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

Circular thin walled structures have wide range of applications. This type of structure is generally exposed to different types of loads, but one of the most important types is a buckling. In this work, the phenomena of buckling was studied by using finite element analysis. The circular thin walled structure in this study is constructed from; cylindrical thin shell strengthen by longitudinal stringers, subjected to pure bending in one plane. In addition, Taguchi method was used to identify the optimum combination set of parameters for enhancement of the critical buckling load value, as well as to investigate the most effective parameter. The parameters that have been analyzed were; cylinder shell thickness, shape of stiffeners section an

A Geographic Information System (GIS) is a computerized database management system for accumulating, storage, retrieval, analysis, and display spatial data. In general, GIS contains two broad categories of information, geo-referenced spatial data and attribute data. Geo-referenced spatial data define objects that have an orientation and relationship in two or three-dimensional space, while attribute data is qualitative data that can be counted for recording and analysis. The main aim of this research is to reveal the role of GIS technology in the enhancement of bridge maintenance management system components such as the output results, and make it more interpretable through dynamic colour coding and more sophisticated visualization

Abstract: The researcher aims to highlight the historical frames of political development in royal regime era (1921-1949) and study its transitions on social-political aspect for the various periods during this consistent era of Iraq history. As some elements played an important role in shaping this era’s features ,as well as 2hat succeed it, which mainly affected the political progression’s configuration, such as : political culture role, social and cultural foundation, state policy essence and the unofficial institutions remarkable role in influencing public awareness and concerning it's relation to the state ,the clan and religious institutions. The researcher employed Alexis de Tocqueville’s evaluation criteria and indi

Massive multiple-input multiple-output (massive-MIMO) is considered as the key technology to meet the huge demands of data rates in the future wireless communications networks. However, for massive-MIMO systems to realize their maximum potential gain, sufficiently accurate downlink (DL) channel state information (CSI) with low overhead to meet the short coherence time (CT) is required. Therefore, this article aims to overcome the technical challenge of DL CSI estimation in a frequency-division-duplex (FDD) massive-MIMO with short CT considering five different physical correlation models. To this end, the statistical structure of the massive-MIMO channel, which is captured by the physical correlation is exploited to find sufficiently

In the present work, the efficiency of Tri-octyl Methyl Ammonium Chloride (TOMAC) ionic liquid was investigated as new and green demulsifier for three types of Iraqi crude oil emulsions (Nafut Khana (NK), Kirkuk and Basrah). The separation efficiency was studied at room temperature and by using microwave heating technique. Several batch experiments were done to specify the suitable conditions for the emulsification and demulsification which were specified as 45 minutes and 3000 rpm for crude oil emulsification while the ionic liquid doses were (500,300,150,50) ppm and the conditions of microwave heating were 1000 watt and 50 second as irradiation time. The results were very encouraging especially for NK and Kirkuk crude oil emulsions whe

This study describe the effect of temperature on the optical
properties of nickel(ii) phthalocyanine tetrasulfonic acid tetrasodium
salt (NiPcTs) organic thin films which are prepared by spin coating
on indium tin oxide (ITO-glass). The optical absorption spectra of
these thin films are measured. Present studies reveal that the optical
band gap energies of NiPcTs thin films are dependent on the
annealing temperatures. The optical band gap decreases with increase
in annealing temperature, then increased when the temperature rising
to 473K. To enhance the results of Uv-Vis measurements and get
more accurate values of optical energy gaps; the Photoluminescence
spectra of as-deposited and annealed NiPcTs thin fi