Molecular Electronics Including Temperature Effects Based on Dyes Pigments.

In this work we used the Density Functional Theory to study the thermodynamic properties from Brazilein (BZE) and Brazilin (BZI) molecules, main pigments responsible for the red color from Brazil wood. We did a comparison between the two dyes to then know which dye has better resistance to temperature (T ) and external electric field (E) values, aiming their potential to possible applications in solar cells, as excitons trainers. We have found that the BZE molecule becomes less stable after a temperature known as degradation temperature, and therefore enters oxidation state. However, BZE is more stable and more resistant to high temperatures. With respect to the applied external electric field, we find that BZE is more reactive to almost all the applied electric fields, thus more easily converted into energy in the form of electrical work.

Impact of the Bowel Management Program on the quality of life in children with fecal incontinence.

PURPOSE: Evaluate the impact of implementing Bowel Management Program (BMP) on quality of life (QoL) in children with fecal incontinence (FI). METHODS: Children 3-12 years referred to the Children's Treatment Center of FI were included and categorized accordingto the tendency to constipation (dilated colon; group 1) or diarrhea (non-dilated colon; group 2). Evaluations were performed before and after 3 months of BMP implementation. Perceptions from parents and children about QoL were assessed by the instrument PedsQL4.0 and the severity of FI by the fecal incontinence index (FII) of Wexner scale. RESULTS: 43 children (mean age of 7.7 ± 3.1) were studied. 32 (74.4 %) belonged to group 1 and 11 (25.6 %) to group 2. After 3 months of BMP, it was noticed a significant improvement of the FII (14.5 ± 3.7 versus 2.8 ± 2.5; p < 0.001) in both groups. QoL perception by the parents and children increased on PedsQL4.0 evaluation (p < 0.001). There was a medium inverse statistically significant correlation of the FII and the total PedsQL4.0 score among children and parents (r = -0.47, p = 0.009 and r = -0, 39, p = 0.03, respectively). CONCLUSION: The BMP applied by a multidisciplinary dedicated team significantly improves the QoL of children with FI in the short-term period.

Enzyme replacement therapy with galsulfase in 34 children younger than five years of age with MPS VI.

BACKGROUND: Mucopolysaccharidosis type VI (MPS VI) is a progressive, chronic and multisystem lysosomal storage disease with a wide disease spectrum. Clinical and biochemical improvements have been reported for MPS VI patients on enzyme replacement therapy (ERT) with rhASB (recombinant human arylsulfatase B; galsulfase, Naglazyme®, BioMarin Pharmaceutical Inc.), making early diagnosis and intervention imperative for optimal patient outcomes. Few studies have included children younger than five years of age. This report describes 34 MPS VI patients that started treatment with galsulfase before five years of age. METHODS: Data from patients who initiated treatment at <5 years of age were collected from patients' medical records. Baseline and follow-up assessments of common symptoms that led to diagnosis and that were used to evaluate disease progression and treatment efficacy were evaluated. RESULTS: A significant negative correlation was seen with treatment with ERT and urinary GAG levels. Of those with baseline and follow-up growth data, 47% remained on their pre-treatment growth curve or moved to a higher percentile after treatment. Of the 9 patients with baseline and follow-up sleep studies, 5 remained unaffected and 1 patient initially with mild sleep apnea showed improvement. Data regarding cardiac, ophthalmic, central nervous system, hearing, surgical interventions and development are also reported. No patient discontinued treatment due to an adverse event and all that were treatment-emergent resolved. CONCLUSIONS: The prescribed dosage of 1mg/kg IV weekly with galsulfase ERT is shown to be safe and effective in slowing and/or improving certain aspects of the disease, although patients should be closely monitored for complications associated with the natural history of the disease, especially cardiac valve involvement and spinal cord compression. A long-term follow-up investigation of this group of children will provide further information on the benefits of early treatment as well as disease progression and treatment efficacy and safety in this young patient population.