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

This deals with estimation of Reliability function and one shape parameter (?) of two- parameters Burr – XII , when ?(shape parameter is known) (?=0.5,1,1.5) and also the initial values of (?=1), while different sample shze n= 10, 20, 30, 50) bare used. The results depend on empirical study through simulation experiments are applied to compare the four methods of estimation, as well as computing the reliability function . The results of Mean square error indicates that Jacknif estimator is better than other three estimators , for all sample size and parameter values

A simple and novel method was developed by combination of dispersive liquid-liquid microextraction with UV spectrophotometry for the preconcentartion and determination of trace amount of malathion. The presented method is based on using a small volume of ethylenechloride as the extraction solvent was dissolved in ethanol as the dispersive solvent, then the binary solution was rapidly injected by a syringe into the water sample containing malathion. The important parameters, such the type and volume of extraction solvent and disperser solvent, the effect of extraction time and rate, the effect of salt addition and reaction conditions were studied. At the optimum conditions, the calibration graph was linear in the range of 2-100 ng mL-1 of ma

This research presents a new study in reactive distillation by using consecutive reaction: the saponification reaction of diethyl adipate (DA) with sodium hydroxide solution .

The effect of three parameters were studied through a design of experiments applying 2³ factorial design . These parameters were : the mole ratio of DA to NaOH solution (0.1 and 1) , NaOH solution concentration (3 N and 8 N) , and batch time (1.5 hr. and 3.5 hr.) . The conversion of DA to sodium monoethyladipate(SMA)(intermediate product) was the effect of these parameters which was detected . Also , the percentage purity of the intermediate product was recorded . The results showed that increasing mole ratio of DA to NaOHsolutio

   The potential application of granules of brick waste (GBW) as a low-cost sorbent for removal of Ni⁺²ions from aqueous solutions has been studied. The properties of GBW were determined through several tests such as X-Ray diffraction (XRD), Energy dispersive X-ray (EDX), Scanning electron microscopy (SEM), and BET surface area. In batch tests, the influence of several operating parameters including contact time, initial concentration, agitation speed, and the dose of GBW was investigated. The best values of these parameters that provided maximum removal efficiency of nickel (39.4%) were 1.5 hr, 50 mg/L, 250 rpm, and 1.8 g/100mL, respectively. The adsorption data obtained by batch experiments subjected to the Three i

This research is studying technique sculptures super - realism, search through, how the method of work, and the search for the materials used in their manufacture, and this is the first study in the field of art and the field of academic study in the country.Research consists of an introduction, And four sections, The introduction containing information on: research problem, Importance of research, Goals of the research, Limits of research, research approach, and research tools.The first section contains a technical study sculptures super -realism in contemporary sculpture, while the second section includes a search for alternative materials available in the local markets, for making sculptures super - realism, the third section dedicate

The current study has beenconduced to evaluate the effect of extracted crude terpens at the concentrations of 6,8% of seeds of Eucalyptuscamaldulensison the 4th larval instar oftheCallosobruchusmaculates and the percentage of the cowpea seed germination.The Results showed that the terpens extract of the concentration of 8% increases the mortality rate of the fourth larval instar and it reach to 63.3%, and then decrease of to 26.6,20% at concentration of 6%and forcontrol treatment respectively The percentage of adult emergence reduces to 0% at the concentration of 8% compared with control treatment in which it reach to 66.6%. The extraction atbothconcentrations 6,8% does not affect the germination rate

The article presents the results of studying the conchological variability of the terrestrial mollusk Chondrulopsina fedtschenkoi (Ancey, 1886), known to occur in three regions of the Zaravshan Range (Central Asia): the Urgutsay Gorge, the vicinity of the Gissarak Reservoir and the Ingichka-Irmak Gorge. Conchological variability was determined based on statistical analysis. The climate of the three regions is different, and environmental factors have led to changes in the mollusk shell. The shells have changed in response to environmental factors, these are their adaptive traits for survival; the variability of conchological features is also reflected in the color of the shell, and the intensive development of the color of the shell in m

The study aimed to describe the anatomical, histological, and histochemical structure of the proventriculuc of adult male Eurasian marsh harrier, Circus aeruginosus (Linnaeus, 1766) (Accipitriformes, Accipitridae). Six birds were used for this study. The histological sections of proventriculus were stained with hematoxylin and eosin, periodic acid Schiff, and alcain blue. The proventriculus, representing the upper stomach, appeared as a conical structure with a thick wall connecting the caudal end of the thoracic esophagus to the gizzard. It had a light brown color. Microscopic examination revealed that the proventriculus wall consists of the outer tunica serosa, the tunica muscularis, tunica sub mucosa, and the inner tunica mucosa.