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.
The finishing operation of the electrochemical finishing technology (ECF) for tube of steel was investigated In this study. Experimental procedures included qualitative
and quantitative analyses for surface roughness and material removal. Qualitative analyses utilized finishing optimization of a specific specimen in various design and operating conditions; value of gap from 0.2 to 10mm, flow rate of electrolytes from 5 to 15liter/min, finishing time from 1 to 4min and the applied voltage from 6 to 12v, to find out the value of surface roughness and material removal at each electrochemical state. From the measured material removal for each process state was used to verify the relationship with finishing time of work piece. Electrochemi
Structural, optical, and electrical properties of thin films of CdS : Zn prepared by the solution – growth technique are reported as a function of zinc concentration. CdS are window layers influencing the photovoltaic response of CIS solar cells. The zinc doping concentration was varied from 0.05 to 0.5 wt %, zinc doping apparently increase the band gap and lowers the resistivity. All beneficial optical properties of chemically deposited CdS thin films for application as window material in heterojunction optoelectronic devices are retained. Heat treatment in air at 400 °C for 1h modify crystalline structure, optical, and electrical properties of solution growth deposited CdS : Zn films.
In this study, gamma ray transmission method have been used to determine the total porosity in four samples: pure Alumina ( Al2O3 ), Al2O3 + (0.2wt%)MgO , Al2O3 + (0.6wt% )Y2O3 and Al2O3+ (8wt% ) ZrO2 .
The experimental setup for the gamma ray transmission consist of 137Cs gamma source ( 662 KeV ), a NaI (Tl) scintillation detector measured the attenuation of strongly collimated gamma beam through alumina samples.
The porosity obtained by the gamma ray transmission method were compare
... Show MoreThis paper reports experimental and computational fluid dynamics (CFD) modelling studies to investigate the effect of the swirl intensity on the heat transfer characteristics of conventional and swirl impingement air jets at a constant nozzle-to-plate distance ( L = 2 D). The experiments were performed using classical twisted tape inserts in a nozzle jet with three twist ratios ( y = 2.93, 3.91, and 4.89) and Reynolds numbers that varied from 4000 to 16000. The results indicate that the radial uniformity of Nusselt number (Nu) of swirl impingement air jets (SIJ) depended on the values of the swirl intensity and the air Reynolds number. The results also revealed that the SIJ that was fitted with an insert of y = 4.89, which correspo
... Show MoreIn the present work is the deposition of copper oxide using the pulsed laser deposition technique using Reactive Pulsed Laser as a Deposition technique (RPLD), 1.064μm, 7 nsec Q-switch Nd-YAG laser with 400 mJ/cm2 laser energy’s has been used to ablated high purity cupper target and deposited on the porous silicon substrates recorded and study the effect of rapid thermal annealing on the structural characteristics, morphological, electrical characteristics and properties of the solar cell. Results of AFM likelihood of improved absorption, thereby reducing the reflection compared with crystalline silicon surface. The results showed the characteristics of the solar cell and a clear improvement in the efficiency of the solar cell in the
... Show MorePhase change materials (PCMs) such as paraffin wax can be used to store or release large amount of energy at certain temperature at which their solid-liquid phase changes occurs. Paraffin wax that used in latent heat thermal energy storage (LHTES) has low thermal conductivity. In this study, the thermal conductivity of paraffin wax has been enhanced by adding different mass concentration (1wt.%, 3wt.%, 5wt.%) of (TiO2) nano-particles with about (10nm) diameter. It is found that the phase change temperature varies with adding (TiO2) nanoparticles in to the paraffin wax. The thermal conductivity of the composites is found to decrease with increasing temperature. The increase in thermal conductivity ha
... Show MoreObjective(s): To assess nurses' practices for neurological unconscious patients in intensive care units.
Methodology: A descriptive study was conducted that included (50) nurse who are working in intensive care
units in hospitals and departments of the nervous system in (4) hospitals (neuroscience hospital, teaching
neurosurgical hospital, surgical specialist hospital, and sheck zaied hospital) in Baghdad city from March, 30th
,
2009 to July, 30th 2009 for the purpose of assessing their skills towards unconscious patients. A purposive "nonprobability
sample" was selected that consisted of (50) nurse who are working in intensive care units. A
questionnaire format and observational checklist were used which consist of
in this article, we present a definition of k-generalized map independent of non-expansive map and give infinite families of non-expansive and k-generalized maps new iterative algorithms. Such algorithms are also studied in the Hilbert spaces as the potential to exist for asymptotic common fixed point.
Due to the importance of egg parasitoids inthe natural and biological control of economically important insects, including egg parasitoid Pseudoligositaba bylonica Viggiani on dubas bug, the spread of the parasitoid and population distribution of parasitoid, In order to estimate the role of parasitoid as one of the biological factors in decreasing population density of dubas bug on date palm. Some aspects of the life of parasitoids were also studied, including the role of insect parasitoids in organizing the population of their families, geographical distribution and hosts range of genus Pseudoligosita, seasonal presence of parasitoid Pseudoligositaba bylonica Viggiani (Hymenoptera: Trichogrammatidae) , life studies and percentages
... Show MoreStatistical methods and statistical decisions making were used to arrange and analyze the primary data to get norms which are used with Geographic Information Systems (GIS) and spatial analysis programs to identify the animals production and poultry units in strategic nutrition channels, also the priorities of food insecurity through the local production and import when there is no capacity for production. The poultry production is one of the most important commodities that satisfy human body protein requirements, also the most important criteria to measure the development and prosperity of nations. The poultry fields of Babylon Governorate are located in Abi Ghareg and Al_Kifil centers according to many criteria or factors such as the popu
... Show More