The Ped-APS registry: The antiphospholipid syndrome in childhood

In recent years, antiphospholipid syndrome (APS) has been increasingly recognised in various paediatric autoimmune and nonautoimmune diseases, but the relatively low prevalence and heterogeneity of APS in childhood made it very difficult to study in a systematic way. The project of an international registry of paediatric patients with APS (the Ped-APS Registry) was initiated in 2004 to foster and conduct multicentre, controlled studies with large number of paediatric APS patients. The Ped-APS Registry is organised as a collaborative project of the European Forum on Antiphospholipid Antibodies and Juvenile Systemic Lupus Erythematosus Working Group of the Paediatric Rheumatology European Society. Currently, it documents a standardised clinical, laboratory and therapeutic data of 133 children with antiphospholipid antibodies (aPL)-related thrombosis from 14 countries. The priority projects for future research of the Ped-APS Registry include prospective enrolment of new patients with aPL-related thrombosis, assessment of differences between the paediatric and adult APS, evaluation of proinflammatory genotype as a risk factor for APS manifestations in childhood and evaluation of patients with isolated nonthrombotic aPL-related manifestations. © The Author(s), 2009.

Artifactual oxidation of cholesterol during the analysis of cholesterol oxidation products: protective effect of antioxidants

To study the influence of the addition of various antioxidants and their combinations on the artifactual oxidation of cholesterol during analysis, 2 factorial experiments were performed in duplicate. In the first experiment, 2 amounts of the following antioxidants were assayed: ethylenediaminetetraacetic acid (EDTA) disodium salt (0 and 1 mg), pyrogallol (0 and 600 microg), and butylated hydroxytoluene (BHT; 0 and 600 microg); in the second, EDTA disodium salt (0 and 1 mg), ascorbyl palmitate (0 and 600 microg), and BHT (0 and 600 microg). Under low oxidative conditions of dim light, evaporation of solvents at low temperatures, and cold saponification in darkness under nitrogen atmosphere, the addition of antioxidants showed no further protective effect. Furthermore, the presence of ascorbyl palmitate significantly increased the formation of cholesterol-5beta,6beta-epoxide, and 7beta-hydroxycholesterol.

Anti-inflammatory and immunosuppressive drugs and reproduction

Rheumatic diseases in women of childbearing years may necessitate drug treatment during a pregnancy, to control maternal disease activity and to ensure a successful pregnancy outcome. This survey is based on a consensus workshop of international experts discussing effects of anti-inflammatory, immunosuppressive and biological drugs during pregnancy and lactation. In addition, effects of these drugs on male and female fertility and possible long-term effects on infants exposed to drugs antenatally are discussed where data were available. Recommendations for drug treatment during pregnancy and lactation are given.

Aprender a ser docentes con el cine de Miyazaki

El presente artículo plantea la incorporación del cine como argumento válido para la formación del profesorado. Consideramos que el cine incluye suficientes mecanismos para involucrar la educación en artes visuales, la educación en medios, y la educación musical. Entre los directores más relevantes del panorama internacional, proponemos la figura de Hayao Miyazaki. Este creador japonés ha sabido atraer a públicos de todas las edades y culturas a lo largo de su dilatada carrera profesional. La calidad técnica de los trabajos de Miyazaki va acompañada de una serie de valores que resultan muy válidos para incorporar acciones educativas de respeto al medio natural y a las tradiciones culturales. Pensamos que los maestros de educación primaria han de estar preparados para fomentar este tipo de valores entre el alumnado. Tanto la educación artística como el cine son entornos muy atractivos para lograrlo. Hemos observado y analizado a un grupo de estudiantes de grado de Maestro de primaria mientras miraban la película Ponyo en el acantilado. Planteamos un mayor uso del cine como recurso educativo (Ambrós y Breu, 2007; Fedorov, 2010). Animamos al profesorado y al alumnado a realizar nuevas lecturas críticas y personales del cine, indagando más allá del conocimiento del medio como mero entretenimiento (Clarembeaux, 2010; Hernández, 2000; Huerta, 2006)

