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 - M ultiple mixing ratios of -transitions from levels of 56Fe populated in 56 56 Fe n n Fe ( , ) reactions are calculated by using const. S.T.M. This method has been used in other works [3,7] but with pure transition or with transitions that can be considered as pure transitions، in our work we used This method for mixed - transitions in addition to pure - transitions. The experimental angular distribution coefficients a2 was used from previous works [1] in order to calculet - values. It is clear from the results that the - values are in good agreement or consistent, within associated errors, with those reported previously [1]. The discrepancies that occur are due to inaccuracies existing in the expe
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... Show MoreIn this study, Yogurt was dried and milled, then shaked with distilled water to remove the soluble materials, then again dried and milled. Batch experiments were carried out to remove hexavalent chromium from aqueous solutions. Different parameters were optimized such as amount of adsorbent, treatment time, pH and concentration of adsorbate. The concentrations of Cr6+ in solutions are determined by UV-Visible spectrophotometer. Maximum percentage removal of Cr6+ was 82% at pH 2. Two equilibrium adsorption isotherms mechanisms are tested Langmuir and Freundlich, the results showed that the isotherm obeyed to Freundlich isotherm. Kinetic models were applied to the adsorption of Cr6+ ions on the adsorbents, ps
... Show MoreThis study was conducted to evaluate the efficacy of 6 isolates of Pseudomonas fluorescens and Trichoderma harzianum and there combination against Fusarium tomato wilt disease caused by Fusarium oxysporum F.sp. Lycopersisi under green house condition .The isolates of bacteria (B3) and Trichoderma (T1) were found to be highly effective in reducing the disease incidence to 13.3% , 21% respectively , compared to control treatment (40%).Furthermore, disease severity was reduced to 28 and 30% respectively in comparison to control (90%) .Colonization of the roots (cfu /g fresh root weight )by the two isolates whether alon or together was extremely high . The combination treatment had a high ability in reducing disease incidenece and sev
... Show MoreWater is a resource and a crucial aspect of living and surviving. In Iraq, the Tigris River is one of the most critical water sources. The present study aimed to provide an insight analysis of some water quality parameters including the microbial content of drinkable tap water and river water. Ten Water samples (T1- T10) in triplicate were collected from sampling sites -Site I (Tap water) from home water taps, supplied by the Water Filtration Station/ Al Karama Project/ Al-Karkh> 10 from Site II (R1- R10)River water from Tigris River (around or near the Water Filtration Station/ Al Karama Project) every week (from September to half of November 2022), then were immediately placed in sterile bottles and transported to Microbiolo
... Show MoreMetal complexes chrome(III), manganese(II), iron(III), cobalt(II), nickel(II), cupper(II) and zinc(II) with diazonium of 3-amino-2-chloropyridine of general formula [2-Cl-C5H3N≡N]n[MXm], where n=2 or 3 for divalent and trivalent metal, m= 4 or 6 were synthesized. The complexes have been characterized by flame atomic absorption, (C.H.N), molar conductance, magnetic susceptibility UV-vis spectra, infrared spectra,1H-NMR spectroscopy and thermo gravimetric analysis (TGA and DTA). The measurements showed that the divalent metal ion complexes (M2+) have (1:2) M:L ratio with tetrahedral geometry around metal ions while the trivalent metal ions (M3+) formed (1:3) m
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... Show MoreAl-Aziz's book was chosen as a subject for research as it is one of the important books in the Islamic world in general and the Shafi'i school in particular and its author Imam Abdul Karim bin Mohammed bin Abdul Karim Al-Rafi's, who in the doctrine of the jurisprudence has made prominent lines and left behind invaluable scientific treasures in the service of religion. Its importance is summarized as follows:
1. Being an explanation of the book (brief) of the argument of Islam Imam Ghazali (God's mercy), one of the five books adopted in Shafi'i jurisprudence.
2. His work is Imam Abu al-Qasim al-Rafii known for the brilliance of the investigation and the power of weighting in the doctrine.
3. It is considered an encyclopedia in Sh