Clinical Features of Dominant and Recessive Interferon γ Receptor 1 Deficiencies

Background Interferon ? receptor 1 (IFN? R1) deficiency is a primary immunodeficiency with allelic dominant and recessive mutations characterised clinically by severe infections with mycobacteria. We aimed to compare the clinical features of recessive and dominant IFN?R1 deficiencies. Methods We obtained data from a large cohort of patients worldwide. We assessed these people by medical histories, records, and genetic and immunological studies. Data were abstracted onto a standard form. Findings We identified 22 patients with recessive complete IFN?R1 deficiency and 38 with dominant partial deficiency. BCG and environmental mycobacteria were the most frequent pathogens. In recessive patients, 17 (77%) had environmental mycobacterial disease and all nine BCG-vaccinated patients had BCG disease. In dominant patients, 30 (79%) had environmental mycobacterial disease and 11 (73%) of 15 BCG-vaccinated patients had BCG disease. Compared with dominant patients, those with recessive deficiency were younger at onset of first environmental mycobacterial disease (mean 3·1 years [SD 2·5] vs 13·4 years [14·3], p=0·001), had more mycobacterial disease episodes (19 vs 8 per 100 person-years of observation, p=0·0001), had more severe mycobacterial disease (mean number of organs infected by Mycobacterium avium complex 4·1 [SD 0·8] vs 2·0 [1·1], p=0·004), had shorter mean disease-free intervals (1·6 years [SD 1·4] vs 7·2 years [7·6], p

Validating Injection Stretch-Blow Molding Simulation Through Free Blow Trials

A 2D isothermal finite element simulation of the injection stretch-blow molding (ISBM) process for polyethylene terephthalate (PET) containers has been developed through the commercial finite element package ABAQUS/standard. In this work, the blowing air to inflate the PET preform was modeled through two different approaches: a direct pressure input (as measured in the blowing machine) and a constant mass flow rate input (based on a pressure-volume-time relationship). The results from these two approaches were validated against free blow and free stretch-blow experiments, which were instrumented and monitored through high-speed video. Results show that simulation using a constant mass flow rate approach gave a better prediction of volume vs. time curve and preform shape evolution when compared with the direct pressure approach and hence is more appropriate in modeling the preblowing stage in the injection stretch-blow molding process

Individual and couple decision behavior under risk: evidence on the dynamics of power balance

This article reports results of an experiment designed to analyze the link between risky decisions made by couples and risky decisions made separately by each spouse. We estimate both the spouses and the couples' degrees of risk aversion, we assess how the risk preferences of the two spouses aggregate when they make risky decisions, and we shed light on the dynamics of the decision process that takes place when couples make risky decisions. We find that, far from being fixed, the balance of power within the household is malleable. In most couples, men have, initially, more decision-making power than women but women who ultimately implement the joint decisions gain more and more power over the course of decision making.