Management of infants with cystic fibrosis: A summary of the evidence for the cystic fibrosis foundation working group on care of infants with cystic fibrosis

Journal of Pediatrics

Volumn 155, Issue 6 SUPPL., 2009, Pages

  Robinson, Karen A ^a   Saldanha, Ian J ^a   McKoy, Naomi A ^a  

^a JOHNS HOPKINS UNIVERSITY SCHOOL OF MEDICINE   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

CYSTIC FIBROSIS; HUMAN; INFANT; NEWBORN; PRACTICE GUIDELINE; REVIEW; TREATMENT OUTCOME;

CYSTIC FIBROSIS; HUMANS; INFANT; INFANT, NEWBORN; OUTCOME AND PROCESS ASSESSMENT (HEALTH CARE); PRACTICE GUIDELINES AS TOPIC;

EID: 77449123680     PISSN: 00223476     EISSN: 10976833     Source Type: Journal    
DOI: 10.1016/j.jpeds.2009.09.002     Document Type: Review

References (82)

1. Comeau AM, Accurso FJ, White TB, Campbell PW 3rd, Hoffman G, Parad RB, et al. Guidelines for implementation of cystic fibrosis newborn screening programs: Cystic Fibrosis Foundation workshop report. Pediatrics 2007;119:e495-518. (Pubitemid 46397760)
2. Cystic Fibrosis Foundation [homepage on the Internet]. Newborn Screening for Cystic Fibrosis. Available from: http://www.cff.org/GetInvolved/Advocate/ WhyAdvocate/NewbornScreening/#What-states-do-newborn-screening-for-CF? Accessed June 1, 2008.
3. Farrell PM. Taking the CF battle to the extremes: healthy starts with newborn screening; North American Cystic Fibrosis Conference; October 25, 2008; Orlando, FL.
4. The Cystic Fibrosis Foundation. Cystic Fibrosis Foundation Patient Registry, 2006 Annual Data Report. Bethesda: The Cystic Fibrosis Foundation; 2006.
5. Farrell PM, Lai HJ, Li Z, Kosorok MR, Laxova A, Green CG, et al. Evidence on improved outcomes with early diagnosis of cystic fibrosis through neonatal screening: enough is enough!. J Pediatr 2005;147:S30-6. (Pubitemid 41400256)
6. Rosenfeld M. Overview of published evidence on outcomes with early diagnosis from large US observational studies. J Pediatr 2005;147: S11-4. (Pubitemid 41400252)
7. Flume P, Robinson K, O'Sullivan B, Finder JVR, Willey-Courand D, White TMB, et al. Cystic fibrosis pulmonary guidelines: airway clearance therapies. Respir Care 2009;54:522-37.
8. Flume PA, O'Sullivan BP, Robinson KA, Goss CH, Mogayzel PJ Jr., 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. (Pubitemid 350127838)
9. American Academy of Pediatrics Committee on Infectious Diseases and Committee on Fetus and Newborn. Revised indications for the use of palivizumab and respiratory syncytial virus immune globulin intravenous for the prevention of respiratory syncytial virus infections. Pediatrics 2003;112:1442-6.
10. McCool FD, Rosen MJ. Nonpharmacologic airway clearance therapies: ACCP evidence-based clinical practice guidelines. Chest 2006;129: 250S-9S. (Pubitemid 43265229)
11. Butte N, Cobb K, Dwyer J, Graney L, Heird W, Rickard K. The start healthy feeding guidelines for infants and toddlers. J Am Diet Assoc 2004;104:442-54. (Pubitemid 38296644)
12. The Cystic Fibrosis Foundation. Clinical practice guidelines for cystic fibrosis. Bethesda: The Cystic Fibrosis Foundation; 1997.
13. Saiman L, Siegel J. Infection control recommendations for patients with cystic fibrosis: microbiology, important pathogens, and infection control practices to prevent patient-to-patient transmission. Infect Control Hosp Epidemiol 2003;24:S6-52. (Pubitemid 36617275)
