Pulmonary complications in cystic fibrosis

Complicaciones pulmonares en fibrosis quística

Cystic fibrosis is an autosomal recessive disease caused by mutations in chromosome 7, and compromises multiple organs. One of the most common complications is the respiratory tract infection due to Pseudomonas aeruginosa.
Objectives: To present the recommendations on the management of the pulmonary complications reported in clinical practice guidelines, of good quality according to AGREE II, with the aim of reducing morbidity and improving the quality of life of the patients.
Methods: A review was performed on cystic fibrosis clinical practice guidelines, in English and Spanish, published between 2005 and 2013, using the AGREE II tool to evaluate their quality.
Results: The search produced a total of 126 guidelines, of which 6 were evaluated, and 4 obtained a score ≥ 60% in the rigour and independence domain according to the AGREE II. The recommendations for the management of pulmonary exacerbations due to Pseudomonas aeruginosa were taken from these, as well as those for those of the airways, pneumothorax, haemoptysis, and allergic bronchopulmonary aspergillosis.
Conclusions: The best inhaled antibiotic for chronic use is tobramycin. Dornase alpha is the best mucolytic. The use of corticosteroids is controversial, but is useful in patients who suffer from asthma and/or allergic bronchopulmonary aspergillosis. The best quality clinical practice guidelines are currently from Ecuador.

1. Rosenstein BJ, Cutting GR. The diagnosis of cystic fibrosis: A consensus statement. Cystic Fibrosis Foundation Consensus Panel. J Pediatr. 1998;132:589–95.

2. Girón R, Salgado A. Monografías de la Sociedad Madrileña de Neumología y Cirugía Torácica. Fibrosis quística. Madrid: ERGON; 2005. p. 192.

3. Ministerio de Salud Pública del Ecuador. Dirección Nacional de Normatización. Fibrosis quística: Guía práctica clínica y manual de procedimientos. Quito: El Ministerio; 2013. 94 p.

4. Parra W. Fibrosis quística. Iatreia. 1991;4:26–9.

5. Ng MY, Flight W, Smith E. Pulmonary complications of cystic fibrosis. Clin Radiol. 2014;69:e153–62.

6. Canton R, Cobos N. Tratamiento antimicrobiano frente a la colonización pulmonar por Pseudomonas aeruginosa en el paciente con fibrosis quística. Arch Bronconeumol. 2005;41:1–25.

7. Federación Española contra la Fibrosis Quística. Los tres pilares del tratamiento de la fibrosis quística. [Documento en Internet]. Valencia: La Federación [citado 15 Nov 2015]. Disponible en: http://media.fqcantabria.org/documentos/123.pdf

8. Ciofu O, Hansen CR, Høiby N. Respiratory bacterial infections in cystic fibrosis. Curr Opin Pulm Med. 2013;19:251–8.

9. Asensio de la Cruz O, Bosque A, de los Ríos C, Montón M, Espasa A. Fibrosis quística. Manifestaciones respiratorias. Pediatr Integral. 2012;16:156–69.

10. Ministerio de la Protección Social. Guía metodológica para la elaboración de guías de práctica clínica con evaluación Económica en el Sistema General de Seguridad Social en Salud colombiano. Bogotá: El Ministerio; 2010. p. 384.

11. Watine J, Friedberg B, Nagy E, Onody R, Oosterhuis W, Bunting PS, et al. Conflict between guideline methodologic quality and recommendation validity: A potential problem for practitioners. Clin Chem. 2006;52:65–72.

12. U.S. Preventive Services Task Force. [Internet]. Rockville [citado 18 Nov 2015]. Disponible en: http://www.uspreventiveservicestaskforce.org/

13. Centre for Evidence-Based Medicine.[Internet]. Oxford: El Centro [citado 18 Nov 2015]. Disponible en: http://www.cebm.net/

14. Lezana JL. Fibrosis quística. Guías clínicas para el diagnóstico y tratamiento. México: Intersistemas; 2008. p. 104.

15. Ministerio de salud de Chile. Guía clínica fibrosis quística. Santiago de Chile: Minsal; 2007. 77 p.

16. Jimenez S, Bousoño C, Ruiz del arbol P. Patología pulmonar en la fibrosis quística. Bol Pediatr. 2007;47:7–13.

17. Flume PA, O’Sullivan BP, Robinson KA, Goss CH, Mogayzel PJ, Willey-Courand DB, et al. Cystic fibrosis pulmonary guidelines: Chronic medications for maintenance of lung health. Am J Respir Crit Care Med. 2007;176:957–69.

18. Flume PA, Mogayzel PJ, Robinson KA, Goss CH, Rosenblatt RL, Kuhn RJ, et al. Cystic fibrosis pulmonary guidelines: Treatment of pulmonary exacerbations. Am J Respir Crit Care Med. 2009;180:802–8.