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.
Back ground: Zygote produce from once a sperm fertilizes an egg cell. Then, the zygote (unicellular) will begin chain of cellular cleavages to produce multicellular mass, its embryo, the differentiated to different tissues and organism. The development of the embryo is called embryogenesis. Coenzyme Q10, is an antioxidant produced in the body. It boosts cellular energy and may enhance the immune system. CoQ10 is present and measurable in seminal fluid, the concentration of CoQ10 directly correlates with both sperm count and motility. It is beneficial in the prevention and treatment a wide range of health problems. Objectives: The present study was aimed to investigate the possibility of using coenzyme Q10 to improve in vitro fertilization (
... Show MoreThis research includes the synthesis of some new different heterocyclic derivatives of 5-Bromoisatin. New sulfonylamide, diazine, oxazole, thiazole and 1,2,3-triazole derivatives of 5-Bromoisatin have been synthesized. The synthesis process started by the reaction of 5-Bromoisatin with different reagents to obtain schiff bases of 5-Bromoisatin intermediate compounds(1, 8, 19) by using glacial acetic acid as a catalyst in three routes. The first route, 5-Bromoisatin reacted with p-aminosulfonylchloride to product compound(1), then converted to sulfonyl amide derivatives(2-7) by the reaction of compound(1) with different substituted primary aromatic amine in absolute ethanol. The second route includes the reaction of 5-Bromoisatin rea
... Show MoreIn this work, a local sunflower husk (SFH) was used as a natural surface for removing Basic Green-4 (BG4) dye, as a watersoluble pollutant. The effect of initial concentration, contact time, the mass of surface of the dye with the SFH as well as the medium temperature was studied. The application of Langmuir, Freundlich isotherms on the collected data of the adsorption process found to harmonize to Freundlich equation more than that of Langmuir. However, the adsorbed mass of BG4 dye showed a direct increase with the increase of SFH mass and equilibrium was achieved within a 60min window. The interaction of BG4 with SFH surface was spontaneous and exothermic. The empirical kinetic outcomes at ambient temperatures were applied to pseudo 1st a
... Show MoreThis study aimed to evaluate the surface changes of commercial pure Titanium disks (CP Ti) and the Ti 13Nb 13 Zr (Alloy) with a zigzag pattern of laser surface treatment. In vitro, experimental study of CNC Laser treatment on the CP Ti and Alloy disks. Texturing the surfaces of CP Ti and Alloy disks via CNC laser, the sample disks were analyzed using surface roughness, wettability and FESEM. The FESEM revealed a proper increase in the surface texturing and roughness on macro and micro measures without crack formation or dramatic change of the core substance of the CP Ti and Alloy disks. The CNC laser is an effective and suitable method for surface texturing CP Ti and Alloy for dental implantology. Keywords: Commercial pure Titanium;
... Show MoreAcetophenone sulfamethoxazole and 3-Nitrobenzophenone sulfamethoxazole were prepared from the reaction of sulfamethoxazole with two ketones. The prepared ligands were identified by (C.H.N) analysis and UV-VIS, FT-IR spectroscopic techniques. Metal complexes of the two ligands were prepared in an aqueous alcohol with Zn (II), Mn (II) and Cu (II) ions with a molar ratio1:1. The proposed general formula for the resulting complexes was [ML.CL2.H2O]H2O .The complexes were characterized by (C.H.N) technique , spectroscopic methods ,conductivity, atomic absorption ,magnetic susceptibility measurements and melting point. According to the results obtained, the suggested geometry is to be octahedral for all the complexes.
The subject of the organizational Ambidexterity of the vital Topics through which it seeks organizations to provide mentalities renewable for their members and maintain its survival and continuity according to different organizational methods of access for strategic success. The research aims to demonstrate the impact of organizational Ambidexterity in achieving strategic success in the National bank of Iraq, and the questionnaire was prepared as a tool for collecting data and information through sample survey of (16) managers and heads of departments , The results were analyzed by using the statistical program (SPSS) in calculating mean, standard deviation, percent and test (f), coefficie
... Show MoreBalance is considered one of the most important components of physical activity in individual and team sports because it allows proper motor response and performance accuracy. The problem of the research lies in the lack of model for motor balance tests in the field of sports that require different positions and movements for classifying, selecting, diagnosing, and comparing athletes. The importance of the research lies in designing a test for motor balance as a reference for specialists in the field of sports and sport sciences. The subjects were first year College of physical education and sport sciences students / Baghdad University 2016 – 2017. The data was collected and treated using proper statistical operations. The researchers con
... Show MoreSome geological phenomena as landslides and the mobilization of the accumulated rocks or soil are discussed in this research by using projectiles that cause mobility and falling of these land masses which are present at the top of mountains and edges of roads and streets to avoid accidents and human disasters which will occur if they are left falling by effect of climate or vibrating factors that are produced by performing dams, bridges and reservoirs. According to the different divisions of land masses groups, primary and secondary, which depend on type of movement and material arrangement that form the mobile masses, this research had shown the effect of projectiles for every type of cannons on the mobility of every groups of these rocks
... Show MoreSome biological aspects of the zebra mussel, Dreissena polymorpha have been studied at Al-Musayab thermal power plant ,sixty km. south west of Baghdad. Data collected during the period extended from November, 2002 to October, 2003 except for the month of April The population consisted of five age groups; O, I, II, III, and IV which have 0, 1, 2, 3 and 4 annuli respectively. The study also proved the validity of annuli readings for age and growth determination. The average annual growth rates for age groups O,I, II, III, and IV were 5.7, 5.5, 5.4, 5.2 and 5.4 respectively. Average calculated length for laboratory reared mussel was 2.5 mm compared to 5.4 mm in natural environment. Correlation coefficients were very high between age an
... Show More