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.
Background: Vascular tumors and malformations, comprising a broad category of lesions often referred to as vascular anomalies. Hemangioma, represents a variety of vascular lesions (both malformations and tumor), while lobular capillary hemangioma is a common vascular lesion of the skin and mucous membranes that occurs mainly in children and young adults. Lymphangiomas are malformations of the lymphatic system. At the level of light microscopy the small lymphatics vessels may be similar to capillaries and sometimes are only tentatively identified by the nature of their contents or by immunohistochemical staining procedure. This study aimed to assess the vascular and lymphatic vessels density in benign vascular lesions using CD34 and D2-40 im
... Show MoreThe research aims at considering the reality of cognitive bias and organizational inertia as determinants of strategic change in a sample of companies listed in Amman Stock Market. To achieve objectives of the research, a model consisting of two independent variables has been designed, namely:
(1) The cognitive bias resulting from (escalating commitment, analogy, previous assumptions, representative generalization, command and control, convergent thinking), and (2) Organizational inertia due to (Icarus discrepancy, power distribution, rooted organizational culture), and a dependent variable, strategic change in (leadership patterns, strategy, the organization per se).
From the model two main hypotheses were derived;
... Show MoreObjectives: To determine the effectiveness of post-abortion family planning counseling program on nurses and midwives' practices and to predict the variables which may effect on their practices Methodology: A quasi experimental study was conducted from 23th April 2017 to 14th March 2018 in three governorates in Middle Euphrates of Iraq: (Holy Karbala, Al - Najef Al Ashraf and Babylon) on nurses and midwives who work at maternity hospitals. Systematic random sampling was used to select 122 nurses and midwives, (60) of them for study group and (62) for control group. A checklist is an instrument that evaluate the practices which included 50 items. Validity of content was determined through reviewing it by (16) experts and reliability of to
... Show MoreComplexes of Cr+3, Ni+2 and Cu+2 with the ligand 2-(p- nitrobenzoyl) thiobenzimidazol) (L) were prepared and characterized by FT-IR ,UV-Visible, TG analysis ,Atomic absorption spectroscopy , Molar conductivity and magnetic moments measurements .The general formula has been suggested for all the prepared complexes [Cr(L)2Cl2]Cl.4H2O, [Ni(L)2(H2O)2]Cl2.2H2O and [Cu(L)2(H2O)2]Cl2.2C2H5OH to be an octahedral geometry. A theoretical treatment of these compounds in gas phase was done using HyperChem.8.7 program. Semi –empirical PM3 method was performed to evaluate the ∆H° ƒ, ∆ Eb and ∆ET for all compounds, also vibration frequencies, electronic spectra and electrostatic potential HOMO and LUMO energies for the ligand was estimated to de
... Show MoreConsequence of thermal and concentration convection on peristaltic pumping of hyperbolic tangent nanofluid in a non‐uniform channel and induced magnetic field is discussed in this article. The brief mathematical modeling, along with induced magnetic field, of hyperbolic tangent nanofluid is given. The governing equations are reduced to dimensionless form by using appropriate transformations. Exact solutions are calculated for temperature, nanoparticle volume fraction, and concentration. Numerical technique is manipulated to solve the highly non‐linear differential equations. The roll of different variables is graphically analyzed in terms of concentration, temperature, volume fraction of nanoparticles, axial induced magnetic fie
... Show MoreFocal adhesion kinase (FAK), ephrin receptor type A4 (EphA4), and adiponectin (ADPN) are important indicators in inflammation, tumor growth, migration, and angiogenesis in some cancers. The predictive impact of their concentrations in acute myeloid leukemia (AML) patients to be identified remains. The research sought to explore the effect of FAK, EphA4, and ADPN as prognostic biomarkers, and their influence on patient survival, and to look for any potential correlation between their levels with hematological parameters in AML patients.
An aromatic ester containing two azo groups namely p-nitro phenyl azo-β-naphthyl-(4'-azobenzoic acid)-4-benzoate was synthesized by esterfiaction of 4,4'-azo dibenzoic acid with p-nitro phenyl azo-β-naphthol. Synthesized ester was characterized by CHN-Elemental analysis, FTIR, 1H NMR and 13C NMR. A modified PVA polymer was obtained by grafting 10 g of PVA-polymer via partial esterification with (2, 3, 4 g) p-nitro phenyl azo-1-naphthyl-4-azobenzoic acid)-4-azo benzoate. Grafting PVA-polymer behaviours was studied, by physical measurements (solubility, swelling), thermal properties (DSC) and tensile.
Polymer metal complexes of poly ethylene glycol acetal and Ag (I), Cu (II), Ni (II), Mn (II), Co (III) and Hg (II) were prepared from the reaction of PEG with aldehyde derived fromErythro-ascorbic acid (pentulosono-ɣ-lactone-2, 3- enedianisoate). All these compounds were characterized by Thin Layer Chromatography (TLC) and FTIR spectra and aldehyde was also characterized by (U.V-Vis), 1HNMR,13CNMR, and mass spectra. It has been established that, the polymer and its metal complexes showedgood activities against four pathogenic bacteria (Escherichia coli , Klebsiellapneumonae,Staphylococcusaureus, Staphylococcus Albus) and two fungal (Aspergillus Niger,Yeast). The polymer metal complexes showed higher activity than the free polymer.Theorder
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