Clinical practice guidelines from the cystic fibrosis foundation for preschoolers with cystic fibrosis

Pediatrics

Volumn 137, Issue 4, 2016, Pages

  Lahiri, Thomas ^a   Hempstead, Sarah E ^b,c   Brady, Cynthia ^d   Cannon, Carolyn L ^e   Clark, Kelli ^f   Condren, Michelle E ^g   Guill, Margaret F ^c,h   Paul Guillerman, R ⁱ   Leone, Christina G ^j   Maguiness, Karen ^k   Monchil, Lisa ^l   Powers, Scott W ^m   Rosenfeld, Margaret ⁿ   Schwarzenberg, Sarah Jane ^o   Tompkins, Connie L ^p   Zemanick, Edith T ^q   Davis, Stephanie D ^k  

^a UNIVERSITY OF VERMONT COLLEGE OF MEDICINE   (United States)

^b DARTMOUTH INSTITUTE FOR HEALTH POLICY AND CLINICAL PRACTICE   (United States)

^c DARTMOUTH MEDICAL SCHOOL   (United States)

^d CHILDREN S HOSPITALS AND CLINICS OF MINNESOTA   (United States)

^e TEXAS A AND M HEALTH SCIENCE CENTER   (United States)

^f UNIVERSITY OF NORTH CAROLINA AT CHARLOTTE   (United States)

^g UNIVERSITY OF OKLAHOMA   (United States)

^h DARTMOUTH HITCHCOCK MEDICAL CENTER   (United States)

ⁱ TEXAS CHILDREN S HOSPITAL   (United States)

^j CHILDREN S HOSPITAL COLORADO   (United States)

^k INDIANA UNIVERSITY SCHOOL OF MEDICINE   (United States)

^l WESTCHESTER MEDICAL CENTER   (United States)

^m UNIVERSITY OF CINCINNATI COLLEGE OF MEDICINE   (United States)

ⁿ UNIVERSITY OF WASHINGTON SCHOOL OF MEDICINE   (United States)

^o UNIVERSITY OF MINNESOTA   (United States)

^p University of Vermont   (United States)

^q University of Colorado Denver and Children s Hospital Colorado   (United States)

more..

Author keywords

[No Author keywords available]

Indexed keywords

AZITHROMYCIN; BRONCHODILATING AGENT; DORNASE ALFA; LEUKOTRIENE DERIVATIVE;

ARTICLE; ASTHMA; CHILD; CHILD CARE; CHILD NUTRITION; CHILDHOOD DISEASE; COMPUTER ASSISTED TOMOGRAPHY; CONTROLLED STUDY; DIET THERAPY; DISEASE EXACERBATION; DISEASE SURVEILLANCE; DRUG RESPONSE; EVIDENCE BASED MEDICINE; HUMAN; LIFESTYLE MODIFICATION; LUNG CLEARANCE; LUNG DISEASE; LUNG FIBROSIS; LUNG FUNCTION; LUNG FUNCTION TEST; MICROBIOLOGY; MULTIPLE BREATH WASHOUT; PARENTING EDUCATION; PATIENT CARE; PRACTICE GUIDELINE; PRESCHOOL CHILD; PRIORITY JOURNAL; PSEUDOMONAS PNEUMONIA; PULSE OXIMETRY; SPIROMETRY; THORAX RADIOGRAPHY; CYSTIC FIBROSIS; FEMALE; MALE; NON PROFIT ORGANIZATION; STANDARDS;

CHILD, PRESCHOOL; CYSTIC FIBROSIS; FEMALE; FOUNDATIONS; HUMANS; MALE; PRACTICE GUIDELINES AS TOPIC;

EID: 84962511867     PISSN: 00314005     EISSN: 10984275     Source Type: Journal    
DOI: 10.1542/peds.2015-1784     Document Type: Article

References (157)

