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 inflammator

The study aimed to know the degree to which Islamic education teachers employ the strategy of directed discovery in teaching to develop higher thinking skills (analysis, synthesis, and evaluation) among sixth-grade students at the basic stage in East Nile Province, Khartoum State - Sudan. The researcher used the descriptive-analytical method, and adopted a questionnaire that consisted of (15) indicators representing the skills of analysis, synthesis, and evaluation as a tool of the study. A random sample of (175) Islamic education teachers was selected, from the Department of Basic Education. The data were analyzed according to the Statistical Packages for Social Sciences (SPSS) program, and the results of the study have shown that the u

Environment suffered in recent years a large corrupting by human; and because of his ignorance of the dimensions of Caliphate in the ground and ignore what it means gearing. The gearing is that the son of Adam, which will benefit the board of Allah Almighty to him in the land of the causes of life, without exaggeration or negligence and without prejudice to the cosmic Balnoames enacted by the Almighty Creator, has urged verses of the Quran Muslim to preserve and protect the environment which is a religious duty, as it showed a great verses he is the author and the splendor and beauty of workmanship and manufacturer greatness of the Almighty, who created all things beautiful.
Koran played a major role in the consolidation of environmen

Care and attention to the structure in the sixties of the last century replaced the mark, and if the structure of Ms. pampered in research and studies, it has become the mark is also a spoiled lady .. But the relationship between the structure and the mark was not a break and break, but the relationship of integration, His themes are structural analysis, and these are intellectual themes that can not be surpassed in contemporary research, especially since semiotics have emerged from the linguistic inflection.

We have tried to distinguish between text and speech, which is a daunting task, as it seems that whenever the difference between them is clear and clear, we come back to wonder whether the text is the same discourse, and is

The aim of the research is to assess the practice of tax administration to diminish the strategic direction of (proactive, analytical, defensive, response) and the nature of its relationship to organizational excellence represented by (leadership, strategic planning, focus on taxpayers, information and analysis and knowledge management, focus on operations, focus on forces. The questionnaire was adopted as a main tool in collecting data and information from the sample members of the General Authority for Taxes (110) who are in the site (m.  Second Division Manager, Ms The statistical program (SPSS) was used to calculate (mean, standard deviation, difference coefficient, correlation coefficient, t-test). The research reached a number

research objectives to:
1. identify the social, economic and cultural factors affecting consumption.
2. detect the consumption culture among the population in the city of Erbil.
3. Identify the GATT consumer protection and rights.
The most important results:
1. that there is variation in the answers of respondents about keep up with modernity in the basic consumption (necessary), it swallowed the proportion of yes answers about keep up with modernity in food consumption (72%), and is an indication of growing consumer awareness of the individual in the side of nutrition. The clothing on the side of the proportion of yes answers amounted to (85%), in the health field note that the percentage of yes answers (83%), who are abr

It is often needed in demographic research to modern statistical tools are flexible and convenient to keep up with the type of data available in Iraq in terms of the passage of the country far from periods of war and economic sanctions and instability of the security for a period of time . So, This research aims to propose the use of style nonparametric splines as a substitute for some of the compounds of analysis within the model Lee-Carter your appreciation rate for fertility detailed variable response in Iraq than the period (1977 - 2011) , and then predict for the period (2012-2031). This goal was achieved using a style nonparametric decomposition of singular value vehicles using the main deltoid , and then estimate the effect of time-s

Reduction of noise and vibration in spur gear experimentally by using asymmetric teeth profiles with tip relief was presented. Both of classical (symmetric) and asymmetric (with and without tip relief) spur gears are used in this work. Gear test rig was constructed to achieve torsional vibration measuring, and two modified cutters are designed and manufactured to achieve tooth profile modifications. First to cut asymmetric gear tooth with pressure angles (14.5^o/25 ^o) without tip relief for loaded and unloaded tooth sides respectively, and second to cut asymmetric gear tooth with pressure angles (14.5^o/25 ^o) for loaded and unloaded tooth sides respectively with tip relief to ach