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Quintana-Gallego E.,University of Seville | Quintana-Gallego E.,CIBER ISCIII | Ruiz-Ramos M.,Consejeria de Igualdad | Delgado-Pecellin I.,Universitario Virgen Del Rocio | And 5 more authors.
Pediatric Pulmonology | Year: 2016

Summary Objective To date, available mortality trends due to cystic fibrosis (CF) have been limited to the analysis of certain countries in different parts of the world showing that mortality trends have been constantly decreasing. However, no studies have examined Europe as a whole. The present study aims to analyze CF mortality trends by gender within the European Union (EU) and to quantify potential years of life lost (PYLL). Design Deaths from the 27 EU countries were obtained from the statistical office of the EU from the years 1994-2010. Crude and age-standardized mortality rates (ASR) were estimated for women and men using the standard European population, expressed in deaths per 1,000,000 persons. The PYLL from ages 0 up to 30 years were estimated. Trends were studied by a joinpoint regression analysis. Results During the study period, 5,130 deaths (2,443 in males and 2,687 in females) were identified. Females had a slightly higher mortality rate than males, with a downward trend observed for both genders. In males, the ASR changed from 1.34 in 1994 to 1.03 in 2010. In females, the ASR changed from 1.42 in 1994 to 0.92 in 2010. The mean age at death and PYLL increased for both genders. The joinpoint analysis did not identify any significant joinpoint for either gender for ASR or PYLL. Conclusions Our data suggest a continued downward trend of CF mortality throughout the EU, with differences by country and gender. © 2015 Wiley Periodicals, Inc. Source


Corrales Gonzalez A.,Hospital Universitario Virgen Del Rocio | Torralbo Carmona A.,Hospital Universitario Virgen Del Rocio | Salazar Quero J.C.,Hospital Universitario Virgen Del Rocio | Espin Jaime B.,Universitario Virgen Del Rocio
Pediatria Integral | Year: 2016

We present a 14 months old infant with abdominal distension, constipation, failure to thrive and malaise. We suggest some questions about diagnosis and management that we had during the hospitalization of this patient. © 2016, Ediciones Ergon SA. All rights reserved. Source


Sanchez-Mejias A.,Hospital Universitario Virgen Del Rocio | Sanchez-Mejias A.,Research Center Biomedica en Red | Fernandez R.M.,Hospital Universitario Virgen Del Rocio | Fernandez R.M.,Research Center Biomedica en Red | And 7 more authors.
Genetics in Medicine | Year: 2010

PURPOSE: Hirschsprung disease is characterized by the absence of intramural ganglion cells in the myenteric and submucosal plexuses within distal intestine, because of a fail in the enteric nervous system formations process. Endothelin-3-endothelin receptor B signaling pathway is known to play an essential role in this process. The aim of this study was to evaluate the implication of the EDN3 and EDNRB genes in a series of patients with Hirschsprung disease from Spain and determinate their mutational spectrum. METHODS: We performed the mutational screening of both genes in 196 patients with Hirschsprung disease using denaturing high-performance liquid chromatography technology. A case-control study using TaqMan Technology was also carried out to evaluate some common polymorphisms and haplotypes as susceptibility factors for Hirschsprung disease. RESULTS: Besides several novel mutations in both genes, we found a truncating mutation in an alternative isoform of EDNRB. Interestingly, we obtained an overrepresentation of a specific EDN3 haplotype in cases versus controls. CONCLUSIONS: Our results suggest that the isoform EDNRBΔ3 might be playing an essential role in the formation of enteric nervous system. In addition, based on the haplotype distribution, EDN3 might be considered as a common susceptibility gene for sporadic Hirschsprung disease in a low-penetrance fashion. © 2010 Lippincott Williams & Wilkins. Source


Martin-Pena A.,Universitario Virgen Del Rocio | Aguilar-Guisado M.,Universitario Virgen Del Rocio | Espigado I.,University of Seville | Cisneros J.M.,Universitario Virgen Del Rocio
Clinical Infectious Diseases | Year: 2014

The outcome of invasive aspergillosis (IA) continues to be associated with significant attributable mortality, especially in patients with hematological malignancies and in hematopoietic stem cell transplant recipients. In this context, antifungal combined therapy (ACT) has become an emerging strategy against IA. In an attempt to evaluate the benefits of ACT, a large number of experimental studies, clinical series, and randomized trials have been performed, with varying results. In addition, several controlled trials have been registered; however, in most cases, their final results have not been made available. In summary, there is an imbalance between the lack of published evidence regarding the benefits of ACT and its extensive and increasing use in current clinical practice, despite its associated cost. Here, we present a critical analysis of the available information regarding ACT for the treatment of IA as well as the authors' opinion with respect to its use. © The Author 2014. Source


Olveira G.,Hospital Regional Universitario Carlos Haya | Olveira G.,CIBER ISCIII | Garcia-Luna P.P.,Universitario Virgen Del Rocio | Pereira J.L.,Universitario Virgen Del Rocio | And 7 more authors.
Nutricion Hospitalaria | Year: 2012

Background & aims: By means of this update, the GARIN working group aims to define its position regarding the treatment of patients with diabetes or stress hyperglycaemia and artificial nutrition. In this area there are many aspects of uncertainty, especially in non-critically ill patients. Methods: Bibliographical review, and specific questions in advance were discussed and answered at a meeting in the form of conclusions. Results: We propose a definition of stress hyperglycaemia. The indications and access routes for artificial nutrition are no different in patients with diabetes/stress hyperglycaemia than in non-diabetics. The objective must be to keep pre-prandial blood glucose levels between 100 and 140 mg/dl and post-prandial levels between 140 and 180 mg/dl. Hyperglycemia can be prevented through systematic monitoring of capillary glycaemias and adequately calculate energy-protein needs. We recommend using enteral formulas designed for patients with diabetes (high monounsaturated fat) to facilitate metabolic control. The best drug treatment for treating hyperglycaemia/diabetes in hospitalised patients is insulin and we make recommendations for adapt the theoretical insulin action to the nutrition infusion regimen. We also addressed recommendations for future investigation. Conclusions: This recommendations about artificial nutrition in patients with diabetes or stress hyperglycaemia can add value to clinical work. Source

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