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: This study aimed to determine whether there is a relationship among the bite force with facial dimensions and dental arches in a sample of Iraqi adults with Class I skeletal and dental relations. Materials and methods: Forty dental students (20 males and 20 females) were selected under certain criteria. For those individuals, dental impressions, frontal facial photographs and maximum bite force at molar and incisor regions were taken. The dental arches widths and facial dimensions were measured using the AutoCAD program 2007, while the bite force was determined using special device. Descriptive statistics for the measured variables were performed and gender difference was determined using independent sample t-test, while the rel
... Show MoreTwo novel demountable shear connectors for precast steel-concrete composite bridges are presented. The connectors use high-strength steel bolts, which are fastened to the steel beam with the aid of a special locking configuration that prevents slip of bolts within their holes. Moreover, the connectors promote accelerated construction and overcome typical construction tolerances issues of precast structures. Most importantly, the connectors allow bridge disassembly, and therefore, can address different bridge deterioration scenarios with minimum disturbance to traffic flow, i.e. (1) precast deck panels can be rapidly uplifted and replaced; (2) connectors can be rapidly removed and replaced; and (3) steel beams can be replaced, while precast
... Show MoreFree radicals are small extremely reactive species that have unpaired electrons. Free radicals include subgroups of reactive species, which are all a product of regular cellular metabolism. Oxidative stress happens when the free radicals production exceeds the capacity of the antioxidant system in the body’s cells.
The current review clarifies the prospective role of antioxidants in the inhibition and healing of diseases.
Information on oxidative stress, free radicals, reactive oxidant species, and natural and synthetic antioxidants was obta
Visceral leishmaniasis(VL) or kala-azar is one of the world most neglected tropical diseases in mortality and fourth in morbidity, rK39 dipstick was used to diagnose the suspected infected patients as easiest and rapid technique for VL diagnostic, the disease out-coming required to the differentiation of cell mediated immunity either T-helper 1(Th-1) or (Th-2). One of main pointers that may be considered as one of immune evasion strategy in the host-parasite interplay is HLA-G level alteration. HLA-G Known as a special proteins (non-classical HLA class I) molecules which can suppress the immune system by T-cell functions impaired in the aid with target receptors as LILRB4. The development of the cell mediated immunity initiated with Interle
... Show MoreS Khalifa E, AM Sabeeh A, AN Adil A…, 2007
Background. To identify the most common retention protocols practiced by Iraqi orthodontists using a specially designed e-survey. Furthermore, this study aimed to assess the effect of sociodemographic factors on the participant’s choice. Methods. Two hundred and twenty-five questionnaires with 23 multiple choice questions were sent to members of the Iraqi Orthodontic Society. The questionnaire was organized into four sections representing information about sociodemographic status of the orthodontists, factors affecting the selection of the retention system, commonly used retainers in the upper arch and lower arch, and duration of the retention system. The chi-square test was used to test the significant association between differe
... Show MoreObjectives: The study aim was to explore the knowledge, perceptions, and attitudes of Iraqi physicians regarding generic and locally manufactured medicines. Methods: A total of 124 physicians were involved in this cross -sectional study. The convenience sample was collected from five public hospitals in Baghdad. A self-administered questionnaire was distributed and collected in-person. Fisher's Exact Test was used to measure the association between physician years of experience, gender and categorical (perception and knowledge) variables. Results: Most respondent answers regarding the knowledge of generic medicines were incorrect. Only up to one-third of the participants knew that generic medicines are therapeutically eq
... Show MoreAbstract* Backgrounds The retention of the maxillary complete denture may be influenced by adequate recording in the patient’s mouth, marking, and carving of the post-dam on the maxillary cast. Failure to do so may lead to loss of retention. Successful denture construction with patient’s comfort could be achieved by effective methods of recording, marking, and carving of the post-dam. The aim of the study was to assess Iraqi dentists in their knowledge and application of the post-dam in maxillary complete dentures. Methods Questionnaires were distributed randomly through online Google Forms and 118 Iraqi dentists with different academic degree participated in the study. A multinomial logistic regression model was also used to ass
... Show MoreBackground: The oral cavity is considered as a complex ecological niche, its complex microbial community is reflected to it. Streptococcus mutans has been implicated as one of the major etiological factor of dental caries. Tooth surfaces colonized with Streptococcus mutans are at a higher risk for developing caries, while lactobacilli are considered as the secondary invaders, not initiators of the carious lesion. The main purpose of this study was to correlate the dental caries (for primary and permanent teeth) in the upper jaw with the streptococcus mutans and lactobacilli count in the dental plaque and saliva, also to correlate the dental caries (for primary and permanent teeth) in the lower jaw with the streptococcus mutans and lactobaci
... Show MoreThe dubbing process for the Foreign Dramas by the Kurdish language became a serious phenomenon in the Kurdish satellite TV, especially in the past few years. It attracted a wide audience, especially young adolescents. And prepared by some breakthrough hostile to Kurdish culture and value coordinated by others remedy for the structural gaps and lapses have, as a result of climate which is brought into existence globalization and secretions variety dimensional, saturated value and cognitive effects bearing a strong identity of its elements. From here, the problem of this research is the ambiguity of their role in a series of developmental processes Kurdish awareness of young people and the extent of the impact that caused it.
This
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