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.
A novel series of mixed-ligand complexes of the type, [ML1(L2)3]Clx [M= Cr(III), Fe(III), Co(II),Ni(II), Cu(II), Cd(II) and Hg(II), n = 2, 3], was synthesized using Schiff base (HL1) as main ligand, nicotinamide (L2) as secondary ligand, and the corresponding metal ions in 1:3:1 molar ratio. The main ligand, HL1 was prepared by the interaction of ampicillin drug and 4-chlorobenzophenone. The synthesized mixed ligand complexes were characterized by elemental analysis, UV-Vis, FT-IR,1H-NMR,13C-NMR and TG/DTG studies. In the mixed-ligand complexes, the Schiff base ligand, HL1 showed coordination to the central metal ion in tridentate manner via azomethine nitrogen, β-lactam ring oxygen and deprotonated carboxylic oxygen atoms, whereas the sec
... Show MoreResveratrol, (RES), a phytoalexin, is well-known for its anti-inflammatory and anti-oxidant properties. SEB, a superantigen, is known to trigger ALI and cause mortality. In the current study, we tested the effect of RES in a dual-dose model of SEB exposure that triggers ALI and causes 100% mortality in C3H/HeJ-mice. The data revealed RES attenuated SEB-induced ALI and prevented mortality. Forty eight hours post-SEB exposure, lung-infiltrating mononuclear cells were tested for microRNA expression profile to determine the epigenetic regulation by resveratrol. SEB-activated splenocytes were pre-treated with 50 μM of RES or vehicle for metabolic profile analysis by measuring oxygen consu
A new Schiff base [1-((2-(1H-indol-3-yl)ethylimino)methyl)naphthalene-2-ol] (HL) has been synthesized by condensing (2-hydroxy-1-naphthaldehyde) with (2-(1H-indol-3-yl)ethylamine). In turn, its transition metal complexes were prepared having the general formula; [Pt(IV)Cl2(L)2], [Re(V)Cl2(L)2]Cl and [Pd(L)2], 2K[M(II)Cl2(L)2] where M(II) = Co, Ni, Cu] are reported. Ligand as well as metal complexes are characterized by spectroscopic techniques such as FT-IR, UV-visible, 13C & 1H NMR, mass, elemental analysis. The results suggested that the ligand behaves like a bidentate ligand for all the synthesized complexes. On the other hand, theoretical studies of the ligand as well its metal complexes were conducted at gas phase using Hyp
... Show MoreThe ligand [Potassium (E)-(4-(((2-((1-(3-aminophenyl) ethylidene) amino)-4-oxo-1, 4-dihydropteridin-6-yl) methyl) amino) benzoyl)-L-glutamate] was prepared from the condensation reaction of folic acid with (3-aminoacetophenone) through Schiff reaction to give a new Schiff base ligand [H2L]. The ligand [H2L] was characterized by elemental analysis CHN, atomic absorption (AA),(FT-IR),(UV-Vis), TLC, ES mass (for spectroscopes), molar conductance, and melting point. The new Schiff base ligand [H2L], reacts with Mn (II), Co (II), Ni (II), Cu (II), Cr (III) and Cd (II) metal ions and (2-aminophenol),(metal: derivative ligand: 2-aminophenol) to give a series of new mixed complexes in the general formula:-K3 [M2 (HL)(HA) 2],(where M= Mn (II) and Cd
... Show MoreIn this work, Schiff base ligands L1: N, N-bis (2-hydroxy-1-naphthaldehyde) hydrazine, L2: N, N-bis (salicylidene) hydrazine, and L3:N –salicylidene- hydrazine were synthesized by condensation reaction. The prepared ligands were reacted with specific divalent metal ions such as (Mn2+, Fe2+, Ni2+) to prepare their complexes. The ligands and complexes were characterized by C.H.N, FT-IR, UV-Vis, solubility, melting point and magnetic susceptibility measurements. The results show that the ligands of complexes (Mn2+, Fe2+) have octahedral geometry while the ligands of complexes (Ni2+) have tetrahedral geometry.
<p><span>A Botnet is one of many attacks that can execute malicious tasks and develop continuously. Therefore, current research introduces a comparison framework, called BotDetectorFW, with classification and complexity improvements for the detection of Botnet attack using CICIDS2017 dataset. It is a free online dataset consist of several attacks with high-dimensions features. The process of feature selection is a significant step to obtain the least features by eliminating irrelated features and consequently reduces the detection time. This process implemented inside BotDetectorFW using two steps; data clustering and five distance measure formulas (cosine, dice, driver & kroeber, overlap, and pearson correlation
... Show MoreIn this study, dead and live anaerobic biomass was used in biosorption of Pb(II), Cr(III) and Cd(II) ions from a synthetic wastewater. The biosorption was investigated by batch adsorption experiments. It was found that, the biosorption capacities were significantly affected by biosorbent dosage. The process follows Langmuir isotherm (regression coefficient 0.995, 0.99 and 0.987 for Pb(II), Cr(III) and Cd(II) ions, respectively, onto dead anaerobic biomass) model with uniform distribution over the biomass surface. The experimental uptake capacity was 51.56, 29.2 and 28 mg/g for Pb(II), Cr(III) and Cd(II), respectively, onto dead anaerobic biomass, compared with 35, 13.6 and 11.8 mg/g for Pb(II), Cr(III) and Cd(II), respectively, onto live
... Show MoreThis research presents a numerical study to simulate the heat transfer by forced convection as a result of fluid flow inside channel’s with one-sided semicircular sections and fully filled with porous media. The study assumes that the fluid were Laminar , Steady , Incompressible and inlet Temperature was less than Isotherm temperature of a Semicircular sections .Finite difference techniques were used to present the governing equations (Momentum, Energy and Continuity). Elliptical Grid is Generated using Poisson’s equations . The Algebraic equations were solved numerically by using (LSOR (.This research studied the effect of changing the channel shapes on fluid flow and heat transfer in two cases ,the first: cha
... Show MoreSix house-hold Abyssinian pumps distributed in different villages of Mansoura (Mans-I, Mans-II and Mans-III) and Talkha (Talk-I, Talk-II and Talk-III) cities, Egypt, have been selected for regular seasonal water quality assessment during 2017. Water samples have been collected within the mid-periods of four seasons Standard assessment tools were employed for the integrated water quality assessment including Water Quality Index (WQI) and ISO standard algal toxicity test. WQI displayed remarkable local and seasonal variations with excellent (≥ 90) and good (70 - 89) only recorded for water samples collected from Mans-I pump located in sparsely populated area and far 50 meters only from the eastern (Damietta) branch of Nile River. WQI of
... Show MoreThe Electro-Fenton oxidation process is one of the essential advanced electrochemical oxidation processes used to treat Phenol and its derivatives in wastewater. The Electro-Fenton oxidation process was carried out at an ambient temperature at different current density (2, 4, 6, 8 mA/cm2) for up to 6 h. Sodium Sulfate at a concentration of 0.05M was used as a supporting electrolyte, and 0.4 mM of Ferrous ion concentration (Fe2+) was used as a catalyst. The electrolyte cell consists of graphite modified by an electrodepositing layer of PbO2 on its surface as anode and carbon fiber modified with Graphene as a cathode. The results indicated that Phenol concentration decreases with an increase in current dens
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