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 new multidentate Schiff-base (E)-6,6′-((1E,1′E)-(ethane-1,2-diylbis(azan-1-yl-1-ylidene))bis(methan-1-yl-ylidene))bis(4-methyl-2-((E)(pyridine-2-ylmethylimino)methyl)phenol) H2L and its polymeric binuclear metal complexes with Cr(III), Mn(II), Fe(II), Co(II), Ni(II), Cu(II), Zn(II), Cd(II) and Hg(II) are reported. The reaction of 2,6-diformyl-4-methyl-phenol with ethylenediamine in mole ratios of 2:1 gave the precursor 3,3′-(1E,1′E)-(ethane-1,2-diylbis(azan-1-yl-1ylidene))bis(methan-1-yl-1-ylidene)bis(2-hydroxy-5-methylbenzaldehyde) W. Condensation of the precursor with 2-(amino-methyl)pyridine in mole ratios of 1:2 gave the new N6O2 multidentate Schiff-base ligand H2L. Upon complex formation, the ligand behaves as a dibasic oct
... Show MoreThis paper investigate a sensorless speed control of a separately excited dc motor fed from a buck type dc-dc converter. The control system is designed in digital technique by using a two dimension look-up table. The performance of the drive system was evaluated by digital simulation using Simulink toolbox of Matlab.
Background: Mental health is integrated into PHC as a strategy of WHO to fill the gap in mental health treatment. Part of this strategy needs a level of task shifting so that mental health care is provided by different level of PHC workers and not only specialists such as psychiatrists and psychologists.
Objectives: To assess the knowledge and attitudes of family doctors regarding the provision of psychological health in PHCC and if there is an association between the certificates of these family doctors and their Knowledge and attitudes to psychological health.
Subjects and Methods: A cross-sectional descriptive study with analytic elements was conducted in 8 famil
... Show MoreA spectrophotometric study of Fe(III) mixed ligand complex has been
performed involving 1,4 phenylenediamine (A) and anthran i lic acid (B) ligand at 25°C and aconstant ionic strength of µ= 0.05M NaC I04• The optimum pH was found to be pH=4.l. The format ion rat io of the new complex is determined to be 2:1:4 of Fe(III):(A):(B). The molar absorptivity was determined to be :::::: 0.5 x I 04• Stepwise spectrum change of the complex formation is recorded by continuous flow system. Keywords: Mixed ligand
... Show MoreZanthoxylum L. 1753 belongs to the family Rutaceae. The plant, with its forest green color and with its distinctive fragrance attracted the attention of the study, specially it was an exotic to the environment of Iraq. Zanthoxylum is an exotic plant produced to Iraq and planted in gardens as an ever-green aromatic plant named (BUKHUR HINDI), as it is known among sellers and farmers in Iraq, because of the aromatic smell that emanated from the leaves and resembles Indian incense. The plant adapted to environmental conditions and succeeded in growing and blooming. It was examined and diagnosed for the first time in Iraq as Zanthoxylum beechyanum K. Koch. (Chinese pepper tree). Morphological characteristics and anatomical characteristi
... Show MoreThe paper studies the description of the colloquial and popular vocabulary used by wholesales tradesmen in Varonech before the Bolshevik Revolution . The vocabulary were used in the personal letters written by the poet and sent to famous poets and critics like Belenisky, kryvesky and Malsheve. And some were sent to his father and his sister. Not only did these letter bear the personal taste, but they represented a literary product reflecting on the poet's impressions, views, ambitions, and opinion in arts, poetry, prose, and theatre. The researcher used about thirty instances of various levels of grammar, style and phonology, all representing colloquial vocabulary used commonly at the time of Alexi 
... Show MoreIn this paper we introduce several estimators for Binwidth of histogram estimators' .We use simulation technique to compare these estimators .In most cases, the results proved that the rule of thumb estimator is better than other estimators.
Cryptosporidiosis is mainly cause a persistent diarrhea in immune compromised patients, BALB/c mice have been suppressed by dexamethasone, tissue Th1, Th2 and Th17 cytokines concentrations in the ileum were significantly diminished in both infected and immunosuppressed mice. Level of IFN-g, TNF-a, IL-12, IL-6, IL-17A was increased in level, IL-4 didn’t increases, in both ileal and spleen tissue. Levels of above cytokines were examined in spleen in order to follow the proliferation of CD4+ T-cell during C. parvum infection.
The problem of research was to identify after the use of cost technology based on specifications in the validity of determining and measuring the costs of the implementation of contracting, by applying to al-Mansour General Construction Contracting Company as an appropriate alternative to the traditional costing system currently adopted, which is characterized by many shortcomings and weaknesses Which has been reflected in the validity and integrity of the calculations. To solve this problem, the research was based on the premise that: (The application of cost technology based on specifications will result in calculating the cost of the product according to the specification required by the customer, to meet his wishes properly and witho
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