109 research outputs found
HLA-identical umbilical cord blood transplantation from a sibling donor in juvenile myelomonocytic leukemia.
No full textThe Wiskott-Aldrich syndrome protein is required for iNKT cell maturation and function
No full textThe Wiskott-Aldrich syndrome (WAS) protein (WASp) is a regulator of actin cytoskeleton in hematopoietic cells. Mutations of the WASp gene cause WAS. Although WASp is involved in various immune cell functions, its role in invariant natural killer T (iNKT) cells has never been investigated. Defects of iNKT cells could indeed contribute to several WAS features, such as recurrent infections and high tumor incidence. We found a profound reduction of circulating iNKT cells in WAS patients, directly correlating with the severity of clinical phenotype. To better characterize iNKT cell defect in the absence of WASp, we analyzed was(-/-) mice. iNKT cell numbers were significantly reduced in the thymus and periphery of was(-/-) mice as compared with wild-type controls. Moreover analysis of was(-/-) iNKT cell maturation revealed a complete arrest at the CD44(+) NK1.1(-) intermediate stage. Notably, generation of BM chimeras demonstrated a was(-/-) iNKT cell-autonomous developmental defect. was(-/-) iNKT cells were also functionally impaired, as suggested by the reduced secretion of interleukin 4 and interferon gamma upon in vivo activation. Altogether, these results demonstrate the relevance of WASp in integrating signals critical for development and functional differentiation of iNKT cells and suggest that defects in these cells may play a role in WAS pathology
Outcome of Non-hematological Autoimmunity After Hematopoietic Cell Transplantation in Children with Primary Immunodeficiency
No full textPurpose: Knowledge of post-hematopoietic cell transplantation (HCT) non-hematological autoimmune disease (AD) is far from satisfactory.
Method: This multicenter retrospective study focuses on incidence, risk factors, and outcomes of post-HCT AD in 596 children with primary immunodeficiency (PID) who were transplanted from 2009 to 2018.
Results: The indications of HCT were severe combined immunodeficiency (SCID, n = 158, 27%) and non-SCID PID (n = 438, 73%). The median age at HCT was 2.3 years (range, 0.04 to 18.3 years). The 5-year overall survival for the entire cohort was 79% (95% cumulative incidence (CIN), 74-83%). The median follow-up of surviving patients was 4.3 years (0.08 to 14.7 years). The CIN of post-HCT AD was 3% (2-5%) at 1 year post-HCT, 7% (5-11%) at 5 years post-HCT, and 11% (7-17%) at 8 years post-HCT. The median onset of post-HCT AD was 2.2 years (0.12 to 9.6 years). Autoimmune thyroid disorder (n = 19, 62%) was the most common post-HCT AD, followed by neuromuscular disorders (n = 7, 22%) and rheumatological manifestations (n = 5, 16%). All patients but one required treatment for post-HCT AD. After multivariate analysis, age at transplant (p = 0.01) and T cell-depleted graft (p < 0.001) were significant predictors of post-HCT AD. None of the T cell-depleted graft recipients developed post-HCT AD. Patients with a lower CD3+ count at 6 months post-HCT had a significant higher incidence of post-HCT AD compared to disease controls. Graft-versus-host disease, viral infection, and donor chimerism had no association with post-HCT AD.
Conclusion: Post-HCT AD occurred in 11% at 8 years post-HCT and its occurrence was associated with older age at HCT and unmanipulated graft
HLA-identical umbilical cord blood transplantation from a sibling donor in juvenile myelomonocytic leukemia
No full textN/
Riccardia innovans Pagan
No full text<i>Riccardia innovans</i> (Steph.) Pagán (Figs 2 -4) <p> <i>The Bryologist</i> 45: 80 (1942). <i>—</i> Basionym: <i>Aneura innovans</i> Steph., in Urban, <i>Symbolae Antillarum</i> 2: 470 (1901). — Type: Guadeloupe, Savane-à-Mulets, «Sur les arbrisseaux », 1901, <i>Duss</i></p> <p> <i>484,</i> ex hb. Urban (lecto-, designated here, G[G00066662!], <i>c</i>. gyn.; isolecto-, NY n.v., fide Pagán 1942).</p> <i>Description</i> <p> The outstanding characters of <i>R. innovans</i> are the very delicate, 2-pinnate plants with a very narrow (150-250 µm in diameter), biconvex, almost wingless axis and numerous long and narrow, linear to subulate branches. The branches are only little narrower than the axis, plano-convex, obliquely to widely spreading, usually tapering to narrow tips, and narrowly winged by 1-2 cell wide wings. The presence of small scales on the calyptra, made up of large cells, may be a further characteristic of the species. The plants are dioicous; gemmae have not been observed.</p> <i>Remarks</i> <p> <i>Riccardia innovans</i> approaches <i>R. regnellii</i> (Ångstr.) K.G.Hell, but the latter is a larger plant with a flat axis (not biconvex) and with broader, frequently tongue-shaped branches. In the type material, some <i>R. regnellii</i> plants are growing mixed in the dense mat of <i>R. innovans</i> and are immediately recognized by their much larger size. <i>Riccardia innovans</i> is thus far only</p> <p> known from the type. A field search by the second author in the type locality and elsewhere has not revealed further populations of the species. The taxonomic relationships of the species will be dealt with in a comprehensive study on the genus <i>Riccardia</i> in Guadeloupe (Lavocat Bernard & Reeb in prep.).</p>Published as part of <i>Gradstein, S. Robbert & Bernard, Elisabeth Lavocat, 2020, An evaluation of the endemic bryophyte flora of Guadeloupe, pp. 205-214 in Cryptogamie, Bryologie 20 (15)</i> on pages 206-207, DOI: 10.5252/cryptogamie-bryologie2020v41a15, <a href="http://zenodo.org/record/7822144">http://zenodo.org/record/7822144</a>
The 2021 IAEA software intercomparison for k<sub>0</sub>-INAA
