Mucopolysaccharidosis VI

Mucopolysaccharidosis VI (MPS VI) is a lysosomal storage disease with progressive multisystem involvement, associated with a deficiency of arylsulfatase B leading to the accumulation of dermatan sulfate. Birth prevalence is between 1 in 43,261 and 1 in 1,505,160 live births. The disorder shows a wide spectrum of symptoms from slowly to rapidly progressing forms. The characteristic skeletal dysplasia includes short stature, dysostosis multiplex and degenerative joint disease. Rapidly progressing forms may have onset from birth, elevated urinary glycosaminoglycans (generally >100 μg/mg creatinine), severe dysostosis multiplex, short stature, and death before the 2nd or 3rd decades. A more slowly progressing form has been described as having later onset, mildly elevated glycosaminoglycans (generally <100 μg/mg creatinine), mild dysostosis multiplex, with death in the 4th or 5th decades. Other clinical findings may include cardiac valve disease, reduced pulmonary function, hepatosplenomegaly, sinusitis, otitis media, hearing loss, sleep apnea, corneal clouding, carpal tunnel disease, and inguinal or umbilical hernia. Although intellectual deficit is generally absent in MPS VI, central nervous system findings may include cervical cord compression caused by cervical spinal instability, meningeal thickening and/or bony stenosis, communicating hydrocephalus, optic nerve atrophy and blindness. The disorder is transmitted in an autosomal recessive manner and is caused by mutations in the ARSB gene, located in chromosome 5 (5q13-5q14). Over 130 ARSB mutations have been reported, causing absent or reduced arylsulfatase B (N-acetylgalactosamine 4-sulfatase) activity and interrupted dermatan sulfate and chondroitin sulfate degradation. Diagnosis generally requires evidence of clinical phenotype, arylsulfatase B enzyme activity <10% of the lower limit of normal in cultured fibroblasts or isolated leukocytes, and demonstration of a normal activity of a different sulfatase enzyme (to exclude multiple sulfatase deficiency). The finding of elevated urinary dermatan sulfate with the absence of heparan sulfate is supportive. In addition to multiple sulfatase deficiency, the differential diagnosis should also include other forms of MPS (MPS I, II IVA, VII), sialidosis and mucolipidosis. Before enzyme replacement therapy (ERT) with galsulfase (Naglazyme®), clinical management was limited to supportive care and hematopoietic stem cell transplantation. Galsulfase is now widely available and is a specific therapy providing improved endurance with an acceptable safety profile. Prognosis is variable depending on the age of onset, rate of disease progression, age at initiation of ERT and on the quality of the medical care provided

evaporative air-conditioning systems

Water droplet carryover phenomena and pressure drop characteristics in drip-type direct evaporative coolers have been experimentally investigated. Ten different evaporative cooling pads were tested for different air frontal velocities, and the onset of carryover has been determined. The onset of water carryover has been found to be at air velocities between 3.5 and 4.2 m/s. Also, the effect of flute height and pad thickness on pressure drop characteristics of cooling pads has been examined. In addition, it has been determined that for the steady-state operation of drip-type direct evaporative coolers, water temperature in the reservoir approaches the wet bulb temperature and can be taken as constant for long term steady-state operations in practice. (C) 2013 Elsevier Ltd. All rights reserved

of confined circular and elliptic impinging jets

The flow field of confined circular and elliptic jets was studied experimentally with a Laser Doppler Anemometry (LDA) system. In addition, heat transfer characteristics were numerically investigated. Experiments were conducted with a circular jet and an elliptic jet of aspect ratio four, jet to target spacings of 2 and 6 jet diameters, and Reynolds number 10 000. The toroidal recirculation pattern was observed in the outflow region for both geometries at dimensionless jet to plate distance 2. Higher spreading rates in the minor axis direction of the elliptic jet have also been mapped. Along the target plate, different boundary layer profiles were obtained for circular and elliptic jets at H/d = 2, but profiles became similar when dimensionless jet to plate distance was increased to 6. Positions of maximum radial and axial velocities and turbulence intensities have been determined for both geometries. For the confined circular and elliptic jet geometries, analysis of flow field measurements and numerical heat transfer results showed that inner peaks in local heat transfer closely relate to turbulence intensities in the jet and radial flow acceleration along the wall. Differences between the circular and elliptic jet, in terms of flow field and heat transfer characteristics, reduced with increase in the jet to plate distance. (c) 2007 Elsevier Masson SAS. All rights reserved