14. Stallings VA, Stark LJ, Robinson KA, Feranchak AP, Quinton H. Evidence-based practice recommendations for nutrition-related management of children and adults with cystic fibrosis and pancreatic insufficiency: results of a systematic review. J Am Diet Assoc 2008;108: 832-9. (Pubitemid 351602057)
15. Borowitz D, Baker RD, Stallings V. Consensus report on nutrition for pediatric patients with cystic fibrosis. J Pediatr Gastroenterol Nutr 2002;35:246-59.
16. Sinaasappel M, Stern M, Littlewood J, Wolfe S, Steinkamp G, Heijerman HG, et al. Nutrition in patients with cystic fibrosis: a European Consensus. J Cyst Fibros 2002;1:51-75.
17. Kerem E, Conway S, Elborn S, Heijerman H. Standards of care for patients with cystic fibrosis: a European consensus. J Cyst Fibros 2005;4:7-26. (Pubitemid 40347773)
18. The CF Trust's Clinical Standards and Accreditation Group. (May 2001). Standards of care. http://www.cftrust.org.uk/aboutcf/publications/consensusdoc/ C-3000Standards-of-Care.pdf. (Accessed June 1, 2008).
19. UK Cystic Fibrosis Trust [homepage on the Internet]. [September 2002]. UK Cystic Fibrosis Trust Antibiotic Group. Antibiotic treatment for cystic fibrosis. Available from: http://www.cftrust.org.uk/aboutcf/publications/ consensusdoc/C-3200Antibiotic-Treatment.pdf. Accessed June 1, 2008.
20. UK Cystic Fibrosis Trust [homepage on the Internet]. [April 2002]. UK Cystic Fibrosis Trust Nutrition Working Group. Nutritional management in cystic fibrosis. A consensus report. Available from: http://www.cftrust.org.uk/aboutcf/ publications/consensusdoc/C-3500Nutritional-Management.pdf. Accessed June 1, 2008.
21. McKarneyC, EverardM,N'Diaye T.Omega-3 fatty acids (from fish oils) for cystic fibrosis. Cochrane Database of Systematic Reviews 2007, Issue 4. Art. No.:CD002201.DOI: 10.1002/14651858.CD002201.pub2. 2007;CD002201. (Pubitemid 351805321)
22. Jones AP, Wallis CE, Kearney CE. Dornase alpha for cystic fibrosis. Cochrane Database of Systematic Reviews 2003, Issue 3 Art. No.: CD001127. DOI: 10.1002/14651858.CD001127.
23. Smyth A, Walters S. Prophylactic antibiotics for cystic fibrosis. Cochrane Database of Systematic Reviews: Reviews 2003, Issue 3. Art. No.: CD001912. DOI: 10.1002/14651858.CD001912.
24. Giebels K, Marcotte JE, Podoba J, Rousseau C, Denis MH, Fauvel V, et al. Prophylaxis against respiratory syncytial virus in young children with cystic fibrosis. Pediatr Pulmonol 2007;43:169-74.
25. Colombo C, Costantini D, Zazzeron L, Faelli N, Russo MC, Ghisleni D, et al. Benefits of breastfeeding in cystic fibrosis: a single-centre follow-up survey. Acta Paediatrica 2007;96:1228-32. (Pubitemid 47106534)
26. Subbarao P, Balkovec S, Solomon M, Ratjen F. Pilot study of safety and tolerability of inhaled hypertonic saline in infants with cystic fibrosis. Pediatr Pulmonol 2007;42:471-6. (Pubitemid 46700947)
27. Lum S, Gustafsson P, Ljungberg H, Hulskamp G, Bush A, Carr SB, et al. Early detection of cystic fibrosis lung disease: multiple-breath washout versus raised volume tests. Thorax 2007;62:341-7. (Pubitemid 46591279)
28. Martinez TM, Llapur CJ, Williams TH, Coates C, Gunderman R, Cohen MD, et al. High-resolution computed tomography imaging of airway disease in infants with cystic fibrosis. Am J Respir Crit Care Med 2005;172:1133-8. (Pubitemid 43066649)
29. Berge MT, Wiel E, Tiddens HA, Merkus PJ, Hop WC, de Jongste JC. DNase in stable cystic fibrosis infants: a pilot study. J Cyst Fibros 2003;2: 183-8. (Pubitemid 37376495)
30. Parker EM, O'Sullivan BP, Shea JC, Regan MM, Freedman SD. Survey of breast-feeding practices and outcomes in the cystic fibrosis population. Pediatr Pulmonol 2004;37:362-7. (Pubitemid 38523950)
