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To expand the application of magnesium oxychloride cement (MOC) as a kind of engineering materials in the construction field, organic–inorganic hybrid modifications were performed. Waterborne epoxy resin/magnesium oxychloride composites (WER/MOC) with high-strength and water-resistance were prepared using a simple and green method with MOC as matrix and WER as reinforcement. The effects of WER on the resulting composite’s viscosity, mechanical strength, water resistance, phase composition, thermal stability, and micromorphology were discussed. The results demonstrated that WER reduced the viscosity of the WER/MOC complex system, and the composite water resistance and compressive strength were improved, which were mainly attributed to chemical reactions between WER and MOC to form a crosslinked network structure. Meanwhile, XRD analysis results showed that the intensity of the diffraction peak of the 3-phase decreased and 5-phase increased with WER addition to the composite and no new crystal phase appeared. The compressive strength, water resistance, thermal stability, and porosity of these WER/MOC composites reached the best values when the WER mass proportion was 4.8%. Here, the composite water absorption rate decreased to 8.7%, softening coefficient increased to 17.8%, middle compressive strength (sealed for 7 d) at 48.0 MPa, and dry bulk density increased to 1.34 g/cm3. Thus, these WER/MOC composites were suitable for use as engineering materials in the construction and road industries.

A new class of organic–inorganic hybrid materials were prepared by combining Cu-HMS with a silylation agent, trimethylchlorosilane (TMCS) via a simple silylation process at different silylation temperatures. They were characterized by a series of techniques including FT-IR, powder XRD, Nitrogen adsorption–desorption, TG analysis and water adsorption capacity test. It was demonstrated that silylation of PdCl2/Cu-HMS catalysts with TMCS enhanced their hydrophobicity, improved their activity and stability and importantly kept the excellent selectivity to diethyl carbonate (DEC) by oxidative carbonylation of ethanol in the gas-phase reaction. Moreover, the silylated samples obtained at 60 °C showed a better conversion of EtOH of 6.1 % and STY of DEC of 140.8 mg g−1 h−1.

Background: Familial hypomagnesemia with secondary hypocalcemia (HSH) is a rare autosomal recessive disease characterized by severe hypomagnesemia and hypocalcemia associated with neurological symptoms, including generalized seizures, tetany and muscle spasms, which are refractory to anticonvulsant treatment. The pathophysiological hallmarks of HSH are the impaired intestinal absorption of magnesium accompanied by renal magnesium wasting as a result of a reabsorption defect in the distal convoluted tubule. Mutations in TRPM6, the gene encoding the transient receptor potential cation channel subfamily member 6, have been found to be responsible for this disease. In the present study, we report a Chinese family with 2 sisters affected with severe HSH, and elucidate the characteristics of TRPM6 gene mutations in these 2 patients. Methods: We evaluated the clinical, laboratory, and radiographic findings. All 39 TRPM6 exons and flanking exon-intron junctions from genomic DNA were amplified and sequenced in 2 affected members suffering from HSH and their family. Results: We found two novel mutations in the family, one frameshift mutation (c.1196delC) and one non-sense mutation (c.4577G>A). These mutations were predicted to result in a complete loss of function of TRPM6. Both of the sisters were compound heterozygotes for these mutations. Conclusion: Our results suggested that the compound heterozygous mutations in TRPM6 were responsible for HSH in the Chinese family.

Pycnodysostosis is a rare autosomal recessive skeletal dysplasia characterized by short stature, osteosclerosis, acro-osteolysis, frequent fractures, and skull deformities. Mutation in the gene encoding cathepsin K (CTSK), which is a lysosomal cysteine protease, has been found to be responsible for this disease. Here we reported a consanguineous Chinese family with 1 affected individual demonstrating autosomal recessive pycnodysostosis with recurrent kidney stone, a new clinical manifestation which has not been reported in patients of pycnodysostosis before. To identify the pathogenic mutation, we evaluated the patient clinically, biochemically, and radiographically. To screen for mutations in the CTSK gene of the patient and his family members, all of its exons and exon-intron junctions were PCR amplified from genomic DNA and sequenced. Sequence analysis of the patient's CTSK gene revealed homozygosity for a missense mutation (c.746T>C) in exon 6, which leads to amino change (p.Ile249Thr) in the mature CTSK protein. This mutation was firstly reported by Michela Donnarumma and his colleagues in 2007 in a Spanish family. Our study strengthens the role of this particular mutation in the pathogenesis of pycnodysostosis.

A decade ago, only two hormones, parathyroid hormone and 1,25(OH)2D, were widely recognized to directly affect phosphate homeostasis. Since the discovery of fibroblast growth factor 23 (FGF23) in 2000 (1), our understanding of the mechanisms of phosphate homeostasis and of bone mineralization has grown exponentially. FGF23 is the link between intestine, bone, and kidney together in phosphate regulation. However, we still do not know the complex mechanism of phosphate homeostasis and bone mineralization. The physiological role of FGF23 is to regulate serum phosphate. Secreted mainly by osteocytes and osteoblasts in the skeleton (2,3), it modulates kidney handling of phosphate reabsorption and calcitriol production. Genetic and acquired abnormalities in FGF23 structure and metabolism cause conditions of either hyper-FGF23 or hypo-FGF23. Hyper-FGF23 is related to hypophosphatemia, while hypo-FGF23 is related to hyperphosphatemia. Both hyper-FGF23 and hypo-FGF23 are detrimental to humans. In this review, we will discuss the pathophysiology of FGF23 and hyper-FGF23 related renal phosphate wasting disorders (4).

Familial hypomagnesemia with secondary hypocalcemia (HSH) is a rare autosomal recessive disease characterized by severe hypomagnesemia and hypocalcemia associated with neurological symptoms, including generalized seizures, tetany and muscle spasms, which are refractory to anticonvulsant treatment. The pathophysiological hallmarks of HSH are the impaired intestinal absorption of magnesium accompanied by renal magnesium wasting as a result of a reabsorption defect in the distal convoluted tubule. Mutations in TRPM6, the gene encoding the transient receptor potential cation channel subfamily member 6, have been found to be responsible for this disease. In the present study, we report a Chinese family with 2 sisters affected with severe HSH, and elucidate the characteristics of TRPM6 gene mutations in these 2 patients.We evaluated the clinical, laboratory, and radiographic findings. All 39 TRPM6 exons and flanking exon-intron junctions from genomic DNA were amplified and sequenced in 2 affected members suffering from HSH and their family.We found two novel mutations in the family, one frameshift mutation (c.1196delC) and one non-sense mutation (c.4577GA). These mutations were predicted to result in a complete loss of function of TRPM6. Both of the sisters were compound heterozygotes for these mutations.Our results suggested that the compound heterozygous mutations in TRPM6 were responsible for HSH in the Chinese family.

The dynamic characteristics of evapotranspiration in latored soil on hills of subtropics regions in south China was analyzed. The results showed that evapotranspiration presented annual and seasonal fluctuations. The maximum monthly evapotranspiration was 10.80-15.41 times of the monthly minimum. The evapotranspiration in wet season (March to September) accounted for about 77% of annual total evapotranspiration, and that in dry season (October to February of next year) accounted for about 23%. Although the amount of annual rainfalls could balance annual total evapotranspiration, rainfalls were insufficient for evapotranspiration in the dry season, and soil water could be depleted by evapotranspiration. Based on the modified Penman equation, the calculation model of evapotranspiration in latored soil on hills of subtropics regions in south China was set up. By comparing modeling results with experimental data, it was proved that the calculation model was very reliable.