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.
Fine aggregates used for concrete works in Sulaymaniyah city frequently fail to meet the standard requirements for gradation and fineness modulus in cement concrete. This paper aims to critically evaluate gradation, fineness modulus, and clay contents of various natural sands produced and used for concrete work in the region. Sixteen field sand samples were collected from various sites in Darbandikhan (5 samples), Qalat Dizah (5 samples), Koysinjaq (5 samples), and Piramagroon (1 sample) confirming to ASTM D75. The field samples were parted into test specimens based on ASTM C702. Then, sieve analysis was carried out on the oven-dry test specimens in compliance with ASTM C136. The test results of fine aggregates wer
... Show MoreIn this research work, a simulator with time-domain visualizers and configurable parameters using a continuous time simulation approach with Matlab R2019a is presented for modeling and investigating the performance of optical fiber and free-space quantum channels as a part of a generic quantum key distribution system simulator. The modeled optical fiber quantum channel is characterized with a maximum allowable distance of 150 km with 0.2 dB/km at =1550nm. While, at =900nm and =830nm the attenuation values are 2 dB/km and 3 dB/km respectively. The modeled free space quantum channel is characterized at 0.1 dB/km at =860 nm with maximum allowable distance of 150 km also. The simulator was investigated in terms of the execution of the BB84 prot
... Show MoreIn this paper, some estimators of the unknown shape parameter and reliability function of Basic Gompertz distribution (BGD) have been obtained, such as MLE, UMVUE, and MINMSE, in addition to estimating Bayesian estimators under Scale invariant squared error loss function assuming informative prior represented by Gamma distribution and non-informative prior by using Jefferys prior. Using Monte Carlo simulation method, these estimators of the shape parameter and R(t), have been compared based on mean squared errors and integrated mean squared, respectively
Abstract :H.pylori is an important cause of gastric duodenal disease, including gastric ulcers, Mucosa-associated lymphoid tissue (MALT), and gastric carcinoma. biosensors are becoming the most extensively studied discipline because the easy, rapid, low-cost, highly sensitive, and highly selective biosensors contribute to advances in next-generation medicines such as individualized medicine and ultrasensitive point-of-care detection of markers for diseases. Five of ten patients diagnosed with H.pylori ranging in age from 15–85 participated in this research. who [gastritis, duodenitis, duodenal ulcer (DU), and peptic ulcer (PU)] Suspected H.pylori colonies w
... Show MoreAs a well-known oral and intravenous antifungal, voriconazole (VRN) has an extensive history of usage in the medical field. Solid lipid nanoparticles (SLNs) have been produced to treat ocular fungal keratitis in the eye. A 32Box-behnken design was used to produce a variety of new formulas for hot-melt extrusion. The SLNs were evaluated by entrapment efficiency (EE percent), particle size (PS), polydispersity index (PDI), and zeta potential (ZP). A series of in-vitro and in-vivo studies were carried out on the new formula. The produced vesicles’ EE, PS, PDI, and ZP values were all good. SLNs eye drops were numerically adjusted to include carbopol, a stabilizer, lipids, and a surfactant, among other substances. ZP of -36.5 ± 0.20 m
... Show MoreThe subject of dumping is considering today one of the subjects in which form an obstruction arise in front of the cycle of growth for some countries , such as the study of dumping is capturing a large attention by the competent because either a big role and effect in growing the economies of nations then the subject of dumping became a field turn around its sides many measures and laws … and may be done resorting to by many states of the world to anti-dumping as approach of determent weapon delimit the impact of dumping and gives the national agriculture sector the opportunity for rising and growing so this section of international economics is capturing a special importance and represent in same time an important
... Show MoreObjective : The study was carried out to construct an initial assessment documentation tool for nursing
recording system in Coronary Care Unit.
Methodology : A descriptive, purposive sample of (65) nurses was selected from CCU of main
teaching hospitals (Al Karama, Al Kindy, Al Kadimia, Al Yarmmok, Baghdad teaching hospital, Ibn
Al Naffis hospital) and Ibn-Al betar hospital in Baghdad city from the 15th of April 2004 to the 15th of
April 2006.
The instrument was constructed and comprised of two sections: section one included the
nurses' demographic characteristic; section two was the initial assessment documentation tool that
contained (2) parts including: General information form and the initial assessment form.
PMMA (Poly methyl methacrylate) is considered one of the most commonly used materials in denture base fabrication due to its ideal properties. Although, a major problem with this resin is the frequent fractures due to heavy chewing forces which lead to early crack and fracture in clinical use. The addition of nanoparticles as filler performed in this study to enhance its selected mechanical properties. The Nano-additive effect investigated in normal circumstances and under a different temperature during water exposure. First, tests applied on the prepared samples at room temperature and then after exposure to water bath at (20, 40, 60) C° respectively. SEM, PSD, EDX were utilized for samples evaluation in this study. Flexural
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