1. Borowitz D, Robinson KA, Rosenfeld M, et al; Cystic Fibrosis Foundation. Cystic Fibrosis Foundation evidence-based guidelines for management of infants with cystic fibrosis. J Pediatr. 2009;155(suppl 6):S73-S93
2. Mogayzel PJ Jr, Naureckas ET, Robinson KA, et al; Pulmonary Clinical Practice Guidelines Committee. Cystic fibrosis pulmonary guidelines. Chronic medications for maintenance of lung health. Am J Respir Crit Care Med. 2013;187(7):680-689
3. Stallings VA, Stark LJ, Robinson KA, Feranchak A P, Quinton H; Clinical Practice Guidelines on Growth and Nutrition Subcommittee; Ad Hoc Working Group. 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(5):832-839
4. Sly PD, Gangell CL, Chen L, et al; AREST CF Investigators. Risk factors for bronchiectasis in children with cystic fibrosis. N Engl J Med. 2013;368(21):1963-1970
5. Aurora P, Bush A, Gustafsson P, et al; London Cystic Fibrosis Collaboration. Multiple-breath washout as a marker of lung disease in preschool children with cystic fibrosis. Am J Respir Crit Care Med. 2005;171(3):249-256
6. Kerby GS, Rosenfeld M, Ren CL, et al. Lung function distinguishes preschool children with CF from healthy controls in a multi-center setting. Pediatr Pulmonol. 2012;47(6):597-605
7. Brumback LC, Davis SD, Kerby GS, et al. Lung function from infancy to preschool in a cohort of children with cystic fibrosis. Eur Respir J. 2013;41(1):60-66
8. Spieth LE, Stark LJ, Mitchell MJ, et al. Observational assessment of family functioning at mealtime in preschool children with cystic fibrosis. J Pediatr Psychol. 2001;26(4):215-224
9. Mitchell MJ, Powers SW, Byars KC, Dickstein S, Stark LJ. Family functioning in young children with cystic fibrosis: observations of interactions at mealtime. J Dev Behav Pediatr. 2004;25(5):335-346
10. Powers SW, Jones JS, Ferguson KS, Piazza-Waggoner C, Daines C, Acton JD. Randomized clinical trial of behavioral and nutrition treatment to improve energy intake and growth in toddlers and preschoolers with cystic fibrosis. Pediatrics. 2005;116(6):1442-1450
11. Yen EH, Quinton H, Borowitz D. Better nutritional status in early childhood is associated with improved clinical outcomes and survival in patients with cystic fibrosis. J Pediatr. 2013;162(3):530-535.e1
12. Robinson KA, Saldanha IJ, McKoy NA. Development of a framework to identify research gaps from systematic reviews. J Clin Epidemiol. 2011;64(12):1325-1330
13. Simon GR, Baker C, Barden III GA, et al; Committee on Practice and Ambulatory Medicine; Bright Futures Periodicity Schedule Workgroup. 2014 recommendations for pediatric preventive health care. Pediatrics. 2014;133(3):568-570
14. Committee on Infectious Diseases. Recommendations for prevention and control of influenza in children, 2013-2014. Pediatrics. 2013;132(4). Available at: www.pediatrics.org/cgi/content/full/132/4/e1089
15. Committee on Infectious Diseases, American Academy of Pediatrics. Recommended childhood and adolescent immunization schedule-United States, 2014. Pediatrics. 2014;133(2):357-363
16. Committee on Environmental Health; Committee on Substance Abuse; Committee on Adolescence; Committee on Native American Child. From the American Academy of Pediatrics: Policy statement-Tobacco use: a pediatric disease. Pediatrics. 2009;124(5):1474-1487
17. Uyan ZS, Ozdemir N, Ersu R, et al. Factors that correlate with sleep oxygenation in children with cystic fibrosis. Pediatr Pulmonol. 2007;42(8):716-722
18. van der Giessen LJ, Gosselink R, Hop WC, Tiddens HA. Recombinant human DNase nebulisation in children with cystic fibrosis: before bedtime or after waking up? Eur Respir J. 2007;30(4):763-768
19. de Castro-Silva C, de Bruin VM, Cavalcante AG, Bittencourt LR, de Bruin P F. Nocturnal hypoxia and sleep disturbances in cystic fibrosis. Pediatr Pulmonol. 2009;44(11):1143-1150
20. Bakker EM, van der Meijden JC, Nieuwhof EM, Hop WC, Tiddens HA. Determining presence of lung disease in young children with cystic fibrosis: lung clearance index, oxygen saturation and cough frequency. J Cyst Fibros. 2012;11(3):223-230
21. Aurora P, Stocks J, Oliver C, et al; London Cystic Fibrosis Collaboration. Quality control for spirometry in preschool children with and without lung disease. Am J Respir Crit Care Med. 2004;169(10):1152-1159
22. Marostica PJ, Weist AD, Eigen H, et al. Spirometry in 3-to 6-year-old children with cystic fibrosis. Am J Respir Crit Care Med. 2002;166(1):67-71
23. Vilozni D, Bentur L, Efrati O, et al. Spirometry in early childhood in cystic fibrosis patients. Chest. 2007;131(2):356-361
