Clinical and Molecular Characterization of Patients with Mucopolysaccharidosis Type I in an Algerian Series

Mucopolysaccharidoses (MPS’s) represent a subgroup of lysosomal storage diseases related to a deficiency of enzymes that catalyze glycosaminoglycans degradation. Mucopolysaccharidosis type I (MPS I) is a rare autosomal recessive disorder caused by a deficiency of α-l-iduronidase encoded by the IDUA gene. Partially degraded heparan sulfate and dermatan sulfate accumulate progressively and lead to multiorgan dysfunction and damage. The aim of this study is to describe the clinical, biochemical, and molecular characteristics of 13 Algerian patients from 11 distinct families. MPS I diagnosis was confirmed by molecular study of the patients’ IDUA gene. Clinical features at the diagnosis and during the follow-up are reported. Eighty-four percent of the studied patients presented with a mild clinical phenotype. Molecular study of the IDUA gene allowed the characterization of four pathological variations at the homozygous or compound heterozygote status: IDUA NM_00203.4:c.1598C>G-p.(Pro533Arg) in 21/26 alleles, IDUA NM_00203.4:c.532G>A-p.(Glu178Lys) in 2/26 alleles, IDUA NM_00203.4:c.501C>G-p.(Tyr167*) in 2/26 alleles, and IDUA NM_00203. 4: c.1743C>G-p.(Tyr581*) in 1/26 alleles. This molecular study unveils the predominance of p.(Pro533Arg) variation in our MPS I patients. In this series, the occurrence of some clinical features linked to the Scheie syndrome is consistent with the literature, such as systematic valvulopathies, corneal opacity, and umbilical hernia; however, storage signs, facial dysmorphic features, and hepatomegaly were more frequent in our series. Screening measures for these debilitating diseases in highly consanguineous at-risk populations must be considered a priority health problem.     

Characterization of rheological and thermophysical properties of HDPE–wood composite

The objective of this study is to develop a new biocomposite material with high deformation ability. In this regard, the thermal, rheological, and thermophysical properties of this new composite were characterized as a function of temperature and filler concentration. High density polyethylene (HDPE) was the matrix of this new composite which was reinforced with six sawdust concentrations 0%, 20%, 30%, 40%, 50%, and 60%. Maleic anhydride grafted polyethylene (PE‐g‐MA) was used as coupling agent. Addition of sawdust with PE‐g‐MA increased significantly the complex viscosity, the storage modulus (G′), and loss modulus (G″) of the matrix. The superposition of the complex viscosity curves using temperature dependent shift factor, allowed the construction of a viscosity master curve covering a wide range of temperatures. Arrhenius law was used for the relationship of the shift factor to temperature. Furthermore, method of Van Gurp and Palmen (tan delta vs. G*) is also used to control the time–temperature superposition. The experimental results can be well fitted with the cross rheological model which allowed the prediction of the thermorheological properties of the composites over a broad frequency range. By increasing wood concentration, both the activation energy and relaxation time for the biocomposites determined using, respectively, the Arrhenius law and the cole–cole rule increased. By contrast, specific heat of the matrix decreased with sawdust addition while its dimensional stability improved. © 2014 Wiley Periodicals, Inc. J. Appl. Polym. Sci. 2014 , 131, 40495.     

Morphology and properties of highly filled iPP/TiO2 nanocomposites

Nanocomposites based on isotactic polypropylene (iPP) and titanium dioxide (TiO₂) nanoparticle containing 1–15 vol% (4.6–45.5 wt%) of the nanoparticle were prepared by the melt blending process. The effect of an anhydride‐modified polypropylene as a compatibilizer on dispersion of TiO₂ nanoparticles was assessed using SEM. TGA and DSC analysis were performed to study the thermal properties of the nanocomposites. Crystalline structures of iPP in the presence of TiO₂ were analyzed by XRD. Mechanical properties of the nanoparticles were measured and a micromechanical analysis was applied to quantify interface interaction between the polymer and particle. SEM results revealed improvement of TiO₂ particle dispersion by adding the compatibilizer. It was shown that the thermal stability and crystalline structure of the nanocomposite are significantly affected by the state of particle dispersion. TiO₂ nanoparticles were shown to be strong β‐nucleating agents for iPP, especially at concentrations less than 5 vol%. Presence of the β‐structure crystals reduced the elastic modulus and yield strength of the nanocomposites. Micromechanical analysis showed enhanced interaction between organic and inorganic phases of the compatibilized nanocomposites. POLYM. ENG. SCI., 54:874–886, 2014. © 2013 Society of Plastics Engineers     

