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 inflammatory conditions which suggest that cystinosis should be included within the differential diagnosis of disorders associated with increased plasma chitotriosidase activity. This study is aimed to estimate serum chitotriosidase level, as a screening marker and therapeutic monitor for cystinosis disease in Iraqi children with cystinosis.
Subjects and Methods: The present study is a case-control study that included samples of 30 children with nephropathic cystinosis, compared to 25 healthy control children from those attending at The Genetic Rare Diseases Center / AL-Emamain AL-Kadhimain Teaching Hospital, Baghdad-Iraq.
Results: Our results reported that cystinotic children had a marked elevation of serum chitotriosidase activity, compared to age-matched healthy children, besides a significant associated with leukocyte-cystine content for cystinotic patients.
CHT1 as a Novel Biomarker
Conclusion: Estimation of serum chitotriosidase activity might aid in monitoring the therapeutic benefits of cysteamine therapy, as well as the prognosis of the disease when WBC cystine assessment is not available.
Key Words: Cystinosis, Cysteamine, Chitotriosidase.
Fluidization process is widely used by a great assortment of industries worldwide and represents a trillion dollar industry [6]. They are currently used in separation, classification, drying and mixing of particles, chemical reactions and regeneration processes; one of these processes is the mass transfer from an immersed surface to a gas fluidized bed
Communication has seen a big advancement through ages; concepts, procedures and technologies, it has also seen a similar advancement of language. What unites language and media is the fact that each one of them guides and contributes to the other; media exists and results from language and from the other sign systems, and what strengthens this connection is the symbolic language system, as media helps it by providing knowledge and information. The change that occurred through time must leave a significant trace in the media, for example Diction, which has changed concerning development and growth, also the ways and mediums of media have become manifold and widespread. This change affected the recipient whether it was a reader, listener o
... Show MoreMass transfer has been studied at rotating cylinder electrodes fabricated with spiral-wound woven-wire meshes using reduction of copper as a test reaction. The experimental data were correlated by an empirical expression between the Sherwood number and the Reynolds number, both regarding the hydraulic diameter as a characteristic length. It was found that the Sherwood number was dependent upon the Reynolds number to the power of 0.521. An enhancement factor was adopted to compare the efficiency of the new rotating cylinder electrode with previous three-dimensional rotating cylinder electrodes. The results showed that the new type has a mass-transfer enhancement factor 2.3 times higher than those obtained with smooth rotating cylinder electr
... Show More
The present study is about tales attributed to certain people and are not traded. Such a study has a great importance for religion scholars and conveyors of hadiths since it is considered as one of the important sources of Islamic legislation that represent the Holy Sunna, and the majority of the recent study is concerned with non-traded tales of religion scholars. The research starts with the definition of such type of tales and then its types, conditions, legality, exceptions, and the benefit from studying such matter. It is mainly concerned with the opinions of the Imami and Hanafi region scholars respectively, and with comparing between the two doctrines i.e. Imami and Hanafi at the end of each
... Show MoreDue to the importance of the extraction process in many engineering and medical industries, in addition to great interest in medicinal plants, in this research, microwave-assisted extraction has been applied to extract some active compounds from Rosmarinus officinalis leaves. The optimal extraction conditions were then determined by calculating the ratio and extraction efficiency. The process has also been described through kinetic study by applying five kinetic models, the Hyperbolic diffusion model, Power low model, the First order reaction model, Elovich's model, and Fick's second law diffusion model and determining their compatibility with the studies operation, and determining the kinetic constants for each model. The result
... Show MoreIn the current analysis, the effects of circumferential scratches along the inner surface of a 170ᵒ -arc partial journal bearing has been numerically investigated. Their impact on the thermo-elasto-hydrodynamic performance characteristics, including maximum pressure, temperature, deformation, and stress, has been examined thoroughly. The ANSYS Fluent CFD commercial code was employed to tackle the iterative solution of flow and heat transfer patterns in the fluid film domain. They are then applied to the ANSYS Static Structure solver to compute the deformation and stress resulted in the solid bearing zone. A wide range of operating conditions has been considered, including the eccentricity ratio ( ) and scratch depth (
... Show MoreThis study deals with the subject of violence, but from another perspective, it has not been emphasized in contemporary studies of violence in Arabic and raqi universities, which is the structural or institutional aspect of violence. Traditional studies have focused their analysis of violence on the direct side the violence, The other side of the violence is the hidden violence, which is characterized by a lack of clarity of the underlying factors and its effects are not arises, which makes it the most serious violence, which requires a deep research into the social, political, economic, cultural and psychological structure of society (individuals and institutions). This study was based on the theory of the Norwegian scientist (J
... Show MoreBackground. After tooth extraction, alveolar bone resorption is inevitable. This clinical phenomenon challenges dental surgeons aiming to restore esthetic and function. Alveolar ridge preservation can be applied to minimize dimensional changes with a new socket grafting material, an autogenous dentin graft, produced by mechanically and chemically processing natural teeth. This study assessed the safety and efficacy of using autogenous dentin biomaterial in alveolar ridge preservation. Materials and Methods. Patients with nonrestorable maxillary anterior teeth bounded by natural sound teeth were included in this study. After a detailed clinical and tomographic examination, eligible participants were randomly allocated into two groups
... Show More