24. Latzin P, Fehling M, Bauernfeind A, Reinhardt D, Kappler M, Griese M. Efficacy and safety of intravenous meropenem and tobramycin versus ceftazidime and tobramycin in cystic fibrosis. J Cyst Fibros. 2008;7(2):142-146
25. Muramatu LH, Stirbulov R, Forte WC. Pulmonary function parameters and use of bronchodilators in patients with cystic fibrosis. J Bras Pneumol. 2013;39(1):48-55
26. Marson FA, Bertuzzo CS, Ribeiro A F, Ribeiro JD. Polymorphisms in ADRB2 gene can modulate the response to bronchodilators and the severity of cystic fibrosis. BMC Pulm Med. 2012;12:50
27. Davies PL, Doull IJ, Child F. The interrupter technique to assess airway responsiveness in children with cystic fibrosis. Pediatr Pulmonol. 2007;42(1):23-28
28. Aurora P, Stanojevic S, Wade A, et al; London Cystic Fibrosis Collaboration. Lung clearance index at 4 years predicts subsequent lung function in children with cystic fibrosis. Am J Respir Crit Care Med. 2011;183(6):752-758
29. Rosenfeld M, Farrell PM, Kloster M, et al. Association of lung function, chest radiographs and clinical features in infants with cystic fibrosis. Eur Respir J. 2013;42(6):1545-1552
30. Terheggen-Lagro SW, Arets HG, van der Laag J, van der Ent CK. Radiological and functional changes over 3 years in young children with cystic fibrosis. Eur Respir J. 2007;30(2):279-285
31. Li Z, Kosorok MR, Farrell PM, et al. Longitudinal development of mucoid Pseudomonas aeruginosa infection and lung disease progression in children with cystic fibrosis. JAMA. 2005;293(5):581-588
32. Kosorok MR, Zeng L, West SE, et al. Acceleration of lung disease in children with cystic fibrosis after Pseudomonas aeruginosa acquisition. Pediatr Pulmonol. 2001;32(4):277-287
33. Sanders DB, Li Z, Rock MJ, Brody AS, Farrell PM. The sensitivity of lung disease surrogates in detecting chest CT abnormalities in children with cystic fibrosis. Pediatr Pulmonol. 2012;47(6):567-573
34. Sanders DB, Li Z, Brody AS, Farrell PM. Chest computed tomography scores of severity are associated with future lung disease progression in children with cystic fibrosis. Am J Respir Crit Care Med. 2011;184(7):816-821
35. Brasfield D, Hicks G, Soong S, Tiller RE. The chest roentgenogram in cystic fibrosis: a new scoring system. Pediatrics. 1979;63(1):24-29
36. Chrispin AR, Norman A P. The systematic evaluation of the chest radiograph in cystic fibrosis. Pediatr Radiol. 1974;2(2):101-105
37. Koscik RE, Kosorok MR, Farrell PM, et al. Wisconsin cystic fibrosis chest radiograph scoring system: validation and standardization for application to longitudinal studies. Pediatr Pulmonol. 2000;29(6):457-467
38. Sly PD, Brennan S, Gangell C, et al; Australian Respiratory Early Surveillance Team for Cystic Fibrosis (AREST-CF). Lung disease at diagnosis in infants with cystic fibrosis detected by newborn screening. Am J Respir Crit Care Med. 2009;180(2):146-152
39. Stick SM, Brennan S, Murray C, et al Bronchiectasis in infants and preschool children diagnosed with cystic fibrosis after newborn screening. J Pediatr. 2009;155(5):623-628.e1
40. de Jong PA, Nakano Y, Lequin MH, et al. Progressive damage on high resolution computed tomography despite stable lung function in cystic fibrosis. Eur Respir J. 2004;23(1):93-97
41. Gustafsson PM, De Jong PA, Tiddens HA, Lindblad A. Multiple-breath inert gas washout and spirometry versus structural lung disease in cystic fibrosis. Thorax. 2008;63(2):129-134
42. Mott LS, Park J, Murray CP, et al; AREST C F. Progression of early structural lung disease in young children with cystic fibrosis assessed using CT. Thorax. 2012;67(6):509-516
43. Davis SD, Fordham LA, Brody AS, et al. Computed tomography reflects lower airway inflammation and tracks changes in early cystic fibrosis. Am J Respir Crit Care Med. 2007;175(9):943-950
44. Kuo W, Ciet P, Tiddens HA, Zhang W, Guillerman R P, van Straten M. Monitoring cystic fibrosis lung disease by computed tomography. Radiation risk in perspective. Am J Respir Crit Care Med. 2014;189(11):1328-1336
45. Thia LP, Calder A, Stocks J, et al; London Cystic Fibrosis Collaboration. Is chest CT useful in newborn screened infants with cystic fibrosis at 1 year of age? Thorax. 2014;69(4):320-327
46. de Jong PA, Ottink MD, Robben SG, et al. Pulmonary disease assessment in cystic fibrosis: comparison of CT scoring systems and value of bronchial and arterial dimension measurements. Radiology. 2004;231(2):434-439
47. Owens CM, Aurora P, Stanojevic S, et al; London Cystic Fibrosis Collaboration. Lung Clearance Index and HRCT are complementary markers of lung abnormalities in young children with C F. Thorax. 2011;66(6):481-488