ENERGY SAVING BY USING AN AXIAL FLOW DEEP WELL PUMP: AN APPLICATION IN

Turkey is home to a large number of lakes, dam lakes and small lakes which have a great potential for growing fishery products. Growing trout in net frame systems manufactured in dam lakes is very common and economically very promising application. For growing trout, certain conditions such as certain water temperature range and minimum dissolved oxygen (DO) level must be satisfied. The water temperature required should be at 17-20 degrees C to grow trout. Also, the amount of dissolved oxygen (pc) should never fall below 6-7 mg/L. As the temperature rises during the months of June-September, water temperature rises above the acceptable limits to grow trout. Producers usually use different types of internal combustion engine-pump (ICE-P) systems to provide the circulation of the colder water in the deeper parts of the lake to lake surface to grow trout in these months. But, this method is not economically feasible. In this study, as an alternative to ICE-P system, an axial flow deep well pump has been proposed for energy saving purposes. To validate the feasibility of the system, total of 40 net frame systems each has dimensions of 5x5x8 meters have been installed to grow trout in net frames in Lake Karacaoren 2 in Burdur, Turkey. To grow trout in the hot seasons, it is necessary to circulate the colder water in the deeper parts of the lake to lake surface economically. For this aim, total of 10 an axial flow deep well pumps (AFDWPs) having a capacity of 300 m(3)/h, head of 4 m and running at 2850 rpm which is driven by 5.5 kW deep well motor (DWM) have been specially designed and manufactured. To compare the classical water circulation method with ICE-P and newly proposed AFDWP, after every two pumps (ICE/AFDWP) are installed, mean water temperatures were measured along the water column in net frame with the depth of 8 meters and also energy consumptions have been compared during the months of June to September. AFDWP and ICE-P were used only in these months where the water has to be circulated. According to the results energy consumption by using AFDWP was about 90 MWh/year, on the other hand, energy consumed by ICE-P was about 1949 MWh/year during the months of June-September. As a result, significant energy saving of 95.3 % (1906.8 MWh/year) can be obtained by using proposed AFDWP instead of the classical ICE-P during these months for total of 40 net frames

on confined impinging jet heat transfer

The influence of natural convection on the local and average heat transfer at increasing temperature differences between the jet and the target plate from confined impinging jets has been experimentally and numerically investigated. Local Nusselt numbers were obtained numerically for jet Reynolds numbers in the range of 250-1000, and jet to target spacings of 2, 4. 8, 12 jet diameters at various modified Grashof numbers, to determine the effect of buoyancy induced natural convection. To determine the overall effect of natural convection on the average heat transfer, experiments have been conducted for Re numbers in the range 250-5000 and dimensionless jet to target spacing 2, 4. 6, 8, and 12 at increasing modified Grashof numbers. It has been determined that buoyancy induced natural convection might have opposing or assisting influence on local heat transfer at different locations of the target plate. It has also been shown that especially at low jet inlet velocities the average heat transfer coefficient at the highest modified Grashof number, where the natural convection is effective, is higher than the value corresponding to the lowest Grashof number at which buoyancy effects are negligible, by as much as 37%. (C) 2008 Elsevier Ltd. All rights reserved

ENERGY SAVING BY USING AN AXIAL FLOW DEEP WELL PUMP: AN APPLICATION IN

Turkey is home to a large number of lakes, dam lakes and small lakes which have a great potential for growing fishery products. Growing trout in net frame systems manufactured in dam lakes is very common and economically very promising application. For growing trout, certain conditions such as certain water temperature range and minimum dissolved oxygen (DO) level must be satisfied. The water temperature required should be at 17-20 degrees C to grow trout. Also, the amount of dissolved oxygen (pc) should never fall below 6-7 mg/L. As the temperature rises during the months of June-September, water temperature rises above the acceptable limits to grow trout. Producers usually use different types of internal combustion engine-pump (ICE-P) systems to provide the circulation of the colder water in the deeper parts of the lake to lake surface to grow trout in these months. But, this method is not economically feasible. In this study, as an alternative to ICE-P system, an axial flow deep well pump has been proposed for energy saving purposes. To validate the feasibility of the system, total of 40 net frame systems each has dimensions of 5x5x8 meters have been installed to grow trout in net frames in Lake Karacaoren 2 in Burdur, Turkey. To grow trout in the hot seasons, it is necessary to circulate the colder water in the deeper parts of the lake to lake surface economically. For this aim, total of 10 an axial flow deep well pumps (AFDWPs) having a capacity of 300 m(3)/h, head of 4 m and running at 2850 rpm which is driven by 5.5 kW deep well motor (DWM) have been specially designed and manufactured. To compare the classical water circulation method with ICE-P and newly proposed AFDWP, after every two pumps (ICE/AFDWP) are installed, mean water temperatures were measured along the water column in net frame with the depth of 8 meters and also energy consumptions have been compared during the months of June to September. AFDWP and ICE-P were used only in these months where the water has to be circulated. According to the results energy consumption by using AFDWP was about 90 MWh/year, on the other hand, energy consumed by ICE-P was about 1949 MWh/year during the months of June-September. As a result, significant energy saving of 95.3 % (1906.8 MWh/year) can be obtained by using proposed AFDWP instead of the classical ICE-P during these months for total of 40 net frames

