Gaucher disease (GD), which is due to a deficiency in the lysosomal enzyme β-glucocerebrosidase, is a rare genetic disorder. It is characterized by a wide variety of clinical manifestations and severity of symptoms, making it difficult to manage. A cross-sectional hospital-based genetic study was undertaken with 32 pediatric patients. We recruited 21 males and 11 females diagnosed with GD, with a male-to-female ratio of 1.91:1. The mean age of the study population was 8.79 ± 4.37 years with an age range from 8 months to 17 years. We included patients on clinical evaluation from 2011 to 2019. An enzyme assay test was used to measure β-glucosidase enzyme activity in leukocytes and the GBA gene s
The internet, unlike other traditional means of communication, has a flexibility to stimulate the user and allows him to develop it. Perhaps, the reason for the superiority of the internet over other traditional means of communication is the possibility of change and transmission from one stage to another in a short period. This means that the internet is able to move from the use to the development of the use and then the development of means and innovation as the innovation of the internet is a logical product of the interaction of the user with the network. The internet invests all the proposals and ideas and does not ignore any even if it is simple. This is represented in social networking sites which in fact reflects personal emotio
... Show Morefashion designers who have benefited greatly from the mobilization of ancient aesthetic ideas in the heritage of the people and guaranteed in their productions so that there is no change In the aesthetic value created by the designers of the research in the ancient heritage to find new signs that reflect the connection of man to the present as the aesthetic value of all the man created by the designs of fabrics and fashion through the ages The problem of research was determined in the absence of a precise understanding of the nature of classical thought in fashion and the absence of a clear perception of the sustainability of this thought in contemporary fashion. He
... Show MoreSeveral stress-strain models were used to predict the strengths of steel fiber reinforced concrete, which are distinctive of the material. However, insufficient research has been done on the influence of hybrid fiber combinations (comprising two or more distinct fibers) on the characteristics of concrete. For this reason, the researchers conducted an experimental program to determine the stress-strain relationship of 30 concrete samples reinforced with two distinct fibers (a hybrid of polyvinyl alcohol and steel fibers), with compressive strengths ranging from 40 to 120 MPa. A total of 80% of the experimental results were used to develop a new empirical stress-strain model, which was accomplished through the application of the parti
... Show MoreThe partial level density PLD of pre-equilibrium reactions that are described by Ericson’s formula has been studied using different formulae of single particle level density . The parameter was used from the equidistant spacing model (ESM) model and the non- equidistant spacing model (non-ESM) and another formula of are derived from the relation between and level density parameter . The formulae used to derive are the Roher formula, Egidy formula, Yukawa formula, and Thomas –Fermi formula. The partial level density results that depend on from the Thomas-Fermi formula show a good agreement with the experimental data.
The downhole flow profiles of the wells with single production tubes and mixed flow from more than one layer can be complicated, making it challenging to obtain the average pressure of each layer independently. Production log data can be used to monitor the impacts of pressure depletion over time and to determine average pressure with the use of Selective Inflow Performance (SIP). The SIP technique provides a method of determining the steady state of inflow relationship for each individual layer. The well flows at different stabilized surface rates, and for each rate, a production log is run throughout the producing interval to record both downhole flow rates and flowing pressure. PVT data can be used to convert measured in-situ r
... Show MoreIn this study, cloud point extraction combined with molecular spectrometry as an eco-friendly method is used for extraction, enrichment and determination of bendiocarb (BC) insecticide in different complex matrices. The method involved an alkaline hydrolysis of BC followed Emerson reaction in which the resultant phenol is reacted with 4-aminoantipyrene(4-AAP) in the presence of an alkaline oxidant of potassium ferric cyanide to form red colored product which then extracted into micelles of Triton X-114 as a mediated extractant at room temperature. The extracted product in cloud point layer is separated from the aqueous layer by centrifugation for 20 min and dissolved in a minimum amount of a mixture ethanol: water (1:1) followed
... Show MoreThis paper analyzes a piled-raft foundation on non-homogeneous soils with variable layer depth percentages. The present work aims to perform a three-dimensional finite element analysis of a piled-raft foundation subjected to vertical load using the PLAXIS 3D software. Parametric analysis was carried out to determine the effect of soil type and initial layer thickness. The parametric study showed that increasing the relative density from 30 % to 80 % of the upper sand layer and the thickness of the first layer has led to an increase in the ultimate load and a decrease in the settlement of piled raft foundations for the cases of sand over weak soil. In clay over weak soil, the ultimate load of the piled raft foundation w
... Show MoreIn this work, we construct projectively distinct (k,3)-arcs in the projective plane PG(2,9) by applying a geometrical method. The cubic curves have been been constructed by using the general equation of the cubic. We found that there are complete (13,3)-arcs, complete (15,3)-arcs and we found that the only (16,3)-arcs lead to maximum completeness