Consideraciones perioperatorias del síndrome de Noonan en el adulto.

Noonan syndrome (SN) is a monogenic disease with an autosomal dominant inheritance pattern with an incidence of 1: 1500-2500. This syndrome is of particular interest to the anesthesiologist because it is considered a probable difficult airway, presenting short stature, facial dysmorphia, haemostasis, musculoskeletal and cardiological alterations, among others, whose evolution determines life expectancy reaching more advanced ages at present thanks to the control of them. Consequently, they require interventions in adulthood where there are not as many cases as in childhood. We report a case of a 53-year-old patient with NS scheduled for cervical neuroma resection with increased bleeding during surgery. Our goal is to establish a specific anesthetic plan after exposing the characteristics of the disease to minimize perioperative complications.  El síndrome de Noonan (SN) es una enfermedad monogénica con patrón de herencia autosómico dominante con una incidencia de uno de 1:1500-2500. Este síndrome es de particular interés para el anestesiólogo por ser considerado probable vía aérea difícil, presentar talla baja, dismorfia facial, alteraciones en la hemostasia, musculoesqueléticas y cardiológicas, entre otras, cuya evolución condicionan la esperanza de vida alcanzando edades más avanzadas en la actualidad gracias al control de las mismas . Por consiguiente requieren intervenciones en la edad adulta donde no hay tantos casos reportados como en la infancia . Presentamos un caso de un paciente de 53 años con SN programado para resección de neurinoma cervical durante el cual destacó un sangrado aumentado durante la cirugía no esperado. Nuestro objetivo es establecer un plan anestésico específico tras exponer las características de la enfermedad para minimizar las complicaciones perioperatorias.      

Identificación de contextos con contenidos científico-económicos y su aplicación al aula

Depto. de Didáctica de las Ciencias Experimentales , Sociales y MatemáticasFac. de EducaciónFALSEsubmitte

NGCPV: a new generation of concentrator photovoltaic cells, modules and systems

Starting on June 2011, NGCPV is the first project funded jointly between the European Commission (EC) and the New Energy and Industrial Technology Development Organization (NEDO) of Japan to research on new generation concentration photovoltaics (CPV). The Project, through a collaborative research between seven European and nine Japanese leading research centers in the field of CPV, aims at lowering the cost of the CPVproduced photovoltaic kWh down to 5 ?cents. The main objective of the project is to improve the present concentrator cell, module and system efficiency, as well as developing advanced characterization tools for CPV components and systems. As particular targets, the project aims at achieving a cell efficiency of at least 45% and a CPV module with an efficiency greater than 35%. This paper describes the R&D activities that are being carried out within the NGCPV project and summarizes some of the most relevant results that have already been attained, for instance: the manufacturing of a 44.4% world record efficiency triple junction solar cell (by Sharp Corp.) and the installation of a 50 kWp experimental CPV plant in Spain, which will be used to obtain accurate forecasts of the energy produced at system level

Extreme Phenotypes With Identical Mutations: Two Patients With Same Non-sense NHEJ1 Homozygous Mutation

Cernunnos/XLF deficiency is a rare primary immunodeficiency classified within the DNA repair defects. Patients present with severe growth retardation, microcephaly, lymphopenia and increased cellular sensitivity to ionizing radiation. Here, we describe two unrelated cases with the same non-sense mutation in the NHEJ1 gene showing significant differences in clinical presentation and immunological profile but a similar DNA repair defect

Case-Control Analysis of the Impact of Anemia on Quality of Life in Patients with Cancer: A Qca Study Analysis

The impact of anemia on the quality of life (QoL) in cancer patients has been studied previously; however, the cut-off point used to define anemia differed among studies, thus providing inconsistent results. Therefore, we analysed the clinical impact of anemia on QoL using the same cut-off point for hemoglobin level to define anemia as that used in ESMO clinical practice guidelines. This post-hoc analysis aimed to determine the impact of anemia on QoL in cancer patients through the European Organization for Research and Treatment of Cancer Quality of life questionnaire version 3.0 (EORTC QLQ-C30) and Euro QoL 5-dimension 3-level (EQ-5D-3L) questionnaire. We found that cancer patients with anemia had significantly worse QoL in clinical terms. In addition, anemic patients had more pronounced symptoms than those in non-anemic patients. Anemia is a common condition in cancer patients and is associated with a wide variety of symptoms that impair quality of life (QoL). However, exactly how anemia affects QoL in cancer patients is unclear because of the inconsistencies in its definition in previous reports. We aimed to examine the clinical impact of anemia on the QoL of cancer patients using specific questionnaires. We performed a post-hoc analysis of a multicenter, prospective, case-control study. We included patients with cancer with (cases) or without (controls) anemia. Participants completed the European Organization for Research and Treatment of Cancer Quality of Life questionnaire version 3.0 (EORTC QLQ-C30) and Euro QoL 5-dimension 3-level (EQ-5D-3L) questionnaire. Statistically significant and clinically relevant differences in the global health status were examined. From 2015 to 2018, 365 patients were included (90 cases and 275 controls). We found minimally important differences in global health status according to the EORTC QLQ-C30 questionnaire (case vs. controls: 45.6 vs. 58%, respectively; mean difference: -12.4, p < 0.001). Regarding symptoms, cancer patients with anemia had more pronounced symptoms in six out of nine scales in comparison with those without anemia. In conclusion, cancer patients with anemia had a worse QoL both clinically and statistically