48. Yankaskas JR, Marshall BC, Sufian B, Simon RH, Rodman D. Cystic fibrosis adult care: consensus conference report. Chest. 2004;125(suppl 1):1S-39S
49. Douglas TA, Brennan S, Berry L, et al; members of AREST CF and ACFBAL Trial. Value of serology in predicting Pseudomonas aeruginosa infection in young children with cystic fibrosis. Thorax. 2010;65(11):985-990
50. Wainwright CE, Vidmar S, Armstrong DS, et al; ACFBAL Study Investigators. Effect of bronchoalveolar lavage-directed therapy on Pseudomonas aeruginosa infection and structural lung injury in children with cystic fibrosis: a randomized trial. JAMA. 2011;306(2):163-171
51. Jain K, Wainwright C, Smyth AR. Bronchoscopy-guided antimicrobial therapy for cystic fibrosis. Cochrane Database Syst Rev. 2013;(12):CD009530
52. De Boeck K, Alifier M, Vandeputte S. Sputum induction in young cystic fibrosis patients. Eur Respir J. 2000;16(1):91-94
53. Mussaffi H, Fireman EM, Mei-Zahav M, Prais D, Blau H. Induced sputum in the very young: a new key to infection and inflammation. Chest. 2008;133(1):176-182
54. Al-Saleh S, Dell SD, Grasemann H, et al Sputum induction in routine clinical care of children with cystic fibrosis. J Pediatr. 2010;157(6):1006-1011.e1
55. Byrnes CA, Vidmar S, Cheney JL, et al; ACFBAL Study Investigators. Prospective evaluation of respiratory exacerbations in children with cystic fibrosis from newborn screening to 5 years of age. Thorax. 2013;68(7):643-651
56. VanDevanter DR, Elkin E P, Pasta DJ, et al Changing thresholds and incidence of antibiotic treatment of cystic fibrosis pulmonary exacerbations, 1995-2005. J Cyst Fibro. 2013;12(4):332-337
57. Wagener JS, Rasouliyan L, VanDevanter DR, et al; Investigators and Coordinators of the Epidemiologic Study of Cystic Fibrosis. Oral, inhaled, and intravenous antibiotic choice for treating pulmonary exacerbations in cystic fibrosis. Pediatr Pulmonol. 2013;48(7):666-673
58. Bilton D, Canny G, Conway S, et al Pulmonary exacerbation: towards a definition for use in clinical trials. Report from the EuroCareCF Working Group on outcome parameters in clinical trials. J Cyst Fibros. 2011;10(suppl 2):S79-81
59. Regelmann WE, Schechter MS, Wagener JS, et al; Investigators of the Epidemiologic Study of Cystic Fibrosis. Pulmonary exacerbations in cystic fibrosis: young children with characteristic signs and symptoms. Pediatr Pulmonol. 2013;48(7):649-657
60. Rabin HR, Butler SM, Wohl ME, et al; Epidemiologic Study of Cystic Fibrosis. Pulmonary exacerbations in cystic fibrosis. Pediatr Pulmonol. 2004;37(5):400-406
61. Pittman JE, Johnson RC, Davis SD. Improvement in pulmonary function following antibiotics in infants with cystic fibrosis. Pediatr Pulmonol. 2012;47(5):441-446
62. Briggs EC, Nguyen T, Wall MA, MacDonald KD. Oral antimicrobial use in outpatient cystic fibrosis pulmonary exacerbation management: a single-center experience. Clin Respir J. 2012;6(1):56-64
63. Padman R, McColley SA, Miller DP, et al; Investigators and Coordinators of the Epidemiologic Study of Cystic Fibrosis. Infant care patterns at epidemiologic study of cystic fibrosis sites that achieve superior childhood lung function. Pediatrics. 2007;119(3). Available at: www.pediatrics.org/cgi/content/full/119/3/e531
64. van Ewijk BE, van der Zalm MM, Wolfs T F, van der Ent CK. Viral respiratory infections in cystic fibrosis. J Cyst Fibros. 2005;4(suppl 2):31-36
65. Olesen HV, Nielsen L P, Schiotz PO. Viral and atypical bacterial infections in the outpatient pediatric cystic fibrosis clinic. Pediatr Pulmonol. 2006;41(12):1197-1204
66. Wat D, Gelder C, Hibbitts S, et al The role of respiratory viruses in cystic fibrosis. J Cyst Fibros. 2008;7(4):320-328
67. Mogayzel PJ Jr, Naureckas ET, Robinson KA, et al; Cystic Fibrosis Foundation Pulmonary Clinical Practice Guidelines Committee. Cystic Fibrosis Foundation pulmonary guideline. Pharmacologic approaches to prevention and eradication of initial Pseudomonas aeruginosa infection. Ann Am Thorac Soc. 2014;11(10):1640-1650
68. Flume PA, Robinson KA, O'Sullivan B P, et al; Clinical Practice Guidelines for Pulmonary Therapies Committee. Cystic fibrosis pulmonary guidelines: airway clearance therapies. Respir Care. 2009;54(4):522-537
69. 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(5):471-476
70. Dellon EP, Donaldson SH, Johnson R, Davis SD. Safety and tolerability of inhaled hypertonic saline in young children with cystic fibrosis. Pediatr Pulmonol. 2008;43(11):1100-1106
71. Rosenfeld M, Davis S, Brumback L, et al. Inhaled hypertonic saline in infants and toddlers with cystic fibrosis: short-term tolerability, adherence, and safety. Pediatr Pulmonol. 2011;46(7):666-671
