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.
Free radicals are small extremely reactive species that have unpaired electrons. Free radicals include subgroups of reactive species, which are all a product of regular cellular metabolism. Oxidative stress happens when the free radicals production exceeds the capacity of the antioxidant system in the body’s cells.
The current review clarifies the prospective role of antioxidants in the inhibition and healing of diseases.
Information on oxidative stress, free radicals, reactive oxidant species, and natural and synthetic antioxidants was obta
This study investigates the impact of spatial resolution enhancement on supervised classification accuracy using Landsat 9 satellite imagery, achieved through pan-sharpening techniques leveraging Sentinel-2 data. Various methods were employed to synthesize a panchromatic (PAN) band from Sentinel-2 data, including dimension reduction algorithms and weighted averages based on correlation coefficients and standard deviation. Three pan-sharpening algorithms (Gram-Schmidt, Principal Components Analysis, Nearest Neighbour Diffusion) were employed, and their efficacy was assessed using seven fidelity criteria. Classification tasks were performed utilizing Support Vector Machine and Maximum Likelihood algorithms. Results reveal that specifi
... Show MoreObjective: To determine the prevalence of Posttraumatic Stress Disorder (PTSD) among Iraqi repatriated
prisoners of Iran-Iraq war, and the relationship with demographic factors.
Methodology: A descriptive study was carried out from Oct. 18th, 2009 through Jan. 10th, 2010. A nonprobability
based snowball sampling technique was used to recruit 92 Iraqi repatriated prisoners of war
(IRPOWs) who had visited Ministry of Human Rights. A data collection instrument was constructed that
consisted of six demographic characteristics, and eight items to measure the level of PTSD in POWs. Data were
collected with the constructed instrument during a brief interview. Data were analyzed through the application of
descriptive statist
Background: The use of the cone beam computed tomography for analysing the position of the greater palatine foramen in relation to various anatomical landmarks is crucial in dentistry. The aims of the current study, firstly is to determine the greater palatine foramen position in relation to various anatomical landmarks by using cone beam computed tomography and secondly is to make a comparison of the measurements according to side, gender, and age. Materials and methods: This prospective study included 60 Iraqi patients (28males and 32 females) who selected according to availability of Inclusion criteria, which include age range (21 - 60 years), with no dentofacial deformities or pathological lesion at the maxilla. All patients had inform
... Show MoreIn this study , Iraqi Bentonite clay was used as a filler for polyvinyl chloride polymer. Bentonite clay was prepared as a powder for some certain particle size ,followed by calcinations process at (300,700,900) OC ,then milled and sieved. The selected sizes were D ~75 µm and D ~150. After that polyvinyl Al-Cohool solution prepared and used as a coated layer covered the Bentonite powder before applied as a filler ,followed by drying , milling and sieving for limited recommend sizes. polyvinyl chloride solutions were prepared and adding of modified Bentonite power at certain quantities were followed .Sheet of these variables on the mechanical and thermal properties of the prepared reinforced particular polyvinyl chloride composite
... Show MoreThe musical sign was associated with the first appearances of acting that are based on human gestures and expressive and silent movements, and they contributed in accentuating and clarifying these gestures and explain their meanings and indications, and they remained with them until these gestures turned into artistic theatrical shows so that the musical sign would have many functions and constitute the main pillars for this kind of show.
The musical sign is a language not much different than the human language in the semiotic analysis. There has been an increased interest in it and its use in the mime shows by the graduates who realized its necessity and significance in the pantomime show intellectually, artistically and aesthetical
The nuclear level density parameter in non Equi-Spacing Model (NON-ESM), Equi-Spacing Model (ESM) and the Backshifted Energy Dependent Fermi Gas model (BSEDFG) was determined for 106 nuclei; the results are tabulated and compared with the experimental works. It was found that there are no recognizable differences between our results and the experimental -values. The calculated level density parameters have been used in computing the state density as a function of the excitation energies for 58Fe and 246Cm nuclei. The results are in a good agreement with the experimental results from earlier published work.
Materials with external dimensions of one or more nanometers are referred to as nanomaterials. These structures result from a number of manufacturing processes. They are used in many industries, including pharmaceuticals, which is the most significant one. Numerous variables, including size, shape, surface morphology, crystallinity, solubility, etc., affect physical properties. While new physical and chemical processes are being created constantly, the biological method is the ideal strategy for synthesizing nanoparticles since it is straightforward, safe, and economical. Different kinds of nanoparticles can be metabolically synthesized by a wide variety of biological sources, including plants, bacteria, fungi, and yeast. There are
... Show MoreSolubility problem of many of effective pharmaceutical molecules are still one of the major obstacle in theformulation of such molecules. Candesartan cilexetil (CC) is angiotensin II receptor antagonist with very low water solubility and this result in low and variable bioavailability. Self- emulsifying drug delivery system (SEDDS) showed promising result in overcoming solubility problem of many drug molecules. CC was prepared as SEDDS by using novel combination of two surfactants (tween 80 and cremophore EL) and tetraglycol as cosurfactant, in addition to the use of triacetin as oil. Different tests were performed in order to confirm the stability of the final product which includes thermodynamic study, determination of self-emulsificat
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