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 early childhood stage, which is usually described as establishing the development and growth of most of the child’s mental, psychological, moral, and physical abilities, so scientists and researchers have been interested in the growth and upbringing of the child, especially for the specified period between (6 until the age of 11) due to the importance of education in this stage. The age stage in terms of developing the child’s senses and experiences, because he is by nature inclined to play and draw in his instinctive activity, according to which his natural responses to stimuli and his contemplation of what surrounds him change. Since drawing plays an important role in raising a child from an emotional and intellectual point of vie
... Show Moreproblems with its unobvious effect on scientific creativity and information. Problem solving is one of main goals of researchers because it develops their right logical thinking methods. The present study aims at measuring logical thinking among female it structures in the university mea swing problem solving among them ,identifying statically differences significance in logical thinking among female instructors in the university according to (Specialization Variable), identifying differences significance in problem Solving among female instructions in the university according to ( Specialization Variable) and identifying the Correlation between logical thinking and problem solving among female instructors in the university. The sample c
... Show MoreThis research investigates the subject of the impact of wars (as a manifestation of crisis) on architecture, and the extent of continuing wars physical and moral results of wars, even after the end of the cause of the crisis. The impact of different rebuilding which exposed to the effects of the war seems different in crisis regions.
The problem of research is about the uncertainty of the impact of the way chooses for reconstructing the buildings after wars in the continuity of the crisis of war. The goals of this research are to clarify the influence of methods of reconstruction of buildings in a city chosen which is Beirut, on the continuation of the war crisis with the argument of demolishing and rebuilding newly or keeping tr
... Show MoreThe research aims to know The Effect Of Flexible Grouping Strategy and Three Step Interview strategy on achievement of the history material among student of the first literary class, The Researcher used the experimental design of the two experimental groups and the control group and with post test, researcher group (a) represent the experimental group taught according the Flexible Grouping Strategy , and Division (c) to represent the second experimental group which studied according Three Step Interview strategy and Division (b ) to represent the control group taught in the traditional method, the number of students (99) students of (33) female students in each division. T
... Show MoreBackground: Reduction in bond strength when bonding was done immediately after intracoronal bleaching procedure has been recognized. The purpose of this study is to assess the effect of antioxidants (10% sodium ascorbate (SA), 0.1M thiourea and7% sodium bicarbonate (SB)) on reversing bonding strength of composite resin to bleached dentin. Materials and method: Sixty upper 1st premolar teeth, were selected, the crowns of the teeth were embedded in acrylic resin blocks exposing a flat dentin from the buccal surface, then divided into 6 groups (10 samples each). Bleaching for the experimental groups was performed using 35% hydrogen peroxide bleaching gel (pola–office).Group A (Negative
... Show MoreThe two researchers selected the problem of research which represented with the following asking: Does the use of the shape of Round house strategy have effectiveness in the collection of students of the Department of Art Education of the subjectof teaching methods?
The research aims to "measure the effectiveness of Strategy shape of Round house in the collection of students of the Department of Art Education for the material teaching methods" and to verify the aim of the research two zeroassumptions was identified to measure the level of achievement in the subject of teaching methods of third stage students in the Department of Art Education –College of Fine Arts.
The research community included the students of Art Education Dep
Seeds, beans, leaves, fruit peel and seeds of five plants (Ferula assa-foetida, Coffea robusta, Olea europaea, Punica granatum and Vitis vinifera, respectively) were extracted with four solvents (distilled water, 80% methanol, 80% acetone and a mixed solvent that included methanol, ethanol, acetone and n-butanol at proportions 7:1:1:1). Such manipulation yielded 20 extracts, which were phytochemically analyzed for total polyphenols (TP) and flavonoids (TF). The DPPH (2,2-diphenyl-1-picrylhydrazyl) radical scavenging activity (RSA) and DPP-4 (dipeptidyl peptidase-4) relative inhibition activity (RIA) were also assessed for each extract. The results revealed that mixed solvent extract of V.
... Show MoreThis study illustrates in vitro effect of cold atmospheric plasma (CAP) on the treatment of Leishmania. In addition, the study evaluated the effect of drug treatment (pentostam) and the combination treatment of pentostan and CAP at different doses and incubation time 24 h in order to assess the most effective treatment. The duration of the cold plasma doses was 1 min, 2 min and 3 min, while the pentostam doses were 2.5 µg/ml and 5 µg/ml. The combinations therapies included combining the three cold plasma doses with the pentostam doses to test 6 combinations of treated in vitro. The maximum growth inhibition was given by combination treated 46% and 44% for donovani and tropica leishamnia respectively; these results give an indi
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