Here we report the case of a 73-year-old Italian woman affected by genetically confirmed oculopharyngeal muscular dystrophy (OPMD) with a negative family history. As OPMD is usually transmitted as an ...autosomal-dominant meiotically stable trait, this case allows us to suggest that putative de novo OPMD mutations might occur more frequently than previously thought; moreover, when compatible with a proper clinical phenotype, OPMD might be included in the differential diagnosis even in the absence of a positive family history.
Objectives: To survey current practice and to compare the opinion of paediatricians and anaesthesiologists regarding laryngeal mask airway (LMA) in neonatal resuscitation.
Design: A structured postal ...questionnaire on the use of the laryngeal mask airway in neonatal resuscitation was sent to the heads of department of the paediatric and anaesthesiology services.
Setting: Forty-three hospitals in the Veneto Region, Italy.
Results: During the year 2000, 1526 out of 33708 (4.5%) neonates in our region needed resuscitation. Of these cases, 101 (6.6%) were ventilated using the LMA. Laryngeal mask airway availability was significantly greater in the anaesthesiology department compared to the paediatric department (90% versus 50%;
P=0.002). However, 52% of anaesthesiologists and 72% of paediatricians had never used the laryngeal mask airway in their practice. The laryngeal mask airway was considered as an essential device more frequently by the anaesthesiologists than by the paediatricians (27% versus 5%;
P=0.015); both groups considered the laryngeal mask airway particularly useful in specific situations. Interestingly, while 16% of the paediatricians described the laryngeal mask airway as having no value, none of the anaesthesiologists did (
P=0.002). Staff competence was considered low by 70% of anaesthesiology heads of department compared with 90% of their pediatric colleagues. In both specialties, use of the laryngeal mask airway was limited to medical staff. With regard to training, 35% of anaesthesiologists and 22.5% of paediatricians had attended a course on laryngeal mask airway use.
Conclusions: Laryngeal mask airway availability and perceived value were higher amongst anaesthesiologists than their paediatric colleagues. However, educational level, competence and utilization rates of the LMA in neonatal resuscitation were low in both groups.
Objectivos: Avaliar a prática corrente e comparar a opinião de Pediatras e Anestesiologistas em relação à máscara ları́ngea (LMA) em reanimação neonatal.
Modelo: Foi enviado um questionário postal estruturado aos directores de departamento dos serviços de pediatria e anestesiologia sobre a utilização da LMA em reanimação neonatal.
Ambiente: Quarenta e três hospitais da região do Veneto, Itália.
Resultados: Durante o ano 2000, de 33708 recém-nascidos na nossa região, 1526 (4.5%) precisaram de reanimação. Destes, 101 (6.6%) foram ventilados com a LMA. A disponibilidade de LMAs foi significativamente maior nos serviços de anestesiologia quando comparada com os serviços de pediatria (90% versus 50%;
P = 0.002). No entanto, 52% dos anestesiologistas e 72% dos pediatras nunca tinham utilizado uma LMA na sua prática clı́nica. A LMA era considerada, instrumento essencial, com maior frequência pelos anestesiologistas do que pelos pediatras (27% versus 5%;
P = 0.015); ambos os grupos consideraram a LMA particularmente útil em situações especı́ficas. 16% dos pediatras consideraram que a LMA não tinha qualquer valor, mas nenhum dos anestesiologistas o fez (
P = 0.002). A competência dos operacionais foi considerada baixa por 70% dos directores de departamento de anestesiologia comparado com 90% dos seus colegas de pediatria. Em ambas as especialidades a utilização da LMA estava limitada ao pessoal médico. Em relação ao treino, 35% dos anestesiologistas e 22.5% dos pediatras tinham frequentado um curso sobre a utilização da LMA.
Conclusões: A disponibilidade e o valor atribuı́do à LMA era maior entre anestesiologistas que entre pediatras. No entanto, o nı́vel educacional, a competência e as taxas de utilização da LMA em ressuscitação neonatal eram baixas em ambos os grupos.
Objetivos: Evaluar la práctica actual y comparar la opinión de pediatras y anestesiólogos en relación con la máscara ları́ngea (LMA) en la reanimación neonatal.
Diseño: Se envió un cuestionario estructurado acerca del uso de la MLA en la reanimación neonatal, a los jefes de departamento de los servicios de pediatrı́a y anestesiologı́a.
Ambiente: Cuarenta y tres hospitales en la región de Veneto, Italia.
Resultados: Durante el año 2000, 1526 de 33708 (4.5%) de los neonatos de nuestra región requirieron resucitación. De estos casos, 101 (6.6%) fueron ventilados usando LMA. La disponibilidad de LMA fue significativamente mayor en el departamento de anestesiologı́a comparado con el departamento pediátrico (90% versus 50%;
P = 0.002). Sin embargo, 52% de los anestesiólogos y 72% de los pediatras nunca habı́an usado una LMA en su práctica. La LMA fue considerada un dispositivo esencial mas frecuentemente por los anestesiólogos que por los pediatras (27% versus 5%;
P =0.015); ambos grupos consideraban la LMA particularmente útil en situaciones especı́ficas. Es interesante que, mientras el 16% de los pediatras describı́an la LMA como carente de valor, ninguno de los anestesiólogos lo hizo (
P = 0.002). La competencia de su grupo de planta fue considerado bajo por el 70% de los jefes de departamento de anestesia comparados con el 90% de sus colegas de pediatrı́a. En ambas especialidades, el uso de la LMA estaba limitado al personal médico. Con respecto al entrenamiento, 35% de los anestesiólogos y 22.5% de los pediatras habı́an asistido a un curso de uso de la LMA.
