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 dynamic thermomechanical properties, sealing ability, and voids formation of an experimental obturation hydroxyapatite-reinforced polyethylene (HA/PE) composite/carrier system were investigated and compared with those of a commercial system [GuttaCore (GC)]. The HA/PE system was specifically designed using a melt-extrusion process. The viscoelastic properties of HA/PE were determined using a dynamic thermomechanical analyser. Human single-rooted teeth were endodontically instrumented and obturated using HA/PE or GC systems, and then sealing ability was assessed using a fluid filtration system. In addition, micro-computed tomography (μCT) was used to quantify apparent voids within the root-canal space. The data were statistically analys
... Show MoreBiosorption of lead, chromium, and cadmium ions from aqueous solution by dead anaerobic biomass (DAB) was studied in single, binary, and ternary systems with initial concentration of 50 mg/l. The metal-DAB affinity was the same for all systems. The main biosorption mechanisms were complexation and physical adsorption of metallic cations onto natural active functional groups on the cell wall matrix of the DAB. It was found that biosorption of the metallic cations onto DAB cell wall component was a surface process. The main functional groups involved in the metallic cation biosorption were apparently carboxyl, amino, hydroxyle, sulfhydryl, and sulfonate. These groups were part of the DAB cell wall structural polymers. Hydroxyle groups (–O
... Show MoreThis work involves the preparation of the ligand [KL] :- ÂÂÂÂÂÂ
K[4-(N-(5-methylisoxazol-3-yl) sulfamyl) phenylcarbamodithioate] from the reaction of sulfamethoxazole with Carbon disulfide in the presence of potassium hydroxide under reflux (4 hours) using methanol as asolvent. The prepared ligand was characterized using FT-IR, UV-Vis, 1H,13C–NMR spectroscopy, molar conductivity and melting point, Complexes for the above ligand [KL] with some bivalent transition and non-transition metals (Mn +2, Co+2 , Ni+2 ,
... Show MoreAbstract: Thorough assessment of the Maxillary,Sinus is very important. Recently 3-dimensional imagewith Cone Beam Computed Tomography (CBCT) is very dependable in Maxillary Sinus diagnosis. The aim of this study is to: shade light on the role of (CBCT) diagnosis of the maxillary sinus anatomical variation and pathological finding among smokers and nonsmokers prior to maxillary sinus lift techniques. Materials and Method: In this study 60 males with age ranged between 20-50 years old, referred for (CBCT) assessment of maxillary sinus in the Specialist Health Center of AL-Sadder city. The scanning were performed using Kodak 9500 (CBCT), the KV was 90, mA10 and scanning time 10 s. Voxel size 0.3mm with( DICOM ) software on a multiplaner re
... Show MoreIt is found that hypersensitive teeth have a larger number and wider patent tubules than those of non-sensitive teeth. Objective: The aim of this study is to compare between the effects of diode laser at different power densities, with and without sodium fluoride on the sealing of exposed dentinal tubules and dentin permeability. Materials and methods: 118 teeth were used. Samples were divided into three major groups. The first consisted of 100 teeth used for permeability test. The second consisted of 16 teeth for measuring external surface temperature elevation while irradiation. The third, in turn, consisted of one pair of teeth observed under SEM for dentine surface morphology analysis. Results: For dentin permeability measurement, th
... Show MoreThe [2-hydroxy -1,2-diphynel-ethanone oxime] was reacted with 1,2- dichloroethan to give the new ligand [H2L].this ligand was reacted with some metal ions (Co(II),Ni(II),Cu(II),Zn(II) and Cd(II) in methanol as a solvent to give a series of new (1:1)complexes of the general formula [ M(HL)]Cl ,( where : M= Co(II),Ni(II),Cu(II),Zn(II) and Cd(II)) are isolated All compounds have been characterized by spectroscopic methods [ I.R , U.V -Vis ] atomic absorption . Chloride content along with conductivity measurements. From the above data the proposed molecular structure for (Co, Cu, Ni, Zn and Cd) complexes adopting a tetrahedral structure.
In study of effective bioactive compounds, we have synthesized the Co((ІІ), Mn(ІІ), Fe(ІІ), Cu(ІІ), Ni(ІІ), and Zn(ІІ) complexes of the Schiff base derived from trimethoprim and2'-amino-4-chlorobenzophenone and characterized by spectroscopic (NMR, IR, Mass, UV–vis,), analytical, TGA studies and magnetic data .The solution electronic spectral study suggests the stoichiometry of the synthesized complexes and Elemental analysis detected the square planer and octahedral geometry of the compounds. The prepared metal complexes presented promoted efficiency versus the screened bacterial (Escherichia Coli and Staphylococcus aureus) antibacterial efficacy against (Staphylococcus aureus, Salmonella spp., E. coli, Vibrio spp., Pseud
... Show MoreTwo Schiff bases, namely, 3-(benzylidene amino) -2-thioxo-6-methyl 2,5-dihydropyrimidine-4(3H)-one (LS])and 3-(benzylidene amino)-6-methyl pyrimidine 4(3H, 5H)-dione(LA)as chelating ligands), were used to prepare some complexes of Cr(III), La(III), and Ce(III)] ions. Standard physico-chemical procedures including metal analysis M%, element microanalysis (C.H.N.S) , magnetic susceptibility, conductometric measurements, FT-IR and UV-visible Spectra were used to identify Metal (III) complexes and Schiff bases (LS) and (LA). According to findings, a [Cr(III) complex] showed six coordinated octahedral geometry, while [La(III), and Ce(III) complexes]were structured with coordination number seven. Schiff's bases a
... Show Moreالوصف A simple chemistry method approach was used to synthesise new ligand derivate from L-ascorbic acid and its complexes. All of them were water-soluble and are used quite extensively in the medical and pharmaceutical fields. This study synthesised the new ligand derivative from L-ascorbic acid-base using the following steps: A 5, 6-O-isopropylidene-L-ascorbic acid was prepared by reacting dry acetone with L-ascorbic acid followed by reacting it with trichloroacetic acid to yield [chloro (carboxylic) methylidene]-5, 6-O-isopropylidene-L-ascorbic acid in the second stage. In the third stage, the derivative was reacted with (methyl (6-methyl-2-pyridylmethyl) amine to create a new ligand (ONMILA). This novel ligand was identified using
... Show MoreThe N-[(2,3-dioxoindolin-1-yl)-N-methylbenzamide] was prepared by the reaction of acetanilide with isatin then in presence of added paraformaldehyde, the prepared ligand was identified by microelemental analysis, FT.IR and UV-Vis spectroscopic techniques. Treatment of the prepared ligand with the following selected metal ions (CoII, NiII, CuII and ZnII) in aqueous ethanol with a 1:2 M:L ratio, yielded a series of complexes of the general formula [M(L)2Cl2]. The prepared complexes were characterized using flame atomic absorption, (C.H.N) analysis, FT.IR and UV-Vis spectroscopic methods as well as magnetic susceptibility and conductivity measurements. Chloride ion content was also evaluated by (Mohr method). From the obtained data the octahed
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