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 research involves examining the influence of partial solar eclipse on the strength of neutral hydrogen from the Sun. Baghdad University Radio Telescope (BURT) was used to monitor the partial solar eclipse on the 25th of October, 2022. Radio observations from the Sun were recorded from 11:30 AM to 03:36 PM. This means that the HI emission from the Sun was recorded before, during and after the event. It was noticed, that at the moment of maximum eclipse, ~ 46% of the Sun’s disk was covered by the Moon. For the purpose of this research, the solar radio wave intensity was monitored and the solar flux density was determined at different times, i.e. before, during and after the partial solar eclipse. The obtained results showed that
... Show Morekindergarten teacher is one of the fundamental pillars upon which the kindergarten environment so that exposure to a number of problems that could affect its functioning in addition to any deficiencies in this environment leads to deprive a child of some activities and acquisition of concepts so the researcher studying the problems of working in kindergartens from the perspective of the parameters, so the researcher based measuring instrument for labour problems of (30) search sample was paragraph (50) parameter that was chosen at random and have been extracted Sincerity and strength tool researcher used statistical methods and discriminatory (Pearson correlation coefficient, t
... Show MoreThe Behavioral Disorders of Primary School pupils the son of Alcohol and Non Alcoholic
Vibration is a source of energy that can be beneficial or harmful based on the application. Vibration can affect the function of any structure; however, Ceramic matrix composite (CMC) is one of these structures. Whereby less studies have been concentrated on study its function specially when electromagnetic wave (microwave) exposed on its surface to perform its designed function. To address this concept, SiC composite has been fabricated which is designed to have a transparent characteristics to microwave. External vibration had been applied on its surface to monitor how much influence could nanoscale amplitude vibration damage the microwave interaction. The source of vibration was applied from piezoelectric and the vibration was monitored
... Show MoreThis research is aiming to find the effective role of the personality characteristics of the Leader in business organization entrepreneurship by studying the effect of the special dimensions of personality characteristics (neuroticism, extraversion, openness, Agreeableness, and conscientiousness) on business organizations entrepreneurship dimensions representing in the two main dimensions (entrepreneurship direction and strategic entrepreneurship) across field study was conducted in thirteen private colleges in Baghdad.
The problem of the research relies in asking the question, is there an effect for Leader personality characteristics on business organizations entrepreneurship. and statistical methods hav
... Show MoreThe present research aims at identifying the impact of S.N.I.P.S strategy on the achievement of fifth grade students in natural geography
For the purpose of verifying the objective of the research, the researcher put the following zero hypothesis: There is no statistically significant difference at the level of (0.05) between the mean score of experimental group who study according to the (S.N.I.P.S) strategy, and the mean score of control group who study according to the traditional method in the achievement test.
In order to verify the validity of this null hypothesis, the researcher adopted the experimental method. Two groups of (60) fifth grade literary students were collecte
... Show MoreThe study aims at showing the effect of basil oil on the sensory properties of the laboratory biscuits. the results show the sensory valuation before and after the storage. the (A4) equation exceeds 15% of T exchange (3 , 4 , 5 g. basil 19 , 55 g . fats )in most of the studied sensuous qualities. Then, the (A5) equation of 20% exchange percentage (4.60g. basil oil + 18.40g. fats). Then, the (A3) equation of 10% exchange percentage (2.30g. basil oil + 20.70g. fats). Then the (A2) equation of 5% exchange percentage (1.5g. basil oil + 21.85g. fats). Finally, the control equation (A1) received the lower value of sensuous evaluation and general acceptance. Abstract differences also appeared at denotation level 0.05 between the (A2)
... Show MoreIn this paper, SiO2 nanoparticles thin films were synthesised at different PH values of solution by sol gel method at fixed temperature (25oC) and molar ratio (R =H2O/precursor) of (Tetra Ethyl Ortho Silicate) TEOS as precursor at (R=1). The structure and optical properties of the thin films have been investigated. All thin films were tested by using X-RAY diffraction. All X-RAY spectrum can be indexed as monoclinic structure with strong crystalline (110) plane. The morphological properties of the prepared films were studied by SEM. The results indicate that all films are in nano scale and the particle size around (19-62) nm .The size of silica particles increases with increasing PH value of solution where both the rate of hydrolysis and
... Show MoreAbraham son of Izra, the poet, the philosopher, the grammarian, and the Turah exegesis, is considered one of the famous poets in the era of Islamic reign in Spain which is called the "Golden Era". He was taught all types of teaching which was rich in Jewish literary products. He learned Arabic and studied Arabic language and literature. He travelled a lot, and made tours within Spanish places, and even reached the North of Africa. He preferred living with Muslim rulers in the south of Spain, like the rest of the Jews who emigrated from the north of Spain to the South in search for freedom and to participate in higher positions of the Islamic Palace. He was influenced by Arabic culture, and even translated "Th
... Show MoreResearch Summary The aim of the search for knowledge of the effect generative learning strategy in: 1 - Achievement of the second grade. 2 - Systemic thinking for the second grade students when studying the biology. The study sample increased (60) students distributed into two equal experimental and control groups. Prepare the test of 40 pieces of multiple choice type and prepare a test for systematic thinking according to three skills 1. Understand the relationships between the parts of the systemic form and complement the sentences given 2 - complement the relationships between parts of the systemic form 3. Building the systemic form. It was a search result 1- There is a difference of statistical significance (at level 0.05) between th
... Show More