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.
Objectives The strategies of tissue-engineering led to the development of living cell-based therapies to repair lost or damaged tissues, including periodontal ligament and to construct biohybrid implant. This work aimed to isolate human periodontal ligament stem cells (hPDLSCs) and implant them on fabricated polycaprolactone (PCL) for the regeneration of natural periodontal ligament (PDL) tissues. Methods hPDLSCs were harvested from extracted human premolars, cultured, and expanded to obtain PDL cells. A PDL-specific marker (periostin) was detected using an immunofluorescent assay. Electrospinning was applied to fabricate PCL at three concentrations (13%, 16%, and 20% weight/volume) in two forms, which were examined through field emission
... Show MoreThe present study aims to get experimentally a deeper understanding of the efficiency of carbon fiber-reinforced polymer (CFRP) sheets applied to improve the torsional behavior of L-shaped reinforced concrete spandrel beams in which their ledges were loaded in two stages under monotonic loading. An experimental program was conducted on spandrel beams considering different key parameters including the cross-sectional aspect ratio (
Abstract:
The great expansion of teaching skills requires finding ways and methods to help teachers acquire experiences of all kinds. The researcher found in the subject of the teaching skills for teachers in public and private schools a fertile field for conducting a study that enables the measurement of these skills. Thus, the study aims to identify the skills of teaching lessons for teachers, the difference in teaching lesson skills for teachers according to the years of service, the differences in teaching lesson skills for teachers according to the specialized teachers and non-specialized teachers, the differences in teaching lesson skills for teachers according to the public and private school. The
... Show MoreLe présent travail aborde la question de l’enseignement de traduction en tant que matière faisant partie du programme élaboré dans des Départements de Français au sein de certaines universités irakiennes, en particulier celle de Bagdad. La méthode d’enseigner suivie constitue une véritable problématique qu’on a bien diagnostiquée à partir de quelques années d’expériences, à la lumière des observations faites dans des cours de traduction professionnelle, et dans la perspective des citations et témoignages établies par des traductologues et pédagogues et principalement par Marianne LEDERER qui a établi la Théorie Interprétative de la traduction. Mais pourquoi l’enseignement lui-même poserait une telle probl
... Show MoreThe present art icle discusses the prob lems of understanding and translating the lingu istic and cult ural aspect of a foreign lite rary text. The article considers the trans lation process through the pr ism of cult ural orientation. In the process of transl ation, the nati onal cultural iden tity should be expressed to the max imum extent, through all me ans of expre ssion that include imagery and inton ation. In addi tion to the author's sty le, special atte ntion should al so be pa id to tro pes, phraseological uni ts, colloquial wo rds and dial&n
... Show MoreWe are, today, facing a torrent of information, ideas, images and videos due to advances of communication technology and electronic publishing. In addition to the proliferation of social networking sites that allow individuals to use them and participate in their channels without any restrictions limiting their freedom in publishing. Due to these sites many terms have emerged like alternative media which use internet and its various techniques to serve its objectives notably the freedom of expression without restrictions. This research studies the phenomenon of interactive media i.e. alternative media through Facebook along with the freedom that makes it spreading in the society and the relation of individual freedom with social diversit
... Show MoreThis study was conducted to examine the discharge capacity of the reach of the Tigris River between Kut and Amarah Barrages of 250km in length. The examination includes simulation the current capacity of the reach by using HEC-RAS model. 247cross sections surveyed in 2012 were used in the simulation. The model was calibrated using observed discharges of 533, 800, 1025 and 3000m3/s discharged at Kut Barrage during 2013, 1995, 1995 and 1988, respectively, and its related water level at three gauge stations located along the reach. The result of calibration process indicated that the lowest Root Mean Square Error of 0.095 can be obtained when using Manning’s n coefficient of 0.026, 0.03 for th
... Show MoreBrainstorming has been a common approach in many industries where the result is not always accurate, especially when procuring automobile spare parts. This approach was replaced with a scientific and optimized method that is highly reliable, hence the decision to optimize the inventory inflation budget based on spare parts and miscellaneous costs of the typical automobile industry. Some factors required to achieve this goal were investigated. Through this investigation, spare parts (consumables and non-consumables) were found to be mostly used in Innoson Vehicle Manufacturing (IVM), Nigeria but incorporated miscellaneous costs to augment the cost of spare parts. The inflation rate was considered first due to the market's
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