Formación

La proteína CFTR en la Fibrosis Quística

La causa subyacente de la enfermedad
Fuente: contenidos cedidos por Vertex Pharmaceuticals


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El mecanismo subyacente de la Proteína CFTR en Fibrosis Quística

Fuente: contenidos cedidos por Vertex Pharmaceuticals


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Disfunción en la proteína CFTR: La causa subyacente de la fibrosis quística

Actualización de las clases de mutaciones: Clase VII
Fuente: contenidos cedidos por Vertex Pharmaceuticals


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Clases de mutaciones del gen CFTR en Fibrosis Quística

CFTR: Cystic Fibrosis Transmembrane conductance Regulator.
*De Boeck K, Amaral MD. Progress in therapies for cystic fibrosis. Lancet Respir Med 2016;4(8):662-674.
Fuente: contenidos cedidos por Vertex Pharmaceuticals


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European Cystic Fibrosis Society Standards of Care: Quality Management in cystic fibrosis

Abstract

Since the earliest days of cystic fibrosis (CF) treatment, patient data have been recorded and reviewed in order to identify the factors that lead to more favourable outcomes. Large data repositories, such as the US Cystic Fibrosis Registry, which was established in the 1960s, enabled successful treatments and patient outcomes to be recognized and improvement programmes to be implemented in specialist CF centres. Over the past decades, the greater volumes of data becoming available through Centre databases and patient registries led to the possibility of making comparisons between different therapies, approaches to care and indeed data recording. The quality of care for individuals with CF has become a focus at several levels: patient, centre, regional, national and international. This paper reviews the quality management and improvement issues at each of these levels with particular reference to indicators of health, the role of CF Centres, regional networks, national health policy, and international data registration and comparisons.

© 2014 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.
Keywords: CF registries; Standards; Models of care; Quality management; Outcomes in CF

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European Cystic Fibrosis Society Standards of Care: Best Practice guidelines

Abstract

Specialised CF care has led to a dramatic improvement in survival in CF: in the last four decades, well above what was seen in the general population over the same period. With the implementation of newborn screening in many European countries, centres are increasingly caring for a cohort of patients who have minimal lung disease at diagnosis and therefore have the potential to enjoy an excellent quality of life and an even greater life expectancy than was seen previously. To allow high quality care to be delivered throughout Europe, a landmark document was published in 2005 that sets standards of care. Our current document builds on this work, setting standards for best practice in key aspects of CF care. The objective of our document is to give a broad overview of the standards expected for screening, diagnosis, pre-emptive treatment of lung disease, nutrition, complications, transplant/end of life care and psychological support. For comprehensive details of clinical care of CF, references to the most up to date European Consensus Statements, Guidelines or Position Papers are provided in Table 1. We hope that this best practice document will be useful to clinical teams both in countries where CF care is developing and those with established CF centres.

© 2014 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.
Keywords: Cystic fibrosis; Standards of care; Multidisciplinary management

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Tratado de Fibrosis Quística / Editores: A. Salcedo Posadas, S. Gartner, R.M. Girón Moreno, M.D. García Novo

Extracto del Prólogo

Hablar de Fibrosis Quística (FQ) en el Siglo XXI no es nada extraño. Hablar de FQ hace 50 años era un hecho extraordinariamente raro. ¿Qué ha sucedido en este período de tiempo? Simplemente, que entonces la enfermedad era siempre mortal a lo largo del primer año de la vida. Que el diagnóstico solo se efectuaba en muy pocos hospitales de nuestro país. Que no se disponía de ningún tratamiento mínimamente eficaz. Que la inmensa mayoría de los médicos no sabíamos prácticamente nada con respecto a la enfermedad. Todos estos hechos y otros muchos, se han ido superando a lo largo de los años, y la finalidad de los editores de este libro de presentarnos cuál es la situación actual de la enfermedad, y cuáles son los mecanismos de que disponemos para su manejo, la han alcanzado de manera sobresaliente.

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