The Ponseti approach for the treatment of congenital clubfoot

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Etiological diagnosis, prognostic significance and role of electrophysiological study in patients with Brugada ECG and syncope.

BACKGROUND: Syncope is considered a risk factor for life-threatening arrhythmias in Brugada patients. Distinguishing a benign syncope from one due to ventricular arrhythmias is often difficult, unless an ECG is recorded during the episode. Aim of the study was to analyze the characteristics of syncopal episodes in a large population of Brugada patients and evaluate the role of electrophysiological study (EPS) and the prognosis in the different subgroups. METHODS AND RESULTS: One hundred ninety-five Brugada patients with history of syncope were considered. Syncope were classified as neurally mediated (group 1, 61%) or unexplained (group 2, 39%) on the basis of personal and family history, clinical features, triggers, situations, associated signs, concomitant therapy. Most patients underwent EPS; they received ICD or implantable loop-recorder on the basis of the result of investigations and physician's judgment. At 62±45months of mean follow-up, group 1 showed a significantly lower incidence of arrhythmic events (2%) as compared to group 2 (9%, p<0.001). Group 2 patients with positive EPS showed the highest risk of arrhythmic events (27%). No ventricular events occurred in subjects with negative EPS. CONCLUSION: Etiological definition of syncope in Brugada patients is important, as it allows identifying two groups with different outcome. Patients with unexplained syncope and ventricular fibrillation induced at EPS have the highest risk of arrhythmic events. Patients presenting with neurally mediated syncope showed a prognosis similar to that of the asymptomatic and the role of EPS in this group is unproven

Postnatal survival after endoscopic equatorial laser for the treatment of twin-to-twin transfusion syndrome

Background Endoscopic laser coagulation of placental anastomoses is the first-line treatment for severe twin-to-twin transfusion syndrome. A recent randomized controlled trial reported that laser coagulation along the entire vascular equator was associated with a similar dual survival and survival of at least 1 twin compared with the group that was treated with the selective technique. In addition, there was a significantly lower incidence of postoperative recurrence of twin-to-twin transfusion syndrome and the development of twin anemia-polycythemia sequence in the equatorial group. Objective The purpose of this study was to report on neonatal survival in twin-to-twin transfusion syndrome pregnancies that were treated with endoscopic laser therapy with the use of the equatorial technique and to examine the relationship between preoperative factors and twin loss. Study Design Endoscopic equatorial laser therapy was carried out as the primary treatment for twin-to-twin transfusion syndrome in all consecutive monochorionic diamniotic twin pregnancies that were referred at a single fetal surgery Center over a 4-year period. All visible placental anastomoses were coagulated; additional laser ablation of the placental tissue between the coagulated vessels was carried out. Pre-laser ultrasound data, periprocedural complications, pregnancy outcome, and postnatal survival at hospital discharge were recorded and analyzed. Results A total of 106 pregnancies were treated during the study period. Median gestational age at laser therapy was 19.7 weeks (range, 15.1-27.6 weeks). There was postoperative recurrence of twin-to-twin transfusion syndrome or the development of twin anemia-polycythemia sequence in 2 (1.9%) and 2 (1.9%) cases, respectively. The survival rates of both and at least 1 twin were 56.6% and 83.0%, respectively. Donor survival was significantly lower compared with the recipient co-twin (64.2% vs 75.5%, respectively; P <.05). The rate of fetal death, which was the most common cause of twin loss, was significantly higher in donors compared with recipient fetuses (23.6% vs 10.4%, respectively; P <.05). In cases with absent or reversed end-diastolic velocity in the donor umbilical artery, dual and donor survival rates were significantly lower compared with the remaining twin-to-twin transfusion syndrome pregnancies (40.0% vs 64.8% and 40.0% vs 76.1%, respectively; P <.05). There were no significant differences between the 2 groups in the survival of at least 1 twin and in the recipient survival. Conclusions Endoscopic equatorial laser therapy was associated with a survival of both and at least 1 twin of approximately 55% and 83%, respectively, with a low rate of recurrent twin-to-twin transfusion syndrome and twin anemia-polycythemia sequence. In addition, the preoperative finding of abnormal donor umbilical artery Doppler on ultrasound identified a subgroup of twin-to-twin transfusion syndrome pregnancies with a lower dual survival rate caused by increased intrauterine deaths of donor twins

