ESPEN-ESPGHAN-ECFS guidelines on nutrition care for infants, children, and adults with cystic fibrosis

Clinical Nutrition

Volumn 35, Issue 3, 2016, Pages 557-577

  Turck, Dominique ^a   Braegger, Christian P ^b   Colombo, Carla ^c   Declercq, Dimitri ^d   Morton, Alison ^e   Pancheva, Ruzha ^f   Robberecht, Eddy ^g   Stern, Martin ^h   Strandvik, Birgitta ⁱ   Wolfe, Sue ^j   Schneider, Stephane M ^k   Wilschanski, Michael ^l  

^a UNIV LILLE   (France)

^b UNIVERSITY CHILDREN S HOSPITAL   (Switzerland)

^c UNIVERSITY OF MILAN   (Italy)

^d GHENT UNIVERSITY HOSPITAL   (Belgium)

^e ST JAMES S UNIVERSITY HOSPITAL   (United Kingdom)

^f MEDICAL UNIVERSITY OF VARNA   (Bulgaria)

^g GHENT UNIVERSITY   (Belgium)

^h UNIVERSITY HOSPITAL TÜBINGEN   (Germany)

ⁱ KAROLINSKA INSTITUTE   (Sweden)

^j Leeds Children's Hospital   (United Kingdom)

^k CENTRE HOSPITALIER UNIVERSITAIRE DE NICE   (France)

^l HADASSAH HEBREW UNIVERSITY MEDICAL CENTER   (Israel)

more..

Author keywords

Cystic fibrosis; ESPEN guidelines; Malnutrition; Nutritional assessment; Nutritional support

Indexed keywords

APPETITE STIMULANT; ELASTASE; ESSENTIAL FATTY ACID; GROWTH HORMONE; PANCREAS ENZYME; PROBIOTIC AGENT; VITAMIN; ZINC;

ADULT; AGE; ARTICLE; BODY HEIGHT; BODY WEIGHT; BONE DENSITY; BREAST FEEDING; CALORIC INTAKE; CHILD; CREATININE URINE LEVEL; CYSTIC FIBROSIS; DIET SUPPLEMENTATION; DIET THERAPY; ENTERIC FEEDING; FAMILY; FOLLOW UP; GLUCOSE TOLERANCE TEST; GROWTH RETARDATION; HUMAN; INFANT; INFLAMMATION; IRON DEFICIENCY; LUNG FUNCTION; MALNUTRITION; MEDICAL SOCIETY; MINERAL SUPPLEMENTATION; NUTRITIONAL DEFICIENCY; NUTRITIONAL STATUS; ONLINE SYSTEM; PATIENT EDUCATION; PRACTICE GUIDELINE; SODIUM EXCRETION; SODIUM URINE LEVEL; SURVIVAL; CONSENSUS; DIETARY SUPPLEMENT; DIETETICS; DISEASE EXACERBATION; EUROPE; EVIDENCE BASED MEDICINE; HEALTH CARE ORGANIZATION; HEALTHY DIET; INTERNATIONAL COOPERATION; MALABSORPTION; MULTIMODALITY CANCER THERAPY; NUTRITIONAL SUPPORT; PATHOPHYSIOLOGY; PERSONALIZED MEDICINE; STANDARDS;

ADULT; CHILD; COMBINED MODALITY THERAPY; CONSENSUS; CYSTIC FIBROSIS; DIETARY SUPPLEMENTS; DIETETICS; DISEASE PROGRESSION; EUROPE; EVIDENCE-BASED MEDICINE; HEALTHY DIET; HUMANS; INFANT; INTERNATIONAL AGENCIES; MALABSORPTION SYNDROMES; MALNUTRITION; NUTRITIONAL SUPPORT; PRECISION MEDICINE; SOCIETIES, MEDICAL; SOCIETIES, SCIENTIFIC;

EID: 84973470912     PISSN: 02615614     EISSN: 15321983     Source Type: Journal    
DOI: 10.1016/j.clnu.2016.03.004     Document Type: Article

References (240)

