Long-term effects of medical management on growth and weight in individuals with urea cycle disorders

Low protein diet and sodium or glycerol phenylbutyrate, two pillars of recommended long-term therapy of individuals with urea cycle disorders (UCDs), involve the risk of iatrogenic growth failure. Limited evidence-based studies hamper our knowledge on the long-term effects of the proposed medical management in individuals with UCDs. We studied the impact of medical management on growth and weight development in 307 individuals longitudinally followed by the Urea Cycle Disorders Consortium (UCDC) and the European registry and network for Intoxication type Metabolic Diseases (E-IMD). Intrauterine growth of all investigated UCDs and postnatal linear growth of asymptomatic individuals remained unaffected. Symptomatic individuals were at risk of progressive growth retardation independent from the underlying disease and the degree of natural protein restriction. Growth impairment was determined by disease severity and associated with reduced or borderline plasma branched-chain amino acid (BCAA) concentrations. Liver transplantation appeared to have a beneficial effect on growth. Weight development remained unaffected both in asymptomatic and symptomatic individuals. Progressive growth impairment depends on disease severity and plasma BCAA concentrations, but cannot be predicted by the amount of natural protein intake alone. Future clinical trials are necessary to evaluate whether supplementation with BCAAs might improve growth in UCDs

Photochromic sol-gel derived hybrid polymer coatings: The influence of matrix properties on kinetics and photo degradation

Photochromic dyes undergo a reversible change in their absorption characteristics upon UV light irradiation. By incorporating such photochromophores into sol-gel derived inorganic-organic hybrid polymers, versatile coatings with a fast photochromic response and high photochromic activity can be obtained. In the present study, the isomerization kinetics of spirooxazine dyes entrapped in hybrid polymer coatings were investigated in situ. The chemical properties of the matrices used were characterised in terms of their inorganic network connectivity (NMR measurements) and paramagnetic properties (EPR spectroscopy). Their photodegradation behaviour was studied by means of artificial weathering

Comparison of PEDOT films obtained via three different routes through spectroelectrochemistry and the differential cyclic voltabsorptometry method (DCVA)

The performance of different poly(3,4-ethylenedioxythiophene) (PEDOT) films was compared by electrochemical, spectroelectrochemical, and time-derivative measurements of absorbance versus potential (linear potential-scan voltabsorptometry) for an overall spectroelectrochemical characterization of the electrochromic properties in ionic liquids such as 1-butyl-3-methylimidazolium bis(trifluoromethylsulfonyl)imide (BMITFSI). The time-derivative signals were monitored at different wavelengths, and information obtained therefrom was complementary to that obtained from conventional cyclic voltammetry. PEDOT films prepared via in situ chemical oxidative polymerization appeared to be much more efficient than electropolymerized and PEDOT−poly(styrenesulfonate) (PSS) reference films, in terms of both contrast ratio and coloration efficiency, which was the case even for PEDOT films deposited on less conductive flexible plastic substrates

One-step preparation of viologen-TiO2 nanoparticles via a hydrothermally assisted sol-gel process for use in electrochromic films and devices

A one‐step method based on a hydrothermally assisted sol–gel process for the preparation of electrochromic viologen‐modified TiO2 nanoparticles is presented. The simplicity of the proposed one‐step synthesis and its usefulness for the subsequent preparation of transparent electrochromic films is demonstrated. In this method, the viologen moieties are attached during the growing/crystallization process of the TiO2 nanoparticles; therefore, neither sintering nor immersion into viologen solution of the resulting nanostructured film as in the standard methods is required. Thus, this novel approach is compatible with plastic substrates and easier to scale‐up. The morphology of the films is characterized by means of X‐ray diffraction, scanning electron microscopy, and ellipsometric porosimetry experiment, while the presence of viologen moieties in and throughout the film is detected by means of diffuse reflectance infrared Fourier transform and X‐ray photoelectron spectroscopy. Finally, full cells are assembled both on conducting glass and polyester substrates to demonstrate that electrochromic devices with excellent performance (ΔT% = 58% and 48%, respectively) can be made with films deposited from viologen‐TiO2 nanoparticulate dispersions obtained by this method

Hybrid-Nanopartikel fuer bioanalytische Anwendungen Schlussbericht

SIGLEAvailable from TIB Hannover: F03B394 / FIZ - Fachinformationszzentrum Karlsruhe / TIB - Technische InformationsbibliothekBundesministerium fuer Bildung und Forschung, Berlin (Germany)DEGerman