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.
In this research, the structural behavior of reinforced concrete columns made of normal and hybrid reactive powder concrete (hybrid by steel and polypropylene fibers) subjected to chloride salts with concentration was 8341.6 mg/l. The study consists of two parts, the first one is experimental study and the second one is theoretical analysis. Three main variables were adopted in the experimental program; concrete type, curing type and loading arrangement. Twenty (120x120x1200) mm columns were cast and tested depending on these variables. The samples were reinforced using two different bars; Ø8 for ties and Ø12 with minimum longitudinal reinforcement (0.01Ag). The specimens were divided into two main groups based o
... Show MoreBackground: Background : Patients with non-rheumatic atrial fibrillation have high risk of thromboembolism especially ischemic stroke usually arising from left atrial appendage .Transoesophageal echocardiography provides useful information for risk stratification in these patients as it detects thrombus in the left atrial or left atrial appendage. Objective : This study was conducted at Al-Kadhimiya Teaching Hospital to assess the prevalence of left atrial chamber thrombi in patients with chronic non-rheumatic atrial fibrillation using transoesophageal echocardiography and its clinical significance as well as to verify the superiority of transoesophageal over transthoracic echocardiography in the detection of these abnormalities. Type of
... Show MoreIn this study, the possible protective effects of daidzein on ifosfamide-induced neurotoxicity in male rats were examined by the determination of changes in selected oxidant–antioxidant markers of male rats’ brain tissue.
Twenty-eight (28) apparently-healthy Wistar male rats weighing (120-150gm) allocated into 4 groups (n=7) were used in this study. Rats orally-administered 1% tween 20 dissolved in distilled water/Control (Group I); rats were orally-administered daidzein suspension (100mg/kg) for 7 days (Group II); rats intraperitoneally-injected with a single dose of ifosfamide (500 mg/kg) (Group III); rats orally-administered for 7 days with the daidzein (100mg/
... Show MoreObjective: to identify the effect of the Instruction program on the knowledge of pregnant women who suffering anemia.
Methodology: A quasi-experimental design was carried out with the application of pre- post test for the study and the control group. Purposive sample, consists of (60) pregnant women diagnosed with anemia attending four health care centers in Baquba city.
Result: The findings indicate that the level of hemoglobin is increasing post instructional program among women in the study group, in which (46.7%) of women are reveal a level of (8.1-9) g/dl that is less than normal pre instructional program and the level is increased to normal level post instructional
... Show MoreIn this work, an important sugar alkynyl ether has been synthesized in two subsequent steps starting from commercially available D-galactose (3). This kind of compounds is highly significant in the synthesis of biologically active molecules such as 1,2,3-triazole and isoxazoles. In the first step, galactose (3) was reacted with acetone in the presence of anhydrous copper (II) sulfate to produce 1,2:3,4-di-O-isopropylidene-α-D-galactose (4) in good yield. The latter was reacted with excess of 3-bromoprop-1-yne in DMF in the presence of NaOH pellets to afford the target molecule 5 in a very good yield. The temperature of this step is crucial in determining the reaction yi
... Show MoreEmulsion Liquid Membrane (ELM) is an emerging technology that removes contaminants from water and industrial wastewater. This study investigated the stability and extraction efficiency of ELM for the removal of Chlorpyrifos Pesticide (CP) from wastewater. The stability was studied in terms of emulsion breakage. The proposed ELM included n-hexane as a diluent, span-80 as a surfactant, and hydrochloric acid (HCl) as a stripping agent. Parameters such as mixing speed, aqueous feed solution pH, internal-to-organic membrane volume ratio, and external-to-emulsion volume ratio were investigated. A minimum emulsion breakage of 0.66% coupled with a maximum chlorpyrifos extraction and stripping efficiency were achieved at 96.1% and 95.7% at b
... Show MoreDetergent is one of the pollutants that poses significant threats to ecological systems. Detergents can also dissolve in wastewater and negatively impact the efficiency of wastewater treatment facilities. They are used for a variety of functions, most notably hygiene, and are an integral aspect of human life. This means that there are a variety of routes by which detergent components can reach the environment. In this Study, twenty-three detergent samples from local markets in Baghdad. The aim of this study is to investigate the concentration of heavy metals Cobalt (Co), Chromium (Cr),Lead (Pb),Zinc (Zn), Iron (Fe) and Cadmium (Cd) in some detergents using Atomic Absorption Spectrophotometer. The results of the concentration of heavy elemen
... Show MoreThis study aimed to evaluate the effectiveness of a novel concrete-encased column (CE) using small circular steel tubes filled with cementitious grouting material (GFST) as the primary reinforcement instead of traditional steel bars. The research involved three different types of reinforcement: conventional steel bars, concrete-filled steel tubes with 30% of the reinforcement ratio of steel bars, and concrete-filled steel tubes with the same reinforcement ratio as steel bars. Twenty-four circular concrete columns were tested and categorized into six groups based on the type of reinforcement employed. Each group comprised four columns, with one subjected to concentric axial load, two subjected to eccentric axial load (with eccentrici
... Show MoreIn this research, a group of complexes were prepared which were derived from Schiff base ligands, which is called (1E,1'E)-1,1'-(1,2-phenylene)bis(N-(2,4-dichlorophenyl) methanimine) (L) with ortho-phenanthroline (o-phen).The prepared complexes areM(II) [Co(II),Ni(II),Cu(II), Zn(II), Cd(II),and Hg(II)].A range of spectroscopic and technical techniques have been used to characterizethese materials, including:The FTIR, 1H-NMR, LC-Mass Spectrum, UV-Visbale, molar conductance, and magnaticmoment, atomic absorbtion, chlorid contents. Spectral results obtainedare showen that (ortho-phen) and (L) behave as neutral coordinating to the central metal ion by the donatingatoms(N2)of the both compounds. The geometry sha
... Show MoreComplexes reaction of Fe+2, Cd+2, Hg+2 and Ag+ with the 2-thiotolylurea were prepared in ethanolic medium with the (1:1) M:L ratio yielded a series of neutral complexes. The prepared complexes were characterized using flame atomic absorption, micoelemental analysis (C.H.N), chloride content (Mohr Method) , FT.IR and UV-Vis spectroscopic, as well as magnetic susceptibility and conductivity measurement. From the above data, the proposed molecular structure for Fe+2, Cd+2 and Hg+2 complexes are tetrahedral geometry while Ag+ complex is trigonal structure.