72. Elkins MR, Robinson M, Rose BR, et al; National Hypertonic Saline in Cystic Fibrosis (NHSCF) Study Group. A controlled trial of long-term inhaled hypertonic saline in patients with cystic fibrosis. N Engl J Med. 2006;354(3):229-240
73. Rosenfeld M, Ratjen F, Brumback L, et al; ISIS Study Group. Inhaled hypertonic saline in infants and children younger than 6 years with cystic fibrosis: the ISIS randomized controlled trial. JAMA. 2012;307(21):2269-2277
74. Subbarao P, Stanojevic S, Brown M, et al. Lung clearance index as an outcome measure for clinical trials in young children with cystic fibrosis. A pilot study using inhaled hypertonic saline. Am J Respir Crit Care Med. 2013;188(4):456-460
75. Quan JM, Tiddens HA, Sy JP, et al; Pulmozyme Early Intervention Trial Study Group. A two-year randomized, placebo-controlled trial of dornase alfa in young patients with cystic fibrosis with mild lung function abnormalities. J Pediatr. 2001;139(6):813-820
76. McPhail GL, Acton JD, Fenchel MC, Amin RS, Seid M. Improvements in lung function outcomes in children with cystic fibrosis are associated with better nutrition, fewer chronic Pseudomonas aeruginosa infections, and dornase alfa use. J Pediatr. 2008;153(6):752-757
77. Jones A P, Wallis C. Dornase alfa for cystic fibrosis. Cochrane Database Syst Rev. 2010;(3):CD001127
78. Furuya ME, Lezana-Fernández JL, Vargas MH, Hernández-Sierra JF, Ramírez-Figueroa JL. Efficacy of human recombinant DNase in pediatric patients with cystic fibrosis. Arch Med Res. 2001;32(1):30-34
79. Shah PL, Conway S, Scott SF, et al A case-controlled study with dornase alfa to evaluate impact on disease progression over a 4-year period. Respiration. 2001;68(2):160-164
80. Hodson ME, McKenzie S, Harms HK, et al; Investigators of the Epidemiologic Registry of Cystic Fibrosis. Dornase alfa in the treatment of cystic fibrosis in Europe: a report from the Epidemiologic Registry of Cystic Fibrosis. Pediatr Pulmonol. 2003;36(5):427-432
81. Konstan MW, Wagener JS, Pasta DJ, et al; Scientific Advisory Group and Investigators and Coordinators of Epidemiologic Study of Cystic Fibrosis. Clinical use of dornase alpha is associated with a slower rate of FEV1 decline in cystic fibrosis. Pediatr Pulmonol. 2011;46(6):545-553
82. Amin R, Subbarao P, Lou W, et al. The effect of dornase alfa on ventilation inhomogeneity in patients with cystic fibrosis. Eur Respir J. 2011;37(4):806-812
83. Robinson TE, Goris ML, Zhu HJ, et al. Dornase alfa reduces air trapping in children with mild cystic fibrosis lung disease: a quantitative analysis. Chest. 2005;128(4):2327-2335
84. 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):377-382
85. Rozov T, de Oliveira VZ, Santana MA, et al; Pulmozyme Study Group. Dornase alfa improves the health-related quality of life among Brazilian patients with cystic fibrosis-a one-year prospective study. Pediatr Pulmonol. 2010;45(9):874-882
86. Wagener JS, Rock MJ, McCubbin MM, Hamilton SD, Johnson CA, Ahrens RC; Pulmozyme Pediatric Bronchoscopy Study Group. Aerosol delivery and safety of recombinant human deoxyribonuclease in young children with cystic fibrosis: a bronchoscopic study. J Pediatr. 1998;133(4):486-491
87. McKenzie SG, Chowdhury S, Strandvik B, Hodson ME; Investigators of the Epidemiologic Registry of Cystic Fibrosis. Dornase alfa is well tolerated: data from the epidemiologic registry of cystic fibrosis. Pediatr Pulmonol. 2007;42(10):928-937
88. Ratjen F, Saiman L, Mayer-Hamblett N, et al. Effect of azithromycin on systemic markers of inflammation in patients with cystic fibrosis uninfected with Pseudomonas aeruginosa. Chest. 2012;142(5):1259-1266
89. Saiman L, Mayer-Hamblett N, Anstead M, et al; AZ0004 Macrolide Study Team. Open-label, follow-on study of azithromycin in pediatric patients with CF uninfected with Pseudomonas aeruginosa. Pediatr Pulmonol. 2012;47(7):641-648
90. Renna M, Schaffner C, Brown K, et al. Azithromycin blocks autophagy and may predispose cystic fibrosis patients to mycobacterial infection. J Clin Invest. 2011;121(9):3554-3563
91. Levy I, Grisaru-Soen G, Lerner-Geva L, et al. Multicenter cross-sectional study of nontuberculous mycobacterial infections among cystic fibrosis patients, Israel. Emerg Infect Dis. 2008;14(3):378-384
92. Binder AM, Adjemian J, Olivier KN, Prevots DR. Epidemiology of nontuberculous mycobacterial infections and associated chronic macrolide use among persons with cystic fibrosis. Am J Respir Crit Care Med. 2013;188(7):807-812
93. Wainwright CE, Quittner AL, Geller DE, et al Aztreonam for inhalation solution (AZLI) in patients with cystic fibrosis, mild lung impairment, and P. aeruginosa. J Cyst Fibros. 2011;10(4):234-242
