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.
Historic city centers are cultural archives where built forms and spatial practices hold the collective memory of generations. In Baghdad, the concept of Cultural DNA (C-DNA) is a tool to understand how cultural codes are the generative rules that shape the evolution and persistence of the historic urban fabric. This research explores the role of C-DNA as a trigger of urban morphogenesis in Rusafa, the historic heart of Baghdad, by looking into how cultural values underpin spatial continuity, change, and adaptability. The study uses Space Syntax methodologies with DepthmapX, supported by historical maps, surveys, and field observations, to analyze two morphological stages of Rusafa: 1850 and now. Through axial analysis, the research
... Show MoreRacism changes its meaning, shape, purpose or function according to various factors such as time, place, motives, and backgrounds; among others. It seems, however, to have deep roots back in the history of mankind. Such a concept stems from a perceived difference with the other. It is fed by the feeling of being hierarchically superior in opposition to another party that is depicted as inferior. Since ancient times, racism is disguised in different forms and manifests itself on various levels. It is a truism that this ideology can be best presented via language.
Due to its negative implication, racism lends itself to a critical kind of research. As such, this paper is a critical pragmatic investigation for this phenomenon a
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Research summary
Backbiting pronouns have gained great importance in contemporary textual linguistic studies, as they play a coordinating function in the text and link its parts.
Interpreters did not overlook the role of backbiting pronouns in achieving the function of consistency and coherence in the Qur’anic text, as they referred to this, including Ibn Ashour, who had many analytical practices related to referral issues and their complexities. The Qur'anic reference to the pronouns of backbiting, so the researcher traced these phenomena and their evidence in the Holy Qur'an, aiming to clarify their role in enriching the significance and in the coherence and harmony
... Show MoreThis work has been accomplished through a dense field work on the Sawa Lake. The aim of this work is to highlight on characteristics that are consistent with outstanding universal values to be a new Iraqi site belongs to the World Natural Heritage. This study sorted many unique characteristics might ensure that Sawa Lake as a world natural heritage sites. This study shows that the lake had met four natural criteria of the outstanding universal values: (1) the seventh criterion which states to contain superlative natural phenomena or areas of exceptional natural beauty and aesthetic importance. (2) The eighth criterion which stated to be outstanding example representing major stages of earth's history, including the rec
... Show MoreThe aim of the current research is to study a topic from the Qur’anic topics, few have researched it and realized its content, so people knew it in one name in the Qur’an in another name, and due to the ancientity of the topic and its contemporaneity, I wanted to write about it. The research has an introduction, three demands, and a conclusion with the most important results of the research:
As for the introduction: It was to indicate the importance of the topic and an optional reason for it.
As for the first requirement: it included the definition of reasoning, its divisions, and its characteristics.
As for the second requirement, it was to indicate the meaning, types, and methods of labeling it.
As for the third require
KE Sharquie, AA Noaimi, SA Al-Hashimy, MM Al-Salih, Journal of Cosmetics, Dermatological Sciences and Applications, 2014 - Cited by 12
Long-term use of non-steroidal anti-inflammatory drugs (NSAIDs) mostly associated with renal and hepatic adverse effects, and the adjunct use of compounds with potent protective effects, like silymarin, may be one of the choices to avoid these effects. This project was designed to evaluate the protective effect of silymarin against the suspected renal and hepatic injury induced with long term use of NSAIDs; 220 patients with osteoarthritis were randomized into 5 groups and treated with either silymarin 300mg/day alone, piroxicam 20mg/day alone, meloxicam 15mg/day alone or the combination of each of them with silymarin for 8 weeks. The renal and hepatic functions were evaluated before starting treatment and after 8 weeks including assessm
... Show MoreSufficient high-quality data are unavailable to describe the management approach and guideline of COVID-19 disease in pediatric and adolescent population which may be due to mild presentation in most of cases and less severe complications than older ages.
World Health Organization was concerned with the establishment of an approved guideline to manage the increasing number of COVID-19 patients worldwide aiming to prevent or lessen COVID-19 global burden.
The clinical features have a wide spectrum starting from uncomplicated mild illness, mild-moderate pneumonia, severe pneumonia, acute respiratory distress syndrome, sepsis, septic shock, and multisystem inflammatory syndrome in children.
Many important definitions
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Background: Drawing blood to measure total serum bilirubin is painful & time consuming. Transcutaneous bilirubinometer working by multiwavelength spectral reflectance from the skin surface on forehead or upper sternum is a quick & painless technique. Objectives: to compare the effectiveness of transcutaneous (over the upper sternum and forehead) and serum bilirubin measurement of neonate with jaundice. Subjects and Methods: This is a cross sectional prospective study. It enrolled 175 jaundiced neonates & excluded those exposed to phototherapy. It was conducted at Child Central Teaching Hospi |