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.
This paper deals with a Twin Rotor Aerodynamic System (TRAS). It is a Multi-Input Multi-Output (MIMO) system with high crosscoupling between its two channels. It proposes a hybrid design procedure that combines frequency response and root locus approaches. The proposed controller is designated as PID-Lead Compensator (PIDLC); the PID controller was designed in previous work using frequency response design specifications, while the lead compensator is proposed in this paper and is designed using the root locus method. A general explicit formula for angle computations in any of the four quadrants is also given. The lead compensator is designed by shifting the dominant closed-loop poles slightly to the left in the s-plane. This has the effect
... Show MoreHerbal remedies are used in induction of lactation. In view of that .Borage Officinalis was employed to ensure an abundant milk supply or rectify milk insufficiency .However, this remedy has not been scientifically tested .The animals were treated with the aqueous extract of Borage Officinalis flowers at a daily concentration of 100 mg / kg body weight /ml for each rat through Oro-gastric tube for 14 days. Animals were subdivided into subgroups according to their physiological status. Mammary glands of these animals were processed for histological, histochemical and immunohistochemical studies. Parameters indicated that the aqueous extract of Borage Officinalis flowers induced lactogenesis in the mammmary glands of virgin and pregnant rats
... Show MoreThe hydrodynamics behavior of gas - solid fluidized beds is complex and it should be analyzed and understood due to its importance in the design and operating of the units. The effect of column inside diameter and static bed height on the minimum fluidization velocity, minimum bubbling velocity, fluidization index, minimum slugging velocity and slug index have been studied experimentally and theoretically for three cylindrical columns of 0.0762, 0.15 and 0.18 m inside diameters and 0.05, 0.07 and 0.09 m static bed heights .The experimental results showed that the minimum fluidization and bubbling velocities had a direct relation with column diameter and static bed height .The minimum slugging velocity had an
... Show MoreUnlike welding, soldering does not involve melting the work pieces. Soldering is a process in which two or more items are joined together by melting and putting a filler metal (solder) into the joint. Failure in the solder joint may make the system components lose their functions. Electrical wiring and electronic components are joined to devices and printed circuit boards using soldering. Soldering and brazing are both used in the assembly of musical instruments. Lead-tin alloy solder employed in the current investigation which has a diameter of 4 mm and a density of 11.0103 kg/m3 with continuous heat flux heating from the domain's left side and complete insulation on the other side. The melting of PCM was simulated using the ANSYS
... Show MoreObjectives: The study aimed to assess the level of pediatric nurses' knowledge toward children with Guillain-Barre
syndrome (GBS) and to find out the relationships between nurses' knowledge and their demographic data.
Methodology: A purposive “non probability” sample of (30) nurses was selected from medical neurological wards and
Respiratory Care Units of Children Welfare Pediatric Teaching Hospital, Child’s Center Pediatric Teaching Hospital,
AL-Kadhemia Teaching Hospital, Baghdad Teaching Hospital, and Neurological Science Hospital which has started
from March 5
th 2009 to April 30th 2009. The study instrument consists of two parts. The first part is concerned with
nurses' demographical characteristic and the
Background: Nutritional Rickets is a condition produced by an absence of Vitamin D, calcium or phosphate. It clues to relaxing and fading of the bones. Dental expression of children with rickets contains enamel hypoplasia and delayed tooth eruption. This study was conducted in order to assess caries experience (dmfs) and enamel defects among study and control groups, and to evaluate and compare the levels of selected salivary biomarkers between children with nutritional rickets and apparently healthy children. Material and methods: Assessment of caries according to WHO in 1987, and assessment of enamel defects according to enamel defect index EDI of WHO in 1997. In addition a stimulated saliva samples were collected according to Palone e
... Show MoreA total of 96 stool samples were collected from children with bloody diarrhea from two hospitals in Baghdad. All samples were surveyed and examined for the presence of the Escherichia coli O157:H7 and differentiate it from other Non -Sorbitol Fermenting Escherichia coli (NSF E. coli). The Bacterial isolates were identifed by using morphological diagnostic methods, Samples were cultured on liquid enrichment medium, incubated at 37C? for 24 hrs, and then cultured on Cefixime Tellurite -Sorbitol MacConkey Agar (CT- SMAC). 32 non-sorbitol fermenting bacterial isolates were obtained of which 11 were identified as Escherichia coli by using traditional biochemical tests and API20E diagnostic system without differentiation between
... Show MoreThe research discusses one of the most critical issues of corporate finance which is related to asset utilization efficiency. Researchers used internal growth rate as independent variable (Proxy of asset utilization efficiency) and sustainable growth rate-dependent variable (proxy of stockholders wealth). According to these two variables, researchers formulate major hypotheses (There is no significant effect of internal growth rate on sustainable growth rate), as well as two sub-hypotheses, examine the components of major variables. Sample of Iraqi industrial companies which listed in the Iraqi stock exchange selected to test and examine main hypotheses. Result of simple and multiple regressions explain there is a significant effect of i
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