PROTOCOLOS CLÍNICOS E DIRETRIZES TERAPÊUTICAS

Protocolos Clínicos e Diretrizes Terapêuticas 10 R egulação/controle/Avaliação pelo gestor Recomenda-se que a prescrição das enzimas pancreáticas 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 das doses prescritas e dispensadas 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 de 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, et al. Comparison 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 at the 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. Nousia-Arvanitakis S. Cystic fibrosis and the pancreas: recent scientific advances. J Clin Gastroenterol. 1999;29(2):138-42. 5. Colin AA, Wohl ME. Cystic fibrosis. Pediatr Rev. 1994;15(5):192-200. 6. Littlewood JM, Wolfe SP, Conway SP. Diagnosis and treatment of intestinal malabsorption in cystic fibrosis. Pediatr Pulmonol. 2006;41(1):35-49. 7. O’Sullivan BP, Freedman SD. Cystic fibrosis. Lancet. 2009:373(9678):1891-904. Epub 2009 May 4. 8. 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. 9. Mishra A, Greaves R, Smith K, Carlin JB, Wootton A, Sterling 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. 10. Erdman SH. Nutritional imperatives in cystic fibrosis therapy. Pediatr Ann. 1999;28(2):129-36. 11. Walkowiak J, Herzig KH, Strzykala K, Przyslawski J, Krawczynski M. Fecal elastase-1 is superior to fecal chymotrypsin in the assessment of pancreatic involvement in cystic fibrosis. Pediatrics. 2002;110(1 Pt 1):e7. 12. Walkowiak J, Lisowska A, Przyslawski J, Grzymislawski M, Krawczynski M, Herzig KH. Faecal elastase-1 test is superior to faecal lipase test in the assessment of exocrine pancreatic function in cystic fibrosis. Acta Paediatr. 2004;93(8):1042-5. 13. Meyts I, Wuyts W, Proesmans M, De Boeck K. Variability of fecal pancreatic elastase measurements in cystic fibrosis patients. J Cyst Fibros. 2002;1(4):265-8. 14. Stern RC, Eisenberg JD, Wagener JS, Ahrens R, Rock M, doPico G, et al. A comparison of the efficacy and tolerance of pancrelipase and placebo in the treatment of steatorrhea in cystic fibrosis patients with clinical exocrine pancreatic insufficiency. Am J Gastroenterol. 2000;95(8):1932-8. 15. Konstan MW, Stern RC, Trout JR, Sherman JM, Eigen H, Wagener JS, et al. Ultrase MT12 and Ultrase MT20 in the treatment of exocrine pancreatic insufficiency in cystic fibrosis: safety and efficacy. Aliment Pharmacol Ther. 2004;20(11- 12):1365-71. 16. Colombo C, Fredella C, Russo MC, Faelli N, Motta V, Valmarana L, et al. Efficacy and tolerability of Creon for Children in infants and toddlers with pancreatic exocrine insufficiency caused by cystic fibrosis: an open-label, single-arm, multicenter study. Pancreas. 2009;38(6):693-9. 17. Santini B, Antonelli M, Battistini A, Bertasi S, Collura M, Esposito I, et al. Comparison of two enteric coated microsphere preparations in the treatment of pancreatic exocrine insufficiency caused by cystic fibrosis. Dig Liver Dis. 2000;32(5):406-11. 18. Häusler M, Heimann G, Meilcke R, Biesterfeld S. Fibrosing colonopathy in an adult caused by over use of pancreatic enzyme supplements. Gut. 2000;47(4):598. 19. Stevens JC, Maguiness KM, Hollingsworth J, Heilman DK, Chong SK. Pancreatic enzyme supplementation in cystic fibrosis patients before and after fibrosing colonopathy. J Pediatr Gastroenterol Nutr. 1998;26(1):80-4. 20. Brady MS, Rickard K, Yu PL, Eigen H. Effectiveness of enteric coated pancreatic enzymes given before meals in reducing steatorrhea in children with cystic fibrosis. J Am Diet Assoc. 1992;92(7):813-7. 21. Stallings VA, Stark LJ, Robinson KA, Feranchak AP, Quinton H; Clinical Practice Guidelines on Growth and Nutrition 310