Aprender a ser docentes con el cine de Miyazaki

El presente artículo plantea la incorporación del cine como argumento válido para la formación del profesorado. Consideramos que el cine incluye suficientes mecanismos para involucrar la educación en artes visuales, la educación en medios, y la educación musical. Entre los directores más relevantes del panorama internacional, proponemos la figura de Hayao Miyazaki. Este creador japonés ha sabido atraer a públicos de todas las edades y culturas a lo largo de su dilatada carrera profesional. La calidad técnica de los trabajos de Miyazaki va acompañada de una serie de valores que resultan muy válidos para incorporar acciones educativas de respeto al medio natural y a las tradiciones culturales. Pensamos que los maestros de educación primaria han de estar preparados para fomentar este tipo de valores entre el alumnado. Tanto la educación artística como el cine son entornos muy atractivos para lograrlo. Hemos observado y analizado a un grupo de estudiantes de grado de Maestro de primaria mientras miraban la película Ponyo en el acantilado. Planteamos un mayor uso del cine como recurso educativo (Ambrós y Breu, 2007; Fedorov, 2010). Animamos al profesorado y al alumnado a realizar nuevas lecturas críticas y personales del cine, indagando más allá del conocimiento del medio como mero entretenimiento (Clarembeaux, 2010; Hernández, 2000; Huerta, 2006)

Aprender a ser docentes con el cine de Miyazaki

El presente artículo plantea la incorporación del cine como argumento válido para la formación del profesorado. Consideramos que el cine incluye suficientes mecanismos para involucrar la educación en artes visuales, la educación en medios, y la educación musical. Entre los directores más relevantes del panorama internacional, proponemos la figura de Hayao Miyazaki. Este creador japonés ha sabido atraer a públicos de todas las edades y culturas a lo largo de su dilatada carrera profesional. La calidad técnica de los trabajos de Miyazaki va acompañada de una serie de valores que resultan muy válidos para incorporar acciones educativas de respeto al medio natural y a las tradiciones culturales. Pensamos que los maestros de educación primaria han de estar preparados para fomentar este tipo de valores entre el alumnado. Tanto la educación artística como el cine son entornos muy atractivos para lograrlo. Hemos observado y analizado a un grupo de estudiantes de grado de Maestro de primaria mientras miraban la película Ponyo en el acantilado. Planteamos un mayor uso del cine como recurso educativo (Ambrós y Breu, 2007; Fedorov, 2010). Animamos al profesorado y al alumnado a realizar nuevas lecturas críticas y personales del cine, indagando más allá del conocimiento del medio como mero entretenimiento (Clarembeaux, 2010; Hernández, 2000; Huerta, 2006)

Obra completa

Contiene: Poesia. I. Volar ; La inquietud en calma ; Jo sense tu ; On vaig, senyor? ; Aires de cançó ; Goig ; Esparses ; Braçat ; Poema dramàticament esperançat. II. Home ; Jo, cap de casa ; D'amar-te, amor ; Silenci ; Jo també tinc set ; Gran Sonata de la Pàtria ; Schumann, el piano i yo ; 50 anys del 7 d'octubre ; Soc qui soc ; Maror ; Vinatea ; Cantata valenciana de l'anima del cor ; Inèdites, diverses, disperses i rares / edició, estudi introductori i notes de Ricard Bellveser -- Prosa. I. Flexions i reflexions ; Memories d'un important sense importància ; Proses en carn ; Confesión con Ausiàs March ; Miscelànea. II. Artículs periodístics / Vicent Ll. Simó Santonja (coord.)

Nuclear translocation of NF-κB is increased in dopaminergic neurons of patients with Parkinson disease

Evidence from postmortem studies suggest an involvement of oxidative stress in the degeneration of dopaminergic neurons in Parkinson disease (PD) that have recently been shown to die by apoptosis, but the relationship between oxidative stress and apoptosis has not yet been elucidated. Activation of the transcription factor NF-κB is associated with oxidative stress-induced apoptosis in several nonneuronal in vitro models. To investigate whether it may play a role in PD, we looked for the translocation of NF-κB from the cytoplasm to the nucleus, evidence of its activation, in melanized neurons in the mesencephalon of postmortem human brain from five patients with idiopathic PD and seven matched control subjects. In PD patients, the proportion of dopaminergic neurons with immunoreactive NF-κB in their nuclei was more than 70-fold that in control subjects. A possible relationship between the nuclear localization of NF-κB in mesencephalic neurons of PD patients and oxidative stress in such neurons has been shown in vitro with primary cultures of rat mesencephalon, where translocation of NF-κB is preceded by a transient production of free radicals during apoptosis induced by activation of the sphingomyelin-dependent signaling pathway with C2-ceramide. The data suggest that this oxidant-mediated apoptogenic transduction pathway may play a role in the mechanism of neuronal death in PD.

