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.
Researchers are increasingly using multimodal biometrics to strengthen the security of biometric applications. In this study, a strong multimodal human identification model was developed to address the growing problem of spoofing attacks in biometric security systems. Through the use of metaheuristic optimization methods, such as the Genetic Algorithm(GA), Ant Colony Optimization(ACO), and Particle Swarm Optimization (PSO) for feature selection, this unique model incorporates three biometric modalities: face, iris, and fingerprint. Image pre-processing, feature extraction, critical image feature selection, and multibiometric recognition are the four main steps in the workflow of the system. To determine its performance, the model wa
... Show MoreSpatial and frequency domain techniques have been adopted in this search. mean
value filter, median filter, gaussian filter. And adaptive technique consists of
duplicated two filters (median and gaussian) to enhance the noisy image. Different
block size of the filter as well as the sholding value have been tried to perform the
enhancement process.
Non-orthogonal Multiple Access (NOMA) is a multiple-access technique allowing multiusers to share the same communication resources, increasing spectral efficiency and throughput. NOMA has been shown to provide significant performance gains over orthogonal multiple access (OMA) regarding spectral efficiency and throughput. In this paper, two scenarios of NOMA are analyzed and simulated, involving two users and multiple users (four users) to evaluate NOMA's performance. The simulated results indicate that the achievable sum rate for the two users’ scenarios is 16.7 (bps/Hz), while for the multi-users scenario is 20.69 (bps/Hz) at transmitted power of 25 dBm. The BER for two users’ scenarios is 0.004202 and 0.001564 for
... Show MoreThere has been increase in banks and financial institutions interest in governance topic, as it focused all it attention on this topic specially after the growing role and massive expansion of the private sector in economic life , this sector become vital and important and basic role in growth and developing countries economy that’s why there been increase interest in governance and its mechanism , using this mechanism ( board of directors mechanism , ownership concentration mechanism , financial compensation mechanism, minimum rate of capital mechanism) who would adjust and guide the path and orientation of banks and financial institutions as to ensure the achievement of their goals and mission of their desired within th
... Show MoreMonaural source separation is a challenging issue due to the fact that there is only a single channel available; however, there is an unlimited range of possible solutions. In this paper, a monaural source separation model based hybrid deep learning model, which consists of convolution neural network (CNN), dense neural network (DNN) and recurrent neural network (RNN), will be presented. A trial and error method will be used to optimize the number of layers in the proposed model. Moreover, the effects of the learning rate, optimization algorithms, and the number of epochs on the separation performance will be explored. Our model was evaluated using the MIR-1K dataset for singing voice separation. Moreover, the proposed approach achi
... Show MoreGivers of foreign Audit about Social Responsibility of Profit Organization. The recent time is charcterstically with big economic Organization activities, because there are many transactions between these Organizations and different financial markets development techniques.
This encourgage business men to increase their efforts for investment in these markets. Because the Accounting is in general terms it represents a language of these Unions Activities and translate them in to fact numbers, for that there is need for Accounting recording for certain of these Organizations behavior and their harmonization with their Objectives.
In this respect the Audit function comes to che
... Show MoreThe present calculation covers the building shield during irradiation process and under water storage of three milion curries Cobalt-60 radiation source the calculation results in design requirement of 8m depth of water in the source stoeage pool
As they are the smallest functional parts of the muscle, motor units (MUs) are considered as the basic building blocks of the neuromuscular system. Monitoring MU recruitment, de-recruitment, and firing rate (by either invasive or surface techniques) leads to the understanding of motor control strategies and of their pathological alterations. EMG signal decomposition is the process of identification and classification of individual motor unit action potentials (MUAPs) in the interference pattern detected with either intramuscular or surface electrodes. Signal processing techniques were used in EMG signal decomposition to understand fundamental and physiological issues. Many techniques have been developed to decompose intramuscularly detec
... Show MoreThis 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.