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.
<p>Energy and memory limitations are considerable constraints of sensor nodes in wireless sensor networks (WSNs). The limited energy supplied to network nodes causes WSNs to face crucial functional limitations. Therefore, the problem of limited energy resource on sensor nodes can only be addressed by using them efficiently. In this research work, an energy-balancing routing scheme for in-network data aggregation is presented. This scheme is referred to as Energy-aware and load-Balancing Routing scheme for Data Aggregation (hereinafter referred to as EBR-DA). The EBRDA aims to provide an energy efficient multiple-hop routing to the destination on the basis of the quality of the links between the source and destination. In
... Show MoreThe water quality index is the most common mathematical way of monitoring water characteristics due to the reasons for the water parameters to identify the type of water and the validity of its use, whether for drinking, agricultural, or industrial purposes. The water arithmetic indicator method was used to evaluate the drinking water of the Al-Muthana project, where the design capacity was (40000) m3/day, and it consists of traditional units used to treat raw water. Based on the water parameters (Turb, TDS, TH, SO4, NO2, NO3, Cl, Mg, and Ca), the evaluation results were that the quality of drinking water is within the second category of the requirements of the WHO (86.658%) and the first category of the standard has not been met du
... Show MoreClassification of imbalanced data is an important issue. Many algorithms have been developed for classification, such as Back Propagation (BP) neural networks, decision tree, Bayesian networks etc., and have been used repeatedly in many fields. These algorithms speak of the problem of imbalanced data, where there are situations that belong to more classes than others. Imbalanced data result in poor performance and bias to a class without other classes. In this paper, we proposed three techniques based on the Over-Sampling (O.S.) technique for processing imbalanced dataset and redistributing it and converting it into balanced dataset. These techniques are (Improved Synthetic Minority Over-Sampling Technique (Improved SMOTE), Border
... Show MorePublic private partnership PPP is a method to procure public projects in order to achieve additional value for money in terms of efficiency and quality of services. This thesis studies the concepts of PPP, advantages and disadvantages of PPP. In addition, current Iraq infrastructure projects situations and needs, as well as, some aspects relating to the Iraq’s construction market, legal and contract systems were discussed. A financial model was carried out and applied to a real-life case study project. Finally, a survey targeted researchers; public and private- sectors were applied.
KE Sharquie, AA Noaimi, SD Hameed, Journal of Cosmetics, Dermatological Sciences and Applications, 2013 - Cited by 15
This study presents an adaptive control scheme based on synergetic control theory for suppressing the vibration of building structures due to earthquake. The control key for the proposed controller is based on a magneto-rheological (MR) damper, which supports the building. According to Lyapunov-based stability analysis, an adaptive synergetic control (ASC) strategy was established under variation of the stiffness and viscosity coefficients in the vibrated building. The control and adaptive laws of the ASC were developed to ensure the stability of the controlled structure. The proposed controller addresses the suppression problem of a single-degree-of-freedom (SDOF) building model, and an earthquake control scenario was conducted and simulat
... Show MoreAbstract: Under high-excitation irradiance conditions to induce fluorescence, the dependence of photobleaching of Coumarin 307 (C307) and acriflavine (ACF) laser dyes in liquid and solid phases have been studied. A cw LD laser source of 1 mW and 407 nm wavelength was used as an exciting source. For one hour exposure time, it was found that the solid dye samples suffer photobleaching more than the liquid dye samples. This is because in liquid solutions the dye molecules can circulate during the irradiation, while the photobleaching is a serious problem when the dye is incorporated into solid matrix and cannot circulate.