PROTOCOLOS CLÍNICOS E DIRETRIZES TERAPÊUTICAS

Protocolos Clínicos e Diretrizes Terapêuticas 10 R egulação/Controle/Avaliação pelo Gestor Recomenda-se que a prescrição da alfadornase seja realizada por médicos especialistas vinculados a Centros de Referência de fibrose cística. Devem ser observados os critérios de inclusão e exclusão constantes neste protocolo, a duração e a monitorização do tratamento, bem como a verificação periódica da dose prescrita e dispensada e a adequação de uso do medicamento. 11 T ermo de Esclarecimento e Responsabilidade – TER É obrigatória a informação ao paciente ou a seu responsável legal dos potenciais riscos, benefícios e efeitos adversos relacionados ao uso do medicamento preconizado neste protocolo. O TER é obrigatório ao se prescrever medicamento do Componente Especializado da Assistência Farmacêutica. 12 R eferências bibliográficas 1. Hamosh A, FitzSimmons SC, Macek M Jr, Knowles MR, Rosenstein BJ, Cutting GR. Comparasion of the clinical manifestations of cystic fibrosis in black and white patients. J Pediatr. 1998;132(2):255-9. 2. Raskin S, Pereira-Ferrari L, Reis FC, Abreu F, Marostica P, Rozov T, et al. Incidence of cystic fibrosis in five different states of Brazil as determined by screening of p.F508del, mutation atthe CFTR gene in newborns and patients. J Cyst Fibros. 2008;7(1):15-22. Epub 2007 Jun 4. 3. Gerritsen J. Cystic Fibrosis [book review]. N Engl J Med. 2008;358(17):1873-4. 4. Konstan MW, Hilliard KA, Norvell TM, Berger M. Bronchoalveolar lavage findings cystic fibrosis patients with stable, clinically mild lung disease suggest ongoing infection and inflammation. Am J Respir Crit Care Med. 1994;150(2):448-54. 5. Paul K, Rietschel E, BallmannM, GrieseM,WorlitzschD, Shute J, ChenC, SchinkT, DoringG, Koningsbruggen S. van, Wahn U, and Ratjen F. Effect of treatment with dornase alpha on airway inflammation in patients with cystic fibrosis. Am J Respir Crit Care Med. 2004;169(6):719-25. Epub 2003 Dec 18. 6. O’Sullivan BP. Freedman SD. Cystic Fibrosis. Lancet. 2009;373(9678):1891-904. Epub 2009 May 4. 7. De Boeck K, Wilschanski M, Castellani C, Taylor C, Cuppens H, Dodge J, et al Cystic fibrosis: terminology and diagnostic algorithms. Thorax. 2006;61(7):627-35. Epub 2005 Dec 29. 8. Mishra A, Greaves R, Smith K, Carlin JB, Wootton A, Stirling R, et al. Diagnosis of cystic fibrosis by sweat testing: age-specific reference intervals. J Pediatr. 2008;153(6):758-63. Epub 2008 Jun 27. 9. Nasr SZ, Kuhns LR, Brown RW, Hurwitz ME, Sanders GM, Strouse PJ. Use of computerized tomography and chest x-rays in evaluating efficacy of aerosolized recombinant human DNase in cystic fibrosis patients younger than age 5 years: a preliminary study. Pediatr Pulmonol. 2001;31(5):377-82. 10. Robinson E, Goris ML, Zhu HJ, Chen X, Bhise P, Sheikh F, et al. Dornase alfa reduces air trapping in children with mild cystic fibrosis lung disease. Chest. 2005;128(4):2327-35. 11. Flume PA, O’Sullivan BP, Robinson KA, Goss CH, Mogayzel PJ Jr, Willey-Courand DB, et al. Cystic Fibrosis Foundation, Pulmonary Therapies Committee. Cystic fibrosis pulmonary guidelines: chronic medications for maintenance of lung health. Am J Respir Crit Care Med. 2007;176(10):957-69. Epub 2007 Aug 29. 12. Jones AP, Wallins C. Dornase alfa for cystic fibrosis. Cochrane Database Syst Rev. 2010;3:CD001127. 13. Shak S. Aerosolized recombinant human DNase I for treatment of cystic fibrosis. Chest. 1995;107(2 Suppl):65S-70S. 14. Fuchs HJ, Borowitz DS, Christiansen DH, Morris EM, Nash ML, Ramsey BW, et al. Effect of aerosolized recombinant human DNase on exacerbations of respiratory symptoms and on pulmonary function in patients with cystis fibrosis. N Engl J Med. 1994;331(4):637-42. 15. Shah PL, Bush A, Canny GJ, Colin AA, Fuchs HJ, Geddes DM, et al. Recombinant human DNase I in cystic fibrosis patients with severe pulmonary disease: a short-term, double-blind study followed by six months open-label treatment. Eur Respir J. 1995;8(6):954-8. 16. McCoy K, Hamilton S, Johnson C. Effects of 12-week administration of dornase alfa in patients with advanced cystic fibrosis lung disease. Pulmozyme Study Group. Chest. 1996;110(4):889-95. 17. Harms HK, Matouk E, Tournier G, von der Hardt H, Weller PH, Romano L, et al. Multicenter, open-label study of recombinant human DNase in cystic fibrosis patients with moderate lung disease. DNase International Study Group. Pediatr Pulmonol. 1998;26(3):155-61. 18. Quan JM, Tiddens HA, Sy JP, McKenzie SG, Montgomery MD, Robinson PJ, et al. A two-year randomized, 326