Fibroblast Growth Factor (FGF) Soluble Receptor 1 Acts as a Natural Inhibitor of FGF2 Neurotrophic Activity during Retinal Degeneration

Fibroblast growth factors (FGF) 1 and 2 and their tyrosine kinase receptor (FGFR) are present throughout the adult retina. FGFs are potential mitogens, but adult retinal cells are maintained in a nonproliferative state unless the retina is damaged. Our work aims to find a modulator of FGF signaling in normal and pathological retina. We identified and sequenced a truncated FGFR1 form from rat retina generated by the use of selective polyadenylation sites. This 70-kDa form of soluble extracellular FGFR1 (SR1) was distributed mainly localized in the inner nuclear layer of the retina, whereas the full-length FGFR1 form was detected in the retinal Muller glial cells. FGF2 and FGFR1 mRNA levels greatly increased in light-induced retinal degeneration. FGFR1 was detected in the radial fibers of activated retinal Muller glial cells. In contrast, SR1 mRNA synthesis followed a biphasic pattern of down- and up-regulation, and anti-SR1 staining was intense in retinal pigmented epithelial cells. The synthesis of SR1 and FGFR1 specifically and independently regulated in normal and degenerating retina suggests that changes in the proportion of various FGFR forms may control the bioavailability of FGFs and thus their potential as neurotrophic factors. This was demonstrated in vivo during retinal degeneration when recombinant SR1 inhibited the neurotrophic activity of exogenous FGF2 and increased damaging effects of light by inhibiting endogenous FGF. This study highlights the significance of the generation of SR1 in normal and pathological conditions.

Flexibility of the Kir6.2 inward rectifier K+ channel pore

Interactions of sulfhydryl reagents with introduced cysteines in the pore-forming (Kir6.2) subunits of the KATP channel were examined. 2-Aminoethyl methanethiosulfonate (MTSEA+) failed to modify Cd2+-insensitive control-Kir6.2 channels, but rapidly and irreversibly modified Kir6.2[L164C] (L164C) channels. Although a single Cd2+ ion is coordinated by L164C, four MTSEA+ “hits” can occur, each sequentially reducing the single-channel current. A dimeric fusion of control-Kir6.2 and L164C subunits generates Cd2+-insensitive channels, confirming that at least three cysteines are required for coordination, but MTSEA+ modification of the dimer occurs in two hits. L164C channels were not modified by bromotrimethyl ammoniumbimane (qBBr+), even though qBBr+ caused voltage-dependent block (as opposed to modification) that was comparable to that of MTSEA+ or 3-(triethylammonium)propyl methanethiosulfonate (MTSPTrEA+), implying that qBBr+ can also enter the inner cavity but does not modify L164C residues. The Kir channel pore structure was modeled by homology with the KcsA crystal structure. A stable conformation optimally places the four L164C side chains for coordination of a single Cd2+ ion. Modification of these cysteines by up to four MTSEA+ (or three MTSPTrEA+, or two qBBr+) does not require widening of the cavity to accommodate the derivatives within it. However, like the KcsA crystal structure, the energy-minimized model shows a narrowing at the inner entrance, and in the Kir6.2 model this narrowing excludes all ions. To allow entry of ions as large as MTSPTrEA+ or qBBr+, the entrance must widen to >8 Å, but this widening is readily accomplished by minimal M2 helix motion and side-chain rearrangement.

Evidence for the Critical Role of Sucrose Synthase for Anoxic Tolerance of Maize Roots using a Double Mutant

The induction of the sucrose synthase (SuSy) gene (SuSy) by low O2, low temperature, and limiting carbohydrate supply suggested a role in carbohydrate metabolism under stress conditions. The isolation of a maize (Zea mays L.) line mutant for the two known SuSy genes but functionally normal showed that SuSy activity might not be required for aerobic growth and allowed the possibility of investigating its importance during anaerobic stress. As assessed by root elongation after return to air, hypoxic pretreatment improved anoxic tolerance, in correlation with the number of SuSy genes and the level of SuSy expression. Furthermore, root death in double-mutant seedlings during anoxic incubation could be attributed to the impaired utilization of sucrose (Suc). Collectively, these data provide unequivocal evidence that Suc is the principal C source and that SuSy is the main enzyme active in Suc breakdown in roots of maize seedlings deprived of O2. In this situation, SuSy plays a critical role in anoxic tolerance.