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The X-linked inhibitor of apoptosis (XIAP) deficiency is a primary immunodeficiency characterized by Epstein-Barr virus (EBV)-driven hemophagocytic lymphohistiocytosis (HLH), splenomegaly, and colitis. Here, we present, for the first time, granulomatous hepatitis and granulomatous and lymphocytic interstitial lung disease (GLILD) as manifestations of XIAP deficiency. We report successful treatment of GLILD in XIAP deficiency with rituximab and azathioprine and discuss the role of XIAP deficiency in immune dysregulation.

Purpose-This study aims to develop a simple analytical model for predicting the throughthickness distribution of residual stresses in a cold spray deposit-substrate assembly. Design/methodology/approach-Layer by layer build up of residual stresses induced by both the peening dominant and thermal mismatch dominant cold spray processes, taking into account the force and moment equilibrium requirements. The proposed model has been validated with the neutron diffraction measurements, taken from the published literature for different combinations of deposit-substrate assemblies comprising Cu, Mg, Ti, Al and Al alloys.Findings-Through a parametric study, the influence of geometrical variables (number of layers, substrate height and individual layer height) on the through-thickness residual stress distribution and magnitude are elucidated. Both the number of deposited layers and substrate height affect residual stress magnitude, whereas the individual layer height has little effect. A good agreement has been achieved between the experimentally measured stress distributions and predictions by the proposed model. Originality/value-The proposed model provides a more thorough explanation of residual stress development mechanisms by the cold spray process along with mathematical representation. Comparing to existing analytical and finite element methods, it provides a quicker estimation of the residual stress distribution and magnitude. This paper provides comparisons and contrast of the two different residual stress mechanisms: the peening dominant and the thermal mismatch dominant. The proposed model allows parametric studies of geometric variables, and can potentially contribute to cold spray process optimisation aiming at residual stress control.

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