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.
Due to the development that occurs in the technologies of information system many techniques was introduced and played important role in the connection between machines and peoples through internet, also it used to control and monitor of machines, these technologies called cloud computing and Internet of Things. With the replacement of computing resources with manufacturing resources cloud computing named converted into cloud manufacturing.
In this research cloud computing was used in the field of manufacturing to automate the process of selecting G-Code that Computer Numerical Control machine work it, this process was applied by the using of this machine with Radio Frequency Identification and a AWS Cloud services and some of py
... Show MoreThis study focused on a fundamental issue which was represented by ability of Iraqi central bank in facing the difficulty of determining the optimal ratio of liquidity in the Iraqi banks in terms of the balancing between its obligations to the depositors and borrowers, and liquidate their funds on one hand and the risks on the other hand.the search aimed for achieving the goals which represented by identifying the possibility of Iraqi banks to apply the regulations rules and instructions issued by central bank of Iraq in determining ratio of liquidity and its appropriate with Iraqi banks action to implement a reasonable profit to&
... Show MoreAcquires this research importance of addressing the subject (environmental problems) with
age group task, a category that children pre-school, and also reflected the importance of
research, because the (environmental problems) constitute a major threat to the continuation
of human life, particularly the children, so the environment is Bmchkladtha within
kindergarten programs represent the basis of a hub of learning where the axis, where the
kindergarten took into account included in the programs in order to help the development of
environmental awareness among children and get them used to the sound practices and
behaviors since childhood .
The research also detected problem-solving skills creative with kids Riyad
BACKGROUND: Sickle cell nephropathy, a heterogeneous group of renal abnormalities resulting from complex interactions of sickle cell disease (SCD)-related factors and non-SCD phenotype characteristics, is associated with an increased risk for morbidity and mortality. AIMS: The aims of this study were to determine the frequency of microalbuminuria (MA) among pediatric patients with SCD and to determine risk factors for MA among those patients. SUBJECTS AND METHODS: A case–control study was carried out on 120 patients with SCD, 2–18 years old, registered at Basrah Center for Hereditary Blood Diseases, and 132 age-and sex-matched healthy children were included as a control group. Investigations included complete blood panel, blood urea, se
... Show MoreThe aim of the present research is to study different protein fractions in sera of children and adolescent with β –thalassemia major and minor and to compare the results with that of healthy control.One hundred fifty children and adolescents were enrolled in this study,including 50 patients with β- thalassemia major , 50 patients with β- thalassemia minor as pathological control group and another apparently 50 healthy individuals as a control group. The age of all studied groups ranged from (4-18)years.Total protein, albumin and immunoglobulins were estimated in sera of all subjects. A Significant decrease was found in the total protein and albumin&nb
... Show MoreThree N-(hydroxylphenyl) dimethylmaleimides were directly prepared in good yields (81-86)% from the reaction of dimethylmaleic anhydride with amino phenols. The prepared imides were esterified to the corresponding benzoates, methacrylates and cinnamates via their reaction with different acid chlorides in the presence of triethylamine. The prepared esters were tested as plasticizers for PVC via preparing of thirty six samples of PVC with the prepared esters in certain weight ratio followed by recording their softening points. Comparison the results with the universal plasticizers for PVC (DOP) and (DBP) indicated that the prepared esters in general have high plasticizing efficiency.
The high carbon dioxide emission levels due to the increased consumption of fossil fuels has led to various environmental problems. Efficient strategies for the capture and storage of greenhouse gases, such as carbon dioxide are crucial in reducing their concentrations in the environment. Considering this, herein, three novel heteroatom-doped porous-organic polymers (POPs) containing phosphate units were synthesized in high yields from the coupling reactions of phosphate esters and 1,4-diaminobenzene (three mole equivalents) in boiling ethanol using a simple, efficient, and general procedure. The structures and physicochemical properties of the synthesized POPs were established using various techniques. Field emission scanning elect
... Show MoreAbstract Introduction: MMP3 plays a crucial role in the process of bone erosion in the pathomechanism of rheumatoid arthritis (RA). It acts by removing the outer osteoid layer, which allows the osteoclasts to tightly connect and carry out the subsequent damage to the underlying bone. MMP3 can trigger the production of other MMPs like MMP-1, MMP-7, and MMP-9, it plays a pivotal role in the remodeling of connective tissues. Aim of the study: to assess the influence of MMP-3 serum levels and single-nucleotide polymorphisms of rs679620 in the rheumatoid arthritis patients' group in comparison to the control group. Subjects: eighty eight samples, 45 rheumatoid arthritis patients after being referred by their treating physician for regular RA
... Show MoreThe elections of the Council of Representatives in Iraq are one of the manifestations of political participation, which makes it attracts the attention of researchers. Where Iraq witnessed in 2005 important political events in the Iraqi arena, a pluralist parliamentary elections or elections in Iraq by direct free election on January 30, the first almost half a century ago. On November 15 of the same year, Iraq adopted a permanent constitution for the country through a popular referendum.