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.
In this paper, we characterize the percolation condition for a continuum secondary cognitive radio network under the SINR model. We show that the well-established condition for continuum percolation does not hold true in the SINR regime. Thus, we find the condition under which a cognitive radio network percolates. We argue that due to the SINR requirements of the secondaries along with the interference tolerance of the primaries, not all the deployed secondary nodes necessarily contribute towards the percolation process- even though they might participate in the communication process. We model the invisibility of such nodes using the concept of Poisson thinning, both in the presence and absence of primaries. Invisibility occurs due to nodes
... Show MoreAmong a variety of approaches introduced in the literature to establish duality theory, Fenchel duality was of great importance in convex analysis and optimization. In this paper we establish some conditions to obtain classical strong Fenchel duality for evenly convex optimization problems defined in infinite dimensional spaces. The objective function of the primal problem is a family of (possible) infinite even convex functions. The strong duality conditions we present are based on the consideration of the epigraphs of the c-conjugate of the dual objective functions and the ε-c-subdifferential of the primal objective functions.
Thermal evaporation method has used for depositing CdTe films
on corning glass slides under vacuum of about 10-5mbar. The
thicknesses of the prepared films are400 and 1000 nm. The prepared
films annealed at 573 K. The structural of CdTe powder and prepared
films investigated. The hopping and thermal energies of as deposited
and annealed CdTe films studied as a function of thickness. A
polycrystalline structure observed for CdTe powder and prepared
films. All prepared films are p-type semiconductor. The hopping
energy decreased as thickness increased, while thermal energy
increased.
This research has discussed the origins of ESP, addressed key notions about ESP and examined issues in ESP syllabus design. The content of the paper was determined by a need identified based on my experience as an ESL instructor designing and delivering the content-based language program - Language Preparation for the Cadets and Employment in the Iraqi College of Police . These issues, where possible, have been supported by current and pertinent academic literature. It is my sincerest hope that these observations will lend insight into the challenges facing the ESL instructor acting as ESP syllabus developer.
Building a system to identify individuals through their speech recording can find its application in diverse areas, such as telephone shopping, voice mail and security control. However, building such systems is a tricky task because of the vast range of differences in the human voice. Thus, selecting strong features becomes very crucial for the recognition system. Therefore, a speaker recognition system based on new spin-image descriptors (SISR) is proposed in this paper. In the proposed system, circular windows (spins) are extracted from the frequency domain of the spectrogram image of the sound, and then a run length matrix is built for each spin, to work as a base for feature extraction tasks. Five different descriptors are generated fro
... Show MoreRegression testing being expensive, requires optimization notion. Typically, the optimization of test cases results in selecting a reduced set or subset of test cases or prioritizing the test cases to detect potential faults at an earlier phase. Many former studies revealed the heuristic-dependent mechanism to attain optimality while reducing or prioritizing test cases. Nevertheless, those studies were deprived of systematic procedures to manage tied test cases issue. Moreover, evolutionary algorithms such as the genetic process often help in depleting test cases, together with a concurrent decrease in computational runtime. However, when examining the fault detection capacity along with other parameters, is required, the method falls sh
... Show MoreJava is a high-level , third generation programming language were introduced Javaoptics Open Source Physics (OSP) as a new simulation for design one of the most important interference optical coating called antireflection coating. It is recent developments in deign thin-film coatings. (OSP) shows multiple beam interferences from a parallel dielectric thin film and the evolution of reflection factors. It is simple to use and efficiently also can serve educational purposes. The obtained results have been compared with needle method
A .technology analysis image using crops agricultural of grading and sorting the test to conducted was experiment The device coupling the of sensor a with camera a and 75 * 75 * 50 dimensions with shape cube studio made-factory locally the study to studio the in taken were photos and ,)blue-green - red (lighting triple with equipped was studio The .used were neural artificial and technology processing image using maturity and quality ,damage of fruits the of characteristics external value the quality 0.92062, of was value regression the damage predict to used was network neural artificial The .network the using scheme regression a of means by 0.98654 of was regression the of maturity and 0.97981 of was regression the of .algorithm Marr
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