Fibrosis quística: consenso sobre el tratamiento del neumotórax y de la hemoptisis masiva y sobre las indicaciones del trasplante pulmonar

Prados, Concepción; Máiz, Luís; Antelo, C.; Baranda, F.; Blázquez, Javier; Borro, J.M.; Gartner, Sílvia; Garzón, Gonzalo; Girón, Rosa; Gracia, Javier de; Lago, J.; Lama, R.; Martínez, María Teresa; Moreno, Abelardo; Oliveira, Carla; Frías, Javier Pérez; Solé, Antonio Gens; Salcedo, Antonio

doi:10.1016/s0300-2896(15)30141-1

Cited by 15 publications

(4 citation statements)

References 21 publications

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“…Non-infectious complications arising throughout the evolution of the disease, such as atelectasis, hemoptysis, and allergic bronchopulmonary aspergillosis, should also be treated [ 165 , 166 ] ( Box 2 ).…”

Section: Treatment Of Non-infectious Respiratory Complicationsmentioning

confidence: 99%

Treatment of Pulmonary Disease of Cystic Fibrosis: A Comprehensive Review

et al. 2021

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Cystic fibrosis (CF) is a genetic disease that causes absence or dysfunction of a protein named transmembrane conductance regulatory protein (CFTR) that works as an anion channel. As a result, the secretions of the organs where CFTR is expressed are very viscous, so their functionality is altered. The main cause of morbidity is due to the involvement of the respiratory system as a result of recurrent respiratory infections by different pathogens. In recent decades, survival has been increasing, rising by around age 50. This is due to the monitoring of patients in multidisciplinary units, early diagnosis with neonatal screening, and advances in treatments. In this chapter, we will approach the different therapies used in CF for the treatment of symptoms, obstruction, inflammation, and infection. Moreover, we will discuss specific and personalized treatments to correct the defective gene and repair the altered protein CFTR. The obstacle for personalized CF treatment is to predict the drug response of patients due to genetic complexity and heterogeneity of uncommon mutations.

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Section: Treatment Of Non-infectious Respiratory Complicationsmentioning

confidence: 99%

Treatment of Pulmonary Disease of Cystic Fibrosis: A Comprehensive Review

et al. 2021

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“…O tratamento consiste em corticoterapia durante 2-3 meses. Deve-se ponderar a associação com itraconazol, se houver má resposta ou toxicidade aos corticóides e em casos de ABPA corticodependente 28 .…”

Section: Vol XIV Nº 1 Janeiro/fevereiro 2008unclassified

Fibrose quística: Revisão

Damas

Amorim

Gomes

2008

Revista Portuguesa de Pneumologia (English Edition)

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Artigo de Revisão Revision Article Resumo A fibrose quística (FQ) é a doença autossómica recessiva mais frequente na raça caucasiana. Caracteriza-se por mutações na CFTR, uma proteína transmembranar responsável pelo transporte de cloretos. Esta proteína tem uma ampla distribuição epitelial, o que dá um carácter sistémico a esta doença e consequentemente múltiplas manifestações clínicas de gravidade variável. A melhoria dos cuidados de saúde, associada ao desenvolvimento do arsenal terapêutico, permitiu um aumento da sobrevida destes doentes, de tal forma que a FQ já não pode ser abordada como doença da idade pediátrica. Também a evolução técnica na transplantação abriu novas perspectivas quanto ao tratamento desta afecção. Assim, cada vez mais esta patologia implica um envolvimento multidisciplinar no qual a pneumologia tem uma parte preponderante.

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“…Los documentos de consenso establecen los paráme-tros que habitualmente se siguen en la selección de receptores 22 . Un excelente trabajo es la guía internacional publicada en 1988 por Maurer et al 23 .…”

Section: Indicacionesunclassified

Actualización del trasplante pulmonar en España

Borro

2005

Archivos de Bronconeumología

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Fibrosis quística: consenso sobre el tratamiento del neumotórax y de la hemoptisis masiva y sobre las indicaciones del trasplante pulmonar

Cited by 15 publications

References 21 publications

Treatment of Pulmonary Disease of Cystic Fibrosis: A Comprehensive Review

Treatment of Pulmonary Disease of Cystic Fibrosis: A Comprehensive Review

Fibrose quística: Revisão

Actualización del trasplante pulmonar en España

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