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.
Biological activity substances was investigated in watery extract of lentil which found to contain phenols, tannin, saponins and resins while, flavons, terpens and steroids were not exist in the extract details explained that 5%, 10% of lentil extract largly inhibited the growth of Psedumonas aeruginosa then Escherichia coli and Bacillus subtilis. The growth of both Staphylococcus aureus and Salmonella typhimurium were slightly affected by all extract concentration. Extracellular protease were screened in all bacterial species under study. Complete inhibition was achieved for extracellular protease while different percentage of protease inhibition were seen for intracellular proteases.
الوصف The synthesis of 2 (N-phenyl dithio carboxamid) benzothiazol Ligand (L) from reaction of 2-Mercaptobenzothiozol with phenylisothiocyanate using ratio 1: 1. The ligand was characterized by elemental analysis (CHN),'H-NMR, IR and UV-Vis. The complexes with bivalent ions (Ni, Cu, Zn, Cd and Hg) have been prepared and characterized. The structural diagnosis was established using IR, UV–Visible spectro photometer, molar conductivity, atomic absorption and molar ratio with selected metal ions (Ni2+, Cu2+). The complexes of (Ni, Cu) gave octahedral structural while the complexes of (Zn, Cd, Hg) gave tetrahedral structural. The study of biological activity of the ligand (L) and its complexes (Ni, Cu, Hg) in two deferent concentration (
... Show MoreThe study involved the effectiveness of Iraqi attapulgite (IQATP) clay as an environmentally friendly material that easily adsorbs brilliant green (BG) dye from water systems and is identified by various complementary methods (e.g., FTIR, SEM‐EDS, XRD, ICP‐OES, pHpzc, and BET), where the result reported that the IQATP specific surface area is 29.15 m2/g. A systematic analysis was selected to evaluate the impact of different effective adsorption performance variables on BG dye decontamination. These variables included IQATP dosage (0.02–0.8 g/L), solution pH (3.05–8.15), contact time (ranging from 2 to 25 min), and initial BG dye concentration from 20 to 80 mg/L. The parameter
... Show MoreThe article is devoted to the issue of word-formation motivation, which does not lose its relevance and plays a role not only in disclosing formal-semantic relations between words of one language and has not only theoretical, but also applied significance. The authors consider word-formation motivation consistently in its varieties in a comparative way on the materials of so different languages as Russian and Arabic and approach the mechanism of achieving semantic equivalence of translation. To the greatest extent, word-formation activity today, due to objective reasons, affects some special branch (technical, medical, etc.) vocabulary, which is increasing from year to year in national dictionaries. This extensive material, selected
... Show MoreThe importance of the present work falls on the pitting corrosion behavior investigation of 304 SS and 316 SS alloys in 3.5 wt% of aqueous solution bearing with chloride and bromide anion at different solutions temperature range starting from (20-50)oC due to the pitting corrosion tremendous effect on the economic, safety and materials loss due to leakage. The impact of solution temperatures on the pitting corrosion resistance at 3.5wt% (NaCl and NaBr) solutions for the 304 SS and 316 SS has been investigated utilizing the cyclic polarization techniques at the potential range -400 to1000 mV vs. SCE at 40 mV/sec scan rate followed by the surface characterization employing Scanning Electron&nbs
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Objective: the idea of this study to improve transdermal permeability of Methotrexate using eucalyptus oil, olive oil and peppermint oil as enhancers.
Method: eucalyptus oil (2% and 4%), peppermint oil (2% and 4%) and olive oil (2% and 4%) all used as natural enhancers to develop transdermal permeability of Methotrexate via gel formulation. The gel was subjected to many physiochemical properties tests. In-vitro release and permeability studies for the drug were done by Franz cell diffusion across synthetic membrane, kinetic model was studied via korsmeyer- peppas equation.
Result: the results demonstrate that safe, nonirritant or cause necrosis to rats' skin and stable till 60 days gel was successfully formulated.<
In this study forty mature albino rats were used wich were randomly divided into five groups ,four groups were adminstrated Phoenix dactylifera pollen grains suspension at concertenrations (18,54,108,and 216)mg/ kg body weight by oral administration while the fifth group was considered as a control group.Experiment continued for 40 days then rats were sacrificed and samples of blood were collected for determination of some biochemical parameters (total protein ,total cholesterol ,LDLc and HDLc).Testis were removed for preparation histological sections to measures the diameters of seminferous tubules ,thickness of seminiferous epithelium and the numbers of spermatogenic cells.
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