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.
A microbial desalination cell (MDC) is a new approach to bioelectrochemical systems. It provides a more sustainable way to electrical power production, saltwater desalination, and wastewater treatment at the same time. This study examined three operation modes of the MDC: chemical cathode, air cathode, and biocathode MDC, to give clear sight of this system's performance. The experimental work results for these three modes were recorded as power densities generation, saltwater desalination rates, and COD removal percentages. For the chemical cathode MDC, the power density was 96.8 mW/m2, the desalination rate was 84.08 ppm/hr, and the COD removal percentage was 95.94%. The air cathode MDC results were different
... Show MoreBackground: Mother-infant bonding is an important psychological step postpartum and disturbed relationship may carry dramatic consequences as a psychological disorder which may affect the periodontal health of the mother. The aim of the present study was to assess the effect of the postpartum Mother-infant bonding on their periodontal condition. Materials and Methods: Mothers in the postpartum period with age range 20-35 years were subjected to postpartum Bonding Questionnaire (PBQ). Periodontal health status was assessed by measuring probing pocket depth and clinical attachment level. Results: The mean values of both probing pocket depth (PPD) and clinical attachment loss (CAL) were higher among disordered mothers than mothers with normal
... Show MoreAbstract: Despite the distinct features of the continuous wave (CW) Terahertz (THz) emitter using photomixing technique, it suffers from the relatively low radiation output power. Therefore, one of effective ways to improve the photomixer emitter performance was using nanodimensions electrodes inside the optical active region of the device. Due to the nanodimension sizes and good electrical conductivity of silver nanowires (Ag-NWs), they have been exploited as THz emitter electrodes. The excited surface plasmon polariton waves (SPPs) on the surface of nanowire enhances the incident excitation signal. Therefore, the photomixer based Ag-NW compared to conventional one significantly exhibits higher THz output signal. In thi
... Show MoreA modified Leslie-Gower predator-prey model with a Beddington-DeAngelis functional response is proposed and studied. The purpose is to examine the effects of fear and quadratic fixed effort harvesting on the system's dynamic behavior. The model's qualitative properties, such as local equilibria stability, permanence, and global stability, are examined. The analysis of local bifurcation has been studied. It is discovered that the system experiences a saddle-node bifurcation at the survival equilibrium point whereas a transcritical bifurcation occurs at the boundary equilibrium point. Additionally established are the prerequisites for Hopf bifurcation existence. Finally, using MATLAB, a numerical investigation is conducted to verify the va
... Show MoreThis study examines the impact of adopting International Financial Reporting Standards (IFRS) on the value of economic units. Given the global push toward standardization of financial reporting to enhance financial statement transparency, comparability, and reliability, this research seeks to understand the implications of these standards for economic valuation within a region characterized by its unique economic and regulatory challenges. A questionnaire was distributed to 86 Iraqi academics specializing in economics, accounting, and finance to collect their views on the impact of adopting international financial reporting standards. Through careful statistical analysis, the study concluded that applying international financial reporting s
... Show MoreThe current research deals with short term forecasting of demand on Blood material, and its' problem represented by increasing of forecast' errors in The National Center for Blood Transfusion because using inappropriate method of forecasting by Centers' management, represented with Naive Model. The importance of research represented by the great affect for forecasts accuracy on operational performance for health care organizations, and necessity of providing blood material with desired quantity and in suitable time. The literatures deal with subject of short term forecasting of demand with using the time series models in order to getting of accuracy results, because depending these models on data of last demand, that is being sta
... Show MoreTo understand what the arts in general, and plastic arts in particular, have to do, it is necessary to know how they perform, not only that, but also to know their history, which we should understand at least in general terms. Which in turn gives an image of the standards of taste prevailing in each age, and some of the social and historical relationship of each era, and the cultural expression of that era, which is shown in the arts. We need to understand that the arts of each stage, although different from each other, yet remain interrelated in what we see through the effects that appear through the succession of times, which creates certain artistic traditions, inherited by one generation after the other. The research in this field de
... Show MoreThe problem of the research represented by knowing the approaches which presented by knowing the design guides and the design styles which the imitation products enter upon it to be able to competitor the other original products .The research aim was to comparison the design approaches of the functional performance and the utilizations for the imitation and the original products , however the research limits contained study products samples of the kitchen electric equipments which available in local markets of Baghdad city for the period (2000 – 2013) .After the view of research back for the subject fundamental basics and investigating research sample to be complete to a group of results and conclusions and the most marked of her were
... Show MoreThe impacts of harvested cropland in the double cropping region (DCR) of the northern China plains (NCP) on the regional climate are examined using surface meteorological data and the satellite-derived normalized difference vegetation index (NDVI) and land surface temperature (LST). The NDVI data are used to distinguish the DCR from the single cropping region (SCR) in the NCP. Notable increases in LST in the period May–June are found in the area identified as the DCR on the basis of the NDVI data. The difference between the mean daily maximum temperature averaged over the DCR and SCR stations peaks at 1.27°C in June. The specific humidity in the DCR is significantly smaller than in
A modified Leslie-Gower predator-prey model with a Beddington-DeAngelis functional response is proposed and studied. The purpose is to examine the effects of fear and quadratic fixed effort harvesting on the system's dynamic behavior. The model's qualitative properties, such as local equilibria stability, permanence, and global stability, are examined. The analysis of local bifurcation has been studied. It is discovered that the system experiences a saddle-node bifurcation at the survival equilibrium point whereas a transcritical bifurcation occurs at the boundary equilibrium point. Additionally established are the prerequisites for Hopf bifurcation existence. Finally, using MATLAB, a numerical investigation is conducted to verify t
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