94. Oermann CM, Retsch-Bogart GZ, Quittner AL, et al. An 18-month study of the safety and efficacy of repeated courses of inhaled aztreonam lysine in cystic fibrosis. Pediatr Pulmonol. 2010;45(11):1121-1134
95. Retsch-Bogart GZ, Quittner AL, Gibson RL, et al. Efficacy and safety of inhaled aztreonam lysine for airway Pseudomonas in cystic fibrosis. Chest. 2009;135(5):1223-1232
96. McCoy KS, Quittner AL, Oermann CM, Gibson RL, Retsch-Bogart GZ, Montgomery AB. Inhaled aztreonam lysine for chronic airway Pseudomonas aeruginosa in cystic fibrosis. Am J Respir Crit Care Med. 2008;178(9):921-928
97. Treggiari MM, Retsch-Bogart G, Mayer-Hamblett N, et al; Early Pseudomonas Infection Control (EPIC) Investigators. Comparative efficacy and safety of 4 randomized regimens to treat early Pseudomonas aeruginosa infection in children with cystic fibrosis. Arch Pediatr Adolesc Med. 2011;165(9):847-856
98. Gibson RL, Emerson J, McNamara S, et al; Cystic Fibrosis Therapeutics Development Network Study Group. Significant microbiological effect of inhaled tobramycin in young children with cystic fibrosis. Am J Respir Crit Care Med. 2003;167(6):841-849
99. Taccetti G, Bianchini E, Cariani L, et al; Italian Group for P aeruginosa Eradication in Cystic Fibrosis. Early antibiotic treatment for Pseudomonas aeruginosa eradication in patients with cystic fibrosis: a randomised multicentre study comparing two different protocols. Thorax. 2012;67(10):853-859
100. Hennig S, McKay K, Vidmar S, et al Safety of inhaled (Tobi(R)) and intravenous tobramycin in young children with cystic fibrosis. J Cyst Fibros. 2014;13(4):428-434
101. Nguyen TT, Thia LP, Hoo AF, et al; London Cystic Fibrosis Collaboration (LCFC). Evolution of lung function during the first year of life in newborn screened cystic fibrosis infants. Thorax. 2014;69(10):910-917
102. 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(3):299-305
103. Smyth AR, Walters S. Prophylactic anti-staphylococcal antibiotics for cystic fibrosis. Cochrane Database Syst Rev. 2012;(12):CD001912
104. Ratjen F, Comes G, Paul K, Posselt HG, Wagner TO, Harms K; German Board of the European Registry for Cystic Fibrosis (ERCF). Effect of continuous antistaphylococcal therapy on the rate of P. aeruginosa acquisition in patients with cystic fibrosis. Pediatr Pulmonol. 2001;31(1):13-16
105. Chatfield S, Owen G, Ryley HC, et al Neonatal screening for cystic fibrosis in Wales and the West Midlands: clinical assessment after five years of screening. Arch Dis Child. 1991;66(spec no. 1):29-33
106. Weaver LT, Green MR, Nicholson K, et al. Prognosis in cystic fibrosis treated with continuous flucloxacillin from the neonatal period. Arch Dis Child. 1994;70(2):84-89
107. Lo DK, Hurley MN, Muhlebach MS, Smyth AR. Interventions for the eradication of methicillin-resistant Staphylococcus aureus (MRSA) in people with cystic fibrosis. Cochrane Database Syst Rev. 2013;2:CD009650
108. Solís A, Brown D, Hughes J, Van Saene HK, Heaf D P. Methicillin-resistant Staphylococcus aureus in children with cystic fibrosis: an eradication protocol. Pediatr Pulmonol. 2003;36(3):189-195
109. Macfarlane M, Leavy A, McCaughan J, Fair R, Reid AJ. Successful decolonization of methicillin-resistant Staphylococcus aureus in paediatric patients with cystic fibrosis (CF) using a three-step protocol. J Hosp Infect. 2007;65(3):231-236
110. Dalboge CS, Pressler T, Hoiby N, Nielsen KG, Johansen HK. A cohort study of the Copenhagen CF Centre eradication strategy against Staphylococcus aureus in patients with CF. J Cyst Fibros. 2013;12(1):42-48
111. Davies J, Sheridan H, Bell N, et al. Assessment of clinical response to ivacaftor with lung clearance index in cystic fibrosis patients with a G551D-CFTR mutation and preserved spirometry: a randomised controlled trial. Lancet Respir Med. 2013;1(8):630-638
112. Ramsey BW, Davies J, McElvaney NG, et al; VX08-770-102 Study Group. A CFTR potentiator in patients with cystic fibrosis and the G551D mutation. N Engl J Med. 2011;365(18):1663-1672
113. Accurso FJ, Rowe SM, Clancy J P, et al. Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation. N Engl J Med. 2010;363(21):1991-2003
114. Davies CA, Robertson S, Green Y, Rosenfeld M. 200. An open-label study of the safety, pharmacokinetics, and pharmacodynamics of ivacaftor in patients aged 2 to 5 years with CF and a CFTR gating mutation: the KIWI study. Pediatr Pulmonol. 2014;(suppl 38):286
115. Rosenfeld M, Robertson S, Green Y, Cooke J, Lawal A, Davies JC. WS01.5 An open-label study of the safety, pharmacokinetics, and pharmacodynamics of ivacaftor in patients aged 2 to 5 years with cystic fibrosis and a CFTR gating mutation: the KIWI study. J Cyst Fibros. 2015;14:S2