On the rehydration of organic layered double hydroxides to form low-ordered carbon/LDH nanocomposites

Low-ordered carbon/layered double hydroxide (LDH) nanocomposites were prepared by rehydration of the oxides produced by calcination of an organic LDH. While the memory effect is a widely recognized effect on oxides produced by inorganic LDH, it is unprecedented from the calcination/rehydration of organic ones. Different temperatures (400, 600, and 1100 °C) were tested on the basis of thermogravimetric data. Water, instead of a carbonate solution, was used for the rehydration, with CO2 available from water itself and/or air to induce a slower process with an easier and better intercalation of the carbonaceous species. The samples were characterized by X-ray powder diffraction (XRPD), infrared in reflection mode (IR), and Raman spectroscopies and scanning electron microscopy (SEM). XRPD indicated the presence of carbonate LDH, and of residuals of unreacted oxides. IR confirmed that the prevailing anion is carbonate, coming from the water used for the rehydration and/or air. Raman data indicated the presence of low-ordered carbonaceous species moieties and SEM and XRPD the absence of separated bulky graphitic sheets, suggesting an intimate mixing of the low ordered carbonaceous phase with reconstructed LDH. Organic LDH gave better memory effect after calcination at 400 °C. Conversely, the carbonaceous species are observed after rehydration of the sample calcined at 600 °C with a reduced memory effect, demonstrating the interference of the carbonaceous phase with LDH reconstruction and the bonding with LDH layers to form a low-ordered carbon/LDH nanocomposite

Extracellular calcium antagonizes forskolin-induced aquaporin 2 trafficking in collecting duct cells

BACKGROUND: Urinary concentrating defects and polyuria are the most important renal manifestations of hypercalcemia and the resulting hypercalciuria. In this study, we tested the hypothesis that hypercalciuria-associated polyuria in kidney collecting duct occurs through an impairment of the vasopressin-dependent aquaporin 2 (AQP2) water channel targeting to the apical membrane possibly involving calcium-sensing receptor (CaR) signaling. METHODS: AQP2-transfected collecting duct CD8 cells were used as experimental model. Quantitation of cell surface AQP2 immunoreactivity was performed using an antibody recognizing the extracellular AQP2 C loop. Intracellular cyclic adenosine monophosphate (cAMP) accumulation was measured in CD8 cells using a cAMP enzyme immunoassay kit. To study the translocation of protein kinase C (PKC), membranes or cytosol fractions from CD8 cells were subjected to Western blotting using anti-PKC isozymes antibodies. The amount of F-actin was determined by spectrofluorometric techniques. Intracellular calcium measurements were performed by spectrofluorometric analysis with Fura-2/AM. RESULTS: We demonstrated that extracellular calcium (Ca2+ o) (5 mmol/L) strongly inhibited forskolin-stimulated increase in AQP2 expression in the apical plasma membrane. At least three intracellular pathways activated by extracellular calcium were found to contribute to this effect. Firstly, the increase in cAMP levels in response to forskolin stimulation was drastically reduced in cells pretreated with Ca2+ o compared to untreated cells. Second, Ca2+ o activated PKC, known to counteract vasopressin response. Third, quantification of F-actin demonstrated that Ca2+ o caused a nearly twofold increase in F-actin content compared with basal conditions. All these effects were mimicked by a nonmembrane permeable agonist of the extracellular CaR, Gd3+. CONCLUSION: Together, these data demonstrate that extracellular calcium, possibly acting through the endogenous CaR, antagonizes forskolin-induced AQP2 translocation to the apical plasma membrane in CD8 cells. In hypercalciuria, this mechanism might blunt water reabsorption and prevent further calcium concentration, thus protecting against a potential risk of urinary calcium-containing stone formation

Chemical characterization of the stained glass window from the rose window, Siena Duomo (Italy, 1288-1289)

