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.
Study Purpose. highlighting the origins of the sport's philosophy. Studying the development of the sport of kendo across different historical periods and its adaptation to societal and cultural changes. Identifying the establishment of the most prominent organizations and federations that contributed to its development, and exploring the sport's basic tools and rules and its most prominent championships. Materials and Methods. The historical approach was adopted in studying the historical process of the development of kendo, as it is appropriate to the nature of the problem. The information has been simplified and presented in a sequential manner that presents the development of the sport of kendo, starting from the origins of its p
... Show MoreThis research studied the effect of magnetized water in concrete preparation and its effect on the presenting of cement in concrete mixtures also to find the ability of reducing the amount of cement in preparing one cubic meter, this is not exceed than 10% in one mixture , The experiments showed the preparation of standard cubes from the concrete which was used two kind of water magnetized water which was prepared by passing the tap water through the systems of different magnetic strength in terms of (6000,9000) Gauss and the ordinary water . The velocity of water through the magnetic field, which gives us the highest value for the compressive strength, was up to 1m/sec. to determine the best magnetic intensity, we examined The comp
... Show MoreThe simulation have been made for 3D flow structure and heat transfer with and without
longitudinal riblet upstream of leading edge vane endwall junction of first stage nozzle guide vane .The research explores concept of weakening the secondary flows and reducing their harmful effects.Numerical investigation involved examination of the secondary flows ,velocity and heat transfer rates by solving the governing equations (continuity, Navier -stokes and energy equations ) using the known package FLUENT version (12.1).The governing equations were solved for three dimentional, turbulent flowe, incompressible with an appropriate turbulent model (k-ω,SST) .The numerical solution was carried out for 25 mode
... Show MoreIn this work , an effective procedure of Box-Behnken based-ANN (Artificial Neural Network) and GA (Genetic Algorithm) has been utilized for finding the optimum conditions of wt.% of doping elements (Ce,Y, and Ge) doped-aluminizing-chromizing of Incoloy 800H . ANN and Box-Behnken design method have been implanted for minimizing hot corrosion rate kp (10-12g2.cm-4.s-1) in Incoloy 800H at 900oC . ANN was used for estimating the predicted values of hot corrosion rate kp (10-12g2.cm-4.s-1) . The optimal wt.% of doping elements combination to obtain minimum hot corrosion rate was calculated using genetic alg
... Show Moreالحمدُ للهِ رب العالمين ، والصلاة والسلام على نبيه الأمين محمد r وعلى آله الطيبين الطاهرين ، وأصحابه الغر الميامين:
تعد الصورة السمعية مفهوما بيانيا نجده في البلاغة العربية واضحاً مؤثرا، مؤديا دورا جوهريا في إيصال الفكرة التي يروم الأديب إيصالها إلى المتلقي ولا تبدو السمعية واضحة إلاّ إذا نظر إليها في حالة أدبيه تهز كيان الشاعر  
... Show MoreThis study aims at recognizing Pesticides and how the process of pesticides biodegradation by microbiology took place, and the effect of environmental condition on this process. And how the research uncovered the efficiency of microbiology in the biodegradation process of pesticides, as the perfect temperature for the biodegradation process is 40 °C and humidity effect on pesticides efficiency, when high humidity reduces pesticide efficiency and the perfect acidity to increase bacteria efficiency is 7, for the incubation period, it was found during the previous studies that the best incubation period is 5-7 days, in this period the bacteria imprint on pesticides and increase biodegradation of it.
The rabid development in life and technology arouse the anxiety, stress, and fear of people. Accordingly the maladjustment and deceived conceptions appear in their behaviors which influence their selves and others.
These changes and development in society influence the university students' life, personality, thoughts and behavior. An exceeding in anxiety and fear has negative effect on everyday life activities and. negative effect on both mind and body.
The problem of the study is centered around cognitive conception anxiety of the university students.
The university students have their special importance in society and nation development, therefore many studies search in their personality and the effect of anxiety on them to fi
Abstract
The study aims to recognize the degree of consumptions awareness of the
Iraqi family; the conscious and studied planning of the family; budget and the
object study of the rationalization s consumption.
The research study 100 specimens of both students and employees who
are housewives at the department of home economics of the college of
Education for women.
Questioner the items are 22.
One of the most important results that the study finds is the availability of
consumption's awareness's level particularly of low income families and
college degrees hdders who are over 35.
The study recommends the necessity of a conscious and studied
planning of the family's budget; its purchase and consumption