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: Symptoms related to the upper gastro-intestinal tract are very common. Attribution of these symptoms to upper G. I. T.diseases are usually done on clinical bases, which could be confirmed by Esophago Gastro Duodenoscopy (EGD). The use of such tools might increase the diagnosis accuracy for such complaints. The indications for upper G I endoscopy might decrease the negative results of endoscopies.Objective: To follow strict indications for Esophago Gastro Duodenoscopy in order to decrease the negative endoscopy results. Methods: One thousand eight hundred and ninety cases were subjected to EGD from Feb. 1999 to Feb 2009 at Alkindy Teaching Hospital and Abd-Al-Majeed private hospital in Baghdad, Iraq. A special endoscopy unit f
... Show MoreDesalination is a process where fresh water produces from high salinity solutions, many ways used for this purpose and one of the most important processes is membrane distillation (MD). Direct contact membrane distillation (DCMD) can be considered as the most prominent type from MD types according to ease of design and modus operandi. This work studies the efficiency of using DCMD operation for desalination brine with different concentration (1.75, 3.5, 5 wt. % NaCl). Frame and plate cell was used with flat sheet PTFE hydrophobic type membrane. The study proves that MD is an effective process for desalination brines with feed temperature less than 60˚C especially for feed with low TDS. 37˚C, 47˚C, and 57˚C was feed t
... Show MoreNd:YAG laser pulses of 9 nanosecond pulse duration and operating wavelength at 1.06 μm, were utilized to drill high thermal conductivity and high reflectivity aluminum and copper foils. The results showed a dependence of drilled holes characteristics on laser power density and the number of laser pulses used. Drilled depth of 74 ϻm was obtained in aluminum at 11.036×108 W/cm2 of laser power density. Due to its higher melting point, copper required higher laser power density and/or larger number of laser pulses to melt, and a maximum depth of 25 μm was reached at 13.46×108 W/cm2 using single laser pulse.
Background: Acute cholecystitis is common surgical
problem, which was treated previously by conservative
treatment .Later early open has been introduced as an
alternative to interval for treatment of acute cholecystitis.
Early open was found to be a safe, successful with
comparable postoperative complication rate. With the
advent of laparoscopy laparoscopic have been used for
chronic cholecystitis and became the first line of
treatment. New reports have shown that laparoscopic can
be used as an alternative to open for surgical treatment of
acute cholecystitis.
Objectives: to compare the success, safety of early
laparoscopic versus early open as a primary treatment of
acute cholecystitis.
Methods:
In this research a new system identification algorithm is presented for obtaining an optimal set of mathematical models for system with perturbed coefficients, then this algorithm is applied practically by an “On Line System Identification Circuit”, based on real time speed response data of a permanent magnet DC motor. Such set of mathematical models represents the physical plant against all variation which may exist in its parameters, and forms a strong mathematical foundation for stability and performance analysis in control theory problems.
In this paper, we characterize the percolation condition for a continuum secondary cognitive radio network under the SINR model. We show that the well-established condition for continuum percolation does not hold true in the SINR regime. Thus, we find the condition under which a cognitive radio network percolates. We argue that due to the SINR requirements of the secondaries along with the interference tolerance of the primaries, not all the deployed secondary nodes necessarily contribute towards the percolation process- even though they might participate in the communication process. We model the invisibility of such nodes using the concept of Poisson thinning, both in the presence and absence of primaries. Invisibility occurs due to nodes
... Show MoreThe current research dealt with the rapid development of industrial product design in recent times, and this development in the field of design led to the emergence of modern trends in many terms and theories to direct greater interest in the cognitive foundations of design and its relationship with the components of other natural sciences, and despite the impressive technological development, nature remains With its content of formative values and structural dimensions, it is the first source of inspiration and the source of all modern mathematical sciences and theories, as God made them tend towards organization to continue to provide us with endless inspiration. Hence, the fractional one, which is an important part of dedicating the d
... Show MoreNowadays, due to our everyday stress and current stressful lifestyle, the loss of items appears a frequent issue and may be very inconvenient. In this regard, until the IoT becomes part of everyday life, we can use the software as an efficient tool to assist a person's searching, verifying, and finding lost belongings. This paper presents an Android-based application that we proposed and implemented to help users find lost items. Utilizing this software will enable the subscriber to record his request to the relevant authority. In addition, a special section offers to insert a contact telephone number or email to communicate between the person who found the item and the person who lost it. During testing, among other services, the p
... Show MoreA niger, a fungus which doesn't have high ability to production lipid, this fungus has been select to investigate the non oleaginicity. In this search, there are explorations about: i) growth profile ii) enzymes profile iii) isoforms. Growth profile shows that this fungus doesn't have ability to accumulate lipid more than 6% while bio mass are around 10g/l in spite of the presence of glucose in the media till the end of cultivation time and excision of nitrogen within 24 hrs. In enzyme study, we investigate all lipogenic enzymes Malic enzyme (ME), Fatty acid synthase (FAS), ATP: Citrate lays (ACL), NAD+ isocitrate dehydrogenase (NAD+ICDH), Glucose-6-phosphate (G6PD), and 6-phosphogluconate dehydrogenase (6PGD), all these enzymes show, ac
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