[EN] The chemical composition of nine medieval coloured stained glasses from Duomo (Catedral) of Siena, Italy, has been characterized. They come from the rose window elaborated under the drawing of Duccio di Buoninsegna masterwork (1288- 89 AD). This note explains the results obtained by EMPA, representative of bulk chemistry of several coloured glasses (deep green, olive green, yellow, purple, pink, deep blue, light blue, red plaqué and also uncoloured), as well as the associated trace elements (obtained by Induced Coupled Plasma Mass Spectrometry) that represent the chemical fingerprint of these glasses. The studied samples are sodium-calcium glass (chemical compositions in the range 13-14 wt% Na2O, 56-64 wt% SiO2, 4 wt% MgO, 9-10 wt% CaO, 2,5-4 wt% K2O); thus of Mediterranean tradition.This fact has been found by our team in previous studies (stained glasses from the church of Monestir de Pedralbes, Barcelona) for XIV century glass made at least 40 years later. As a general rule, the conservation state of these sodium glasses is good, except for the cohesion of grisaille to glass mesostase. This study allowed to identify three glass groups developed from different raw material formulations: a first group is constituted by deep green, olive green, light blue and yellow glass; a second one comprises by uncoloured, deep purple, deep blue and pink glass; and finally, the plaqué red glass that shows deeply different composition. Pink and yellow glass was produced following the traditional recipe compiled by Theophilus, a monk who lived at the beginning of XII century. This implies a separate process of raw material purification and a careful control of the redox kiln conditions; thus, these glasses can be considered as traditional or technologically not evolved. The deep blue, light blue and deep purple were obtained after the artisan dosed addition of a cobalt salt. Olive green and deep green glasses were produced with addition of copper (together with manganese and iron), previously prepared as a pigment that has as excipient a potassium glass. The use of potassium glass instead of the sodium glass locally produced strongly suggests that the pigment could be bought directly from Middle or Northern Europe markets and/or could be a sub-product of copper ore melting. On the other hand, the production of sodium red ruby plaqué glass, technologically more evolved and comparable to the coeval potassium glass coming from the Central Europe glass factories, would be outstanding in the XIV century; but we interpret (taking into account its chemical composition) that was produced later and introduced during the restoration conducted at the end of XVII century. We can also note the use of well-dosed lead additions in order to increase the lightness and transparency of coloured glass. Manganese has been a very important element in the Siena workshop glassmaker colour palette achievements. On the other hand, the trace-element chemical fingerprint of the glass allows arguing what kind of mineral salts were used as pigments, as well as the way to introduce it in the uncoloured original glass.[ES] Se han caracterizado químicamente una colección de vidrios arquitectónicos coloreados originales del rosetón del Duomo (catedral) de Siena, Italia, realizados bajo diseño del artista Duccio di Buoninsegna en 1288-89. Esta comunicación expone los resultados obtenidos mediante el empleo de microsonda electrónica de Castaing (mayoritarios) y espectrometría de masas con fuente de plasma acoplado inductivamente (ICP-MS, elementos en traza) en vidrios de varios colores (incoloro, verde oscuro, verde oliva, amarillo, violeta, rosa, azul oscuro, azul celeste, rojo plaqué). Se trata de vidrios sódico-cálcicos (valores en peso alrededor del 13-14 % de Na2O, 56-64 % SiO2, 4% MgO, 9-10 % CaO, 2,5-4 K2O) de tradición por tanto mediterránea. Un resultado semejante se encontró precedentemente para vidrios realizados a lo largo del siglo XIV, cuanto menos unos 40 años después (vidriera de la iglesia del Monestir de Pedralbes, Barcelona). En general, el estado de conservación de estos vidrios es bueno, excepto por lo que se refiere a la adherencia de las grisallas al vidrio base. El estudio desarrollado ha permitido identificar tres grupos de vidrios desarrollados a partir de formulaciones de diferentes composiciones: un primer grupo constituido por los vidrios de color verde claro, verde oscuro, azul celeste y amarillo; un segundo grupo constituido por los vidrios incoloro, violeta, azul oscuro, y malva; y finalmente el vidrio rojo plaqué, de composición netamente diferente a todos los demás. Desde el punto de vista de la obtención de los colores, cabe destacar que los datos químicos permiten deducir que el malva y el amarillo han sido elaborados siguiendo la receta tradicional del monje Theophilus de inicios del siglo XII, utilizando un proceso de purificación especial de las materias primas, y controlando artesanalmente las condiciones redox del horno; en este sentido, estos vidrios se pueden calificar como tradicionales o tecnológicamente poco evolucionados. Los colores azul oscuro, violeta y azul celeste se han obtenido mediante la adición de una misma sal de cobalto en diferentes dosis, y los colores verde oliva y verde oscuro mediante la adición de cobre (junto con manganeso y hierro), previamente preparados en un colorante que tiene como excipiente un vidrio potásico. Este último hecho (el empleo de vidrio potásico no usual en los centros productores del área mediterránea) permite suponer que el color puede haber sido comprado directamente a un fabricante centroeuropeo o que se tratara de un subproducto de fundición de minerales de cobre. Por su parte, la fabricación del vidrio plaqué, tecnológicamente más complejo y comparable al vidrio rojo plaqué potásico centroeuropeo contemporáneo indicaría, si fuera original, la incorporación de este proceso tecnológico al taller local. En opinión de los autores se trata de un vidrio de producción local mucho más tardia, incorporado en la restauración documentada a finales del siglo XVII. Destaca también, como en Pedralbes, el empleo del plomo para aumentar la luminosidad y transparencia del vidrio, en dosis variables atendiendo al color del vidrio. Por otro lado hay que señalar que en Siena el manganeso es un elemento muy importante con el que el fabricante del vidrio jugó intensamente para obtener la paleta de colores. El análisis de los metales presentes como elementos traza, y de las tierras raras, permite establecer sólidas hipótesis sobre el tipo de sales minerales empleadas como colorantes y como fueron incorporados al vidrio incoloro original.Este trabajo se ha podido realizar en el marco de diferentes proyectos financiados (proyecto 3338 de la Fundació Bosch i Gimpera, UB; acción integrada hispano-italiana HI2006-0190: La producción y el comercio del vidrio plano en Europa y en el Mediterráneo Occidental, entre los siglos XI y XV: un estudio arqueométrico) . La caracterización geoquímica de los vidrios mediante MSE e ICP-MS se ha llevado a cabo en los Serveis Científico-Tècnics de la Universitat de Barcelona (SCT-UB);Peer reviewe