116. Centers for Disease Control and Prevention. Data table of weight-forage charts. August 2001. Available at: www.cdc.gov/growthcharts/html- charts/wtage. htm. Accessed July 28, 2014
117. AAP Committee on Nutrition. Kleinman RE, Greer FR, eds. Pediatric Nutrition. 7th ed. Elk Grove Village, IL: American Academy of Pediatrics; 2014
118. McCammon RW. Human Growth and Development. Springfield, IL.: Thomas; 1970
119. US Department of Agriculture. Office of Disease Prevention and Health Promotion. Dietary guidelines for Americans 2010. Available at: www. dietaryguidelines. gov. Accessed July 28, 2014
120. Dietary Reference Intakes for Energy. Carbohydrate, Fiber, Fat, Fatty Acids, Cholesterol, Protein, and Amino Acids (Macronutrients). Washington D.C.: The National Academies Press; 2005
121. Driscoll KA, Modi AC, Filigno SS, et al. Quality of life in children with CF: psychometrics and relations with stress and mealtime behaviors. Pediatr Pulmonol. 2015;50(6):560-567
122. Groleau V, Schall JI, Dougherty KA, et al Effect of a dietary intervention on growth and energy expenditure in children with cystic fibrosis. J Cyst Fibros. 2014;13(5):572-578
123. Poustie VJ, Russell JE, Watling RM, Ashby D, Smyth RL; CALICO Trial Collaborative Group. Oral protein energy supplements for children with cystic fibrosis: CALICO multicentre randomised controlled trial. BMJ. 2006;332(7542):632-636
124. Piazza-Waggoner C, Driscoll KA, Gilman DK, Powers SW. A comparison using parent report and direct observation of mealtime behaviors in young children with cystic fibrosis: Implications for practical and empirically based behavioral assessment in routine clinical care. Child Health Care. 2008;37(1):38-48
125. Powers SW, Mitchell MJ, Patton SR, et al Mealtime behaviors in families of infants and toddlers with cystic fibrosis. J Cyst Fibros. 2005;4(3):175-182
126. Ward C, Massie J, Glazner J, et al. Problem behaviours and parenting in preschool children with cystic fibrosis. Arch Dis Child. 2009;94(5):341-347
127. Sheehan J, Massie J, Hay M, et al. The natural history and predictors of persistent problem behaviours in cystic fibrosis: a multicentre, prospective study. Arch Dis Child. 2012;97(7):625-631
128. Rosenfeld M, Casey S, Pepe M, Ramsey BW. Nutritional effects of long-term gastrostomy feedings in children with cystic fibrosis. J Am Diet Assoc. 1999;99(2):191-194
129. Best C, Brearley A, Gaillard P, et al. A pre-post retrospective study of patients with cystic fibrosis and gastrostomy tubes. J Pediatr Gastroenterol Nutr. 2011;53(4):453-458
130. Efrati O, Mei-Zahav M, Rivlin J, et al. Long term nutritional rehabilitation by gastrostomy in Israeli patients with cystic fibrosis: clinical outcome in advanced pulmonary disease. J Pediatr Gastroenterol Nutr. 2006;42(2):222-228
131. Bradley GM, Carson KA, Leonard AR, Mogayzel PJ Jr, Oliva-Hemker M. Nutritional outcomes following gastrostomy in children with cystic fibrosis. Pediatr Pulmonol. 2012;47(8):743-748
132. Tangpricha V, Kelly A, Stephenson A, et al; Cystic Fibrosis Foundation Vitamin D Evidence-Based Review Committee. An update on the screening, diagnosis, management, and treatment of vitamin D deficiency in individuals with cystic fibrosis: evidence-based recommendations from the Cystic Fibrosis Foundation. J Clin Endocrinol Metab. 2012;97(4):1082-1093
133. Brodlie M, Orchard WA, Reeks GA, et al. Vitamin D in children with cystic fibrosis. Arch Dis Child. 2012;97(11):982-984
134. Scurati-Manzoni E, Fossali EF, Agostoni C, et al. Electrolyte abnormalities in cystic fibrosis: systematic review of the literature. Pediatr Nephrol. 2014;29(6):1015-1023
135. Borowitz DS, Grand RJ, Durie PR; Consensus Committee. Use of pancreatic enzyme supplements for patients with cystic fibrosis in the context of fibrosing colonopathy. J Pediatr. 1995;127(5):681-684
136. Powers SW, Piazza-Waggoner C, Jones JS, Ferguson KS, Daines C, Acton JD. Examining clinical trial results with single-subject analysis: an example involving behavioral and nutrition treatment for young children with cystic fibrosis. J Pediatr Psychol. 2006;31(6):574-581
137. Stark LJ, Quittner AL, Powers SW, et al. Randomized clinical trial of behavioral intervention and nutrition education to improve caloric intake and weight in children with cystic fibrosis. Arch Pediatr Adolesc Med. 2009;163(10):915-921
138. Stark LJ, Opipari-Arrigan L, Quittner AL, Bean J, Powers SW. The effects of an intensive behavior and nutrition intervention compared to standard of care on weight outcomes in CF. Pediatr Pulmonol. 2011;46(1):31-35