Conclusiones: La disponibilidad y el valor percibido de la LMA fueron mas altos entre anestesiólogos que entre pediatras. Sin embargo, el nivel educacional, la competencia y las tasas de utilización de la LMA en resucitación neonatal fue baja en ambos grupos.
We evaluate the results of an elective cardiopulmonary bypass conceived to minimize the surgical risk related to its use with temporary circulatory arrest and deep hypothermia in the treatment of ...patients with renal tumor extending into the right atrium.
From July 1996 to December 2000, 19 patients with renal neoplasm and venous involvement were admitted to our department. Three patients 4, 57 and 58 years old with a right (2) and left (1) renal tumor extending into the right atrium underwent radical nephrectomy and tumor thrombus removal using a normothermic cardiopulmonary bypass. The bypass circuit was connected with a vacuum assisted venous drainage giving a negative pressure of 20 to 40 mm. Hg. Neither circulatory arrest nor hypothermia was used. Tumor thrombus was extracted through a longitudinal “cavotomy” and removed along with the kidney.
Total cardiopulmonary bypass time was 14, 19 and 22 minutes, respectively. No intraoperative or postoperative complications due to surgical technique occurred. No significant bleeding was observed at the time of cavotomy and all neoplastic tissue was removed. Pathologicalexamination documented renal cell carcinoma in 2 cases and Wilms tumor in 1. All the patients are alive 30, 42 and 15 months, respectively, after the operation.
Normothermic cardiopulmonary bypass with vacuum assisted venous drainage makes circulatory arrest and hypothermia unnecessary and avoids the potential complications associated with these procedures. With respect to veno-venous shunts this technique guarantees complete surgical control of the thrombus and avoids the need for extensive dissection of the retrohepatic vena cava and Pringle maneuver.
Mounted on the International Space Station(ISS), the Extreme Universe Space Observatory, on-board the Japanese Experimental Module (JEM-EUSO), relies on the well established fluorescence technique to ...observe Extensive Air Showers (EAS) developing in the earth's atmosphere. Focusing on the detection of Ultra High Energy Cosmic Rays (UHECR) in the decade of 10 super(20)eV, JEM-EUSO will face new challenges by applying this technique from space. The EUSO Simulation and Analysis Framework (ESAF) has been developed in this context to provide a full end-to-end simulation frame, and assess the overall performance of the detector. Within ESAF, angular reconstruction can be separated into two conceptually different steps. The first step is pattern recognition, or filtering, of the signal to separate it from the background. The second step is to perform different types of fitting in order to search for the relevant geometrical parameters that best describe the previously selected signal. In this paper, we discuss some of the techniques we have implemented in ESAF to perform the geometrical reconstruction of EAS seen by JEM-EUSO. We also conduct thorough tests to assess the performances of these techniques in conditions which are relevant to the scope of the JEM-EUSO mission. We conclude by showing the expected angular resolution in the energy range that JEM-EUSO is expected to observe.
Hyperbolic metamaterials (HMMs) offer unconventional properties in the field of optics, enabling opportunities for confinement and propagation of light at the nanoscale. In-plane orientation of the ...optical axis, in the direction coinciding with the anisotropy of the HMMs, is desirable for a variety of novel applications in nanophotonics and imaging. Here, a method for creating localized HMMs with in-plane optical axis, based on block copolymer (BCP) blend instability, is introduced. The dewetting of BCP thin film over topographically defined substrates generates droplets composed of highly ordered lamellar nanostructures in hierarchical configuration. The hierarchical nanostructures represent a valuable platform for the subsequent pattern transfer into a Au/air HMM, exhibiting hyperbolic behavior in a broad wavelength range in the visible spectrum. A computed Purcell factor as high as 32 at 580 nm supports the strong reduction in the fluorescence lifetime of defects in nanodiamonds placed on top of the HMM.
Cet article porte sur un logiciel d'annotation collaborative de films. L'outil, Lignes de Temps, permet d'inscrire des commentaires dans le flux du film et d'échanger les annotations entre les ...différents participants. L'étude articule quatre niveaux d'attaque de la description de l'outil : les fonctionnalités logicielles, le discours philosophique qui l'accompagne, la sémiotique visuelle de l'écran et les premiers usages constatés dans une phase d'expérimentation originale. Il situe le discours d'escorte dans l'histoire de la pensée, débordant la stricte innovation technique. Il montre le caractère instituant du design visuel de l'écran, en parallèle de l'idéologie et des fonctionnalités logicielles qui le façonnent. Il confronte la promesse politique et cognitive aux premières pratiques et retours d'usage relevés dans le cadre de l'expérimentation.
Cet article porte sur un logiciel d’annotation collaborative de films. L’outil, Lignes de Temps, permet d’inscrire des commentaires dans le flux du film et d’échanger les annotations entre les ...différents participants. L’étude articule quatre niveaux d’attaque de la description de l’outil : les fonctionnalités logicielles, le discours philosophique qui l’accompagne, la sémiotique visuelle de l’écran et les premiers usages constatés dans une phase d’expérimentation originale. Il situe le discours d’escorte dans l’histoire de la pensée, débordant la stricte innovation technique. Il montre le caractère instituant du design visuel de l’écran, en parallèle de l’idéologie et des fonctionnalités logicielles qui le façonnent. Il confronte la promesse politique et cognitive aux premières pratiques et retours d’usage relevés dans le cadre de l’expérimentation.
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