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.
هدف البحث إلى بناء مقياسين تخصصين للنزاعات اللاشعورية وحرية الإرادة لمدربي بعض ألعاب القوى والتعرف على مستوى كل منهما لديهم، وإعداد برنامج إرشادي نفسي مستند لرفع مستوى الحالات اللاشعورية وحرية الإرادة للمدربين الذين يعانون من انخفاض مستوى النزاعات اللاشعورية وحرية الإرادة، والتعرف على تأثير البرنامج الإرشادي نفسي في النزاعات اللاشعورية وحرية الإرادة لدى مدربي بعض ألعاب القوى، لتفترض بذلك الباحثة بإ
... Show MoreBiodiesel production from microalgae depends on the biomass and lipid production. Both biomass and lipid accumulation is controlled by several factors. The effect of various culture media (BG11, BBM, and Urea), nutrients stress [nitrogen (N), phosphorous (P), magnesium (Mg) and carbonate (CO3)] and gamma (γ) radiation on the growth and lipid accumulation of Dictyochloropsis splendida were investigated. The highest biomass and lipid yield of D. splendida were achieved on BG11 medium. Cultivation of D. splendida in a medium containing 3000 mg L−1 N, or 160 mg L−1 P, or 113 mg L−1 Mg, or 20 mg L-1 CO3, led to enhanced growth rate. While u
... Show MoreThe objective of an Optimal Power Flow (OPF) algorithm is to find steady state operation point which minimizes generation cost, loss etc. while maintaining an acceptable system performance in terms of limits on generators real and reactive powers, line flow limits etc. The OPF solution includes an objective function. A common objective function concerns the active power generation cost. A Linear programming method is proposed to solve the OPF problem. The Linear Programming (LP) approach transforms the nonlinear optimization problem into an iterative algorithm that in each iteration solves a linear optimization problem resulting from linearization both the objective function and constrains. A computer program, written in MATLAB environme
... Show Moreforty-six patients with asthma were tested for the scrum levels of total sialic and diffrental the results reveled a significant increased in the scra of asthmatic patients
The tagged research (realism in the Paintings of Iraqi Kurdistan artists, “a study of expression methods”) dealt with realism in an objective way, as well as the complexity of its concepts through its formations and formations. On realism and its historical dimension in concept and meaning, as for the second chapter, the research was focused on the methods of expression in painting, while the third chapter was concerned with the procedural applications of realistic methods of expression in the drawings of Iraqi Kurdistan, and according to these axes and to achieve the goal of the research, a number of Among the results are:
1- Realism documented the life of the Kurdish society in line with the developments of the era, as the sty
Asthma is a chronic inflammatory disease of respiratory airways characterized by distinctive history of respiratory symptoms due to variable airflow obstruction which reverses either spontaneously or in response to certain medications. Acetylcholine is a parasympathetic neurotransmitter which plays fundamental roles in the development of persistent asthma. Treatment guidelines recommend using medium doses of inhaled corticosteroids in addition to another controller bronchodilator instead of using high doses inhaled steroid alone for treatment of moderate to severe persistent asthma. The inhaled long acting muscarinic antagonist, tiotropium, was approved recently to control unresponsive asthma to inhaled corticosteroid with or without a long
... Show MoreThis research deals with the use of a number of statistical methods, such as the kernel method, watershed, histogram and cubic spline, to improve the contrast of digital images. The results obtained according to the RSME and NCC standards have proven that the spline method is the most accurate in the results compared to other statistical methods
This research deals with the use of a number of statistical methods, such as the kernel method, watershed, histogram, and cubic spline, to improve the contrast of digital images. The results obtained according to the RSME and NCC standards have proven that the spline method is the most accurate in the results compared to other statistical methods.
This article suggests and explores a three-species food chain model that includes fear effects, refuges depending on predators, and cannibalism at the second level. The Holling type II functional response determines food consumption between stages of the food chain. This study examined the long-term behavior and impacts of the suggested model's essential elements. The model's solution properties were studied. The existence and stability of every probable equilibrium point were examined. The persistence needs of the system have been determined. It was discovered what conditions could lead to local bifurcation at equilibrium points. Appropriate Lyapunov functions are utilized to investigate the overall dynamics of the system. To support the a
... Show More