31. Farrell PM, Li Z, Kosorok MR, Laxova A, Green CG, Collins J, et al. Longitudinal evaluation of bronchopulmonary disease in children with cystic fibrosis. Pediatr Pulmonol 2003;36:230-40. (Pubitemid 37055971)
32. Button BM, Heine RG, Catto-Smith AG, Olinsky A, Phelan PD, Ditchfield MR, et al. Chest physiotherapy in infants with cystic fibrosis: to tip or not? A five-year study. Pediatr Pulmonol 2003;35:208-13. (Pubitemid 36246158)
33. Stutman HR, Lieberman JM, Nussbaum E, Marks MI. Antibiotic prophylaxis in infants and young children with cystic fibrosis: a randomized controlled trial. J Pediatr 2002;140:299-305. (Pubitemid 34289705)
34. Heinzl B, Eber E,Oberwaldner B, HaasG, ZachMS. Effects of inhaled gentamicin prophylaxis on acquisition of Pseudomonas aeruginosa in children with cystic fibrosis: a pilot study. Pediatr Pulmonol 2002;33:32-7. (Pubitemid 34031552)
35. Feranchak AP, Sontag MK, Wagener JS, Hammond KB, Accurso FJ, Sokol RJ. Prospective, long-term study of fat-soluble vitamin status in children with cystic fibrosis identified by newborn screen. J Pediatr 1999;135:601-10. (Pubitemid 30180451)
36. Ellis L, Kalnins D, Corey M, Brennan J, Pencharz P, Durie P. Do infants with cystic fibrosis need a protein hydrolysate formula? A prospective, randomized, comparative study. J Pediatr 1998;132:270-6. (Pubitemid 28125179)
37. van Egmond AW, Kosorok MR, Koscik R, Laxova A, Farrell PM. Effect of linoleic acid intake on growth of infants with cystic fibrosis. Am J Clin Nutr 1996;63:746-52. (Pubitemid 26126595)
38. Beardsmore CS. Lung function from infancy to school age in cystic fibrosis. Arch Dis Child 1995;73:519-23.
39. Turner DJ, Lanteri CJ, LeSouef PN, Sly PD. Improved detection of abnormal respiratory function using forced expiration from raised lung volume in infants with cystic fibrosis. Eur Respir J 1994;7: 1995-9. (Pubitemid 24366290)
40. Beardsmore CS, Thompson JR, Williams A, McArdle EK, Gregory GA, Weaver LT, et al. Pulmonary function in infants with cystic fibrosis: the effect of antibiotic treatment. Arch Dis Child 1994;71:133-7. (Pubitemid 24261116)
41. Weaver LT, Green MR, Nicholson K, Mills J, Heeley ME, Kuzemko JA, et al. Prognosis in cystic fibrosis treated with continuous flucloxacillin from the neonatal period. Arch Dis Child 1994;70:84-9. (Pubitemid 24088956)
42. McClean P, Harding M, Coward WA, Green MR, Weaver LT. Measurement of fat digestion in early life using a stable isotope breath test. Arch Dis Child 1993;69:366-70. (Pubitemid 23291325)
43. Tepper RS, Montgomery GL, Ackerman V, Eigen H. Longitudinal evaluation of pulmonary function in infants and very young children with cystic fibrosis. Pediatr Pulmonol 1993;16:96-100.
44. Bronstein MN, Sokol RJ, Abman SH, Chatfield BA, Hammond KB, Hambidge KM, et al. Pancreatic insufficiency, growth, and nutrition in infants identified by newborn screening as having cystic fibrosis. J Pediatr 1992;120:533-40.
45. Greer R, Shepherd R, Cleghorn G, Bowling FG, Holt T. Evaluation of growth and changes in body composition following neonatal diagnosis of cystic fibrosis. J Pediatr Gastroenterol Nutr 1991;13: 52-8.
46. Singer LT, Nofer JA, Benson-Szekely LJ, Brooks LJ. Behavioral assessment and management of food refusal in children with cystic fibrosis. J Dev Behav Pediatr 1991;12:115-20.
47. Holliday KE, Allen JR, Waters DL, Gruca MA, Thompson SM, Gaskin KJ. Growth of human milk-fed and formula-fed infants with cystic fibrosis. J Pediatr 1991;118:77-9.
