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.
Convolvulus arvensis is a species of bindweed that is rhizomatous and is in the morning glory family (Convolvulaceae) native to Europe and Asia. The plant is naturally grown in Iraq. The plant was reported to be used in traditional medicine from as early as 1730s.
The Aerial parts of Convolvulus arvensis were macerated in 80% ethanol for 6 days. The concentrated extract was partitioned with n-hexane, chloroform, ethyl acetate- and n-butanol successively. The n-hexane and ethyl acetate, fractions were examined for the presence of phytochemicals by thin l
... Show Moreيحتل موضوع الاستهلاك اهمية كبيرة في الدراسات الاقتصادية في حالتي السلم والحرب وذلك لارتباط هذا الموضوع بالانسان والمجتمع ولكونه احد مؤشرات مستوى الرفاهية الاقتصادية والاجتماعية وتزداد اهمية ضبط حركة هذا المتغير السلوكي والكمي في زمن الحرب اكثر مما هو عليه في حالة السلم، في هذا البحث تم استخدام بيانات احصائية عن الانفاق الاستهلاكي الخاص ونصيب الفرد من الدخل القومي اضافة الى الرقم القياسي لاسعار المس
... Show MoreThis research is a result of other studies made about the iraqi public and its relationship with different states institutions, until recently, such studies were almost non-existent. The main characteristic that distinguishes scientific research is that it involves a specific problem that needs to be studied and analysed from multiple aspects. What is meant by identifying the problem, is to limit the topic to what the researcher wants to deal with, rather than what the title suggests as topics which the researcher doesn’t want to deal with. The problem of this research is the absence of thoughtful and planned scientific programs to build a positive mental image of the institutions of the modern state in general and the House of Represe
... 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 MoreA novel series of liquid crystalline compounds containing 2,4-thiazolidinedione units with varying terminal alkyl chain lengths was successfully synthesized and characterized. The chemical structures of the synthesized compounds were confirmed by FT-IR, ¹H-NMR, and mass spectrometry. The mesomorphic behavior was investigated using polarized optical microscopy (POM) and differential scanning calorimetry (DSC). Compounds [V]₄, [V]₅, and [V]₆ exhibited enantiotropic nematic phases, while compound [V]₈ displayed a smectic A (SmA) phase. No liquid crystalline behavior was observed for compound [V]₃. The liquid crystalline properties were found to depend on the terminal-to-lateral chain length ratio, molecular geometry, and the nature
... Show MoreUnconfined 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 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
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