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.
Unconfined Compressive Strength is considered the most important parameter of rock strength properties affecting the rock failure criteria. Various research have developed rock strength for specific lithology to estimate high-accuracy value without a core. Previous analyses did not account for the formation's numerous lithologies and interbedded layers. The main aim of the present study is to select the suitable correlation to predict the UCS for hole depth of formation without separating the lithology. Furthermore, the second aim is to detect an adequate input parameter among set wireline to determine the UCS by using data of three wells along ten formations (Tanuma, Khasib, Mishrif, Rumaila, Ahmady, Maudud, Nahr Um
... Show MoreThe official spokesperson considered responsible for the transmission of information and communication messages that convey the government’s view on the various political and economic issues that affect the public opinion towards the performance achieved by the various governmental institutions and for the purpose of communicating with the media in a positive and effective manner, a number of institutions in the world in general and in Iraq in particular have nominated a spokesman to coordinate the efforts and the dissemination of news and the preparation of press conferences to contribute to the achievement of a positive and effective level between the government and the media, so this study has tackled to deal with the credibility of
... Show MoreObjectives: To assess the psychological adjustment of middle school students and to identify the relationship between differences in demographic characteristics and psychological adjustment.
Methodology: A descriptive correlation design was used. The study utilized a simple random sample to select (381) students using self-report to select students who will be recruited into the study for the period from November 26th, 2021 to April 1st, 2022. A scale approved in the study consisting of 40 items was chosen. It measures four trends which are personal, social, familial, and emotional adjustment. The data were analyzed by applying the descriptive statistical dat
... Show MoreBackground: Monocyte chemotactic protein-1 (MCP-1) is a chemokine expressed by inflammatory and endothelial cells. It has a crucial role in initiating, regulating, and mobilizing monocytes to active sites of periodontal inflammation. Its expression is also elevated in response to pro-inflammatory stimuli and tissue injury, both of which are linked to atherosclerotic lesions. Aim of the study: To determine the serum level of MCP-1 in patients with periodontitis and atherosclerotic cardiovascular disease in comparison to healthy control and evaluate the biomarker's correlations with periodontal parameters. methods: This study enrolled 88 subjects, both males and females, ranging in age from 36-66 years old, and divided into four groups: 1<
... Show MoreBackground: Systemic sclerosis (SSc) is a chronic autoimmune illness, which is consider by three main features: Sclerotic changes in the skin and internal organs, Vasculopathy of small blood vessels, Particular autoantibodies (1). The most important autoantibodies appeared significantly in SSc patients are anti-topoisomerase I autoantibody (Scl-70), anti-centromere autoantibody (ACA), and anti-RNA polymerase III autoantibody (RNAP3) (2). Anti-centromere antibodies (ACA) are infrequent in rheumatic conditions and in healthy persons but occur commonly in limited systemic sclerosis (CREST syndrome), and rarely appeared in the diffuse form of systemic sclerosis (3). Anti-Ro/SSA and antiLa/SSB, antibodies directed against Ro/La ribonucleoprot
... Show MoreBackground: The iron deficiency anemia along with hyperphosphatemia are the main complications of dialysis patients. Traditional iron supplement has been failed to correct iron deficiency anemia, therefore, the current study aimed to investigate the efficacy and tolerability of new phosphate binder, ferric citrate, in a sample of Iraqi patients with end stage renal disease on maintenance hemodialysis. Method: Prospective, randomized, open label, active controlled trial was conducted in one center for dialysis in Babylon governance. Patients were randomized to receive ferric citrate with dose of 6 g/d and calcium carbonate with dose of 3 g/d for eight weeks. Hemoglobin concentration, mean corpuscular hemoglobin concentration and count o
... Show MoreBackground: Chief complaint of patients attending dental clinic represents the first step towards treatment plan. However, most of patients are not aware but the extent and severity of periodontal disease, which could be also, misdiagnose by the dentist. Aim of the study: To investigate whether reported chief complaint(s) are consistent with oral hygiene status Materials and methods: Records of 1102 patients, attending periodontics clinics in the college of dentistry/ university of Baghdad, were used to determine ten most commonly reported chief complaints. Sample of patients was further subdivided according to gender and age. In addition, plaque and gingival index were recorded to determine oral hygiene status. Results: Patients mostly
... Show More Despite the availability of information technology banking features and benefits of the banking sector, they involve many risks and challenges and put in the face of the administrative authorities and regulatory institutions in the banking system, organizational matters and control sensitive and bear direct responsibility for conducting independent assessments of their regulatory and information and determine the degree of its durability and its ability to confront problems imposed by the technical challenges and technological .
And the success of the administrative authorities and regulatory institutions in achieving its objectives in the management of risks and threats oversight resulting from the act
In this research the specific activity of natural radionuclides 226Ra, 232Th and 40K were determined by sodium iodide enhanced by thallium NaI(TI) detector and assessed the annual effective dose in Dielac 1 and 2 and Nactalia 1 and 2 for children of less than 1 year which are available in Baghdad markets. The specific activity of 40K has the greater value in all the types which is in the range of allowed levels globally that suggested by UNSCEAR. The mean value of annual effective doses were 2.92, 4.005 and 1.6325 mSv/y for 226Ra, 232Th and 40K respectively.