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.
Background: Diabetic mellitus type ? is a metabolic disorder of diverse etiological factors, characterized by hyperglycemia resulting from an absolute deficiency of insulin affected childhood and adolescent. Some of these patients seek an orthodontic care .The orthodontist who is treating these medically compromised patients should have a working knowledge of the multitude of medically complex problems. This information will support and enable for delivery of high standards of dental care in general and orthodontic care in particular. The aim of this study was to analyze serum IgG levels and salivary secretory IgA (sIgA) levels in human dentine extract (HDE) before (T0) and 6 months after (T6) orthodontic treatment and to correlate anti-HDE
... Show MoreThis research is devoted to design and implement a Supervisory Control and Data Acquisition system (SCADA) for monitoring and controlling the corrosion of a carbon steel pipe buried in soil. A smart technique equipped with a microcontroller, a collection of sensors and a communication system was applied to monitor and control the operation of an ICCP process for a carbon steel pipe. The integration of the built hardware, LabVIEW graphical programming and PC interface produces an effective SCADA system for two types of control namely: a Proportional Integral Derivative (PID) that supports a closed loop, and a traditional open loop control. Through this work, under environmental temperature of 30°C, an evaluation and comparison were done for
... Show MoreDiabetic kidney disease is an illness of the glomerulus that interferes with the glomerular filtration barrier (GFB), which is worked to enable kidney to selective purification of water and solutes in addition to limiting the movement of large macromolecules such as albumin. In the glomerular endothelium, mesangial cells, foot cells, and the brush border of the proximal tubules, ACE-2 is expressed and that the kidneys represent the highest-expressing region of this enzyme. Thus, the current study aimed to evaluate ACE-2 level in this case compared to healthy condition. The study Conducted with 120 male and female ranging in age (30-65) years old. Ninety patients with type 2 diabetes subdivided into three groups on the basis of A
... Show MoreThe advancements in Information and Communication Technology (ICT), within the previous decades, has significantly changed people’s transmit or store their information over the Internet or networks. So, one of the main challenges is to keep these information safe against attacks. Many researchers and institutions realized the importance and benefits of cryptography in achieving the efficiency and effectiveness of various aspects of secure communication.This work adopts a novel technique for secure data cryptosystem based on chaos theory. The proposed algorithm generate 2-Dimensional key matrix having the same dimensions of the original image that includes random numbers obtained from the 1-Dimensional logistic chaotic map for given con
... Show MoreRheumatoid arthritis (RA) is a chronic inflammatory disease associated with decreased antioxidant state .This study aim to investigate the status of oxidant/antioxidant in a sample of Iraqi patients with RA and the role of peroxynitrite and its natural scavenger uric acid in them .This case-controlled study was conducted at Baghdad teaching hospital /Baghdad from December 2010-May 2011 . Twenty-five patients with mean age 39 years and 25 apparently healthy subject as controls with mean age 29 years were included in the study .Investigations include estimation of serum levels of nitric oxide (NO) ,peroxynitrite (PN) , malondialdehyde (MDA) , and uric acid (UA) .Serum PN levels were significantly elevated in RA patients a
... Show MoreKE Sharquie, KI Al-Hamdi, AA Noaimi, AA Al-Mohammadi, J Clin Exp Invest www. clinexpinvest. org Vol, 2011 - Cited by 1
Behavioral and emotional disorders represent one of the commonest problems facing children in particular and disturbing their parents and educators in general. This is because Iraqi people have long been living in difficult conditions and faced various terrorists’ operations, such as killing, destruction, robbery, and looting. Such operations are said to be one of the reasons that leads to the development of such disorders, and may negatively affect the psyche of the child and be reflected on his behavior as represented by playing with the IPad for hours, and suffering from nightmares. Accordingly, the current research aims to examine specifically the commonest negative behavioral disorders among kindergarten children during the post-t
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