatment Consortium centers and European centers. Our data showed that PIRD patients often had immunodeficient and autoimmune features affecting multiple organ systems. Transplantation resulted in resolution of disease manifestations in more than half of the patients with an overall 5-years survival of 67%. This study, the first to encompass disorders across the PIRD spectrum, highlights the need for further research in PIRD management

Behavior of Hybrid NSM Reinforced and Externally Confined Reinforced Concrete Columns under Eccentric Compression –Experimental and Numerical Studies

The effectiveness of hybrid combination of ne ar surface mounted (NSM) and externally confined (EC) FRP strengthenin g on the performance of RC column elements under uniaxial eccentric compression is investigated. In total, ten short RC column elements were cast. Carbon FRP is used for strengthening due to its inherent stiffness and strength properties on par with other FRP materials. The columns were strengthened using NSM CFRP laminates, EC using CFRP fabrics and their hybrid combi nations. A non-linear finite element model is developed using ABAQUS and the numerical model is calibrated using the experimental results to improve the accuracy of the predict ions. Experimental results revealed that hybrid strengthening of RC columns was able to show a better performance in terms of stiffness, strength, ultimate displacement ductility when compared to other FRP strengthening techniques. The numerical predictions obtained were able to better capture the initial stiffness, peak load and post-peak behavior. Thus, the proposed hybrid strengthening technique for RC columns possess the capability of restoring the loss in stiffness, strength and ductility due to additional bending moment induced by the eccentric compression loading

Bonded overlay strengthening of hollow core slab with and without interface shearkeys connection

Precast Prestressed Hollow Core Slabs (PPHCS) are most commonly used as flooring and roofing elements. Usually, a new layer of concrete is placed on the top of hollow core slabs to create a continuous and levelled surface. The common thickness of this bonded overlay will be around 50 mm to 75 mm deep. The provision of Bonded Overlay (BO) will increase the cracking load and flexural strength of hollow core slab after the full composite action is developed. In the present study, the effect of shear keys at the interface of bonded overlay and hollow core slab is studied. The hollow core slab and bonded overlay is expected to have a full composite action until failure without any interface separation. The dimension of hollow core used in this investigation is 600mm wide, 150mm depth and 3500mm length. In total, three full-scale hollow core slabs were tested under shear span (a) to depth (d) ratio of 7.5. The three specimens which include un-strengthened slab denoted as control slab, slab strengthened with bonded overlay without any shear keys at the interface and bonded overlay with shear keys. Bonded overlay specimens without shear keys resulted in interfacial failure and it was able to increase the peak load by 38.4% compared to the control specimen. However, the bonded overlay with shear keys resulted in full composite action till the final failure and it was able to increase the peak load by 59.6% compared to the control specimen. The provision of shear keys at the interface of hollow core slabs and bonded overlay resulted in full composite action

Efficacy of Antenatal Education on childbirth in last decade – A Systematic Review

Background: Antenatal education classes are intended to improve health behaviors and deliver comprehensive information about the psychological and physical changes that occur during pregnancy. These classes have long been a vital element of antenatal care services. Antenatal education should be standardized to clearly define its actual impact on both mental and physical health. The implementation of structured antenatal education programs is essential for gaining a better understanding of the relationship between prenatal care and maternal and infant health outcomes.Aim: To summarize the various antenatal education or classes in different country practices and its impact on childbirth outcomes in the last decade.Methods: We conducted a search for relevant literature published in English from 2013 to 2024 related to antenatal education. This systematic review included articles involving pregnant women willing to participate in antenatal or childbirth education, as well as studies on antenatal education guidelines published within the specified timeframe. Our search strategy yielded approximately 93 articles. The reviewers screened these articles based on eligibility criteria, excluding 67 due to irrelevant data, conclusions, or failure to meet our study\u27s criteria. Ultimately, 26articles were included in this systematic review.Conclusion: This systematic review concludes that the existing evidence base for antenatal education is inconclusive. However, emerging evidence from future well-conducted and well-reported trials may help establish definitive conclusions

Development and application of C - scan ultrasonic facility

This paper presents the in-house development and application of a C-scan ultrasonic facility ULTIMA 200M2 at the Indira Gandhi Centre for Atomic Research (IGCAR), Kalpakkam, carried out in collaboration with the Electronics Division, Bhabha Atomic Research Centre (BARC), Mumbai. The paper describes various constituents of the system developed and also highlights the typical results obtained using this system, including bond integrity assessment of explosive welds and imaging of fuel sub-assembly heads of the Fast Breeder Test Reactor. The system has also been used for imaging both the sides of a one rupee Indian coin. All the finer details of the coin could be extracted, demonstrating the resolution capabilities of the system

Chronic Granulomatous Disease-Associated IBD Resolves and Does Not Adversely Impact Survival Following Allogeneic HCT.

