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

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

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

Evaluation of Urea Treated Acacia nilotica Pods as a Feed for Grower Lambs

Background: Acacia pods are non-conventional protein rich feed resources that could be harnessed as a feed ingredient in sheep production. Though many workers have observed the utility of raw Acacia nilotica pods as a noteworthy feed for small ruminants, the information on nutritive value of urea treated pods is lacking. Hence the present study was undertaken to evaluate the nutritional potential of urea treated Acacia nilotica pods at different dietary levels for supporting growth and nutrient utilisation in lambs. Methods: Acacia nilotica pods were collected, sun dried and ground in a hammer mill. The ground pods and seeds were treated in bulk with urea. Then the urea treated pods (UTP) was incorporated in three experimental concentrate mixtures @ 0, 15 or 30 per cent (R1, R2, R3) replacing deoiled rice bran. The concentrate mixtures were made isoproteinic and isocaloric. Napier Bajra hybrid grass (CNCO4) was provided as the basal roughage component in the rations. The ingredient and proximate composition of concentrate mixtures. Fifteen ram lambs of native Kilakarsal breed of about six months of age and with an average initial body weight of 9.39 kg were divided on the basis of body weight into three groups with five lambs per dietary group in a completely randomized design. Daily record of feed consumption was maintained. The lambs were weighed at fortnightly intervals. At the end of the feeding experiment, digestion trial was conducted using the same ram lambs. The samples were analysed for proximate composition and the digestibility of nutrients in the three experimental rations were determined. Result: Based on the data analyzed the DMI worked out to be 64.90, 65.84 and 65.43 g/kg W0.75 in lambs fed with rations containing 0, 15 and 30% UTP in the concentrate mixture respectively. The corresponding values for ADG in the present study were 48.14, 47.43 and 48.57 g. The feed efficiency in terms of DMI required to gain one kg body weight were 8.28, 8.58 and 8.35 kg which did not vary between the treatment groups. DCP of experimental rations increased significantly (P≤0.05) as the level of UTP in the concentrate mixture increased which could be attributed to the increased crude protein digestibility. The TDN decreased significantly (P≤0.05) at 15% and level and was unaffected thereafter. Hence, it was concluded that Acacia nilotica pods treated with 5% urea at 40% moisture and stored for 14 days could safely be included up to 30 per cent level in the concentrate mixture of growing lamb ration. </jats:p

Effect of Nano Zinc Supplementation on Production Performance, Immune Response and Carcass Characteristics in Japanese Quail Broiler

Background: Nano-technology is currently an emerging field and giving promising results with cost economics in the poultry nutrition sector. A study was conducted to investigate the effects of nano Zn supplementation on production performance, immune response and carcass characteristicsin Japanese quail broiler. Methods: Synthesis of zinc oxide nano-particles was carried out by chemical method and characterized by standard techniques. Day old Japanese quail chicks (n=240) were randomly allotted in four treatment groups for feeding trial and fed with basal diet and source of zinc viz. for T1 inorganic Zinc Oxide, for T2, T3 and T4 nano Zinc Oxide was used at 75%, 50% and 25% levels of BIS recommendation for Zinc. Production parameters, immune response, carcass characteristics and cost economics were studied. Result: Based on the data analysed T3 and T4 showed significantly (P less than 0.05) improved performance in production parameters like body weight gainand cumulative feed conversion ratio than T1 and T4. HI, titer value and carcass characteristics data also showed similar trend. </jats:p