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

Iraqi industrial units face strong competition due to many problems including1- high production costs2- weak interest in studying the market3- lack of government support for their products4- dumping the market with imported products with specifications and a competitive price as well as adopting the traditional cost system in calculating costs that do not provide appropriate information for pricing decisions Which requires studying and analyzing these problems and dealing with them by adopting modern technologies so that they can compete, so the research aims to show the knowledge bases of technology Activity- Based Costing, with an indication of the role of technology Activity- Based Costing in rationalizing the tax In, and the

 The financial statements   must accurately, clearly and fairly present the financial situation and financial statements should be reliable as well. Pursuant to Board Decision No. (74)  2020, CBI obliges banks, according to the Banking Law No. (94) for the year 2004, Article (33) paragraph 2  concerning real estate owned from previous years and expropriated as a result debt settlement , to apply a real estate liquidation provision  at  a  rate of 20% for each property expropriated and at a rate of 100% for each year of delay up to (5) years  .also from  the significance of the issue of  real estates that become a property of   banks as a result of debt settlement and the pr

Salicylaldehyde was react with 4-amino-2,3-dimethyl-1-phenyl-3-Pyrazoline-5-on to produce the novel Schiff base ligand 2,3-dimethyl-1-phenyl-4-salicylidene-3-pyrazoline-5-on (HL). A new complexes of VO(II), Cr(Ш), Zn(II), Cd(II), Hg(II) and UO2(II) with mixed ligands of bipyridyl and new shiff base ( 2,3-dimethyl-1-phenyl-4-salicylidene-3-pyrazoline-5-on) (HL) were prepared . All prepared compounds were identified by atomic absorption, FT.IR , UV-Visable spectra and molar conductivity. From the above data, the proposed molecular structure for VO(II) complex is squre pyramidal while (Zn(II), Cd(II), Hg(II)) and ( UO2(II),Cr(III)) complexes are forming tetrahedral and octahedral geometry respectively.

The complexes of Schiff base of 4-aminoantipyrine and 1,10-phenanthroline with metal ions Mn (II), Cu (II), Ni (II) and Cd (II) were prepared in ethanolic solution, these complexes were characterized by Infrared , electronic spectra, molar conductance, Atomic Absorption ,microanalysis elemental and magnetic moment measurements. From these studies the tetrahedral geometry structure for the prepared complexes were suggested.The prepared ligand of 4-aminoantipyrine was characterized by using Gc-mass spectrometer .

This paper focuses on Load distribution factors for horizontally curved composite concrete-steel girder bridges. The finite-element analysis software“SAP2000” is used to examine the key parameters that can influence the distribution factors for horizontally curved composite steel
girders. A parametric study is conducted to study the load distribution characteristics of such bridge system due to dead loading and AASHTO truck loading using finite elements method. The key parameters considered in this study are: span-to-radius of curvature ratio, span length, number of girders, girders spacing, number of lanes, and truck loading conditions. The results have shown that the curvature is the most critical factor which plays an important

Self-repairing technology based on micro-capsules is an efficient solution for repairing cracked cementitious composites. Self-repairing based on microcapsules begins with the occurrence of cracks and develops by releasing self-repairing factors in the cracks located in concrete. Based on previous comprehensive studies, this paper provides an overview of various repairing factors and investigative methodologies. There has recently been a lack of consensus on the most efficient criteria for assessing self-repairing based on microcapsules and the smart solutions for improving capsule survival ratios during mixing. The most commonly utilized self-repairing efficiency assessment indicators are mechanical resistance and durab