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.
Background: Lymphomas are group of diseases caused by malignant lymphocytes that accumulate in lymph nodes and caused the characteristics lymphadenopathy. Occasionally, they may spill over into blood or infiltrate organs outside the lymphoid tissue. The major subdivision of lymphomas is into Hodgkin lymphoma and non–Hodgkin lymphoma and this is based on the histologic presence of Reed-Sternberg cells in Hodgkin lymphoma. Salivary immunoglobulin A is the prominent immunoglobulin and is considered to be the main specific defense mechanism in oral cavity. The aim of this study was to determine the level of salivary immunoglobulin A in lymphoma patients before and after chemotherapy treatment. Subjects, materials and methods: The study i
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Among the things that have happened and that have emerged from the developments in society is the phenomenon of dairy banks, where institutions collect milk from donating mothers or sellers of milk and benefit from it by sterilizing and selling it.
This topic is considered one of the important topics, as Islam considers breastfeeding as a link as well as parentage, and it has the same genealogy as the spread of sanctity. Therefore, Imamate jurists addressed this topic with research despite its absence in Islamic societies.
The importance of r
... Show MorePhotonic crystal fiber interferometers are used in many sensing applications. In this work, an in-reflection photonic crystal fiber (PCF) based on Mach-Zehnder (micro-holes collapsing) (MZ) interferometer, which exhibits high sensitivity to different volatile organic compounds (VOCs), without the needing of any permeable material. The interferometer is robust, compact, and consists of a stub photonic crystal fiber of large-mode area, photonic crystal fiber spliced to standard single mode fiber (SMF) (corning-28), this splicing occurs with optimized splice loss 0.19 dB In the splice regions the voids of the holey fiber are completely collapsed, which allows the excitation and recombination of core and cladding modes. The device reflection
... Show MoreVanadium dioxide nanofilms are one of the most essential materials in electronic applications like smart windows. Therefore, studying and understanding the optical properties of such films is crucial to modify the parameters that control these properties. To this end, this work focuses on investigating the opacity as a function of the energy directed at the nanofilms with different thicknesses (1–100) nm. Effective mediator theories (EMTs), which are considered as the application of Bruggeman’s formalism and the Looyenga mixing rule, have been used to estimate the dielectric constant of VO2 nanofilms. The results show different opacity behaviors at different w
Pragmatics of translation is mainly concerned with how social contexts have their own influence on both the source text (ST) initiator's linguistic choices and the translator's interpretation of the meanings intended in the target text (TT). In translation, socio-pragmatic failure(SPF), as part of cross-cultural failure, generally refers to a translator's misuse or misunderstanding of the social conditions placed on language in use. In addition, this paper aims to illustrate the importance of SPF in cross-cultural translation via identifying that such kind of failure most likely leads to cross-cultural communication breakdown. Besides, this paper attempts to answer the question of whether translators from English into Arabic or vice versa h
... Show MoreTerrorism is a serious problem for many societies today. This research aims to identify the impact of terrorism and displacement crisis on human security, which was a shock to the Iraqi society in terms of its impact on the psychological, social and economic conditions of the individual, family, and society. The variety of methods of carrying out the terrorist operations that resulted from the phenomenon of human displacement witnessed by Iraq since the middle of 2014. This phenomenon has its demographic, political and social dimensions.
In order to achieve the goal of this study and the importance of the subject, the social survey method was used by selecting a sample of 200 IDPs in a compou
... Show MoreFibromuscular dysplasia (FMD) is a noninflammatory and nonatherosclerotic arteriopathy that is characterized by irregular cellular proliferation and deformed construction of the arterial wall that causes segmentation, constriction, or aneurysm in the intermediate-sized arteries. The incidence of FMD is 0.42–3.4%, and the unilateral occurrence is even rarer. Herein, we report a rare case of a localized extracranial carotid unilateral FMD associated with recurrent transient ischemic attacks (TIAs) treated by extracranial-intracranial bypass for indirect revascularization. The specific localization of the disease rendered our case unique.
In modern technology, the ownership of electronic data is the key to securing their privacy and identity from any trace or interference. Therefore, a new identity management system called Digital Identity Management, implemented throughout recent years, acts as a holder of the identity data to maintain the holder’s privacy and prevent identity theft. Therefore, an overwhelming number of users have two major problems, users who own data and third-party applications will handle it, and users who have no ownership of their data. Maintaining these identities will be a challenge these days. This paper proposes a system that solves the problem using blockchain technology for Digital Identity Management systems. Blockchain is a powerful techniqu
... Show Moreالمستخلص [English] Peace-building is a science that has its foundations and needs mechanisms, components, studies and plans based on the needs of the parties responsible for achieving peace and meeting the necessary needs of conflict societies, as it has become a goal sought by many local and regional actors and parties. The State of South Africa has realized this fact and has devoted space in its conception and planning to addressing the issues of conflict and war within the framework of its unitary work.Since security and stability in general seek to create peaceful and legal means through which existing problems can be expressed and addressed. The differences have varied to build peace and create cooperation between groups and th
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