Charge-transfer interactions stabilize g-quadruplex-forming thrombin binding aptamers and can improve their anticoagulant activity

In the search for optimized thrombin binding aptamers (TBAs), we herein describe the synthesis of a library of TBA analogues obtained by end-functionalization with the electron-rich 1,5-dialkoxy naphthalene (DAN) and the electron-deficient 1,8,4,5-naphthalenetetra-carboxylic diimide (NDI) moieties. Indeed, when these G-rich oligonucleotides were folded into the peculiar TBA G-quadruplex (G4) structure, effective donor–acceptor charge transfer interactions between the DAN and NDI residues attached to the extremities of the sequence were induced, providing pseudo-cyclic structures. Alternatively, insertion of NDI groups at both extremities produced TBA analogues stabilized by π–π stacking interactions. All the doubly-modified TBAs were characterized by different biophysical techniques and compared with the analogues carrying only the DAN or NDI residue and unmodified TBA. These modified TBAs exhibited higher nuclease resistance, and their G4 structures were markedly stabilized, as evidenced by increased Tm values compared to TBA. These favorable properties were also associated with improved anticoagulant activity for one DAN/NDI-modified TBA, and for one NDI/NDI-modified TBA. Our results indicated that TBA pseudo-cyclic structuring by ad hoc designed end-functionalization represents an efficient approach to improve the aptamer features, while pre-organizing and stabilizing the G4 structure but allowing sufficient flexibility to the aptamer folding, which is necessary for optimal thrombin recognition