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 current study used extracts from the aloe vera (AV) plant and the hibiscus sabdariffa flower to make Ag-ZnO nanoparticles (NPs) and Ag-ZnO nanocomposites (NCs). Ag/ZnO NCs were compared to Ag NPs and ZnO NPs. They exhibited unique properties against bacteria and fungi that aren't present in either of the individual parts. The Ag-ZnO NCs from AV showed the best performance against E. coli, with an inhibition zone of up to 27 mm, compared to the other samples. The maximum absorbance peaks were observed at 431 nm and 410 nm for Ag NPs, at 374 nm and 377 nm for ZnO NPs and at 384 nm and 391 nm for Ag-ZnO NCs using AV leaf extract and hibiscus sabdariffa flower extract, respectively. Using field emission-scanning electron microscopes (FE-
... Show MoreA series of new aromatic polyesters have been synthesized by polycondensation of different aromatic diols (M1, M2 ,M3) with different dicarboxlic acids (4,4'azo di benzoic acid, malic acid and adipic acid) using dibutyltine dilaurate as catalyst . The preparation of thermally stable polyesters was successfully applied with good yields (60-85%). All polyesters (PE1-PE4) containing aliphatic methylene linkage, and azo group in the main chain. The resulted polymers are readily soluble in aprotic polar solvents , such as (pyridine , CHCl3 , CH2Cl2 , NaOH , H2SO4 , HNO3 , acetone , benzene , DMF , DMSO , THF) without need for heating. Thermal analysis of polyesters by Thermo Gravimetric Analysis (TGA) reveals that these aromatic polyesters po
... Show MoreNew complexes of the type [ML2(H2O)2] ,[FeL2(H2O)Cl] and [VOL2] were M=Co(II),Ni(II) and Cu(II) ,L=4-(2-methyl-4-oxoquinazoline-3(4H)-yl) benzoic acid were synthesized and characterized by element analysis, magnetic susceptibility ,molar conductance ,FT-IR and UV-visible. The studies indicate that the L acts as doubly monodentate bridge for metal ions and form mononuclear complexes. The complexes are found to be octahedral except V(IV) complex is square pyrimde shape . The structural geometries of compounds were also suggested in gas phase by theoretical treatments, using Hyper chem-6 program for the molecular mechanics and semi-empirical calculations, addition heat of formation(?Hf ?) and binding energy (?Eb)for the free ligan
... Show MoreThis study wass carried out to investigate the incedence of powdery mildew disease on ornamental plants (Nasturtium) Tropaeolum majus L. caused by Oidiopsis haplophylli in some nurseries of Baghdad area and in fields at college of Agriculture /University of Baghdad. This study was conducted in tow succesive seasons of 2011-2012 (April and May). The survey indicated that the Mildew disease existe in the following nurseries (Al-Adhamiya 97.5% ,Palestine street 93.8%, Zayouna 86.0%, and 100% in two fields at college of Agriculture. It has been found that the disease severity was developed in Agriculture college fields successively from 12-4-2011 to 20-5-2011 and from 12-4-2012 to 20-5-2012 (18.0–98.0 % and 22.7–96.0% )for the two sea
... Show MoreThe current study sheds light on the measurement and estimation of the radioactivity of radionuclides (238U, 226Ra, 232Th, and 40k) in natural waters of different regions of Nineveh Governorate in Iraq.15 samples were collected from different sources of natural waters, where gamma-ray spectroscopy was used using NaI)TI) sodium iodide detector to determine the concentration of radioactivity in the samples. According to the results, the radioactivity concentration in the tested water sample were ranged from 0.36 ± 0.04-1.57 ± 0.09with an average value of 0.69 ± 0.06 Bq/l for 238U, and 2.9 ± 0.02-0.88 ± 0.03 with an average value of 0.65 ± 0.03 Bq/l for 226Ra Bq/l
... Show MoreObjectives: The study aims: (1) To determine effectiveness of instructional health education vascular access on hemodialysis patients' knowledge, (2) To find out the association between effects of instructional health education vascular access and demographic characteristics of (age, gender and educational level). Methodology: A quasi experimental study –control study design is carried out at AL-Hussein Teaching Hospital in AL-Nasiriyah City, from 3November,2015 to 2 June, 2016. A non-probability (Purposive sample) of (80) patients with vascular access devices on maintenance hemodialysis patients divided int
Human Herpes Virus-8 (HHV-8) is a sexually transmitted viral infection that can infect the prostate epithelium in immunocompromised adults. Recently, HHV-8 was related to the development and progression of several human malignancies like prostatic adenocarcinoma. This retrospective research was designed to analyze the distribution and possible impact of HHV-8 infection on prostatic adenocarcinogenesis. A total number of one hundred formalin-fixed prostatic tissues were enrolled in this research; forty Prostate Adenocarcinoma (PAC) biopsies, forty biopsies from Benign Prostatic Hyperplasia (BPH), and twenty Apparently Normal Prostatic Tissues (ANPT) as a control group. Detection of HHV -8 DNA was achieved by a highly-sensitive variant of
... Show MoreHypertension is a major health problem throughout the world because of its high prevalence and its association with increased risk of cardiovascular diseases. It is defined as systolic blood pressure ≥ 140 mmHg and/or diastolic blood pressure ≥ 90 mmHg. The aim of this study was to compare the efficacy, safety and cardiovascular disease risk lowering ability, of three antihypertensive drug regimens.
A retrospective study was carried out on 66 hypertensive patients, divided in to three groups based on their antihypertensive drug regimens (ACE inhibitors, β-blockers treated and combination antihypertensive therapy, the combination therapy consist of two or more of the following antihypertensive drugs ACE inhibitor di
... Show MoreIn the present investigation, new polymers of Poly [ N - acryl - N - sulfonic acid - N\ yL - 2 - substitute - 4 - oxo - thiazolidine ] Maleic and Succinic diimide were synthesized by seven steps; first step includes esterification of different cyclic anhydride, using one mole of absolute methanol in the presence few drops of conc. H2SO4, yielded mono group ester [I] , which was reaction with thionyl chloride to give ester acid chloride [II] .Then reacted with sulfanilic acid to product compound [III] , which was condensation with hydrazine hydrate to give acid hydrazide [IV] . The new different Schiff bases [V-VIII] were synthesized by reaction of acid hydraizide with different (aliphatic and aromatic) aldehyedes and ketones in the prese
... Show MoreRheumatoid arthritis is a chronic inflammatory autoimmune disease its etiology is unknown . The classical autoimmune diseases, have adaptive immune genetic associations with autoantibodies and major histocompatibility complex(MHC) class II such as rheumatoid arthritis (RA), diabetes mellitus type two (DM II). Serum of99 males suffering from RA without DMII as group (G1), 45 males suffering from RA with DM II as group (G2) and 40 healthy males as group (G3) were enrolled in this study to estimation of alkaline phosphates (ALP),C-reactive protein(CRP) and Pentraxin-3(PTX). Results showed a highly significant increase in PTX3 levels in G1 and G2 compared to G3 and a significant decrease in G1comparing to G2. Results also revealed a si
... Show More