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 present study was designed to investigate the effect of different concentrations of Maxxthor on some hematological and oxidative stress parameters in male albino rats.Twenty male rats with age of 6-8 weeks and weight of 200-250gm were equally divided into four groups as follow:G1 treated with normal saline as control group,while G2,G3andG4groups were treated with(0.01,0.1,1)mg\kg body weight of Maxxthor respectively for 40 days.The following hematological parameters were measured: red blood cell(RBC),hemoglobin (Hb),white blood cell(WBC), platelets(PLT),malondialdehyde(MDA),glutathione(GSH),catalase and vitamin E. The hematological parameters results revealed highly significant(p<0.01)decreasein RBC and H,while a highly significant(p<0.
... Show MoreThe main aim of this study was to molecular identification and determine the antagonistic impact of rhizosphere Trichoderma spp. against some phytopathogenic fungi, including (Magnaporthe grisea) pyricularia oryzae, Rhizoctonia solani and Macrophomina phasolina. Four Trichoderma isolates were isolated from rhizosphere soils of the different host plants in different locations of Egyptian governorates. The morphological characterization of isolated Trichoderma as well as using of (ITS1-5.8S-ITS2) ribosomal gene sequence acquisition and data analyses. By comparing the results of DNA sequences of ITS region, the fungi represented one isolate were positively identified as T. asperellum (1 isolate T1) and one as T. longibrachiatum (1 isolate T2)
... Show MorePseudomonas aeruginosa is a Gram-negative opportunistic pathogen and a model bacterium for studying virulence and bacterial social traits. While it can be isolated in low numbers from a wide variety of environments including soil and water, it can readily be found in almost any human/animal-impacted environment. It is a major cause of illness and death in humans with immunosuppressive and chronic conditions, and infections in these patients are difficult to treat due to a number of antibiotic resistance mechanisms and the organism’s propensity to form multicellular biofilms. One hundred twenty clinical samples and forty hospital environmental samples (various sources) were collected from hospitals in Baghdad city during the period from Oc
... Show MoreThe meteorite with a single total mass of 630 gm as a visible meteorite has fallen on 22 March 2021, at 10:00 a.m. in Al-Sherqat subdistrict within Salah Al-Din, northern Iraq; and therefore, was named Al-Sherqat meteorite by the authors. It is characterized by a uniform structure of coherent and medium degree of malleability. It is of a well-crystalline structure and not homogeneous in composition. The Al-Sherqat meteorite is composed of metallic phases of 7.6 gm/cm3 density exhibiting an oriented intergrowth of kamacite (α-FeNi) with taenite showing a Widmanstätten pattern on an etched polished section with the finest octahedrite kamacite bandwidth of less than 0.2 mm. It is composed of Fe (86.9 wt%), Ni (9.63 wt%), P (1.31 wt%)
... Show MoreZnS:Ce3+ nanoparticles were prepared by a simple microwave irradiation method under mild condition. The starting materials for the synthesis of ZnS:Ce3+ quantum dots were zinc acetate (R & M Chemical) as zinc source, thioacetamide as a sulfur source, cerium chloride as cerium source and ethylene glycol as a solvent. All chemicals were analytical grade products and used without further purification. The quantum dots of ZnS:Ce3+ with cubic structure were characterized by X-ray powder diffraction (XRD), the morphology of the film is seen by scanning electron microscopy (SEM) also by field effect scanning electron microscopy (FESEM) and XRD. Upon exposure to 460 nm light at zero bias voltage, ZnS:Ce3+/p-Si showed a high sensitivity of 4000% an
... Show MoreThis research is a continued efforts for a project on the fire tube boiler control for Al Rasheed edible oil factory. The aim is to enhance the control system with new integral control one. A functional blocks diagram (FBD) was built and simulated. With Schneider smart relays, FBD differs than ladder logic programming in which the PID option is active. An extensive work was done to understand the operation sequence, emergency shutdown, and faults causing the trips. A control program was designed to control logical sequence of operation. Furthermore temperature is controlled via cascade control with fuel and air controllers. The temperature controller output is send as remote set point to the fuel controller in a serial cascade manner. The f
... Show MoreDate palm (Phoenix dactylifera) is one of the world’s oldest cultivated fruit crops and belongs to the Arecaceae family. It originated in Mesopotamia (Iraq) in 4000 BC. Large areas of palm groves in Iraq produce various types of dates for internal consumption and export. Their cultivation has spread and has become a significant crop in the Arabian Peninsula, North Africa, and the Middle East. Date fruits are widely consumed in Iraq, and radiological monitoring of this crop is necessary as activity concentrations of 238U, 232Th, 40K, and 137Cs were measured in 12 soil samples and 12 date samples from
The unprejudiced of this education is to gauge the ability of the retinoic acid to induce apoptotic cell death in hematological tumors through caspase dependent or independent apoptotic pathway, The cytotoxicity effects of retinoic acid of different concentrations (400,350,300,250,200,150,100,50,25,12.5 μg\ml) and exposure for all hematological malignancy cell lines (Human non-Hodgkin lymphoma SR and human multiple myeloma (COLO 677) and Human Monocytic Leukemia THP1 and Acute promyelocytic leukemia NB4) have been determined using a microtetrazolium (MTT) assay. Propodeum iodide and alcidine orange (AO/PI) paired discoloration was used to study the ability of retinoic acid to induce apoptosis in the infected cells and examined under fluore
... Show MoreBackground: Periodontal disease (PD) is a chronic inflammatory condition characterized by destruction of supporting structures of the teeth. Intelligence quotient (IQ) was potentially reported to significantly associated with prevalence of gingivitis. Mild gingivitis was obtained in high IQ levels while moderate gingivitis may be attributed to poor oral hygiene seen among the subjects having low IQ levels. Method: One hundred volunteers aged between 20-45 years old were enrolled in this study, patients were equally divided into right- and left-handed (50 patients each)and each group then subdivided into patients with healthy gingiva(10), patients with gingivitis (20), and patients suffering from periodontitis (20).An IQ questionnaire was p
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