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 inflammator

Conditional logistic regression is often used to study the relationship between event outcomes and specific prognostic factors in order to application of logistic regression and utilizing its predictive capabilities into environmental studies. This research seeks to demonstrate a novel approach of implementing conditional logistic regression in environmental research through inference methods predicated on longitudinal data. Thus, statistical analysis of longitudinal data requires methods that can properly take into account the interdependence within-subjects for the response measurements. If this correlation ignored then inferences such as statistical tests and confidence intervals can be invalid largely.

Grass trimming operation is widely done in Malaysia for the purpose of maintaining highways. Large number of operators engaged in this work encounters high level of noise generated by back pack type grass trimmer used for this purpose. High level of noise exposure gives different kinds of ill effect on human operators. Exact nature of deteriorated work performance is not known. For predicting the work efficiency deterioration, fuzzy tool has been used in present research. It has been established that a fuzzy computing system will help in identification and analysis of fuzzy models fuzzy system offers a convenient way of representing the relationships between the inputs and outputs of a system in the form of IF-THEN rules. The paper presents

The electric quadrupole moments for some scandium isotopes (41, 43, 44, 45, 46, 47Sc) have been calculated using the shell model in the proton-neutron formalism. Excitations out of major shell model space were taken into account through a microscopic theory which is called core polarization effectives. The set of effective charges adopted in the theoretical calculations emerging about the core polarization effect. NushellX@MSU code was used to calculate one body density matrix (OBDM). The simple harmonic oscillator potential has been used to generate the single particle matrix elements. Our theoretical calculations for the quadrupole moments used the two types of effective interactions to obtain the best interaction compared with the exp

        A newly developed analytical method was conducted for the determination of Ketotifen fumarate (KTF) in pharmaceuticals drugs via quenching of continuous fluorescence of 9(10H)-Acridone (ACD). The method was applied using flow injection system of a new homemade ISNAG fluorimeter with fluorescence measurements at ± 90◦ via 2×4 solar cell. The calibration graph was linear in the range of 1-45 mmol/L, with correlation coefficient r = 0.9762 and the limit of detection 29.785 µg/sample from the stepwise dilution for the minimum concentration in the linear dynamic ranged of the calibration graph. The method was successfully applied to the determination of Ketotifen fumarate in two different pharma

Maximum power point tracking (MPPT) is used in photovoltaic (PV) systems to enhance efficiency and maximize the output power of PV module, regardless the variation of temperature, irradiation, and the electrical characteristics of the load. A new MPPT system has been presented in this research, consisting of a synchronous DC-DC step-down Buck converter controlled by an Arduino microcontroller based unit. The MPPT process with Perturb and Observe method is performed with a DC-DC converter circuit to overcome the problem of voltage mismatch between the PV modules and the loads. The proposing system has high efficiency, lower cost and can be easily modified to handle more energy sources. The test results indicate that the u

In this research ,Undoped Nio and 1%Li doped Nio thin films were deposited utilizing chemical spray pyrolysis on the glass substrates heated (450C). The effects of non-thermal plasma on the structural and optical properties were studied. XRD measurement shows that Nio and Nio:1%Li films were found to be polycrystalline and have cubic structure with a preferred orientation (111). Decreased crystal size after exposure especially at (7) sec. AFM data indicate that the surface roughness average and (RMS) values of the prepared doped films are increasing after exposure to plasma, the transmittance increases after doped samples exposure to plasma, it was found that the energy gap value decreased when doped samples exposure to plasma, also, thickn

Gingival carcinoma is a malignant neoplasm affecting the oral mucosa and is associated with significant morbidity and mortality. Allium ampeloprasum var. porrum water extracts have gotten a lot of attention because of their bioactive components, such as polyphenols, flavonoids, and alkaloids, which have a variety of pharmacological activities, including antiproliferative actions. This study aimed to evaluate the histological and molecular effects of Allium ampeloprasum (leek) water extract on the proliferation of the murine gingival cancer cell line. Histological evaluation was conducted to examine morphological changes induced by extract treatment. Molecular mechanisms underlying the observed histological changes were investigated