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.
Several million tons of solid waste are produced each year as a result of construction and demolition activities around the world, and brick waste is one of the most widely wastes. Recently, there has been growing number in studies that conducted on using of recycling brick waste (RBW) to produce environmentally friendly concrete. The use of brick waste (BW) as potential partial cement or aggregate replacement materials is summarized in this review where the performance is discussed in the form of the mechanical strength and properties that related to durability of concrete. It was found that, because the pozzolanic activity of clay brick powder, it can be utilized as substitute for cement in replacement level up t
... Show MoreSeveral million tons of solid waste are produced each year as a result of construction and demolition activities around the world, and brick waste is one of the most widely wastes. Recently, there has been growing number in studies that conducted on using of recycling brick waste (RBW) to produce environmentally friendly concrete. The use of brick waste (BW) as potential partial cement or aggregate replacement materials is summarized in this review where the performance is discussed in the form of the mechanical strength and properties that related to durability of concrete. It was found that, because the pozzolanic activity of clay brick powder, it can be utilized as substitute for cement in replacement level up to 10%. Whereas,
... Show MoreThe theme of love has been and still is the interest of authors and writers for it is closely related to people’s lives. That great passion has helped them express genuine thoughts pertinent to that theme that has given rise to many debates, some are vague and others controversial. In his (strait is the gate), (La Porte étroite), André Gide has sought to shed light on his characters perspective of love and its relationship with sacredness and divine happiness. Indeed these characters have found often themselves restrained, chained and exhausted, by tough religious commands that have imposed on them spiritual commitments and duties too hard to break.
André Gide has revealed a protestant deviation from the perfection theme tha
... Show MoreThe division partitioning technique has been used to analyze the four electron systems into six-pairs electronic wave functions for ( for the Beryllium atom in its excited state (1s2 2s 3s ) and like ions ( B+1 ,C+2 ) using Hartree-Fock wave functions . The aim of this work is to study atomic scattering form factor f(s) for and nuclear magnetic shielding constant. The results are obtained numerically by using the computer software (Mathcad).
An anal fissure which does not heal with conservative measures as sits baths and laxatives is a chronic anal fissure. Physiologically, it is the high resting tone of the internal anal sphincter that chiefly interferes with the healing process of these fissures. Until now, the gold standard treatment modality is surgery, either digital anal dilatation or lateral sphincterotomy. However, concerns have been raised about the incidence of faecal incontinence after surgery. Therefore, pharmacological means to treat chronic anal fissures have been explored.A Medline and pub med database search from 1986-2012 was conducted to perform a literature search for articles relating to the non-surgical treatment of chronic anal fissure.Pharmacological s
... Show MoreContemporary life is racing against time in its temptations and variables, and it has become shaped and changed in an amazing way in its various aspects and fields. This was facilitated by intellectual and scientific communication between civilizations, and the rapid progression in successive inventions and discoveries in the fields of science and arts of knowledge. This contributed to a great economic and commercial renaissance. Then, these economic developments entered the world into a very strong competition, which forced producers to calculate all production costs, to reach the highest profits by reducing the price of the produced commodity on the one hand, and achieving quality in appearance (especially) on the other hand. Since the ma
... Show MoreSpergularia iraqensis sp. nov. is described as a new species from Iraq. This species has been collected from Diyala Province in the central east of Iraq; it is closely related to Spergularia rubra (L.) J. Presl & C. Presl, 1819 and Spergularia bocconei (Scheele) Graebn., 1919.
The distinguishing of the morphological characteristics of the new species alongside the two similar species are discussed with photographs, and an identification key is given for Spergularia iraqensis and other closely related species.
Worldwide, enormous amounts of waste cause major environmental issues, including scrap tires and plastic, and large waste, a consequence of the demolition of buildings, including crushed concrete, crushed clay bricks, and crushed thermo-stone. From that point, it’s possible to consider that the recycling processes for these materials and using them in the manufacturing field will reduce the adverse effects on the environment of these wastes and the consumption of natural resources. Sustainable concrete blocks can be considered as one of the products produced by using these materials as partial volume replacement of the coarse, fine aggregate, or cement content, considering their dry density, workability, absorption, compressive st
... Show More