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.
Three 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 deliberative system of communicative studies is exposed to cultural openness, according to modern cultural studies, to establish a verbal language system that achieves reciprocal and cross-cultural relations. This research has examined the concept of deliberative approach in the contemporary Iraqi theater text by studying the deliberative system of text and its interrelationship with The stage of the verbal embodiment of the dramatic event, because the dramatic text achieves in its construction of racism, a deliberative approach between the verbal event and the language, and what later leads to the completion of the dramatic action. The research included four chapters. The first chapter was the systematic framework of the research, T
... Show MoreABSTRACT Background: chronic periodontitis and rheumatoid arthritis are widely prevalent diseases and are characterized by tissue destruction due to chronic inflammation. Recently, there is growing evidence that the two diseases share many pathological features the aims of the study To determine the periodontal health status in patient have chronic periodontitis with rheumatoid arthritis and compare it with those having chronic periodontitis without Rheumatoid arthritis and determine the serum levels of interleukin -17(IL-17), tumor necrosis factor- alpha (TNF-α ) in both groups and compare with the control group (subject samples neither have periodontitis nor arthritis ) and correlate these immunological markers with the periodontal p
... Show MoreThe present study deals with the effect of self -observation on EFL University students` achievement in conversation classes. The process of self-observation helps the teacher to understand one’s own actions and the reactions in the process of teaching. The sample of this study is EFL students in the third stage at the Department of English Language, morning studies, College of Education /Ibn-Rushd .The sample of the study consists of (84) students distributed on experimental group(A) includes (42) students, and (42) students as control group(B). In order to achieve the aim of the study ,and to gain a closer idea about the impact of reflective teaching technique(self-observation) on the students achievement in conversation classes, a chec
... Show Moreunacceptable social behaviors, particularly withdrawal behavior that appears in children with autism represent a major problem hindering the process of communication with those around them and therefore the process of mergence with them be difficult.
The withdrawal causes a real affect deficit for children with autism limits the possibility of development of their intellectual and mental growth due to their solitude and the weakness of their focus in the acquisition of pedagogical skills and lack the necessary social skills to maintain the relations of friendship and enjoyment of them.
withdrawal children fail to participate
... Show MorePlantation of humic acid nanoparticles on the inert sand through simple impregnation to obtain the permeable reactive barrier (PRB) for treating of groundwater contaminated with copper and cadmium ions. The humic acid was extracted from sewage sludge which is byproduct of the wastewater treatment plant; so, this considers an application of sustainable development. Batch tests signified that the coated sand by humic acid (CSHA) had removal efficiencies exceeded 98 % at contact time, sorbent dosage, and initial pH of 1 h, 0.25 g/50 mL and 7, respectively for 10 mg/L initial concentration and 200 rpm agitation speed. Results proved that physicosorption was the predominant mechanism for metals-CSHA interaction because the sorption data followed
... Show MoreBackground: Mycoplasma pneumoniae (M. pneumoniae) is an important respiratory bacterial pathogen, especially among children. It causes acute upper and lower respiratory infections.Objective: This study was aimed to measure anti- M. pneumoniae antibodies among hospitalized children who were admitted to hospital diagnosed with acute respiratory tract infections.Method: Automated ELISA technique was performed to detect anti- M. pneumoniae antibodies (IgM and IgG antibodies) in serum from 108 children less than 5 years old. The children were admitted to the Pediatric Teaching Hospital in Suleimani city/Kurdistan Region/Iraq because of acute respiratory tract infections. A questionnaire was designed to collect demographic and clinical data fr
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