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

A fixed firefighting system is a key component of fire safeguarding and reducing fire danger. It is installed as a permanent component in a structure to protect the entire or a portion of the building and its contents. The study aims to review the previous studies that deal with the evaluation of fire safety measures and their use in resolving problems associated with fire threats in buildings. For this reason, a number of previous studies in this field were reviewed compared with the NFPA code. The findings revealed that regulatory developments over the last several decades had created an atmosphere conducive to innovation. This has resulted in a growth in the number of fixed firefighting system types now obtainable. Th

The investigation of machine learning techniques for addressing missing well-log data has garnered considerable interest recently, especially as the oil and gas sector pursues novel approaches to improve data interpretation and reservoir characterization. Conversely, for wells that have been in operation for several years, conventional measurement techniques frequently encounter challenges related to availability, including the lack of well-log data, cost considerations, and precision issues. This study's objective is to enhance reservoir characterization by automating well-log creation using machine-learning techniques. Among the methods are multi-resolution graph-based clustering and the similarity threshold method. By using cutti

This research depends on the relationship between the reflected spectrum, the nature of each target, area and the percentage of its presence with other targets in the unity of the target area. The changes occur in Land cover have been detected for different years using satellite images based on the Modified Spectral Angle Mapper (MSAM) processing, where Landsat satellite images are utilized using two software programming (MATLAB 7.11 and ERDAS imagine 2014). The proposed supervised classification method (MSAM) using a MATLAB program with supervised classification method (Maximum likelihood Classifier) by ERDAS imagine have been used to get farthest precise results and detect environmental changes for periods. Despite using two classificatio

In order to scrutinize the impact of the decoration of Sc upon the sensing performance of an XN nanotube (X = Al or Ga, and XNNT) in detecting sarin (SN), the density functionals M06-2X, τ-HCTHhyb, and B3LYP were utilized. The interaction of the pristine XNNT with SN was a physical adsorption with the sensing response (SR) of approximately 5.4. Decoration of the Sc metal into the surface of the AlN and GaN led to an increase in the adsorption energy of SN from −3.4 to −18.9, and −3.8 to −20.1 kcal/mol, respectively. Also, there was a significant increase in the corresponding SR to 38.0 and 100.5, the sensitivity of metal decorated XNNT (metal@XNNT) is increased. So, we found that Sc-decorating more increases the sensitivity of GaNN

Introduction/Aim. Seminal fluid analysis (SFA) plays a crucial role in helping infertility clinics diagnose the underlying reason of male infertility. The aim of the study was to investigate seminal fluid patterns of male partners of an infertile couple with apparently fertile female partners. Materials and methods. A cross-sectional study was conducted between January 2019 and December 2022. Patients were attending consultations for delayed conception for more than 12 months with apparently fertile female partner. Results. Four hundred fifty-three patients were included in the study. The distribution of patients according to age groups showed that 277 patients were young, aged 21 - 30 years (61%). Two hundred sixty-two (58%) patien

This research tries to reveal how to manage and control the competitive edge for business by building managerial skills in various organizational levels. Our research aims at finding out the nature of various technical, human and in tellectual skills of a new president whose superiority is his competitive ness in the application field at general company for construe tioual  industriesand testing the surveyed minor and major changes through a questionnaire to collect information from officials. The sample was composed of (45) director. The data was analyzed  using some methods and statistical programs. The most prominent of these is (SPSS) that was used to extract the arithmetic mean, standard deviation, correlation coefficient

Public budget is the government's tool in achieving the objectives of economic and social development is the accounting curriculum to estimate state revenues and expenditures for years to come, as well as to have legal status as it is after the adoption of an official statement of government units to spend funds on items planned at the same time is a statement collect resources to finance these appropriations, if the primary objective of the budget initially limited to the achievement of financial and legislative control have evolved this function in the area of public administration turned attention from mere imposition of control over the money, which provides information to assist the Department to utilize available resources and prog