No full textIn order to establish the variation between results in mass fractions due to software implementation, as measured by the k0-method for INAA, the IAEA has organized a software intercomparison. A complete set of test spectra and associated information was assembled. Efficiency curves, neutron spectrum parameters, correction factors and mass fractions were calculated with the participating programs (k0-IPEN, k0-INRIM, k0-DALAT, k0-IAEA and KayWin) using identical peak areas. In this paper, we report on the observed discrepancies, causes, remedies and future software developments. The test data, as well as intermediate results and observed mass fractions of the certified reference material BCR-320R “channel sediment” are available through the IAEA on request. The variations in concentrations attributed to differences between the programs were initially found to be 5.6 and 7.9%, for certified and uncertified concentrations, respectively. After the certified concentrations had been made available to the participants and they had been allowed to improve their programs, the variations found were 2.7 and 3.4%, respectively. The main identified remaining causes of variation are differences in the procedures used for detector efficiency characterisation and neutron spectrum parameter determination.</p
Deterioration of the anammox process at decreasing temperatures and long SRTs
No full textThe implementation of autotrophic nitrogen removal in the mainstream of a municipal wastewater treatment plant is currently pursued. Among the crucial unknown factors are the kinetic properties of anaerobic ammonium oxidising (anammox) bacteria at low temperatures. In this study we investigated the adaptation of a fast-growing anammox culture to a lower temperature. In a membrane bioreactor a highly enriched anammox community was obtained at 30°C, 25°C and 20°C. This culture was exposed to long- and short-term temperature changes. In short-term experiments the decrease in biomass-specific activity due to decrease in temperature can be described by an activation energy of 64 ± 28 kJ mol−1. Prolonged cultivation (months) implies that cultivation at low temperatures resulted in deterioration of biomass-specific activity (EaLT 239 kJ mol−1). The growth rate and specific anammox activity in the system decreased from 0.33 d−1 and 4.47 g NO2-N g VSS−1 d−1 at 30°C to 0.0011 d−1 and 0.037 g NO2-N g VSS−1 d−1 at 20°C. The reason for the deterioration of the system was related to the required long SRT in the system. The long SRT leads to an increase of non-active and non-anammox cells in the reactor, thereby decreasing the biomass-specific activity.BT/Environmental BiotechnologyBT/Bioprocess Engineerin
Pharmacokinetics of rituximab in a pediatric patient with therapy-resistant nephrotic syndrome
No full textDeterminants of the relationship between cytokine production in pregnant women and their infants.
No full textExposure to environmental factors during fetal life and infancy is thought to play an important role in the early development of innate and adaptive immunity. The immunological relationship between mother and infant and the effect that environmental exposures have during pregnancy and early childhood have not been studied extensively. Here the production of cytokines was measured in 146 pairs of mothers and their 2- month-old infants. The effect of place of residence, socio-economic variables, parasitic infections as well as maternal and child characteristics on measured cytokine production was determined. Mothers producing high levels of IL-10, IFN-gamma and IL-5 were more likely to have infants who also produced high levels of these cytokines either spontaneously (OR 2.6(95%CI 1.2-5.4), OR 2.9(CI 1.3-6.6), OR 11.2(CI 4.6-27.2), respectively) or in response to PHA (IL-10: OR 3.0(CI 1.4-6.6), IFN-gamma: OR 2.0(CI 1.0-4.2), respectively) even after adjustment for potential confounding variables. This was not the case for TNF-alpha. In response to LPS, place of residence was a strong determinant of infant IL-10 (OR 0.2(CI 0.1-0.9)) and TNF-alpha (OR 0.3(CI 0.1-0.9)) production. Maternal protozoan infections was independently associated with reduced infant IL10 in response to PHA and to LPS as well as reduced TNF-alpha and IFN-gamma in response to PHA. These results indicate strong relationship between maternal and infant's cellular immune responses even after taking into account many environmental influences that could affect infant's response directly or indirectly through uterine microenvironment. However, place of residence and intestinal infections may still directly affect the immune responses of the infant. Taken together, the study provides evidence for imprinted cytokine responses of an infant which may have implications for their reaction to incoming antigens, warranting further investigation into the role that genetics or epigenetics play in shaping the cytokine response by an infant to self or external antigens
Effect of different salt adaptation strategies on the microbial diversity, activity, and settling of nitrifying sludge in sequencing batch reactors
No full textThe effect of salinity on the activity of nitrifying bacteria, floc characteristics, and microbial community structure accessed by fluorescent in situ hybridization and polymerase chain reaction–denaturing gradient gel electrophoresis techniques was investigated. Two sequencing batch reactors (SRB1 and SBR2) treating synthetic wastewater were subjected to increasing salt concentrations. In SBR1, four salt concentrations (5, 10, 15, and 20 g NaCl/L) were tested, while in SBR2, only two salt concentrations (10 and 20 g NaCl/L) were applied in a more shock-wise manner. The two different salt adaptation strategies caused different changes in microbial community structure, but did not change the nitrification performance, suggesting that regardless of the different nitrifying bacterial community present in the reactor, the nitrification process can be maintained stable within the salt range tested. Specific ammonium oxidation rates were more affected when salt increase was performed more rapidly and dropped 50% and 60% at 20 g NaCl/L for SBR1 and SBR2, respectively. A gradual increase in NaCl concentration had a positive effect on the settling properties (i.e., reduction of sludge volume index), although it caused a higher amount of suspended solids in the effluent. Higher organisms (e.g., protozoa, nematodes, and rotifers) as well as filamentous bacteria could not withstand the high salt concentrations.BiotechnologyApplied Science
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