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.
The effect of mental and physical health on the feelings of subject welfare is known as health-related quality of life. The chronic obstructive pulmonary disease is a common respiratory diseases. Assessment of health-related quality of life is considered important in such chronic disease. The objective of the current study was to measure health-related quality of life in a sample of chronic obstructive pulmonary patients in AL- Diwanyia city/Iraq. This study was carried out on 150 already diagnosed COPD patients who attended to the Center of Respiratory Diseases/AL-Diwaniyah Teaching Hospital during September 2019 to January 2020. The Arabic version of St George’s Respiratory Questionnaire was used to asses
... Show MoreThe enhancement of the thermal and thermo-hydraulic performance of a semi-circular solar air collector (SCSAC) is numerically investigated using porous semi-circular obstacles made of metal foam with and without longitudinal porous Y-shaped fins. Two 10 and 40 PPI porous material samples are examined. Three-dimensional models are built to simulate the performance of SCSAC: model (I) with clear air passage; model (II) with only metal foam obstacles, and model (III) with metal foam obstacles as well as porous Y-fins. COMSOL Multiphysics software version 6.2 based on finite element methodology is employed. A conjugate heat transfer with a (k-ε) turbulence model is selected to simulate both heat transfer and fluid flow across the entir
... Show MoreNo country in the world has an ancient heritage of its own. It represents the product of the civilizations left behind by previous eras. It represents the development of urban life and its capital of the interest of its people in the field of construction and reconstruction. The urban heritage and its associated arts may seem to be a material heritage at first glance, but it is not free from the spiritual side. Therefore, the nations in different parts of the earth cherish it and care for it with all due diligence, because it mixes with its history, memories and emotions.
This research discusses the use of local environment materials in the production of urban fabric constituents. I
The aim of the research is the knowing of the academic Scientific Journals of the colleges of University of Baghdad , through searching in the University of Baghdad website and the websites of colleges and thus studying the presentation of these journals in those website , and this is done by surfing the main pages of the websites for the colleges included in the research , and by analyzing the research made a menu for all the academic Scientific Journals for all institutes of the University of Baghdad , which simplifies the way for the researchers to publish these researches in the specific journals for their specialties .
The importance of the accounting profession in creating data and accounting information for economic resources and their sources and changes therein , through the effectiveness of this information and the degree of suitability and providing accurate and timely manner and in a form that makes it able to rationalize decisions and performance reporting , and there are several elements and behaviors control how the effectiveness of these reports and these behaviors work ethic that govern the work of management accountant when preparing performance reports , which are the ethics of the work of professional control principles and rules of accounting , legal, such as objectivity and reliability , neutrality and timeliness that must be a
... Show MoreThis research is concerned with studying the representations of the event in the drawings of the ancient civilizations of the world, and the research consists of two axes, the axis of the theoretical framework, which included (the research problem, its aim, its limits, and the definition of its terminology).
The research aims to reveal how the event pattern was formulated by the artist on the surface of his visual achievement, and the limits of the search were spatial in the ancient civilizations of Iraq, Egypt, Greece and Rome, but the limits of the temporal research could not be determined because they were before birth, and objectively:
representations of the event in the civilizations of the ancient world This axis also in