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

n this study new derivatives of Schiff bases (5-10) were synthesized from the new starting material 1 . Which has been synthesized by the reaction of (1 mol.) of dichloroacetic acid with two moles of morpholine, in the presence of potassium hydroxide, Ester derivatives 2 and 3 were synthesized by the reaction of 1 with methanol or ethanol respectively in the presence of sulphuric acid as catalyst . Compound 2 was also prepared from dimethylsulphate with high yield , 2 and 3 was used to synthesized 2,2-dimorpholinylacetohydrazide 4 via reaction with NH2NH2.H2O 80% .Imines (5-10) were synthesized via the reaction of 4 with appropriate aromatic aldehydes in the presence of G.A.A as a catalyst . Derivatives compounds (1-10) were identifie

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(

  The relationship between the vision of the scenario and the director is represented in the second search for new mechanisms and possibilities that possess the direct effect and synonyms of the vision of the scenario, so he works on investing them in achieving his ideas and visions with more effectiveness and flexibility to achieve creative potentials, without resorting to the installations and expressive structures themselves that have exhausted their meaning, and lost their luster due to Its prevalence and familiarity on the one hand, and its activation on the other hand to achieve modernity at the level of aromas, after the director takes a method or approach that has privacy and exclusivity, and based on the foregoing, getting

      Strategies to reduce obesity have become main priority for many health institution and health staff around the world, as the prevalence of obesity has risen and exacerbated in most of the world mainly because of the modern life style which tend to be more sedentary with an increase eating unhealthy fast western food. Many years ago, the lipid-lowering drug simvastatin; and omega-3 were considered as a traditional lipid-lowering drug that have been well-documented to possess anti-inflammatory, cardioprotective and triglyceride-lowering properties; and their co-administration may demonstrate a complementary effect in lowering patients' triglycerides and total cholesterol to treat atherosclerosis. Many previous studies have

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

In this work, a vertical pulsating heat pipe heat exchanger (PHPHE)  was designed for waste heat recovery, exchanging thermal energy between two air streams in a counterflow configuration. The heat exchanger consists of six rows, each row consists of one pulsating heat pipe (PHP), and each PHP has six turns. The working fluid used in the heat pipe was acetone with fill ratios of 50%, 60%, and 70%. The effect of evaporator inlet temperature at 40, 45, and 50°C and air velocity at 0.5, 0.7, and 0.9 m/s on the pulsating heat pipes consisting of three sections- evaporator, condenser, and adiabatic, whose dimensions were 25 x 25 x 10 cm, was studied. At the same time, the condenser temperature was maintained at 26°C. The system’s th

A new novel series of metalcomplexes are prepared from reactions between 2-benzoylthiobenzimidazole (L) with metal salts of Co (II) , Fe(III) and Rh (III) , while Pd(II) complex was obtained by mixing ligandsof 2-benzoylthiobenzimidazole (L) as primary ligand and bipyridine (L/ )as secondary ligand as well as palladium chloride as metal salt in an ethanoic medium. The geometry of these compounds were identified using C.H.N.microanalysis, Ultraviolet–visible, Fourier transforms infrared, magnetic susceptibility, molar conductivity and flame atomic absorption (A.A). From the dataobtained by these spectral analyses, the molecular structures for Rh and Fe complexes were proposed to be octahedral geometry. A square planar construction is propo