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.
Eriobotrya japonica Lindl., named as loquat, is a subtropical fruit tree of the family Rosaceae which is well known medical plant originated in Japan and China. Loquat portions, like leaves, peels and fruits have been shown to possess various health usefulnesses. In Chinese classical medicine, it is vastly utilized in many illnesses, like gastroenteric disorders, diabetes mellitus, pulmonary inflammatory diseases and chronic bronchitis. Loquat plant contain many active constituents, such as flavonoids, carotenoids, vitamins, polyphenolic compounds, other that have many biological effects like anti-tumor, anti-diabetic, anti-inflammatory, anti-mutagenic, antioxidant, antiviral, antitussive, hepatoprotective and hypoli
... Show MoreA simple, accurate, precise, rapid, economical and a high sensitive spectrophotometric method has been developed for the determination of tadalafil in pharmaceutical preparations and industrial wastewater samples, which shows a maximum absorbance at 204 nm in 1:1 ethanol-water. Beer's law was obeyed in the range of 1-7?g/ mL ,with molar absorptivity and Sandell ? s sensitivity of 0.783x105l/mol.cm and 4.97 ng/cm2respectively, relative standard deviation of the method was less than 1.7%, and accuracy (average recovery %) was 100 ± 0. 13. The limits of detection and quantitation are 0.18 and 0.54 µg .ml-1, respectively. The method was successfully applied to the determination of tadalafil in some pharmaceutical formulations
... Show MoreTreatment of a high strength acidic industrial wastewater was attempted by activated carbon
adsorption to evaluate the feasibility of yielding effluents of reusable qualities. The experimental
methods which were employed in this investigation included batch and column studies. The
former was used to evaluate the rate and equilibrium of carbon adsorption, while the latter was
used to determine treatment efficiencies and performance characteristics. Fixed bed and expanded
bed adsorbers were constructed in the column studies. In this study, the adsorption behavior of acetic acid onto activated carbon was examined as a function of the concentration of the adsorbate, contact time and adsorbent dosage. The adsorption data was mo
In this paper, a new analytical method is introduced to find the general solution of linear partial differential equations. In this method, each Laplace transform (LT) and Sumudu transform (ST) is used independently along with canonical coordinates. The strength of this method is that it is easy to implement and does not require initial conditions.
الخلفية: العقدية المقيحة المعروفة أيضًا باسم ""(GAS) هي احدى مسببات الأمراض ذات الأهمية الصحية العامة، حيث تصيب 18.1 مليون شخص في جميع أنحاء العالم وتقتل 500000 شخص كل عام. الهدف: حددت هذه المراجعة المقالات المنشورة حول عوامل الخطر واستراتيجيات الوقاية والسيطرة لأمراض المكورات العقدية. المواد والأساليب: تم إجراء بحث منهجي لتحديد الأوراق المنشورة على قواعد البيانات الإلكترونية Web of Science و PubMed و Scopus و Google Scholar في مح
... Show MoreThe need for quick airborne transportation is critical, especially in emergencies. Drones with suspended payloads might be used to accomplish quick airborne transportation. Due to the environment or the drone's motion, the slung load may oscillate and lead the drone to fall. The altitude and attitude controls are the backbones of the drone's stability, and they must be adequately designed. Because of their symmetrical and simple structure, quadrotor helicopters are one of the most popular drone classes. In this work, a genetic algorithm with two weighted terms fitness function is used to adjust a Proportional-Integral-Derivative (PID) controller to compensate for the altitude and attitude controllers in a quadrotor drone
... Show MoreAs they include both nucleophilic and electrophilic moieties on the same skeleton, enaminones are an important subclass of chemical compounds that contain conjugated NC=CC=O fragments. These active sites aid in the production of organic molecules containing linear or cyclic heteroatoms. Enaminones and the chemica1 compounds produced from them are both biologically active against the most dangerous bacteria. As a result, they have been utilized as starting materials for the synthesis of anti-inf1ammatory, antibacteria1, anticonvulsant, anticancer, anti-urease, anti-malaria1, optically luminescent, corrosion inhibition, and antitumor agents. Their synthesis has usually a terrific deal of interest and a plethora of synthetic paths have been na
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