IntroductionInflammatory bowel disease (IBD) affects approximately 1/3 of patients with chronic granulomatous disease (CGD). Comprehensive investigation of the effect of allogeneic hematopoietic cell transplantation (HCT) on CGD IBD and the impact of IBD on transplant outcomes is lacking.MethodsWe collected data retrospectively from 145 patients with CGD who had received allogeneic HCT at 26 Primary Immune Deficiency Treatment Consortium (PIDTC) centers between January 1, 2005 and June 30, 2016.ResultsForty-nine CGD patients with IBD and 96 patients without IBD underwent allogeneic HCT. Eighty-nine percent of patients with IBD and 93% of patients without IBD engrafted (p = 0.476). Upper gastrointestinal acute GVHD occurred in 8.5% of patients with IBD and 3.5% of patients without IBD (p = 0.246). Lower gastrointestinal acute GVHD occurred in 10.6% of patients with IBD and 11.8% of patients without IBD (p = 0.845). The cumulative incidence of acute GVHD grades II-IV was 30% (CI 17-43%) in patients with IBD and 20% (CI 12-29%) in patients without IBD (p = 0.09). Five-year overall survival was equivalent for patients with and without IBD: 80% [CI 66-89%] and 83% [CI 72-90%], respectively (p = 0.689). All 33 surviving evaluable patients with a history of IBD experienced resolution of IBD by 2 years following allogeneic HCT.ConclusionsIn this cohort, allogeneic HCT was curative for CGD-associated IBD. IBD should not contraindicate HCT, as it does not lead to an increased risk of mortality. This study is registered at clinicaltrials.gov NCT02082353

Hematopoietic Cell Transplantation in Patients With Primary Immune Regulatory Disorders (PIRD): A Primary Immune Deficiency Treatment Consortium (PIDTC) Survey

\ua9 Copyright \ua9 2020 Chan, Leiding, Liu, Logan, Burroughs, Allenspach, Skoda-Smith, Uzel, Notarangelo, Slatter, Gennery, Smith, Pai, Jordan, Marsh, Cowan, Dvorak, Craddock, Prockop, Chandrakasan, Kapoor, Buckley, Parikh, Chellapandian, Oshrine, Bednarski, Cooper, Shenoy, Davila Saldana, Forbes, Martinez, Haddad, Shyr, Chen, Sullivan, Heimall, Wright, Bhatia, Cuvelier, Goldman, Meyts, Miller, Seidel, Vander Lugt, Bacchetta, Weinacht, Andolina, Caywood, Chong, de la Morena, Aquino, Shereck, Walter, Dorsey, Seroogy, Griffith, Kohn, Puck, Pulsipher and Torgerson.Primary Immune Regulatory Disorders (PIRD) are an expanding group of diseases caused by gene defects in several different immune pathways, such as regulatory T cell function. Patients with PIRD develop clinical manifestations associated with diminished and exaggerated immune responses. Management of these patients is complicated; oftentimes immunosuppressive therapies are insufficient, and patients may require hematopoietic cell transplant (HCT) for treatment. Analysis of HCT data in PIRD patients have previously focused on a single gene defect. This study surveyed transplanted patients with a phenotypic clinical picture consistent with PIRD treated in 33 Primary Immune Deficiency Treatment Consortium centers and European centers. Our data showed that PIRD patients often had immunodeficient and autoimmune features affecting multiple organ systems. Transplantation resulted in resolution of disease manifestations in more than half of the patients with an overall 5-years survival of 67%. This study, the first to encompass disorders across the PIRD spectrum, highlights the need for further research in PIRD management

Current treatment in macrophage activation syndrome worldwide: a systematic literature review to inform the METAPHOR project

Objective: To assess current treatment in macrophage activation syndrome (MAS) worldwide and to highlight any areas of major heterogeneity of practice. Methods: A systematic literature search was performed in both EMBASE and PubMed databases. Paper screening was done by two independent teams based on agreed criteria. Data extraction was standardized following the PICO framework. A panel of experts assessed paper validity, using the Joanna Briggs Institute appraisal tools and category of evidence (CoE) according to EULAR procedure. Results: Fifty-seven papers were finally included (80% retrospective case-series), describing 1148 patients with MAS: 889 systemic juvenile idiopathic arthritis (sJIA), 137 systemic lupus erythematosus (SLE), 69 Kawasaki disease (KD) and 53 other rheumatological conditions. Fourteen and 11 studies specified data on MAS associated to SLE and KD, respectively. All papers mentioned glucocorticoids (GCs), mostly methylprednisolone and prednisolone (90%); dexamethasone was used in 7% of patients. Ciclosporin was reported in a wide range of patients according to different cohorts. Anakinra was used in 179 MAS patients, with a favourable outcome in 83% of sJIA-MAS. Etoposide was described by 11 studies, mainly as part of HLH-94/04 protocol. Emapalumab was the only medication tested in a clinical trial in 14 sJIA-MAS, with 93% of MAS remission. Ruxolitinib was the most reported Janus kinase inhibitor in MAS. Conclusion: High-dose GCs together with IL-1 and IFN gamma inhibitors have shown efficacy in MAS, especially in sJIA-associated MAS. However, the global level of evidence on MAS treatment, especially in other conditions, is still poor and requires standardized studies to be confirme