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.
Internal Audit is one of the most important backers of corporate governance, the researcher expanded his interest in this subject to examine the efficiency of Internal Auditors at the Arab Bank and its branches in Jordan to achieve Accountability which enhances the Corporate Governance and to identify the effect of the International Internal Audit Standards in strengthening the role of Internal Auditors in Accountability, and the effect of Attribute and Performance Standards in Accountability. The researcher applied descriptive analysis method to define the role of Internal Audit in the Arab Bank in achieving one of the basic principles of Corporate Governance assimilated in Accountability. The researcher’s sources include
... Show More The integration of AI technologies is revolutionizing various aspects of the apparel and textile industry, from design and manufacturing to customer experience and sustainability. Through the use of artificial intelligence algorithms, workers in the apparel and textile industry can take advantage of a wealth of opportunities for innovation, efficiency and creativity.
The research aims to display the enormous potential of artificial intelligence in the clothing and textile industry through published articles related to the title of the research using the Google Scholar search engine. The research contributes to the development of the cultural thought of researchers, designers, merchants and the consumer with the importance of integ
The current study is concerned with presenting a descriptive study of the history of the city of Astrabath in the sixth century AH. The problem that the research tried to answer is represented in the investigation of historical news, and the study of the most prominent cultural aspects which characterizing that city. The research also aims to reach the geographical location of that city, its name and its suburbs. The importance of the research lies in the knowledge of everything related to the role of the civilization of Astrabath, as well as tracing the entry of the Arabs to it, so that it became a cultural, economic and scientific center and its historical role in the sixth century AH. The research reminde us with the scientific role o
... Show MoreApproaching the turning of the millennium, the American theatre witnessed an arousing
interest much shown in patients suffering of severe diseases as a subject matter to drama. In a
discussion of Margaret Edson's Wit, the light is shed on how far such patients, who were literally
involved in secular visions during their life-time, become apt to create a different one on their
death beds. The vision newly blossomed becomes much rooted in the spiritual life; it is a
redemptive vision that can amend what those patients' hearts and minds have long ignored.
Further, the human touch that has been ignored during man's healthy secular life is ultimately
needed for the time being. It helps to enhance man's vision towards the
Thi s study included the effect of chronic exposure of di fferent concentrations of Nan on the biology of fresh water zroDplonk ton species Moina 41.112' ( Cladcxe'ra The concentrations used for chronic exposure were 03, 0.75. 1 960 to investigate iI effects on the life cycle and la lables of this species after exposure period and the range of this exposure effects on the rate of expectation for further life, also the effect of salinity on reproduotive parameters was studied which included average of total number of eggs and young produced, volume clutch's (6.95. indiclutch for control group , 14.516 indi clutch for 0.596a ,3_65 ind./ clutch for (175 5(.. and 03 Ind} cruel] for 1960) and number of Clutch's (5.5 clutch/ female for control gr
... Show MoreThis study was aimed to investigate the load of bacterial contaminant in fresh meat with different types of bacteria.One handered and seven samples were collected from different regions of Baghdad . These samples included 37 of fresh beef 70 of fresh sheep meat. All samples were cultured on different selective media to identitfy of contaminated bacteria .The result revealed that The percentage of bacterial isolate from raw sheep meat were, % 23.8of StreptococcusgroupD,29.4 % of Staphylococcus aureus ,14.7 % of E.coli , %4.9of Salmonella spp, ,%3.5 of pseudomonas aeruginosa, %14.7.%14.7 of Proteus spp.% 2.1 of Listeria spp while the raw beef meat content %5.55 of Staphylococcus aureus, %8.14 of streptococcus group D , %5.18 %1.85 of E.coli,
... Show MoreThe ideas and principles formulated by Ibn-Jamaah in the field of education occupy a central place in the historical origins of education for Muslims. These views and principles have an active role in the educational reorganization that the Islamic world aspires to. These ideas have had a great impact on the educational process that had preceded the opinions of Russo, Pestalutzi, Fruel, Herbert, and Dewey. Moreover, we have seen that the Sheikh of Ibn-Jamaah has taken part in formulating the origins of education and leadership.
The seventh international scientific conference held at the "University of Baghdad" College of Education "Ibn Rushd" 14-15 / 4/2019, confirmed in its recommendations the need to draw clear strategic planning to invest the qualitative inputs from the Ministry of Education and focus on it in higher education, which requires educational institutions to amend from its current strategies or adopting new strategies in a way that contributes to presenting new ways to compete and keep pace with scientific progress and to find realistic and objective working methods. The aim of the current study is to identify the reality of university strategic planning to invest the outputs of the distinguished schools of the heads of public universities. The r
... Show MoreThis paper reflects the evidence of the Islamic Development Bank's role in building human and institutional capacities in Member States and assisting governments in achieving economic development. The Bank's activities in the area of capacity development are an important part in following up the economic developments, And help governments to build and formulate effective policies and institutions to implement more effective macro policies leading to better economic outcomes. It is therefore helpful to help Member States increase their resources, modernize their banking systems, establish effective legal frameworks and improve Reports on macroeconomic and financial statistics. The Bank has faced many challenges and risks tha
... Show More