139. Powers SW, Stark LJ, Chamberlin LA, et al. Behavioral and nutritional treatment for preschool-aged children with cystic fibrosis: a randomized clinical trial. JAMA Pediatr. 2015;169(5):e150636
140. Opipari-Arrigan L, Powers SW, Quittner AL, Stark LJ. Mealtime problems predict outcome in clinical trial to improve nutrition in children with C F. Pediatr Pulmonol. 2010;45(1):78-82
141. McDonald CM, Haberman D, Brown N. Self-efficacy: empowering parents of children with cystic fibrosis. J Cyst Fibros. 2013;12(5):538-543
142. McClellan CB, Cohen LL, Moffett K. Time out based discipline strategy for children's non-compliance with cystic fibrosis treatment. Disabil Rehabil. 2009;31(4):327-336
143. Hourigan SE, Helms SW, Christon LM, Southam-Gerow MA. Improving nutrition in toddlers with cystic fibrosis: feasibility of a behavioral parent-training intervention. Clin Pract Pediatr Psychol. 2013;1(3):235-249
144. Children's Hospital Medical Center Cincinnati. National Institute of Diabetes and Digestive and Kidney Diseases. ClinicalTrials.gov [Internet]. Bethesda (MD): National Library of Medicine (US). 2000-2014 Aug 20. A Multi-Site Randomized Clinical Trial of Behavioral and Nutrition Treatment Designed to Help Preschoolers With Cystic Fibrosis Optimize Growth. NLM Identifier: NCT00241969. Available at: https://clinicaltrials. gov/ct2/show/NCT00241969?term= Behavioral+and+Nutrition+Treatment +for+Preschoolers+with+Cystic+Fibr osis& rank= 1. Accessed January 22, 2015
145. Baker SS, Borowitz D, Duffy L, Fitzpatrick L, GyamfiJ, Baker RD. Pancreatic enzyme therapy and clinical outcomes in patients with cystic fibrosis. J Pediatr. 2005;146(2):189-193
146. Tabbers MM, DiLorenzo C, Berger MY, et al; European Society for Pediatric Gastroenterology, Hepatology, and Nutrition; North American Society for Pediatric Gastroenterology. Evaluation and treatment of functional constipation in infants and children: evidence-based recommendations from ESPGHAN and NASPGHAN. J Pediatr Gastroenterol Nutr. 2014;58(2):258-274
147. Houwen RH, van der Doef HP, Sermet I, et al; ESPGHAN Cystic Fibrosis Working Group. Defining DIOS and constipation in cystic fibrosis with a multicentre study on the incidence, characteristics, and treatment of DIOS. J Pediatr Gastroenterol Nutr. 2010;50(1):38-42
148. Vandenplas Y, Rudolph CD, Di Lorenzo C, et al; North American Society for Pediatric Gastroenterology Hepatology and Nutrition; European Society for Pediatric Gastroenterology Hepatology and Nutrition. Pediatric gastroesophageal reflux clinical practice guidelines: joint recommendations of the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition (NASPGHAN) and the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition (ESPGHAN). J Pediatr Gastroenterol Nutr. 2009;49(4):498-547
149. Lisowska A, Wójtowicz J, Walkowiak J. Small intestine bacterial overgrowth is frequent in cystic fibrosis: combined hydrogen and methane measurements are required for its detection. Acta Biochim Pol. 2009;56(4):631-634
150. Hill ID, Dirks MH, Liptak GS, et al; North American Society for Pediatric Gastroenterology, Hepatology and Nutrition. Guideline for the diagnosis and treatment of celiac disease in children: recommendations of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition. J Pediatr Gastroenterol Nutr. 2005;40(1):1-19
151. Agréus L, Svärdsudd K, Nyrén O, Tibblin G. Reproducibility and validity of a postal questionnaire. The abdominal symptom study. Scand J Prim Health Care. 1993;11(4):252-262
152. Brunner HI, Johnson AL, Barron AC, et al. Gastrointestinal symptoms and their association with health-related quality of life of children with juvenile rheumatoid arthritis: validation of a gastrointestinal symptom questionnaire. J Clin Rheumatol. 2005;11(4):194-204
153. Wilschanski M, Novak I. The cystic fibrosis of exocrine pancreas. Cold Spring Harb Perspect Med. 2013;3(5):a009746
154. Ooi CY, Dorfman R, Cipolli M, et al. Type of CFTR mutation determines risk of pancreatitis in patients with cystic fibrosis. Gastroenterology. 2011;140(1):153-161
155. Terlizzi V, Tosco A, Tomaiuolo R, et al Prediction of acute pancreatitis risk based on PIP score in children with cystic fibrosis. J Cyst Fibros. 2014;13(5):579-584
156. Simpson RM, Lloyd DJ, Gvozdanovic D, Russell G. Vitamin B12 deficiency in cystic fibrosis. Acta Paediatr Scand. 1985;74(5):794-796
157. Garcia-Naveiro RUJ. Maldigestion and malabsorption. In: Wyllie R, Hyams JS, Kay M, eds. Pediatric Gastrointestinal and Liver Disease, 4th ed. Philadelphia, PA: Elsevier Saunders; 2011:337-349