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.
Providing useful information in estimating the amount and timing and the degree of uncertainty concerning the future cash flows is one of the three main objectives of the financial reporting system, which is done through the main financial statements. The interest on standard-setting bodies in the forecasting of future cash flows, especially Financial Accounting Standards Board (FASB) explain under Accounting Standard (1) of the year 1978 "Objectives of Financial Reporting by Business Enterprises", paragraph (37) thereof that accounting profits better than cash flows when forecasting future cash flows, In contrast, IAS (7) as amended in 1992 aims to compel economic units to prepare statement of c
... Show MoreThe Diwan of Imam Al-Shafi’i acquires great importance, as Al-Shafi’i is an authority in the language, and when I saw that no one had preceded me in exploring its depths, I took my tool and turned my face towards it intending to study the triple verb in it. I stop at these verbs and the student pauses for their morphological forms, looking at the significance of the triple verb more with one letter, two letters, and three letters, and I found that they are many, and such research cannot contain them all, so the choice came to choose the triple verb more with one letter, and the significance of the increase in it, as the increase in The building necessitates an increase in the meaning, and from here the study was limited to the triple
... Show MoreThe postmodern ideas and concepts have produced social, political and economic variables that have been affected by wars, crises, the role of globalization and the information revolution. They have created many variables in concepts and great variables in technological, artistic and cultural innovations. All these changes have contributed to changing the form of the theatrical show aesthetically and intellectually, which cast a shadow over the nature of the actor's performance who has become more demanding to change his performance and to find the mechanisms and new nature of work governing him corresponding to those variables and this prompted the researcher to adopt the subject (the performance variable of the actor's techniques in pos
... Show MoreThe current study aims to identify:The meta-motivation and Uniqueness seeking of the study sample. The correlated relationship among them. The present study sample consists of (400) students from the colleges of engineering, University of Baghdad, and the University of Technology in the academic year (2019-2020), and the researcher has adopted the Chen Scale (1995)to measure the meta-motivation after its translation into Arabic by(Al-Samawi,2011).The scale includes six dimensions. The researcher has also adopted the Snyder&Fromkin scale (1980) to measure the uniqueness seeking after translating and adapting it into the Arabic environment. The scale consists of three dimensions. The results show that students of the Facult
... Show MoreThis study aims at re-transmit the spirit of the national democratic project of Iraq post of the chaos left by the Daesh terrorist group stage(The Islamic State of Iraq and Syria, ISIS) , Which controlled large parts of Iraq, and had an adverse effect on the political scene in Iraq, inspired everyone that there are deficiencies in the political, economic, religious and ethnic structure of the Iraqi people in non-response to democratic thought.The researcher relied on the hypothesis that " the requirements of building a democratic project are based on the need to provide the requirements ranging from two interrelated aspects, like the institutional aspect and the aspect that related to the general and necessary requirements for the e
... Show MoreThe aim of this work is studying the binary system ??'??? Ni?)with two ratios (y=36,80) by using casting method for preparing the samples.Magnetic and Mechanical properties have been studidt different httrea^nttem^rature.All the alloys were found a ferromagnetic behavior and sensitive to the heat treatment. Best properties were found at the heat treatment 1100 C°.A significant different results were found above 1100C° for lower magnetic and mechanical values. This is possibly due to the change on the degree of magnetic moment orders, in which most of the moments are started to remove from coupled ferromagnetically.?
This study presents the effect of laser energy on burning loss of magnesium from the holes' drilled in aluminum alloy 5052. High energy free running pulsed Nd:Glass laser of 300 µs pulse duration has been used to perform the experiments. The laser energy was varied from 1.0 to 8.0 Joules, The drilling processes have been carried out under atmospheric pressure and vacuum inside a specially designed chamber. Microhardness of the blind drilled holes has been investigated .The results indicated that the magnesium loss could be manipulated by adjusting the focusing conditions of the laser beam. Almost, the obtained holes were free of cracks with low taper and low sputter deposition. .The holes performed under atmospheric conditions have high
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