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
... Show MoreZ-scan has been utilized for studying the non-linear properties and optical limiting behaviors of the dye Copper Phthalocyanine thin films. The refractive index is negative, which indicates a self-defocusing behavior and non-linear absorption coefficient (
KE Sharquie, AA Al-Nuaimy, WJ Kadhum, Saudi medical journal, 2006 - Cited by 3
Globally, Sustainability is very quickly becoming a fundamental requirement of the construction industry as it delivers its projects; whether buildings or infrastructures. Throughout more than two decades, many modeling schemes, evaluation tools, and rating systems have been introduced en route to realizing sustainable construction. Many of these, however, lack consensus on evaluation criteria, a robust scientific model that captures the logic behind their sustainability performance evaluation, and therefore experience discrepancies between rated results and actual performance. Moreover, very few of the evaluation tools available satisfactorily address infrastructure projects. The res