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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

النظام السياسي في كوريا الشمالة

The study aimed at identifying the strategic gaps in the actual reality of the management of public organizations investigated to determine the strategy used based on the study model. The study relied on the variable of the general organization strategy in its dimensions (the general organization strategy, the organization's political strategy and the defense strategy of the organization) The sample of the study was (General Directorate of Traffic, Civil Status Directorate and Civil Defense Directorate), formations affiliated to the Ministry of the Interior, for the importance of the activity carried out by these public organizations by providing them In order to translate the answers into a quantitative expression in the analysi

Diversity the terms and practice the organizational filed with different concepts and environment, which Iraqi environment part from them. Some organizational in Iraqi environment leave its basic oriented to agreement with the leader desire, their fore this research focus tow basic variable (organizational citizenship behavior & transparence), we supposition which is dependent to explanation the response variable (strategic leadership). The results justification in part and not justification in another part. For example the organizational citizenship behavior effect on some parte of the strategic leadership. The transparence have faraway to fly from the relation with organizational citizenship behavior and strategic leadershi

The purpose of this research is to identify the youth issues in Talk Shows in the Iraqi satellite channels via monitoring a sample of episodes of the Talk Shows  episodes which are concerned and analyzed the youth issues in the Iraqi satellite channels, namely, «Hala Shabab Program» at Al-Iraqia satellite Channel and «Shabab wa Banat Program» at Al-Sumaria satellite Channel by recording and re-watching them again. This research is classified as one of descriptive researches. The survey method was adopted in this study.

For this purpose, the researcher prepared an analysis form. The researcher de

This note reported the first record of Spotted Flycatcher Muscicapa striata (Pallas, 1764) (Passeriformes, Muscicapidae) from the state of Odisha, India. This species was recorded from the north and western part of the country as well as from the Western Ghats, but this note reports the first record from the Eastern Ghats of India.