The kinetic and thermodynamic analysis of ultrasound-extraction of minerals from aerial parts of white lady's bedstraw (Galium mollugo L.)

The present paper deals with the extraction of some minerals (potassium, calcium and magnesium) from ground, dried aerial parts of white lady's bedstraw (Galium mollugo L.) using an aqueous ethanol solution (50% by volume) at different temperatures (from 23 to 40 °C) in the presence and absence of ultrasound. The main goal was to establish the kinetics and the thermodynamics of the two extraction processes. A phenomenological model involving simultaneous washing and diffusion was proved for both ultrasound-assisted and silent extraction of the minerals. The minerals extraction both in the presence and the absence of ultrasound is endothermic and irreversible since the enthalpy change and the entropy change are positive in the ranges of extraction temperature applied. The Gibbs free energy change for the extraction of minerals is negative, indicating that the process is feasible and spontaneous. The minerals yield increased by a factor of 1.02–1.06 and 1.14–1.27 in the absence and the presence of ultrasound, respectively for every 10 °C rise in temperature.     

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.     

Real Porous Media: Local Geometry and Transports

Our recent progress in the analysis and reconstruction of real porous media are surveyed and some emphasis is put on the two-field and on the grain reconstruction methods. Then, after a short summary of our previous analyses of transports, two recent developments are presented, namely, the determination of the resistivity index and of the dispersion tensor in multiphase flow.     

Study of the Magneto-Resistivity and Dependence of Percolation in La0.75Ca0.1Sr0.15Mn1−x Ga x O3 Compounds

We present a study of the effect of Ga-doping on the physical properties of La_0.75Ca_0.1Sr_0.15Mn_1?x Ga _x O₃ (x=0.025, 0.075 and 0.1) compounds prepared by the sol-gel method. The variation of the magnetization (M) vs. temperature (T), under an applied magnetic field of 0.05 T, reveals a ferromagnetic–paramagnetic transition for all samples. The magnetization behavior and the Curie temperature T _C have shown a large dependence on the fractional composition x. In fact, the M(T) curves have revealed the presence of a long-range ferromagnetic state below T _C. The magnetotransport properties have been investigated based on the temperature dependence of the resistivity ρ(T) measurements. We have noted that these samples present an electrical transition from a paramagnetic-semiconductor state to a ferromagnetic-metallic one, when decreasing temperature. We have used the phenomenological equation for conductivity under a percolation approach, which is based upon an approach that the system consists of the phase separated ferromagnetic metallic and paramagnetic insulating regions. We have then fitted the resistivity data measured in the range of 15–300 K and found that the activation barrier decreases with the raising Ga³⁺ ion concentration.     

Synthesis,Crystal Structure,Vibrational and Magnetic Properties of a New Organic–inorganic Hybrid Material: 2-Amonium-methyl-1-Ethylpyrrolidinium Tetrachlorocuprate (II)

Synthesis, crystal structure, vibrational study and magnetic properties of the compound (C₇H₁₈N₂) CuCl₄ are reported. The latter crystallizes in the monoclinic system (space group P2 ₁, Z = 4) with the following unit cell dimensions: a = 7.569(5) Å b = 14.174(5) Å, c = 13.193(2) Å and β = 105.53(3)^°. Besides, its structure was solved using 5754 independent reflections down to R = 0.0475. The atomic arrangement can be described by alternating organic and inorganic layers stacked in the [\(\bar {1}\)01] direction, made up of tetrahedral of tetrachlorocuprate CuCl\(_{4}^{2-}\) sandwiched between two organic layers. In crystal structure, the inorganic layer, built up by independent monomeric [CuCl₄]^2?, is connected to the organic ones through hydrogen bonding in order to build a three-dimensional network. The magnetic behavior of (C₇H₁₈N₂) CuCl₄ samples was investigated as a function of temperature and applied field. At hightemperature paramagnetic behavior, and at low temperature, evidence for weak ferromagnetism, reinforced by a hysteresis loop at 2 K is observed. The magnetic behavior of (C₇H₁₈N₂) CuCl₄ is attributed to Cu (II) due to the presence of an active Jahn–Teller effect in the d ⁹ electronic system, which give rise to short-range weak ferromagnetism.     

Heterogenous Clinical Landscape in a Consanguineous Malonic Aciduria Family

Malonic aciduria is an extremely rare inborn error of metabolism due to malonyl-CoA decarboxylase deficiency. This enzyme is encoded by the MLYCD (Malonyl-CoA Decarboxylase) gene, and the disease has an autosomal recessive inheritance. Malonic aciduria is characterized by systemic clinical involvement, including neurologic and digestive symptoms, metabolic acidosis, hypoglycemia, failure to thrive, seizures, developmental delay, and cardiomyopathy. We describe here two index cases belonging to the same family that, despite an identical genotype, present very different clinical pictures. The first case is a boy with neonatal metabolic symptoms, abnormal brain MRI, and dilated cardiomyopathy. The second case, the cousin of the first patient in a consanguineous family, showed later symptoms, mainly with developmental delay. Both patients showed high levels of malonylcarnitine on acylcarnitine profiles and malonic acid on urinary organic acid chromatographies. The same homozygous pathogenic variant was identified, c.346C > T; p. (Gln116*). We also provide a comprehensive literature review of reported cases. A review of the literature yielded 52 cases described since 1984. The most common signs were developmental delay and cardiomyopathy. Increased levels of malonic acid and malonylcarnitine were constant. Presentations ranged from neonatal death to patients surviving past adolescence. These two cases and reported patients in the literature highlight the inter- and intrafamilial variability of malonic aciduria.