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.
The concept of environmental debt, which has several dimensions, emerged in the nineties. One of these dimensions is spatial, referring to the debts that southern countries sustain due to the extraction of natural resources and environmental damage inflicted upon northern countries. Another dimension, the temporal dimension, concerns the environmental debts that current generations owe to their predecessors. That means past generations are culpable for overuse of natural resources and mismanagement. The debt issue is complex, despite many mechanisms to resolve it. Most important are the recently introduced environmental bonds, an important step forward in the field of sustainable financing of environmental debts.
The major of DDoS attacks use TCP protocol and the TCP SYN flooding attack is the most common one among them. The SYN Cookie mechanism is used to defend against the TCP SYN flooding attack. It is an effective defense, but it has a disadvantage of high calculations and it doesn’t differentiate spoofed packets from legitimate packets. Therefore, filtering the spoofed packet can effectively enhance the SYN Cookie activity. Hop Count Filtering (HCF) is another mechanism used at the server side to filter spoofed packets. This mechanism has a drawback of being not a perfect and final solution in defending against the TCP SYN flooding attack. An enhanced mechanism of Integrating and combining the SYN Cookie with Hop Count Filtering (HCF) mech
... Show MoreThe study aimed to show the jurisprudential impact of the multiplicity of directing the syntactic movement of the Qur’anic readings, and it was based on the inductive, descriptive and analytical approach. , such as the difference in explaining the ruling on washing the feet, and the one who meditates on the Qur’anic readings notices in it a picture of the Qur’anic miracles, and this is what is manifested by the brevity of the speech. Collapse the research, and the research tried to trace the Qur’anic readings, contained in the verses, and the readings were limited to what is related to the explanatory meaning mentioned by the commentators, by clarifying the explanatory relationship between the Qur’anic readings and the me
... Show MoreThe phenomenon of spatial variation in the economic, social and urban development levels is considered prevalent in most of the economic and social systems,this relates to the concentration of most of those activities in certain regions and because of their rarity in other regions , that led to the emergence of the problem of the sharp contrast between the most developed areas and least developed areas within the same region or within the regions of the same country,
Reduction of this variables , in addition to the development of areas through following up and relying on an effective regional development enabling to reduce unemployment as well as to stop the migration of the unplanned for population,
And the ideal use of available
The study of the relationship between the coordinates of the sun and the moon with the crescent visibility factors has not been previously treated in a detailed and accurate way in research and previous studies, despite its religious importance. Accordingly, this paper aims to study the relationship between the crescent visibility factors (age, lag time, elongation (ARCL), arc of vision or relative altitude (ARCV), relative azimuth (DAZ), and crescent width (W), with coordinates of the sun and the moon), and how it varies during the day of the crescent's observation. In this paper, Matlab programs were designed to calculate the ecliptic sun and moon coordinates (λ, β) and in the presence of all perturbation impacts (planets), th
... Show MoreThe weight of larvae virgins and Alcamlat for males more than the weight of females of the roles themselves that the highest rate of loss in weight of larvae developed to virgins when field conditions were (21.5,22,21.3) mg during June and July and August respectively, recorded the highest degrees of heat and less attributed to moisture
The research aims at recognition of The rate of performing tasks done by the scientific committees in the scientific departments in the University of Baghdad, within the evaluative perspective of the departments’ Heads. To find any statistically significant differences in the responses of the research sample to the rates performance of the scientific committees in scientific departments in the University of Baghdad, within the evaluative perspective of the departments’ Heads, according to the two variables of the social gender; male and females, and field of specialization of Scientific and Humanities. The research sample consisted if (107) heads of the scientific department in the University of Baghdad. The researchers designed a
... Show MoreThe Vogue of the Restoration Comedy of Manners and George Etherage's The Man of Mode; or, Sir Fopling Flutter