48. Lynch DA, Brasch RC, Hardy KA, Webb WR. Pediatric pulmonary disease: assessment with high-resolution ultrafast CT. Radiology 1990;176: 243-8. (Pubitemid 20208144)
49. Sokol RJ, Reardon MC, Accurso FJ, Stall C, Narkewicz M, Abman SH, et al. Fat-soluble-vitamin status during the first year of life in infants with cystic fibrosis identified by screening of newborns. Am J Clin Nutr 1989;50:1064-71. (Pubitemid 19280033)
50. Hardy KA, Wolfson MR, Schidlow DV, Shaffer TH. Mechanics and energetics of breathing in newly diagnosed infants with cystic fibrosis: effect of combined bronchodilator and chest physical therapy. Pediatr Pulmonol 1989;6:103-8.
51. Kraemer R. Early detection of lung function abnormalities in infants with cystic fibrosis. J R Soc Med 1989;82(Suppl 16):21-5. (Pubitemid 19264695)
52. Maayan C, Bar-Yishay E, Yaacobi T, Marcus Y, Katznelson D, Yahav Y, et al. Immediate effect of various treatments on lung function in infants with cystic fibrosis. Respiration 1989;55:144-51. (Pubitemid 19203971)
53. Beardsmore CS, Bar-Yishay E, Maayan C, Yahav Y, Katznelson D, Godfrey S. Lung function in infants with cystic fibrosis. Thorax 1988; 43:545-51.
54. Tepper RS, Hiatt P, Eigen H, Scott P, Grosfeld J, Cohen M. Infants with cystic fibrosis: pulmonary function at diagnosis. Pediatr Pulmonol 1988; 5:15-8.
55. Farrell PM, Mischler EH, Sondel SA, Palta M. Predigested formula for infants with cystic fibrosis. J Am Diet Assoc 1987;87:1353-6. (Pubitemid 18021895)
56. Tepper RS, Hiatt PW, Eigen H, Smith J. Total respiratory system compliance in asymptomatic infants with cystic fibrosis. Am Rev Resp Dis 1987;135:1075-9. (Pubitemid 18021493)
57. Canciani M, Mastella G. Absorption of a new semielemental diet in infants with cystic fibrosis. J Pediatr Gastroenterol Nutr 1985;4: 735-40. (Pubitemid 15048885)
58. Harrison CJ, Marks MI, Welch DF, Sharma BB, Baker D, Dice J. A multicenter comparison of related pharmacologic features of cephalexin and dicloxacillin given for two months to young children with cystic fibrosis. Pediatr Pharmacol 1985;5:7-16. (Pubitemid 15116090)
59. Bellentani S, Grisendi A, Rinaldi M, Bertolani P, Costa G, Agostini M, et al. BT-Paba test in the diagnosis of pancreatic exocrine insufficiency in cystic fibrosis: urinary and serum determinations compared. Eur J Pediatr 1984;143:145-8. (Pubitemid 15222536)
60. Gillard BK, Cox KL, Pollack PA, Geffner ME. Cystic fibrosis serum pancreatic amylase. Useful discriminator of exocrine function. Am J Dis Child 1984;138:577-80. (Pubitemid 14100984)
61. Durie PR, Largman C, Brodrick JW, Johnson JH, Gaskin KJ, Forstner GG, et al. Plasma immunoreactive pancreatic cationic trypsinogen in cystic fibrosis: a sensitive indicator of exocrine pancreatic dysfunction. Pediatr Res 1981;15:1351-5. (Pubitemid 11018692)
62. Cavell B. Gastric emptying in infants with cystic fibrosis. Acta Paediatr Scand 1981;70:635-8. (Pubitemid 12196515)
63. Durie PR, Newth CJ, Forstner GG, Gall DG. Malabsorption of mediumchain triglycerides in infants with cystic fibrosis: correction with pancreatic enzyme supplement. J Pediatr 1980;96:862-4. (Pubitemid 10068215)
64. Godfrey S, Mearns M, Howlett G. Serial lung function studies in cystic fibrosis in the first 5 years of life. Arch Dis Child 1978;53:83-5. (Pubitemid 8301030)
65. Simmonds EJ, Wall CR, Wolfe SP, Littlewood JM. A review of infant feeding practices at a regional cystic fibrosis unit. J Hum Nutr Diet 1994;7:31-8. (Pubitemid 24066002)
