Heritable Connective Tissue Disorders (HCTD) show an overlap in the physical features that can evolve in childhood. It is unclear to what extent children with HCTD experience burden of disease. This study aims to quantify fatigue, pain, disability and general health with standardized validated questionnaires. Methods. This observational, multicenter study included 107 children, aged 4–18 years, with Marfan syndrome (MFS), 58%; Loeys-Dietz syndrome (LDS), 7%; Ehlers-Danlos syndromes (EDS), 8%; and hypermobile Ehlers-Danlos syndrome (hEDS), 27%. The assessments included PROMIS Fatigue Parent–Proxy and Pediatric self-report, pain and general health Visual-Analogue-Scales (VAS) and a Childhood Health Assessment Questionnaire (CHAQ). Results. Compared to normative data, the total HCTD-group showed significantly higher parent-rated fatigue T-scores (M = 53 (SD = 12), p = 0.004, d = 0.3), pain VAS scores (M = 2.8 (SD = 3.1), p < 0.001, d = 1.27), general health VAS scores (M = 2.5 (SD = 1.8), p < 0.001, d = 2.04) and CHAQ disability index scores (M = 0.9 (SD = 0.7), p < 0.001, d = 1.23). HCTD-subgroups showed similar results. The most adverse sequels were reported in children with hEDS, whereas the least were reported in those with MFS. Disability showed significant relationships with fatigue (p < 0.001, r_s = 0.68), pain (p < 0.001, r_s = 0.64) and general health (p < 0.001, r_s = 0.59). Conclusions. Compared to normative data, children and adolescents with HCTD reported increased fatigue, pain, disability and decreased general health, with most differences translating into very large-sized effects. This new knowledge calls for systematic monitoring with standardized validated questionnaires, physical assessments and tailored interventions in clinical care.

To our knowledge, the potential use of CO₂ as a heat-transmitting fluid for cooling applications in power plants has not been explored very extensively. In this paper, we conduct a theoretical analysis to explore the use of CO₂ as the heat transmission fluid. We evaluate and compare the thermophysical properties of both dry air and CO₂ and perform a simple analysis on a steam-condensing device where steam flows through one of the flow paths and the cooling fluid (CO₂ or air) is expanded from a high-pressure container and flows through the other. Sample calculations are carried out for a saturated-vapor steam at 0.008 MPa and 41.5 °C with the mass flow rate of 0.01 kg/s. The pressure of the storage container ranges from 1 to 5 MPa, and its temperature is kept at 35 °C. The pressure of the cooling fluid (CO₂ or dry air) is set at 0.1 MPa. With air as the heat-removing fluid, the steam exits the condensing device as a vapor-liquid steam of 53% to 10% vapor for the container pressure of 1 to 5 MPa. With CO₂ as the heat-removing fluid, the steam exits the device still containing 44% and 7% vapor for the container pressure of 1 MPa and 2 MPa, respectively. For the container pressure of 3 MPa and higher, the steam exits the device as a single-phase saturated liquid. Thus, due to its excellent Joule–Thomson cooling effect and heat capacity, CO₂ is a better fluid for power plant cooling applications. The condensing surface area is also estimated, and the results show that when CO₂ is used, the condensing surface is 50% to 60% less than that when dry air is used. This leads to significant reductions in the condenser size and the capital costs. A rough estimate of the amount of CO₂ that can be stored and utilized is also carried out for a steam power plant which operates with steam with a temperature of 540 °C (813 K) and a pressure of 10 MPa at the turbine inlet and saturated-vapor steam at 0.008 MPa at the turbine outlet. The results indicate that if CO₂ is used as a cooling fluid, CO₂ emitted from a 1000 MW power plant during a period of 250 days could be stored and utilized.

To store CO2 in geological reservoirs, expansion valves have been used to intentionally release supercritical CO2 from high-pressure containers at a source point to lower-pressure pipelines and transport to a selected injection site. Using expansion valves, however, has some shortcomings: (i) the fluid potential, in the form of kinetic energy and pressure which can produce mechanical work or electricity, is wasted, and (ii) due to the Joule-Thomson cooling effect, the reduction in the temperature of the released CO2 stream might be so dramatic that it can induce thermal contraction of the injection well causing fracture instability in the storage formation. To avoid these problems, it has been suggested that before injection, CO2, should be heated to a temperature slightly higher than that of the reservoir. However, heating could increase the cost of CO2 injection. This work explores the use of a Tesla Turbine, instead of an expansion valve, to harvest the potential of CO2, in the form of its pressure and kinetics, to generate mechanical work when it is released from a high-pressure container to a lower-pressure transport pipeline. The goal is to avoid throttling losses and to produce useful power because of the expansion process. In addition, due to the friction between the gas and the turbine disks, the expanded gas temperature reduction is not as dramatic as in the case when an expansion valve is used. Thus, as far as CO2 injection is concerned, the need for preheating can be minimized.