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 study, a 3 mm thickness 7075-T6 aluminium alloy sheet was used in the friction stir welding process. Using the design of experiment to reduce the number of experiments and to obtain the optimum friction stir welding parameters by utilizing Taguchi technique based on the ultimate tensile test results. Orthogonal array of L9 (33) was used based on three numbers of the parameters and three levels for each parameter, where shoulder-workpiece interference depth (0.20, 0.25, and 0.3) mm, pin geometry (cylindrical thread flat end, cylindrical thread with 3 flat round end, cylindrical thread round end), and thread pitch (0.8, 1, and 1.2) mm) this technique executed by Minitab 17 software. The results showed th
... Show MoreThis study aims at creating an analogy between Quran text Contents as meanings that have representations as visional shapes within ornamental figures in Islamic architecture. The theoretical framework of the study deals with the concept of semantics and its parts, artistic contents of Quran texts, and ornamental figures in Islamic architecture. The study procedures included a population of (69) figures, (5) of them were chosen deliberately for analysis in accordance with a form that had been presented to a number of experts to ensure its validity. The study reached a number of conclusions, the most significant among them are: adopting natural denotation of direct reference in order to link the ornamental figure to the source it was taken
... Show MoreThis prospective study investigates the prevalence of methicillin-resistant S.aureus (MRSA)
in burn unit of Al-Kindy Iraqi hospital, their susceptibility to antibiotics and bactericidal effect of near
infrared light from high powered 1064nm Nd: YAG laser and green light 532nm from SHG Nd: YAG laser
using various energy densities on these bacteria. Twenty four clinical isolates of S.aureus out of sixty
four examined patients with sever burn ulcers.MRSA was associated with 50% of S.aureus infections
.Results of antimicrobial susceptibility revealed that MRSA were multidrug resistant. After laser treatment
of non MRSA with Nd:YAG with wavelength of 1.064nm, 4mm beam diameter, energy density of
0.636 kh/cm2 and 180sec ex
This paper is interested in certain subclasses of univalent and bi-univalent functions concerning to shell- like curves connected with k-Fibonacci numbers involving modified Sigmoid activation function θ(t)=2/(1+e^(-t) ) ,t ≥0 in unit disk |z|<1 . For estimating of the initial coefficients |c_2 | , |c_3 |, Fekete-Szego ̈ inequality and the second Hankel determinant have been investigated for the functions in our classes.
The icon and the symbol represent the constituents of the communication process, through their intellectual and philosophical concepts that have been addressed by the current research that it has touched upon their importance in conveying the design idea for the recipient and showing the specificity of each one of them in the communication process. The research problem has been limited by the following question: what are the communication roles that the symbol and the icon present for the user in designing the industrial product?
The research objective is to reveal the theoretical visualization that describes the icon and the symbol in the industrial product and its importance in the communication process through stating the of simil
Phenol is one of the worst-damaging organic pollutants, and it produces a variety of very poisonous organic intermediates, thus it is important to find efficient ways to eliminate it. One of the promising techniques is sonoelectrochemical processing. However, the type of electrodes, removal efficiency, and process cost are the biggest challenges. The main goal of the present study is to investigate the removal of phenol by a sonoelectrochemical process with different anodes, such as graphite, stainless steel, and titanium. The best anode performance was optimized by using the Taguchi approach with an L16 orthogonal array. the degradation of phenol sonoelectrochemically was investigated with three process parameters: current de
... Show MoreThe Aaliji Formation in wells (BH.52, BH.90, BH.138, and BH.188) in Bai Hassan Oil Field in Low Folded Zone northern Iraq has been studied to recognize the palaeoenvironment and sequence stratigraphic development. The formation is bounded unconformably with the underlain Shiranish Formation and the overlain Jaddala Formation. The microfacies analysis and the nature of accumulation of both planktonic and benthonic foraminifera indicate the two microfacies associations; where the first one represents deep shelf environment, which is responsible for the deposition of the Planktonic Foraminiferal Lime Wackestone Microfacies and Planktonic Foraminiferal Lime Packstone Microfacies, while the second association represents the deep-sea environme
... Show MoreIn Iraq, the domestic goat
Hypertension is identified as one of the most significant risk factors for cardiovascular diseases (CVDs). There is growing evidence showing that oxidative stress plays a major role in hypertension. Increased production of reactive oxygen species and decrease bioavailability of antioxidant have been demonstrated in experimental and human hypertension. The present study was directed to determine the beneficial effect of the antioxidant vitamin C in patients with essential hypertension treated with the calcium channel blocker (amlodipine) or with the angiotensin converting enzyme inhibitor (enalapril). Ninety six patients (50 females and 46 males) with essential hyp
... Show MoreObjective: To evaluate nurses' practices toward coronary artery bypass grafting (CABG) patients in the
intensive care units (ICU) and to find out the relationship with some vanables.
Methodology: A purposive sample of (50) staff nurses was selected out of three hospitals for cardiac surgery.
The study instmment consisted of two major sections was constructed. It is based on the review of literature.
First it is concerned with demographic data for nurses; and the second section is observationaL tool (checklist) is
composed of six parts in which there mere (78) items. Reliability and validity of the observational checklist were
determined through the analysis of a pilot study.
Data were collected through the use of the