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.
Chronic kidney disease (CKD) is described as an abnormalities of renal function, existing for a long period of time. By reason of the early grades of Chronic kidney disease can be experiences no symptoms, its premature identification is strenuous. initial stage CRD can cause various complications, such as anemia, matabolyic disorders of bone mineral. The study was done to assess the effect the chronic renal disease stage on the Osteoprotegerin, 1,25 dihydroxyvitamin D, Obestatin levels and some biochemical parameters in patients not undertaken dialysis therapy. In this case-control study fifty-five patients with Kidney failure and fourty healthy people were examined. Circulating concentrations of Osteoprotegerin, 1,25 dihydroxyvitamin D
... Show MoreSixty albino female mice were used in this experiment to study the possible effect of the crude alcoholic extract of the celery leaves on their fertility. These animals were randomly and equally divided into three experimental groups (20 females/group). The first and second groups were orally given a daily dose of 500 and 1000 mg/kg body weight, respectively, of the crude alcoholic extract, while the third group (control) was similarly treated, at the same time, with 0.1 ml/gm body weight of physiological saline for comparison. The treatment, however, lasted for 25 consecutive days. On day 26, after treatment was stopped, the life body weight of all animals was recorded before sacrificing the animals. Thereafter blood
... Show MoreEighteen new cyclic imides (maleimides) conncted to benzothiazole moiety through sulfonamide group were synthesized via multistep synthesis.The first step involved preparation of two maleamic acids N-phenylmaleamic acid and N-benzylmaleamic acid via reaction of maleic anhydride with aniline or benzyl amine.Dehydration of the prepared amic acids by treatment with acetic anhydride and anhydrous sodium acetate in the second step afforded N-phenylmaleimide and N- benzyl maleimide which in turn were treated with chlorosulfonic acid in the third step to afford 4-(N-maleimidyl) phenyl sulfonyl chloride and 4-(N-maleimidyl) benzyl sulfonyl chloride respectively.In the Fourth step of this work each one of the two prepared maleimidyl sulfonyl chlorid
... Show MoreEthanolic extracts of Thyme (Thymbra spicata) were tested for their inhibitory action on Paenibacillus larvae the causative agent of American foul brood with different concentration by using disc assay and mixed with culture media . Results showd that 3000mg / disc and 3000 mg / ml was the effective concentroation for the both methods .Thymol was isolated by using TLC technique . The effective concentration of thymol on growth of bacteria was 1000 mg / disc . Thymol and crude extracts of thyme 3000 mg / ml were tested on infected hives by mixed with sugar solution . The symptoms of AFB disease was full disappearance within 21 , 27 days after treatment with thymol and thyme
... Show MoreThis paper presents a method to organize memory chips when they are used to build memory systems that have word size wider than 8-bit. Most memory chips have 8-bit word size. When the memory system has to be built from several memory chips of various sizes, this method gives all possible organizations of these chips in the memory system. This paper also suggests a precise definition of the term “memory bank” that is usually used in memory systems. Finally, an illustrative design problem was taken to illustrate the presented method practically
Klebsiella pneumoniae has been found in the urinary tract of some bladder cancer patients. Bacterial presence within tumor tissue may affect the tumor-microenvironment and consequently influence cancer behavior, development, and treatment response. This study investigated mesenchymal and stemness transdifferentiation of bladder cancer cell line due to environmental stress of K. pneumoniae. Cultures of urothelial bladder cancer cell line (T24) were infected with K. pneumoniae with different multiplicity of infection (MOI) for two and four days. Transdifferentiation-associated features were morphologically assessed.
Moreover, transdifferentiation markers were estimated using Q-PCR and immunohistoc
... Show MoreCopper and Zinc powders with different particle sizes were subjected to sieving of range (20-100?m) and He-Ne laser system to determine the particle size . 1wt% from each powders was blended carefully with 99wt% from Iraqi oil . Microscopic examination were carried for all samples to reveal the particle size distribution . XRF intensity measurements were conducted for all suspended samples , and the relation between XRF intensity and the particle size was found .
Background: Polycystic ovary syndrome is a heterogeneous disorder and its etiology appears to be complex and multifactorial; characterized by hyperandrogenism, chronic anovulation and infertility. It’s associated with evidence of low-grade chronic inflammation, as indicated by the presence of elevated levels of high sensitive C- reactive protein levels, interleukin-6 and tumor necrosis factor-α. The source of excess circulating tumor necrosis factor-α in obese Polycystic ovary syndrome patient is likely to be the adipose tissues while in lean women increased visceral adiposity has been proposed as a source of excess tumor necrosis factor-α.Objectives: to evaluate the levels of high sensitive C- reactive protein, tumor necrosis facto
... Show MoreOne of the serious problems in any wireless communication system using multi carrier modulation technique like Orthogonal Frequency Division Multiplexing (OFDM) is its Peak to Average Power Ratio (PAPR).It limits the transmission power due to the limitation of dynamic range of Analog to Digital Converter and Digital to Analog Converter (ADC/DAC) and power amplifiers at the transmitter, which in turn sets the limit over maximum achievable rate.
This issue is especially important for mobile terminals to sustain longer battery life time. Therefore reducing PAPR can be regarded as an important issue to realize efficient and affordable mobile communication services.
... Show More
Female infection with HPV (human papilla virus) has been established as an essential cause of CIN (cervical intraepithelial neoplasia). The danger of transformation from CIN to frank malignancy should be considered. Objective: The goal of this study is to evaluate the effectiveness of CO2 laser vaporization of ectocervical lesion high grade squamous intraepithelial lesion (HGSIL). Patients and Methods: Four Female out of 150 affected with HGSIL lesions were submitted to CO2 laser vaporization and followed up in 4 months later, and 10 women with HGSIL lesion submitted to electrocautery diathermy for the comparison. Results: Among women treated by CO2 laser vaporization, 3 women had negative results (clear cervix), at 4 months follow up; o
... Show More