and experiments

Effects of jet inlet geometry and aspect ratio on local and average heat transfer characteristics of totally nine confined impinging jets have been investigated experimentally using thermochromic liquid crystals and numerically by using a 3-D low Reynolds number k-epsilon model. Experimental study by using liquid crystals for temperature measurement was conducted for three different jet exit geometries (circular, elliptic, rectangular). In addition, simulations were performed at the same mass flow rate for totally nine jet exit geometries including circular, elliptic and rectangular jets with different aspect ratios for dimensionless jet to plate distances 2, 6, and 12.As the aspect ratio of equal cross-sectional area elliptic and rectangular jets increases, heat transfer enhancement in the stagnation region was obtained. As a result higher aspect ratio jets can be used as a passive enhancement technique for localized heating or cooling especially at small jet to plate distances. Wall jet region comprises very large portion of the impinging plate under study and generally lower heat transfer rates were attained for higher aspect ratio jets in this region especially at small jet to plate distances. Therefore as the aspect ratio increases, lower average heat transfer rates were acquired. The effect of aspect ratio on local and average heat transfer decreases with increasing jet to plate distance. Even though the mass flow rate is the same, heat transfer rate of rectangular jets were reduced with increasing the cross-sectional area. With increasing jet to plate distance very similar heat transfer characteristics were observed along the major and minor axis directions. (C) 2010 Elsevier Masson SAS. All rights reserved

EXPERIMENTAL PERFORMANCE EVALUATION OF COMBI-HEATERS WITH DIFFERENT HEAT

In this study, characteristics of three different combi-heaters and heat exchangers were experimentally investigated. All combi-heaters tested have similar technical specifications and have almost the same heat load. Main difference among the combi-heaters is the type of heat exchanger used. Tests were performed for combi heaters containing a combined heat exchanger (K1), a flat plate heat exchanger (K2), double heat exchanger (K3). The objective of this study is to determine the performance of all combi heaters and to determine the benefits and advantages of K1. Performance of all combi heaters were investigated for the heating cycle, domestic water cycle with and without the heating cycle in use, and similarly heating cycle with domestic water in use and finally for different mass flow rates. According to the results of different and parametric experiments for performance evaluation, for the combi heater with combined heat exchanger (K1), advantages in heat transfer characteristics have been detected compared to the other two combi-heaters tested

direct evaporative cooling systems

Radial basis function network method is used for modeling a direct evaporative cooling system. Air dry exit temperature, air pressure drop across the cooler and cooler efficiency are predicted using these models. The inputs are pad thickness, air inlet speed, air dry inlet temperature, relative humidity at the inlet and feed water temperature. The data for the models are taken from the experiments performed specifically for this purpose. Model validation is performed using twofold cross validation method. A grid search is used to determine optimal network parameters, such as, optimum number of radial basis elements and spread parameter. Validated models are tested against ordinary least squares models for the output variables. The results indicate that it is feasible to apply radial basis function networks to model direct evaporative coolers. (C) 2018 Elsevier Ltd. All rights reserved

jet

Thermal control of electronic components is a continuously emerging problem as power loads keep increasing. The present study is mainly focused on experimental and numerical investigation of impinging jet cooling of 18 (3 x 6 array) flash mounted electronic components under a constant heat flux condition inside a rectangular channel in which air, following impingement, is forced to exit in a single direction along the channel formed by the jet orifice plate and impingement plate. Copper blocks represent heat dissipating electronic components. Inlet flow velocities to the channel were measured by using a Laser Doppler Anemometer (LDA) system. Flow field observations were performed using a Particle Image Velocimetry (PIV) and thermocouples were used for temperature measurements. Experiments and simulations were conducted for Re 4000 8000 at fixed value of H 10 x D-h. Flow field results were presented and heat transfer results were interpreted using the flow measurement observations. Numerical results were validated with experimental data and it was observed that the results arc in agreement with the experiments