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

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

Experimental and Numerical Investigation on the Shear Behavior of Engineered Cementitious Composite Beams with Hybrid Fibers

The shear behavior of innovative engineered cementitious composites (ECC) members with a hybrid mix of polyvinyl alcohol (PVA) and polypropylene (PP) fibers is examined. The overall objective of the investigation is to understand the shear behavior of ECC beams with different mono and hybrid fiber combinations without compromising the strength and ductility. Four different configurations of beams were prepared and tested, including 2.0% of PP fibers, 2.0% of PVA fibers, 2.0% of steel fibers and hybrid PVA and PP fibers (i.e., 1% PP and 1% PVA). In addition to the tests, a detailed nonlinear finite element (FE) analysis was accomplished using the commercial ABAQUS software. The validated FE model was used to perform an extensive parametric investigation to optimize the design parameters for the hybrid-fiber-reinforced ECC beams under shear. The results revealed that the use of hybrid PVA and PP fibers improved the performance by enhancing the overall strength and ductility compared to the steel and PP-fiber-based ECC beams. Incorporating hybrid fibers into ECC beams increased the critical shear crack angle, indicating the transition of a failure from a brittle diagonal tension to a ductile bending

Bisphosphonates in Langerhans cell histiocytosis: An international retrospective case series

Background: Bone is the most common organ of involvement in patients with Langerhans cell histiocytosis (LCH), which is often painful and associated with significant morbidity from pathological fractures. Current first-line treatments include chemotherapy and steroids that are effective but often associated with adverse effects, whereas the disease may reactivate despite an initial response to first-line agents. Bisphosphonates are osteoclast inhibitors that have shown to be helpful in treating bone lesions of LCH. To date, there are no large international studies to describe their role in treating bone lesions of LCH. Method: We conducted a multicenter retrospective review of 13 patients with histologically proven LCH, who had received bisphosphonates either at diagnosis or at disease reactivation. Results: Ten patients (77%) had a single system bone disease, and 3 (23%) had bone lesions as part of multisystem disease. Median follow-up time post-bisphosphonate therapy was 4.6 years (range, 0.8 to 8.2 years). Treatment with bisphosphonates was associated with significant pain relief in almost all patients. Twelve (92%) achieved resolution of active bone lesions, and 10 out of them had no active disease for a median of 3.5 years (range, 0.8 to 5 years). One patient did not respond. No major adverse effects were reported in this series. Conclusion: Bisphosphonates are well-tolerated drugs that can significantly improve bone pain and induce remission in active bone LCH. Future prospective studies evaluating the role of bisphosphonates in LCH are warranted. © 2016

Bisphosphonates in Langerhans Cell Histiocytosis: An International Retrospective Case Series

Bone is the most common organ of involvement in patients with Langerhans cell histiocytosis (LCH), which is often painful and associated with significant morbidity from pathological fractures. Current first-line treatments include chemotherapy and steroids that are effective but often associated with adverse effects, whereas the disease may reactivate despite an initial response to first-line agents. Bisphosphonates are osteoclast inhibitors that have shown to be helpful in treating bone lesions of LCH. To date, there are no large international studies to describe their role in treating bone lesions of LCH. We conducted a multicenter retrospective review of 13 patients with histologically proven LCH, who had received bisphosphonates either at diagnosis or at disease reactivation. Ten patients (77%) had a single system bone disease, and 3 (23%) had bone lesions as part of multisystem disease. Median follow-up time post-bisphosphonate therapy was 4.6 years (range, 0.8 to 8.2 years). Treatment with bisphosphonates was associated with significant pain relief in almost all patients. Twelve (92%) achieved resolution of active bone lesions, and 10 out of them had no active disease for a median of 3.5 years (range, 0.8 to 5 years). One patient did not respond. No major adverse effects were reported in this series. Bisphosphonates are well-tolerated drugs that can significantly improve bone pain and induce remission in active bone LCH. Future prospective studies evaluating the role of bisphosphonates in LCH are warrante