Rheological,crystal structure,barrier, and mechanical properties of PA6 and MXD6 nanocomposite films

In this study, rheological, crystal structure, barrier, and mechanical properties of polyamide 6 (PA6), poly(m‐xylene adipamide) (MXD6) and their in situ polymerized nanocomposites with 4 wt % clay were studied. The extent of intercalation and exfoliation as well as type of crystals, crystallinity, and thermal transitions were investigated using X‐ray diffraction (XRD) and differential scanning calorimetry (DSC), respectively. Dynamic rheological measurements revealed that incorporation of nanoclay significantly increases complex viscosity of MXD6 nanocomposites at low frequencies, which was related to the formation of a nanoclay network and exchange reaction between MXD6 chains. The comparative study of dynamic characteristics (G′ (ω) and G″ (ω)) for aliphatic and aromatic polyamide nanocomposites with their neat resins as well as the relaxation spectra for both polymer systems confirmed the possibility of the aforementioned phenomena. Although, the crystallinity of MXD6 films was lower as compared to PA6 films, the permeability to oxygen was more than 5 times better for the former. Incorporating 4 wt% clay enhanced the barrier property, tensile modulus, and yield stress of PA6 and MXD6 nanocomposite films in both machine and transverse directions without sacrificing much puncture and tear resistances. The PA6‐based films showed higher tear and puncture strength as compared to MXD6 films. POLYM. ENG. SCI., 54:2617–2631, 2014. © 2013 Society of Plastics Engineers     

Handwritten characters recognition based on nature-inspired computing and neuro-evolution

The enormous services obtainable by bank and postal systems are not 100 % guaranteed due to variability of handwriting styles. Various methods based on neural networks have been suggested to address this issue. Unfortunately, they often fall into local optima that arises from the use of old learning methods. Global optimization methods provided new directions for neural networks evolution that may be useful in recognition. This paper develops efficient algorithms that compute globally optimal solutions by exploiting the benefits of both swarm intelligence and neuro-evolution in a way to improve the overall performance of a character recognition system. Various adaptations implied to both MLP and RBF networks have been suggested namely: particle swarm optimization (PSO) and the bees algorithm (BA) for characters classification, MLP training or RBF design by co-evolution and effective combinations of MLPs, RBFs or SVMs as an attempt to overcome the drawbacks of old recognition methods. Results proved that networks combination proposals ensure the highest improvement compared to either standard MLP and RBF networks, the co-evolutionary alternatives or other classifiers combination based on common combination rules namely majority voting, the fusion rules of min, max, sum, average, product and Bayes, Decision template and the Behavior Knowledge Space (BKS).     

UWB-based sensor networks for localization in mining environments

Wireless sensor networks (WSN) have attached a great attention in the last few years. WSN offer several advantages over the traditional sensor networks, such as elimination of costly wires, security and larger area coverage. In the last few years, there has been great interest commercial applications based on Ultra Wideband (UWB). It has potentially low complexity, low cost and an excellent time domain resolution which facilitates location and tracking applications. Therefore, UWB provide a good combination of high performance with low complexity for WSN applications. Localization has attracted considerable attention in many fields. Accurate estimation of an individual location can be a difficult task, producing ambiguous results particularly in harsh environment. In this paper, we focus on feasibility study (via simulation and measurements) of using UWB based-WNS as future solution for localization in underground mines.     

Arghel Extract: a Promising Green Corrosion Inhibitor

Protection of Metals and Physical Chemistry of Surfaces - The corrosion of copper in an aqueous 0.5 M H2SO4 has been studied under the effect of Arghel plant extracts using potentiodynamic...     

Stabilization of controlled positive discrete‐time T‐S fuzzy systems by state feedback control

This paper deals with sufficient conditions of asymptotic stability and stabilization for nonlinear discrete‐time systems represented by a Takagi–Sugeno‐type fuzzy model whose state variables take only nonnegative values at all times t for any nonnegative initial state. This class of systems is called positive systems. The conditions of stabilizability are obtained with state feedback control. This work is based on multiple Lyapunov functions. The results are presented in linear matrix inequalities form. A real plant is studied to illustrate this technique. Copyright © 2010 John Wiley & Sons, Ltd.