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.
Gas hydrate formation poses a significant threat to the production, processing, and transportation of natural gas. Accurate predictions of gas hydrate equilibrium conditions are essential for designing the gas production systems at safe operating conditions and mitigating the problems caused by hydrates formation. A new hydrate correlation for predicting gas hydrate equilibrium conditions was obtained for different gas mixtures containing methane, nitrogen and carbon dioxide. The new correlation is proposed for a pressure range of 1.7-330 MPa, a temperature range of 273-320 K, and for gas mixtures with specific gravity range of 0.553 to 1. The nonlinear regression technique was applie
PPSU hollow fiber nanofiltration membranes are prepared by applying two concentrations and various extrusion pressures according to the phase inversion method. Cross-sectional area and outer structures were characterized by using scanning electron microscope (SEM) and atomic force microscopy (AFM). In additional to the pore size distribution, either the mean roughness or the mean pore size of the PPSU hollow fiber surfaces was evaluated by AFM. It was found that the morphology of the PPSU fibers had both sponge-like and finger-like structures through different extrusion pressures and PPSU concentrations. The mean pore size and mean roughness for inner and outer surfaces were seen to be decreased with the increase of extrusion pressure at
... Show MoreIn this paper a dynamic behavior and control of a jacketed continuous stirred tank reactor (CSTR) is developed using different control strategies, conventional feedback control (PI and PID), and neural network (NARMA-L2, and NN Predictive) control. The dynamic model for CSTR process is described by a first order lag system with dead time.
The optimum tuning of control parameters are found by two different methods; Frequency Analysis Curve method (Bode diagram) and Process Reaction Curve using the mean of Square Error (MSE) method. It is found that the Process Reaction Curve method is better than the Frequency Analysis Curve method and PID feedback controller is better than PI feedback controller.
The results s
... Show MoreIn this work ,medical zinc oxide was produced from zinc scraps instead of traditional method which used for medical applications such as skin diseases, Iraq is importing around 50 ton/year for samarra plant the producted powder has apartical size less than 5 micron and the purity was more than 99.98%,also apilot plant of yield capacitiy 15 kg/8hours wsa designed and manufactured .
Before the start of delivery, any membranes rupture could be named as a premature rupture of membranes (PROM), which may need special obstetrical interactions to minimize perinatal complications, it is important topromptly diagnose PROM, the method used should be accurate, cheap, simple, and widely available. This was exactly the idea behind this study to use an ordinary pregnancy test kit aiming to confirm presence of PROM.Over a 6 months’ period, 60 pregnant women with a history of leaking liquor and a positive speculum examination for amniotic fluid pooling were collected prospectively and compared with other 60 women (control group) with uneventful pregnancy. Majority of patients with positive leaking liquor signs and symptoms had a p
... Show MoreIn this research, we have added nano anatase TiO2 as a partial replacement of Portland cement by a weight percentage of (0.25 to 1%) for the development of properties for protection against bacteria. The control mix was made by using "the cement to sand" proportion about (1: 2.75) with the "water to cement" proportion of (0.5) to study the structure, porosity, water absorption, density, mechanical properties, as well as anti-bacterial behavior. Inspections have been done such as scanning electron microscopy (SEM), and atomic force microscope (AFM) for mortar. Experimental results showed that after the addition of Nano powders in cement mortar, the structural properties improved significantly with the development of hydration o
... Show MoreSupport vector machine (SVM) is a popular supervised learning algorithm based on margin maximization. It has a high training cost and does not scale well to a large number of data points. We propose a multiresolution algorithm MRH-SVM that trains SVM on a hierarchical data aggregation structure, which also serves as a common data input to other learning algorithms. The proposed algorithm learns SVM models using high-level data aggregates and only visits data aggregates at more detailed levels where support vectors reside. In addition to performance improvements, the algorithm has advantages such as the ability to handle data streams and datasets with imbalanced classes. Experimental results show significant performance improvements in compa
... Show MoreThe study examined the assessment of raw water and drinking water projects of Diyala Governorate for the year 2017, amounting to (24) projects, The average per capita supply of potable water (0.396 m3 / day/person), which is less than the global standard for the average per capita of drinking water, and constitute water rumors within the network of water transport in the province (3%), and the water of raw and drinking value within the limits allowed to be used by Iraq and the global indicators of {Total acidity, alkaline, acidic function, chlorides, magnesium, Electrical conductivity, total soluble salts, sodium, potassium, sulfates, turbidity other than (raw water)}. While the index of calcium only a value higher than the limits
... Show MorePolypyrrole (PPy) nanocomposites were prepared using chemical oxidation and were combined with manganese oxide (MnO2) nanoparticles. The PPY-MnO2 nanocomposite was synthesized by integrating PPy nanofibers with varying volume ratio percentages of MnO2 dopant (10, 30, and 50% vol. ratio). The structural features of the PPy and PPy-MnO2 nanocomposite were investigated using X-ray diffraction (XRD). Fourier transfor infrared (FTIR) spectroscopy was used to demonstrate the molecular structures of primary materials and the final product of PPy, MnO2, and PPy- MnO2 nanocomposites. Field Emission Scanning Electron Microscopy (FESEM) showed that the morphology of PPy consisted of a network of nanofibers. Increasing the volume ratios of ma
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