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.
Iraq has suffered since 1991 from international sanctions imposed on it by the dictatorial regime that existed at the time, invading Kuwait, which led to the decline of the status of Iraq and the isolation of international and regional (Arab) and clear Iraq as a strange entity living within its regional environment, after April 2003 did not change much In fact, there were no signs of détente before the Arab League summit in Baghdad in 2011, and the signs of a break in the stalemate in inter-relations over the past years have become evident. Disruption and refraction was not high (Islamic Republic of Iran, Turkey, Syrian Arab Republic, Hashemite Kingdom of Jordan, Saudi Arabia, Kuwait). Each side is governed by perceptions about Iraq, es
... Show MoreThe response of the combustor’s liner to the air-flow that passes through it is the key reason for the combustion chambers noise, hence the instabilities of those chambers that decreases the mechanical efficiency of such sections, by increased its mechanical vibrations, which increases the failure rate created during originating of the cracks spreading by the shakes producing by the series of high-level frequencies. Accordingly, any work debating the impact of the context of liners in the combustion chamber can provide grasping for the combustion noise generated by the undesirable vibrations, and benefits the industrial firms to design an ideal production procedure which increases the lifespan of the combustor. The goal of this work is
... Show MoreThe art of calligraphy is regarded as one of the tremendous and distinct products in the history of Islamic arts through the unparalleled high ranks it reached to, until it gained Islam's indication and became a symbol for it, in terms of its function linked to the calligraphy of the holy Qur'anic texts, where this merit formed a strong impulse in seeking its development, the emergence of the calligraphic constructs, including the calligraphic straps, is one of this continual seeking products, and an advanced stage at the level of technical and expressive development, and also one of the natural results generated by the development of fonts and the increase of their employing methods through what they recorded at the level of formal char
... Show MoreMost of us are tired of the circumstances that surround us because of their deficiency, deprivation, and sullenness, even though troubles and pains are the soil in which the seeds of strong personality sprout.
The content of the research is summed up in the fact that there is no need to frown, so let our face be free, and our word good and tolerant, so that we can be the most loving people of those who give them, and the Messenger of God (may God’s prayers and peace be upon him and his family) was known to be the heaviest concerns of people, but he was most smiling of people, by smiling, we buy lives, so we should get used to it, because that gives us hope and finds the world in our hands, an
... Show MoreCarbides or nitrides thin films present materials with good mechanical properties for industrial applications as they can be coatings at low temperatures serve temperature sensitive surfaces. In this work the effect of the C percentage on the mechanical properties represented by the Young modulus (E) of combinatorial magnetron sputtered TiCx (34%x˂65%) has been studied. The structure of the produced films is TiC independent on the C concentration. The mechanical properties are increased with increasing the C concentration up to 50%, and then decreasing with further C % increasing. These results can be explained by considering the resultant residual stresses.
Background: The microhardness of a composite resin is a vital parameter that is used to determine its clinical behavior. Measuring the microhardness of a composite resin has been used as an indirect method to assess its degree of conversion and extent of polymerization. The purpose of this in vitro study was to evaluate the effect of three curing distances (0, 2, and 4 mm) on the microhardness of the top and bottom surfaces of three types of flowable bulk-fill composite resins (smart dentin replacement, Opus bulk fill flow, and Tetric N). Material and method: Sixty-three specimens from the three types of composite resins (n=21) were fabricated using Teflon mold with a 4mm depth and a 5 mm internal diameter and cured for 20 seconds. For e
... Show MoreThe physical, the thermal and the mechanical properties of Nano-composites, that consisted of Polyprime EP epoxy that reinforced by multi-walled carbon nanotubes (MWCNTs), have been studied. Various loading ratios, 0.1, 0.5, and 1 wt. %of MWCNT shave been infused into epoxy by a magnetic stirrer and then the hardener mixed with the mthat supplied with the epoxy. All sample shave been cutting using CNC machine. Tensile test, three-point bending, hardness tests, lee's disk, differential scanning calorimetry, water absorption and dielectric and electrical conductivity test were utilized on unfilled, MWCNT-filled epoxy to identify the loading effect on the properties of materials. Scanning electron microscopy (SEM) was used to determine the
... Show MoreAbstract
In order to enhance the efficiency of flat plate solar water collectors without changing in its original shape and with low additional cost, twisted strips are inserted inside its riser pipes. Three flat plate collectors are used for test. Family of twisted strips are inserted inside each collector risers with different twisted ratios (TR=3,4,5). The collectors are connected in parallel mode (Z-Configuration) and are exposed to the same conditions (solar radiation and ambient temperature) .The experimental results show that, the highest heat transfer rate occurs at twisted ratio (3) .Consequently, for the same twisted ratio the daily efficiencies for the solar collector at d
... Show MoreThis research aims to investigate the effect of four types of nanomaterial on the Marshall properties and durability of warm mix asphalt (WMA). These types are; nano silica(NS), nano carbonate calcium (NCC), nano clay(NC), and nanoplatelets (NP). For each type of Nanomaterial, three contents are tried as following; NS(1%, 3%, and 5%), NCC(2%, 4%, and 6%), NC(3%, 5%, and 7%), and NP (2%, 4%, and 6%) by weight of asphalt cement. Following Marhsall mix design method, the optimum asphalt cement content is determined, thereafter the optimum dosage for each nanomaterial is obtained based on the highest Marshall stability value. The durability of the control mix (no nanomaterial) and modified mixtures have been compared based on moisture damage, r
... Show MoreThe advancement of cement alternatives in the construction materials industry is fundamental to sustainable development. Geopolymer is the optimal substitute for ordinary Portland cement, which produces 80% less CO2 emissions than ordinary Portland cement. Metakaolin was used as one of the raw materials in the geopolymerization process. This research examines the influence of three different percentages of sulfate (0.00038, 1.532, and 16.24) % in sand per molarity of NaOH on the compressive strength of metakaolin-based geopolymer mortar (MK-GPM). Samples were prepared with two different molarities (8M and 12M) and cured at room temperature. The best compressive strength value (56.98MPa) was recorded with 12M w
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