Background: Cystinosis is a rare autosomal recessive lysosomal storage disease with high morbidity and mortality. It is caused by mutations in the CTNS gene that encodes the cystine transporter, cystinosin, which leads to lysosomal cystine accumulation. It is the major cause of inherited Fanconi syndrome, and should be suspected in young children with failure to thrive and signs of renal proximal tubular damage. The diagnosis can be missed in infants, because not all signs of renal Fanconi syndrome are present during the first months of life. Elevated white blood cell cystine content is the cornerstone of the diagnosis. Since chitotriosidase (CHIT1 or chitinase-1) is mainly produced by activated macrophages both in normal and inflammator

The current study investigated the stability and the extraction efficiency of emulsion liquid membrane (ELM) for Abamectin pesticide removal from aqueous solution. The stability was investigated in terms of droplet emulsion size distribution and emulsion breakage percent. The proposed ELM included a mixture of corn oil and kerosene (1:1) as a diluent, Span 80 (sorbitan monooleate) as a surfactant and hydrochloric acid (HCl) as a stripping agent without utilizing a carrier agent. Parameters such as homogenizer speed, surfactant concentration, emulsification time and internal to organic volume ratio (I/O) were evaluated. Results show that the lower droplet size of 0.9 µm and higher stable emulsion in terms of breakage percent of 1.12 % we

natural and non-natural disasters, is an environmental challenges the society and the economy as well as a direct and indirect economic affect, and the units are part of the system overlapping among themselves and thus affected by external indicators, directly or indirectly, these direct effects appear in the destruction or damage inflicted by disasters in property , infrastructure , superstructure , accounting information systems and indirectly in the outcome of future business, comes research problem through access to accounting treatments issued by the Federal Office of financial supervision to address the damage caused by the disasters and prepare the missing financial accounts it turns out us that there is negligence of a nu

Sequencing technologies have reshaped the study of the subgingival microbiome, but selecting the appropriate method remains challenging because of differences in resolution, cost, host DNA contamination, and computational complexity. This review compares 16S rRNA sequencing, full-length 16S, shotgun metagenomics, and metatranscriptomics with respect to taxonomic resolution, functional output, sample requirements, and analytical limitations. Key practical issues, including low microbial biomass, contamination control, and the choice of appropriate bioinformatic tools, are emphasized to help researchers avoid common pitfalls. A decision-making framework is provided to link study goals to suitable sequencing methods while outlining rea

Future generations of wireless networks are expected to heavily rely on unmanned aerial vehicles (UAVs). UAV networks have extraordinary features like high mobility, frequent topology change, tolerance to link failure, and extending the coverage area by adding external UAVs. UAV network provides several advantages for civilian, commercial, search and rescue applications. A realistic mobility model must be used to assess the dependability and effectiveness of UAV protocols and algorithms.  In this research paper, the performance of the Gauss Markov (GM) and Random Waypoint (RWP) mobility models in multi-UAV networks for a search and rescue scenario is analyzed and evaluated. Additionally, the two mobility models GM and RWP are descr

This study presents the results of atmospheric particulates sampling using high volume air sampler for selected places at Al Tuwaitha nuclear site. The collected samples were analyzed for gross alpha /beta radioactivity using Ludlum model 3030 and measurement particles activity in Al Tuwaitha nuclear site and the surrounding areas for the period from 28/12/2016 to 13/4/2017.The measurement of activity concentrations ranged from (0.42±0.03 to 4.18±0.13) Bq/m3 for alpha particles and from(0.93±0.06 to 9.21±0.26) Bq/m3for beta particles. The activity concentration of nuclides inversely proportional with air temperature and wind speed while humidity is directly proportional with it. Highest value of activity concentration has been found at(

Erosion and deposition are natural phenomena in many estuaries that can cause morphological changes, leading to navigation and offshore structure problems. Many previous studies have focused on investigating the morphological changes of estuaries depending on different parameters and their combined effects with erosion and deposition processes, such as maximum flow amplitude, waves, currents, tidal flow, storms, rising sea elevation, maximum turbidity, salinity, and bed roughness height as input parameters. These studies are based on field measurements and numerical simulations by using topographic surveys, soil samples, satellite images, geological data, and bathymetric maps with the aid of ArcGIS. Some of these studies examine the