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 performance and durability of the asphalt pavement structure mainly depend on the strength of the bonding between the layers. Such a bond is achieved through the use of an adhesive material (tack coat) to bond the asphalt layers. The main objective of this study is to evaluate the effect of moisture in conjunction with repeated traffic loads on the strength of the bonding between asphalt layers using two types of tack coats with different application rates. Using the nominal maximum size of aggregate (NMAS), the layers were graded (25/19) and (19/9.5) mm. The slabs of multilayer asphalt concrete were prepared using a roller compactor using two types of tack coats to bond between layers, namely rapid curing cut back a

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

S Ali…, Journal of Physical Education, 2019 - Cited by 1

Aim: To evaluate the effect of two bonding systems and two curing systems on sealing ability of class V composite restorative materials. Materials and methods: This study was performed in vitro on 40 caries free upper first premolar teeth. The Standardized class V cavity preparation on buccal and lin- gual surfaces of each tooth was done. Then the teeth were randomly divided into two major groups each of twenty. 40 cavities were performed on these teeth and the first group7th generation bonding agent (i Bond) were applied according to the manufacturer instructions and single increment of univer- sal composite (XRV Herculite) from kerr were applied and twenty of the cavities were cured with con- ventional light cure device (astralis-5) and t

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

In this article, the influence of group nano transition metal oxides such as {(MnO₂), (Fe₂O₃) and (CuO)} thin films on the (ZnO-TiO2) electric characteristics have been analyzed. The prepared films deposited on glass substrate laser Nd-YAG with wavelength (ℷ =1064 nm) ,energy of (800mJ) and number of shots (400). The density of the film was found to be (200 nm) at room temperature (RT) and annealing temperature (573K).Using DC Conductivity and Hall Effect, we obtained the electrical properties of the films. The DC Conductivity shows that that the activation energies decrease while the σRT at annealing temperature with different elements increases the formation of mixed oxides. The Hall effect, the elec