66. Armstrong DS, Grimwood K, Carlin JB, Carzino R, Olinsky A, Phelan PD. Bronchoalveolar lavage or oropharyngeal cultures to identify lower respiratory pathogens in infants with cystic fibrosis. Pediatr Pulmonol 1996;21:267-75. (Pubitemid 26159822)
67. Rosenfeld M, Emerson J, Accurso F, Armstrong D, Castile R, Grimwood K, et al. Diagnostic accuracy of oropharyngeal cultures in infants and young children with cystic fibrosis. Pediatr Pulmonol 1999;28: 321-8. (Pubitemid 29523443)
68. Ranganathan SC, Bush A, Dezateux C, Carr SB, Hoo AF, Lum S, et al. Relative ability of full and partial forced expiratory maneuvers to identify diminished airway function in infants with cystic fibrosis. AmJ Resp Crit Care Med 2002;166:1350-7. (Pubitemid 35292701)
69. Hendeles L, Dorf A, Stecenko A, Weinberger M. Treatment failure after substitution of generic pancrelipase capsules. Correlation with in vitro lipase activity. JAMA 1990;263:2459-61. (Pubitemid 20169457)
70. Gaskin K, Waters D, Dorney S, Gruca M, O'Halloran M, Wilcken B. Assessment of pancreatic function in screened infants with cystic fibrosis. Pediatr Pulmonol Suppl 1991;7:69-71.
71. Wright GL, Harper J. Fusidic acid and lincomycin therapy in staphylococcal infections in cystic fibrosis. Lancet 1970;1:9-14.
72. Villa MP, Pagani J, Lucidi V, Palamides S, Ronchetti R. Nocturnal oximetry in infants with cystic fibrosis. Arch Dis Child 2001;84:50-4. (Pubitemid 32060137)
73. Phillips GE, Pike SE, Rosenthal M, Bush A. Holding the baby: head downwards positioning for physiotherapy does not cause gastro-oesophageal reflux. Eur Resp J 1998;12:954-7. (Pubitemid 28491673)
74. Colombo C, Maiavacca R, Ronchi M, Consalvo E, Amoretti M, Giunta A. The steatocrit: a simple method for monitoring fat malabsorption in patients with cystic fibrosis. J Pediatr Gastroenterol Nutr 1987;6:926-30. (Pubitemid 18046923)
75. Remtulla MA, Durie PR, Goldberg DM. Stool chymotrypsin activity measured by a spectrophotometric procedure to identify pancreatic disease in infants. Clin Biochem 1986;19:341-7. (Pubitemid 17025966)
76. Abman SH, Ogle JW, Harbeck RJ, Butler-Simon N, Hammond KB, Accurso FJ. Early bacteriologic, immunologic, and clinical courses of young infants with cystic fibrosis identified by neonatal screening. J Pediatri 1991;119:211-7.
77. Hudson VL, Wielinski CL, Regelmann WE. Prognostic implications of initial oropharyngeal bacterial flora in patients with cystic fibrosis diagnosed before the age of two years. J Pediatr 1993;122:854-60. (Pubitemid 23158420)
78. Ranganathan SC, Stocks J, Dezateux C, Bush A, Wade A, Carr S, et al. The evolution of airway function in early childhood following clinical diagnosis of cystic fibrosis. Am J Resp Crit Care Med 2004;169:928-33. (Pubitemid 38457319)
79. Speer ME, Fernandes CJ, Boron M, Groothuis JR. Use of palivizumab for prevention of hospitalization as a result of respiratory syncytial virus in infants with cystic fibrosis. Pediatr Infect Dis J 2008;27:559-61.
80. Button BM, Heine RG, Catto-Smith AG, Phelan PD, Olinsky A. Postural drainage and gastro-oesophageal reflux in infants with cystic fibrosis. Arch Dis Child 1997;76:148-50. (Pubitemid 27086886)
81. Watkins JB, Schoeller DA, Klein PD, Ott DG, Newcomer AD, Hofmann AF. 13C-trioctanoin: a nonradioactive breath test to detect fat malabsorption. J Lab Clin Med 1977;90:422-30. (Pubitemid 8183276)
82. Button BM, Heine RG, Catto-Smith AG, Phelan PD, Olinsky A. Chest physiotherapy, gastro-oesophageal reflux, and arousal in infants with cystic fibrosis. Arch Dis Child 2004;89:435-9.