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 national educational systems both in the Russian Federation and Iraq Republic have to adjust the training programs to duly prepare the pedagogical university students for the modern challenges and situations on the market of educational services. For success of the service, the education specialists have to fully mobilize their physical, mental and emotional resources and persistently advance their skills and knowledge using the relevant online education courses; practical research conferences; persistent self-education to master theoretical fundamentals of the modern physical education and sport service; and be active in trainings and competitions in their vocational individual game sports including badminton, table tennis and tennis.
... Show MoreIn this research a study of the effect of quality, sequential and directional layers for three types of fibers are:(Kevlar fibers-49 woven roving and E- glass fiber woven roving and random) on the fatigue property using epoxy as matrix. The test specimens were prepared by hand lay-up method the epoxy resin used as a matrix type (Quick mast 105) in prepared material composit . Sinusoidal wave which is formed of variable stress amplitudes at 15 Hz cycles was employed in the fatigue test ( 10 mm )and (15mm) value 0f deflection arrival to numbers of cycle failure limit, by rotary bending method by ( S-N) curves this curves has been determined ( life , limit and fa
... Show MoreA series of overbased magnesium fatty acids such as caprylate, caprate, laurate, myristate, palmitate, stearate and oleate) were synthesized by the reaction of the fatty acids with active – 60 magnesium oxide and carbon dioxide (CO2) gas at 60 oC in the presence of ammonia solution as catalyst, toluene / ethanol solvent mixture (9:1vol/vol) was added.
The prepared detergent additives were characterized by FTIR, 1HNMR and evaluated by blending each additive in various concentrations with medium lubricant oil fraction (60 stock) supplied by Iraqi Midland Refineries Company. The total base number (TBN, mg of KOH/g) was determined, and the results of TBN were treated by using two-way analysis of variance (ANOVA) test. It was found that
Background : It has been suggested that pretreatment with a statin agent prior to
myocardial infarction limits myocardial
creatine kinase release, and thus may act to
limit myocardial infarct size in humans.
Objective : To examine the effect of very
early statin initiation for acute myocardial
infarction (AMI), to the extent of
myonecrosis as manifested by peak serum
creatine kinase levels.
Methods : Patients with AMI admitted to AlKindy teaching hospital cardiac care unit
from 1st February 2007 to 28th February
2008, who fulfilled the inclusion criteria
cited in the present study, were randomly
assigned into two study groups. The statin
group patients have received a single oral
dose of 40 mg
Shell model and Hartree-Fock calculations have been adopted to study the elastic and inelastic electron scattering form factors for 25Mg nucleus. The wave functions for this nucleus have been utilized from the shell model using USDA two-body effective interaction for this nucleus with the sd shell model space. On the other hand, the SkXcsb Skyrme parameterization has been used within the Hartree-Fock method to get the single-particle potential which is used to calculate the single-particle matrix elements. The calculated form factors have been compared with available experimental data.
The ground charge density distributions (CDD), elastic charge form factors and proton, charge, neutron, and matter root mean square (rms) radii for stable 40Ca and 48Ca have been calculated using single-particle radial wave functions of Woods-Saxon (WS) and harmonic-oscillator (HO) potentials. Different central potential depths are used for each subshell which is adjusted so as to reproduce the experimental single-nucleon binding energies. An excellent agreement between the calculated rms charge radii and experimental data are found for both nuclei using WS and HO potentials. The calculated proton rms radii for 40Ca are found to be in good agreement with experiment data using both WS and HO potentials while the results for 48Ca showed an ov
... Show MoreThe bound radial wave functions of Cosh potential which are the solutions to the radial part of Schrodinger equation are solved numerically and used to compute the size radii; i.e., the root-mean square proton, neutron, charge and matter radii, ground density distributions and elastic electron scattering charge form factors for nitrogen isotopes 14,16,18,20,22N. The parameters of such potential for the isotopes under study have been opted so as to regenerate the experimental last single nucleon binding energies on Fermi's level and available experimental size radii as well.
The measurement of vitamin B1 in pure and pharmaceutical formulations was proposed by using a straightforward and sensitive spectrophotometric approach. Sulfacetamide (SFA) is diazotized, then coupled with vitamin B1 in alkaline media to produce a colored azo dye complex with a stability constant of 5.597 × 105 L/mol. The product is stable, with a maximum absorption wavelength of 489.5 nm, molar absorptivity of 10108 L/mol∙cm, Sandell's sensitivity of 0.0334 μg/cm2, detection limit of 0.0135 μg/mL, and Beer's law being observed over the concentration range of 0.2–20.0 μg/mL. The stability constant and stoichiometry of the produced azo dye were calculated using the continuous variation (Job's) and mole ratio methods. The suggested ap
... Show MoreThe ejector refrigeration system is a desirable choice to reduce energy consumption. A Computational Fluid Dynamics CFD simulation using the ANSYS package was performed to investigate the flow inside the ejector and determine the performance of a small-scale steam ejector. The experimental results showed that at the nozzle throat diameter of 2.6 mm and the evaporator temperature of 10oC, increasing boiler temperature from 110oC to 140oC decreases the entrainment ratio by 66.25%. At the boiler temperature of 120oC, increasing the evaporator temperature from 7.5 to 15 oC increases the entrainment ratio by 65.57%. While at the boiler temperature of 120oC and
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