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 inflammatory conditions which suggest that cystinosis should be included within the differential diagnosis of disorders associated with increased plasma chitotriosidase activity. This study is aimed to estimate serum chitotriosidase level, as a screening marker and therapeutic monitor for cystinosis disease in Iraqi children with cystinosis.
Subjects and Methods: The present study is a case-control study that included samples of 30 children with nephropathic cystinosis, compared to 25 healthy control children from those attending at The Genetic Rare Diseases Center / AL-Emamain AL-Kadhimain Teaching Hospital, Baghdad-Iraq.
Results: Our results reported that cystinotic children had a marked elevation of serum chitotriosidase activity, compared to age-matched healthy children, besides a significant associated with leukocyte-cystine content for cystinotic patients.
CHT1 as a Novel Biomarker
Conclusion: Estimation of serum chitotriosidase activity might aid in monitoring the therapeutic benefits of cysteamine therapy, as well as the prognosis of the disease when WBC cystine assessment is not available.
Key Words: Cystinosis, Cysteamine, Chitotriosidase.
The objective of this study is to estimate the effect of the hydro-ethanolic catechin extract toward blood glucose, lipid profile and liver functions in alloxan diabetic mice. 50 healthy mice (25-30 g) were divided into five groups of ten animals for each. Group A received normal saline as normal control group. To induce diabetes, alloxan (150 mg/kg), intraperitoneal (i.p.) single dose was injected to groups B, C, D and E. Group B represents diabetic control group. Groups C, D and E received ethanolic catechin extract (30 mg/kg and 40 mg/kg) for different periods of 1, 2 and 3 weeks as treated groups. Blood glucose, serum lipids [Total Cholesterol (TC), Triglycerides (TGs) and High Density Lipoproteins (HDL)], asparagine transaminase (AST),
... Show MoreThis paper develops the work of Mary Florence et.al. on centralizer of semiprime semirings and presents reverse centralizer of semirings with several propositions and lemmas. Also introduces the notion of dependent element and free actions on semirings with some results of free action of centralizer and reverse centralizer on semiprime semirings and some another mappings.
This study was aimed to produce AuNPs biologically using Klebsiella pneumoniae and study their synergistic effect with some antibiotics.Technologies of nanoparticles are quick and are employed in many applications in biomedicine. The potential of metallic nanoparticle as an anti-microbial agent is greatly investigated which considered as an alternative method to reduce the challenges of multi-drug resistance microbes. The present study discusses the novel approach to synthesize nanoparticles involving eco-friendly synthesis of gold nanoparticles using Klebsiella pneumoniae and study their effect as antimicrobial spectrum .Also study synergism effect of gold nanoparticles with antibiotic against Acinetobacter baumannii. These approac
... Show MoreGroundwater is very important for different uses in the present study area which represents Jisr Diyala, located in southeastern Baghdad and covered with quaternary deposits which include the shallow aquifer in the area. Groundwater and surface water were investigated to determine their suitable uses. The main ion concentrations of the wet period seemed to be lower than those in the dry period. According to TDS values, the water is classified as brackish to salty with a high degree of hardness. Most of water samples were of NaCl type due to pollution with sewage water and rock-water interaction. The results show that the water of the study area is suitable for livestock and irrigation purposes only.
In this work, a series of new Nucleoside analogues (D-galactopyranose linked to oxepanebenzimidazole moiety) was synthesized via multisteps synthesis. The first step involved preparation of two benzimidazoles 2-styrylbenzimidazole and 2-(phenyl ethynyl) benzimidazole via reaction of phenylenediamine with cinnamic acid or ?-phenyl propiolic acid. Electrophilic addition of the prepared benzimidazoles by three anhydrides in the second step afforded (4-6) and (14-16) which in turn were treated with 1,2,3,4-di-O-isopropylidene galactopyranose in the third step to afford a series of the desirable protected nucleoside analogues (7-9) ,(17-19)which after hydrolysis in methanolic sodium methoxidein the fourth step afforded the free nucleoside analog
... Show MoreThis paper includes the synthesis of some new nucleoside analogues starting with 2-substituted benzimidazole derivative (7-9), that synthesized by condensation of O-phenylenediamine with p-chloro benzaldehyde and two substituted benzoic acid , which on nucleophilic substitution with propargyl bromide gave a new N-substituted compounds (10-12). D-Fructose and D-galactose were chosen as a sugar moiety which were protected, brominated and azotated to give azido sugars (5) and (6), then they were subjected to 1,3-dipolar cycloaddition reaction with N-substuted compounds afforded bloked nucleoside analoges (13-16), which after hydrolysis gave our target the free nucleoside analogues (17-20). All prepared compounds were identified by FT-IR
... Show MoreAn interpretive study of two-dimension seismic data of the Huwaiza oilfield was carried out using Petrel 2015 program. Twenty seismic section were used, these seismic sections were carried out at three-time stages: HH survey in 1976, 2HH survey in 1978 and AM in 1980. Mishrif and Nahr Umr reservoirs were selected to study because they are the most important reservoirs in the neighbor fields. The study showed that the structure is an anticline trending North-South, so that the field may belong to Zubair tectonic subzone rather than Tigris subzone, which is northwest-southeast trending. A hypothetical model was drawn up showing the extension of the field in the Iraqi and Iranian territories based on information from both sides, it showed t
... Show MoreThe insulation system of a machine coil includes several layers made of materials with different characteristics. The effective insulation design of machine coils, especially in the machine end winding, depends upon an accurate model of the stress grading system. This paper proposes a modeling approach to predict the transient overvoltage, electric field, and heat generation in machine coils with a stress grading system, considering the variation of physical properties in the insulation layers. A non-uniform line model is used to divide the coil in different segments based on material properties and lengths: overhang, stress grading and slot. The cascaded connection of chain matrices is used to connect segments for the representation of the
... Show MoreMixed phase rutile/anatase of TiO2 was prepared and studied by a closed field DC magnetron sputtering configuration (CFDCMS). It was found that the contents of rutile increased from the ratio of 38% to 53% as the deposition time increased from 3.5 hours to 4.5 hours.
The photocatalytic activity of the mixed phase rutile/anatase TiO2 was measured by monitoring the degradation of the blue methylene dye in an aqueous solution, under exposure to UV-radiation, using UV-vis absorption spectroscopy. It was proven that the photocatalytic activity in the mixed phase (TiO2) is a function of rutile content reaching a maximum value at 53% rutile. Thus, the effect of synergy between anatase- TiO2 and rutile- TiO2 was observed. It was observed that