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.
The research aims to formulate a model for research on the dimensions of the Leader-member exchange theory according to the ideas and thoughts of Liden /Maslyn, Hammer and impact on job satisfaction and measure these dimensions at asiacell communications to identify the extent of convergence between theoretical dimensions share leader – member Exchange (LMX) with leadership style used in this company, we have been building scale contains two variables, independent variable Leader-member exchange theory five dimensions (effect , Loyalty, contribution, respect professional, and support) the certified job satisfaction variable dimensions (job security, The Style of management, organizational climate, appreciation, and work itself)
... Show MoreThe attention of civil society organizations, governments and international non-governmental organizations in recent years has focused on the acute practices adopted by companies with the aim of reducing the tax burden, and the results of these practices have a negative impact on the tax revenues that the state uses to finance public needsThe aim of the research is to measure the impact of the voluntary disclosure of companies on tax planning for the period from 2011 to 2015 and the research community is in the General Tax Authority. The research sample is 6 companies within the private sector in the financial, banking, insurance and industrial fields. The research was based on a special model to measure the optional disclosure,
... Show MoreObjective. This study aimed to evaluate and compare the force degradation of two types of elastomeric chains following different periods of immersion in zinc-containing mouthwashes. Materials and Methods. Four hundred and forty pieces of Elasto-Force and Super Elasto-Force elastomeric chains were divided into two control and eight experimental groups. The pieces were stretched to 25 mm on pins mounted on an acrylic block and stored in distilled water at 37°C. The experimental groups were immersed in four different types of mouthwash for one minute twice a day throughout the test period. Ten continuous thermocycles per day between cold and hot water baths (5–55°C) were carried out. Forces were measured at six-time intervals (in
... Show MoreThin films of ZnSe arc deposited on glass substrates by thermal evaporation in vacuum with different thickness (1000, 2700, 4000) A° temperature (293-373) °K are studies the electrical properties before and after annealing. The result show decrease D.0 conductivity and increasing the activation energy Eat.
In this investigation a high density polyethylene (HDPE) was used as a substitute to polyvinylchloride in the production of lead acid battery separators. This has been achieved by preparing mixtures of different percentages of the feed materials which include a high density polyethylene (HDPE) locally produced, filler materials such as silica and oils such as dioctylphthalate (DOP) or paraffin which were added to the mixture to improve the final properties of the separator. The materials were compounded by two roll-mills under the same conditions. The following parameters are involved: &nb
... Show MoreIn this research entitled "Internal music in the poetry of guidance in the era of Islam and its artistic and semantic implications" dealt with the following:
Preface, which addressed the importance of internal music in poetry, and its close association with poetry, and the poet himself, to reveal what is touring himself and revolves in his imagination.
Repetition of all kinds: repetition of letters, repetition of words, and repetition of phrases: It showed what this repetition of strengthening the melody, and linking parts of words within the same house, or the poem, and create a special musical atmosphere to spread a certain significance and to confirm the meanings and accompanied by musical accommodation.
Anaphysic
The usual methods of distance determination in Astronomy parallax and Spectroscopic with Expansion Methods are seldom applicable to Nebulae. In this work determination of the distances to individual Nebulae are calculated and discussed. The distances of Nebulae to the Earth are calculated. The accuracy of the distance is tested by using Aladin sky Atlas, and comparing Nebulae properties were derived from these distance made with statistical distance determination. The results showed that angular Expansions may occur in a part of the nebulae that is moving at a velocity different than the observed velocity. Also the results of the comparison of our spectroscopic distances with the trig
The nonlinear optical properties response of nematic liquid crystal (6CHBT) and the impact of doping with two kinds of nanoparticles; Fe3O4 magnetic nanoparticles and SbSI ferroelectric nanoparticles have been studied using the non-linear dynamic method through z-scan measurement technique. This was achieved utilizing CW He-Ne laser. The pure LC and magnetic LC nanoparticle composite samples had a maximum absorption while the ferroelectric LC nanoparticle composite had a minimum absorption of the incident light. The nonlinear refractive index was positive for the pure LC and the rod-like ferronematic LC composite samples, while it was negative for the ferroelectric LC composite. The studying of the nonlinear optical
... Show More