Inside the Outbreak of the 2009 Influenza A (H1N1)v Virus in Mexico

Influenza viruses pose a threat to human health because of their potential to cause global disease. Between mid March and mid April a pandemic influenza A virus emerged in Mexico. This report details 202 cases of infection of humans with the 2009 influenza A virus (H1N1)v which occurred in Mexico City as well as the spread of the virus throughout the entire country.From May 1st to May 5th nasopharyngeal swabs, derived from 751 patients, were collected at 220 outpatient clinics and 28 hospitals distributed throughout Mexico City. Analysis of samples using real time RT-PCR revealed that 202 patients out of the 751 subjects (26.9%) were confirmed to be infected with the new virus. All confirmed cases of human infection with the strain influenza (H1N1)v suffered respiratory symptoms. The greatest number of confirmed cases during the outbreak of the 2009 influenza A (H1N1)v were seen in neighbourhoods on the northeast side of Mexico City including Iztapalapa, Gustavo A. Madero, Iztacalco, and Tlahuac which are the most populated areas in Mexico City. Using these data, together with data reported by the Mexican Secretariat of Health (MSH) to date, we plot the course of influenza (H1N1)v activity throughout Mexico.Our data, which is backed up by MSH data, show that the greatest numbers of the 2009 influenza A (H1N1) cases were seen in the most populated areas. We speculate on conditions in Mexico which may have sparked this flu pandemic, the first in 41 years. We accept the hypothesis that high population density and a mass gathering which took in Iztapalapa contributed to the rapid spread of the disease which developed in three peaks of activity throughout the Country

New insights into the pathogenesis and transmission of Brucella pinnipedialis: systemic infection in two bottlenose dolphins ( Tursiops truncatus)

The emergence of Brucella infections in marine mammals is a growing concern. The present study reports two cases of systemic Brucella pinnipedialis infection detected in bottlenose dolphins (Tursiops truncatus) pair stranded together in the Cantabrian coast of Spain. Both animals showed systemic lesions associated with the Brucella infection, more severe in the younger dolphin, considered the likely offspring of the other individual. Real-time PCR, bacterial culture, and whole-genome sequencing were used to detect and characterize the Brucella strains involved in both dolphins. The phylogenetic analysis performed on the Brucella genomes retrieved revealed that the species involved was B. pinnipedialis (ST25). Both animals resulted seropositive in a commercial multispecies blocking ELISA but tested negative in the standard Rose Bengal test. To the best of our knowledge, this is the first report of a systemic infection resulting in various lesions associated with Brucella pinnipedialis (ST25) in two bottlenose dolphins. It is also the initial isolation of Brucella in the milk of a non-pregnant or non-aborting female cetacean likely stranded with its offspring. These findings provide new insights into the epidemiology and clinical impact of B. pinnipedialis infection in cetaceans and underscore the importance of continued diagnostic surveillance to gain better understanding of brucellosis effects and transmission in marine mammal populations.Este trabajo ha contado con el apoyo de un convenio de colaboración entre la Fundación Oceanogràfic (Valencia), la Consejería de Medio Rural, Ganadería, Pesca, Alimentación y Medio Ambiente del Gobierno de Cantabria y el Centro de Vigilancia Sanitaria Veterinaria (VISAVET) de la Universidad Complutense de Madrid. Este convenio permite la recogida de muestras de cadáveres de cetáceos varados y aporta fondos para la realización de estudios post-mortem. Ignacio Vargas-Castro es beneficiario de una beca FPU del Ministerio de Ciencia, Innovación y Universidades. El trabajo del CITA fue apoyado por el Gobierno de Aragón (Grupo de Investigación A21_23R). André E. Moura recibió el apoyo del Centro Nacional de Ciencia de Polonia (beca de investigación Sonata 2018/31/D/NZ8/02835) y de la Agencia Nacional Polaca de Intercambio Académico (programa NAWA Ulam PPN/ ULM/2019/1/00162).milkBrucella pinnipedialisbottlenose dolphinmarine mammalssystemic infectionhealth surveillancetransmissionwhole-genome sequencingPublishe

Evolving trends in the management of acute appendicitis during COVID-19 waves. The ACIE appy II study

Background: In 2020, ACIE Appy study showed that COVID-19 pandemic heavily affected the management of patients with acute appendicitis (AA) worldwide, with an increased rate of non-operative management (NOM) strategies and a trend toward open surgery due to concern of virus transmission by laparoscopy and controversial recommendations on this issue. The aim of this study was to survey again the same group of surgeons to assess if any difference in management attitudes of AA had occurred in the later stages of the outbreak. Methods: From August 15 to September 30, 2021, an online questionnaire was sent to all 709 participants of the ACIE Appy study. The questionnaire included questions on personal protective equipment (PPE), local policies and screening for SARS-CoV-2 infection, NOM, surgical approach and disease presentations in 2021. The results were compared with the results from the previous study. Results: A total of 476 answers were collected (response rate 67.1%). Screening policies were significatively improved with most patients screened regardless of symptoms (89.5% vs. 37.4%) with PCR and antigenic test as the preferred test (74.1% vs. 26.3%). More patients tested positive before surgery and commercial systems were the preferred ones to filter smoke plumes during laparoscopy. Laparoscopic appendicectomy was the first option in the treatment of AA, with a declined use of NOM. Conclusion: Management of AA has improved in the last waves of pandemic. Increased evidence regarding SARS-COV-2 infection along with a timely healthcare systems response has been translated into tailored attitudes and a better care for patients with AA worldwide

A922 Sequential measurement of 1 hour creatinine clearance (1-CRCL) in critically ill patients at risk of acute kidney injury (AKI)

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