Neuromuscular disease: health care accessibility in the Nord-Pas-de- Calais region

A survey was conducted to estimate accessibility to current diagnostic techniques for patients with neuromuscular disease living in the Nord-Pas-de-Calais. In association with the "Association fran?aise contre les myopathies", we invited 200 adults with neuromuscular disease to fill out a questionnaire concerning diagnosis of their neuromuscular disease and medical and paramedical follow-up. Each subject answered the questionnaire anonymously providing data on sociodemographic items, diagnosis and medical and paramedical follow-up. The results showed a lack of follow-up for lung disease whereas respiratory failure is known as a frequent complication of neuromuscular disease. Genetic counselling was not suggested often enough. A large proportion (80 p. 100) of the patients had had physiotherapy. Whereas cardiomyopathy and orthopedic buckling with subsequent decreased autonomy are observed in 90 p. 100 of patients with Duchenne muscular dystrophy, only 42 p. 100 of the patients with this disease were followed by a cardiologist and a physical therapists. We suggest more cooperation between specialists in order to improve medical care for patients with neuromuscular diseases.     

Development of receptoral responses in pigmented and albino guinea-pigs (Cavia porcellus)

We describe the postnatal development of the electroretinogram (ERG) receptoral response in the guinea pig. In addition, the time course and nature of maturation was compared between albino and pigmented strains to consider the role that melanogenesis might have in this process. Electroretinograms were collected on groups of albino and pigmented animals from postnatal day (PD) PD1 to PD60. A-wave amplitudes and implicit times were extracted from filtered data (0–75 Hz). Receptoral components were modelled using the delayed gaussian model of Hood and Birch [1] fitted as an ensemble to the raw data. Guinea pigs show saturated amplitudes (Rm_P3) that are 50% of adult values at birth, these mature by PD12. Receptoral delay (t_d) also undergoes some postnatal maturation, while phototransduction gain (log S) is adult-like at birth. Albino animals had significantly (p<0.05) larger Rm_P3 and log S across all ages. Guinea pigs have significant postnatal development in their receptoral response. Maturation of Rm_P3 implies a postnatal increase in rod outer segment length. Whereas the adult values of log S implies a mature phototransduction process at birth. We argue that the likely cause for the larger log S of albino eyes is compatible with theories of increased levels of internal light. Whereas the larger Rm_P3, even after allowing for increased light effectiveness, may reflect a lower ocular resistance in albino eyes due to their lower levels of melanin. Furthermore, decreased Rm_P3 and log S with age is observed in the pigmented group only and is consistent with increased ocular resistance due to melanin development in this strain. This revised version was published online in July 2006 with corrections to the Cover Date.     

Association of KCNJ11 and ABCC8 single-nucleotide polymorphisms with type 2 diabetes mellitus in a Kinh Vietnamese population

Type 2 diabetes mellitus (T2DM) is a genetically influenced disease, but few studies have been performed to investigate the genetic basis of T2DM in Vietnamese subjects. Thus, the potential associations of KCNJ11 and ABCC8 single nucleotide polymorphisms (SNPs) with T2DM were investigated in a Kinh Vietnamese population. A cross-sectional study consisting of 404 subjects including 202 T2DM cases and 202 non-T2DM controls was designed to examine the potential associations of 4 KCNJ11 and ABCC8 SNPs (rs5219, rs2285676, rs1799859, and rs757110) with T2DM. Genotypes were identified based on restriction fragment length polymorphism and tetra-primer amplification refractory mutation system polymerase chain reaction. After statistically adjusting for age, sex, and BMI, rs5219 was found to be associated with an increased risk of T2DM under 2 inheritance models: codominant (OR = 2.15, 95% confidence intervals [CI] = 1.09–4.22) and recessive (OR = 2.08, 95%CI = 1.09–3.94). On the other hand, rs2285676, rs1799859, and rs757110 were not associated with an increased risk of T2DM. Haplotype analysis elucidated a strong linkage disequilibrium between the 3 SNPs, rs5219, rs2285676, and rs757110. The haplotype rs5219(A)/rs2285676(T)/rs757110(G) was associated with an increased risk of T2DM (OR = 1.42, 95%CI = 1.01–1.99). The results show that rs5219 is a lead candidate SNP associated with an increased risk of developing T2DM in the Kinh Vietnamese population. Further functional characterization is needed to uncover the mechanism underlying the potential genotype-phenotype associations.     

One-step hydrothermal preparation of Ta-doped ZnO nanorods for improving decolorization efficiency under visible light

In this work, Ta-doped ZnO (Ta-ZnO) nanomaterials were synthesized by the hydrothermal method at different temperatures (110, 150, and 170 °C) for the photodegradation of methylene blue (MB) under visible light. Ta doping significantly affects the crystal defects, optical properties, and MB photocatalytic efficiency of ZnO materials. The optical absorption edge of Ta-ZnO 150 was redshifted compared to undoped ZnO, correlating to bandgap narrowing (E_gTa–ZnO = 2.92 eV; E_gZnO = 3.07 eV), implying that Ta doped ZnO is capable of absorbing visible light. Besides, Ta-doping was the reason for enhanced blue light emission in the photoluminescence spectrum, which is related to the oxygen defect V⁰_O. It is also observed in the XPS spectra, where the percentage of oxygen in the oxygen-deficient regions (O_531.5 eV) of Ta-ZnO150 is higher than that of ZnO150. It is an important factor in enhancing ZnO''s photocatalytic efficiency. The MB degradation efficiency of Ta-doped ZnO reached the highest for Ta-ZnO 150 and was 2.5 times higher than ZnO under a halogen lamp (HL). Notably, the influence of hydrothermal temperature on the structural, morphological, and photoelectrochemical properties was discussed in detail. As a result, the optimal hydrothermal temperature for synthesizing the nanorod is 150 °C. Furthermore, photocatalytic experiments were also performed under simulated sunlight and natural sunlight. The nature of the photo-oxidative degradation of MB was also investigated.

The Ta doped ZnO nanorods (Ta-ZnO) were synthesized by a hydrothermal method at different temperatures (110, 150, and 170 °C) for the photodegradation of methylene blue (MB) under visible light.     

High Pressure Compression-Molding of α-Cellulose and Effects of Operating Conditions

Commercial α-cellulose was compression-molded to produce 1A dog-bone specimens under various operating conditions without any additive. The resulting agromaterials exhibited a smooth, plastic-like surface, and constituted a suitable target as replacement for plastic materials. Tensile and three-points bending tests were conducted according to ISO standards related to the evaluation of plastic materials. The specimens had strengths comparable to classical petroleum-based thermoplastics. They also exhibited high moduli, which is characteristic of brittle materials. A higher temperature and higher pressure rate produced specimens with higher mechanical properties while low moisture content produced weaker specimens. Generally, the strong specimen had higher specific gravity and lower moisture content. However, some parameters did not follow the general trend e.g., thinner specimen showed much higher Young’s Modulus, although their specific gravity and moisture content remained similar to control, revealing a marked skin-effect which was confirmed by SEM observations.