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.
An experimental study was conducted with low cost natural waste adsorbent materials, barley husks and eggshells, for the removal of Levofloxacine (LEVX) antibacterial from synthetic waste water. Batch sorption tests were conducted to study their isothermal adsorption capacity and compared with conventional activated carbon which were, activated carbon > barley husks > eggshells with removal efficiencies 74, 71 and 42 % with adsorbents doses of 5, 5 and 50 g/L of activated carbon, barley husks, and eggshells respectively. The equilibrium sorption isotherms had been analyzed by Langmuir, Freundlich, and Sips models, and their parameters were evaluated. The experimental data were correlated well with the Langmuir model which gives the
... Show MoreFumonisin B1 (FB1) is a mycotoxin produced in some grains (mainly corn) by Fusarium species. Due to a structural similarity between FB1 and sphinganine, sphingolipids metabolism is inhibited. Such inhibition plays a critical role in cell to cell singling and structure of lipoprotein; therefore FB1 has been suggested to have a relationship with human and animal cancer. This research is planned to study the effect of FB1 on male mice at two doses (20 and 30 µg/ ml) on the expression of TGF-β1 and p16 in liver cells. Three groups of Swiss albino male mice; each group was orally administrated with FB1 toxin as the following: normal saline (control group); 20 and 30 µg/ ml. All groups were sacrificed after two weeks of oral manage
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... Show MoreFollowing model educational offenders in collection and Alasbaka of fifth grade students preparatory in history A. M. Dr Prepared by: Dr. Bashaer Mawloud Tawfeeq, The Center of Educational and Psychological Studies Baghdad University - There is no difference statistically significant at the 0.05 level of significance between the average scores of the following students studying using model and offenders and who are studying in the usual manner (traditional) in the collection - There is no difference statistically significant at the 0.05 level of significance between the mean scores for the following students studying using model and offenders and who are studying in the usual manner (traditional) in retention Find limits: Current search
... Show MoreNanoparticles (NPs) have unique capabilities that make them an eye-opener opportunity for the upstream oil industry. Their nano-size allows them to flow within reservoir rocks without the fear of retention between micro-sized pores. Incorporating NPs with drilling and completion fluids has proved to be an effective additive that improves various properties such as mud rheology, filtration, thermal conductivity, and wellbore stability. However, the biodegradability of drilling fluid chemicals is becoming a global issue as the discharged wetted cuttings raise toxicity concerns and environmental hazards. Therefore, it is urged to utilize chemicals that tend to break down and susceptible to biodegradation. This research presents the pra
... Show MoreTo reduce the effects of discharging heated water disposed into a river flow by a single thermal source, two parameters were changed to get the minimum effect using optimization. The first parameter is to distribute the total flow of the heated water between two disposal points (double source) instead of one and the second is to change the distance between these two points. In order to achieve the solution, a two dimensional numerical model was developed to simulate and predict the changes in temperature distribution in the river due to disposal of the heated water using these two points of disposal.
MATLAB-7 software was used to build a program that could solve the governing partial equations of thermal pollution in rivers by using t
Cataract is an opacity in the normally transparent focusing lens of the eye which leads to blindness. The aim of current study is to investigate the possible protective and therapeutic effects of aqueous extract of Foeniculum vulgare seed eye drops (0.5%) against selenite induced cataract in rabbits. Aqueous extract of Foeniculum vulgare seed prepared then formulated as eye drops. Sodium selenite used to induce cataract in rabbits' right eye by single intravitreal injection. Thirty-six rabbits included in the study and divided into three equal groups (12 rabbits in each group): healthy control group, Cataract-Induced group and group treated by Foeniculum vulgare seed extract eye drops. Parameters include the score of lens opacity which was
... Show MoreChemical bath deposition was used to synthesize ZnO nanorods (NRs) on glass and fluorine_doped tin oxide (FTO) substrates. X-ray diffraction was performed to examine the crystallinity of ZnO nanorod. Results showed that ZnO NRs had a wurtzite crystal structure. Field emission scanning electron microscopy images showed that glass sample had rod-like structure distribution with (50 nm) diameter and average length of approximately (700 nm), whereas the FTO-coated glass sample had 25 nm diameter and average length of approximately 950 nm. The direct optical transition band gaps of the glass and FTO_coated glass samples were( 4 and 4.43 eV), respectively. The structural and optical properties of the synthesized ZnO p
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