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: Oral Lichen Planus (OLP) is a chronic inflammatory mucosal disease, presenting in various clinical forms WHO had regarded OLP as a precancerous conditions in 1978 because of its potential with cancer. Both antigen-specific and nonspecific mechanisms involved in the pathogenesis of OLP. Oral Squamous Cell Carcinoma (OSCC) is the most common malignant neoplasm of the oral cavity representing more than 94% of oral cancer. It occurs in different sites and has many etiological factors. Cyclin Dl is a proto-oncogene which consider as the key protein in the regulation of cell proliferation and its overexpression led to the occurrence and progression of malignant tumors.NF-KB p65 is a member ofNF-kB family of transcription factors that
... Show MoreObjectives: To assess nurses-midwives knowledge and performance regarding immediate newborn care in delivery rooms, and to identify the relationship between nurses-midwives knowledge and performance and their socio-demographic and professional variables regarding immediate newborn care.
Methodology: A descriptive study was conducted from 1st of October, 2019 to 20TH of October, 2020 at three maternity teaching hospitals in Baghdad city/Al-Russafa Health Directorate: Al-Elwiya Maternity Teaching Hospital, Fatema Al- Zahra Maternity and Pediatric Hospital, Al- Za'faraniyah General Hospital. Non-probability (purposive sample) include
... Show MoreNewly 4-amino-1,2,4-triazole-3-thione ring 2 was formed at position six of 2-methylphenol from the reaction of 6-(5-thio1,3,4-oxadiazol-2-yl)-2-methylphenol 1 with hydrazine hydrochloride in the presence of anhydrase sodium acetate. Seven newly fused heterocyclic compounds were synthesized from compound 2. First fused heterocyclic was 6-(6-(3,5-di-tertbutyl-4-hydroxyphenyl)-[1,2,4]triazolo[3,4-b][1,3,4]thiadiazol-3-yl)-2-methylphenol 3 synthesized from reaction compound 2 with 3,5-di-tert-butyl-4-hydroxybenzoic acid in POCl3. Reaction compound 2 with bromophencylbromide afford 6-(6-(4-bromophenyl)-5H-[1,2,4]triazolo[3,4-b][1,3,4]-thiadiazin-3-yl)-2-methylphenol 4. 6-(6-thio-1,7a-dihydro-[1,2,4] triazolo[3,4-b][1,3,4]-thiadiazol-3-yl)-2
... Show MoreObjectives: The study aims: (1) To determine effectiveness of instructional health education vascular access on hemodialysis patients' knowledge, (2) To find out the association between effects of instructional health education vascular access and demographic characteristics of (age, gender and educational level). Methodology: A quasi experimental study –control study design is carried out at AL-Hussein Teaching Hospital in AL-Nasiriyah City, from 3November,2015 to 2 June, 2016. A non-probability (Purposive sample) of (80) patients with vascular access devices on maintenance hemodialysis patients divided int
Abstract
The research aims to measure the level of critical thinking skills among students of A’Sharqiah University in the Sultanate of Oman, as well as identify the level of their availability based on the variables: gender, academic level, school year, cumulative average, and general diploma / high school ratio. The researchers used the descriptive approach. To achieve the objectives of the study, they used The California Test for Critical Thinking Skills Picture (A) after evaluation (Farraj, 2006). It was applied to a sample of (487) students from A’sharqiah University. The results of the study found that the critical thinking skills of A’sharqiah University students are below the educationally acceptabl
... Show MoreNew complexes of the type [ML2(H2O)2] ,[FeL2(H2O)Cl] and [VOL2] were M=Co(II),Ni(II) and Cu(II) ,L=4-(2-methyl-4-oxoquinazoline-3(4H)-yl) benzoic acid were synthesized and characterized by element analysis, magnetic susceptibility ,molar conductance ,FT-IR and UV-visible. The studies indicate that the L acts as doubly monodentate bridge for metal ions and form mononuclear complexes. The complexes are found to be octahedral except V(IV) complex is square pyrimde shape . The structural geometries of compounds were also suggested in gas phase by theoretical treatments, using Hyper chem-6 program for the molecular mechanics and semi-empirical calculations, addition heat of formation(?Hf ?) and binding energy (?Eb)for the free ligan
... Show MoreThe doping process with materials related to carbon has become a newly emerged approach for achieving an improvement in different physical properties for the obtained doped films. Thin films of CuPc: C60 with doping ratio of (100:1) were spin-coated onto pre-cleaned glass substrates at room temperature. The prepared films were annealed at different temperatures of (373, 423 and 473) K. The structural studies, using a specific diffractometry of annealed and as deposited samples showed a polymorphism structure and dominated by CuPc with preferential orientation of the plane (100) of (2θ = 7) except at temperature of 423K which indicated a small peak around (2θ = 3
This paper tries to understand the poetic reference in the images of woman, she-camel, horse and their manifestations in Tarafah-ibnulAbd's poetry. There has got my attention the fact that these three images have their own distinct taste which is characterised by a clear rhythm, let alone the lively nature that is filled with liveliness and activity to be in harmony with the poet's youth. For these three images represented the best manifestations of his psychological and artistic poetics. The paper adopts an artistic analysis to arrive at the psychological aspects of these experiences-the woman, the she-camel, and the horse- and to understand the functions of their images and symbolic reference.