1. Bonifant C.M., Shevill E., Chang A.B. Vitamin A supplementation for cystic fibrosis. Cochrane Database Syst Rev 2014, 5:CD006751.
2. Chinuck R., Dewar J., Baldwin D.R., Hendron E. Appetite stimulants for people with cystic fibrosis. Cochrane Database Syst Rev 2014, 7:CD008190.
3. Ciofu O., Lykkesfeldt J. Antioxidant supplementation for lung disease in cystic fibrosis. Cochrane Database Syst Rev 2014, 8:CD007020.
4. Conwell L.S., Chang A.B. Bisphosphonates for osteoporosis in people with cystic fibrosis. Cochrane Database Syst Rev 2014, 3:CD002010.
5. Ferguson J.H., Chang A.B. Vitamin D supplementation for cystic fibrosis. Cochrane Database Syst Rev 2014, 5:CD007298.
6. Okebukola P.O., Kansra S., Barrett J. Vitamin E supplementation in people with cystic fibrosis. Cochrane Database Syst Rev 2014, 12:CD009422.
7. Smyth R.L., Rayner O. Oral calorie supplements for cystic fibrosis. Cochrane Database Syst Rev 2014, 11:CD000406.
8. Somaraju U.R., Solis-Moya A. Pancreatic enzyme replacement therapy for people with cystic fibrosis. Cochrane Database Syst Rev 2014, 10:CD008227.
9. Borowitz D., Baker R.D., Stallings V. Consensus report on nutrition for pediatric patients with cystic fibrosis. J Pediatr Gastroenterol Nutr 2002, 35:246-259.
10. Cystic Fibrosis Trust Nutritional management of cystic fibrosis 2002, Cystic Fibrosis Trust, Bromley, UK.
11. Sinaasappel M., Stern M., Littlewood J., Wolfe S., Steinkamp G., Heijerman H.G., et al. Nutrition in patients with cystic fibrosis: a European Consensus. J Cyst Fibros 2002, 1:51-75.
12. Preiser J.C., Schneider S.M. ESPEN disease-specific guideline framework. Clin Nutr 2011, 30:549-552.
13. Petrie G., Barnwell E., Grimshaw J. Clinical guidelines: criteria for appraisal for national use 1995, on behalf of the Scottish Intercollegiate Guidelines Network, Royal College of Physicians, Edinburgh.
14. Agency for Health Care Policy and Research Acute pain management, operative or medical procedures and trauma Rockville, MD, USA 1992, p. 92-0032.
15. Atkins D., Best D., Briss P.A., Eccles M., Falck-Ytter Y., Flottorp S., et al. Grading quality of evidence and strength of recommendations. BMJ 2004, 328:1490.
16. Edenborough F.P., Borgo G., Knoop C., Lannefors L., Mackenzie W.E., Madge S., et al. Guidelines for the management of pregnancy in women with cystic fibrosis. J Cyst Fibros 2008, 7(Suppl. 1):S2-S32.
17. Smith C., Winn A., Seddon P., Ranganathan S. A fat lot of good: balance and trends in fat intake in children with cystic fibrosis. J Cyst Fibros 2012, 11:154-157.
18. Culhane S., George C., Pearo B., Spoede E. Malnutrition in cystic fibrosis: a review. Nutr Clin Pract 2013, 28:676-683.
19. FitzSimmons S.C. The changing epidemiology of cystic fibrosis. J Pediatr 1993, 122:1-9.
20. Southern K.W., Munck A., Pollitt R., Travert G., Zanolla L., Dankert-Roelse J., et al. A survey of newborn screening for cystic fibrosis in Europe. J Cyst Fibros 2007, 6:57-65.
21. Farrell P.M. The prevalence of cystic fibrosis in the European Union. J Cyst Fibros 2008, 7:450-453.
22. Cohen-Cymberknoh M., Shoseyov D., Kerem E. Managing cystic fibrosis: strategies that increase life expectancy and improve quality of life. Am J Respir Crit Care Med 2011, 183:1463-1471.
23. Li L., Somerset S. Digestive system dysfunction in cystic fibrosis: challenges for nutrition therapy. Dig Liver Dis 2014, 46:865-874.
24. Engelen M.P., Com G., Deutz N.E.P. Protein is an important but undervalued macronutrient in the nutritional care of patients with cystic fibrosis. Curr Opin Clin Nutr Metab Care 2014, 17:515-520.
25. Kalnins D., Wilschanski M. Maintenance of nutritional status in patients with cystic fibrosis: new and emerging therapies. Drug Des Devel Ther 2012, 6:151-161.
26. Strandvik B. Fatty acid metabolism in cystic fibrosis. Prostagl Leukot Essent Fat Acids 2010, 83:121-129.
27. Gaskin K.J. Nutritional care in children with cystic fibrosis: are our patients becoming better?. Eur J Clin Nutr 2013, 67:558-564.
28. Corey M., McLaughlin F.J., Williams M., Levison H. A comparison of survival, growth, and pulmonary function in patients with cystic fibrosis in Boston and Toronto. J Clin Epidemiol 1988, 41:583-591.
29. Stallings V.A., Stark L.J., Robinson K.A., Feranchak A.P., 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, 108:832-839.
30. McCormick J., Mehta G., Olesen H.V., Viviani L., Macek M., Mehta A., et al. Comparative demographics of the European cystic fibrosis population: a cross-sectional database analysis. Lancet 2010, 375:1007-1013.
31. Maqbool A., Schall J.I., Gallagher P.R., Zemel B.S., Strandvik B., Stallings V.A. Relation between dietary fat intake type and serum fatty acid status in children with cystic fibrosis. J Pediatr Gastroenterol Nutr 2012, 55:605-611.
32. Smyth A.R., Bell S.C., Bojcin S., Bryon M., Duff A., Flume P., et al. European cystic fibrosis Society standards of care: best practice guidelines. J Cyst Fibros 2014, 13(Suppl. 1):S23-S42.
33. Stephenson A.L., Mannik L.A., Walsh S., Brotherwood M., Robert R., Darling P.B., et al. Longitudinal trends in nutritional status and the relation between lung function and BMI in cystic fibrosis: a population-based cohort study. Am J Clin Nutr 2013, 97:872-877.
34. Zolin A., McKone E., van Rens J., Fox A., Iansa P., Preftitsi A., et al. ECFSPR annual report 2010 2014, European Cystic Fibrosis Society, Karup, Denmark.
35. Cystic Fibrosis Foundation Patient Registry 2011 annual data report 2011, Cystic Fibrosis Foundation, Bethesda, Maryland.
36. Perano S., Rayner C.K., Couper J., Martin J., Horowitz M. Cystic fibrosis related diabetes-a new perspective on the optimal management of postprandial glycemia. J Diabetes Complicat 2014, 28:904-911.
37. Debray D., Kelly D., Houwen R., Strandvik B., Colombo C. Best practice guidance for the diagnosis and management of cystic fibrosis-associated liver disease. J Cyst Fibros 2011, 10(Suppl. 2):S29-S36.
38. Dodge J.A., Turck D. Cystic fibrosis: nutritional consequences and management. Best Pract Res Clin Gastroenterol 2006, 20:531-546.
39. Vaisman N., Pencharz P.B., Corey M., Canny G.J., Hahn E. Energy expenditure of patients with cystic fibrosis. J Pediatr 1987, 111:496-500.
40. Castro M., Diamanti A., Gambarara M., Bella S., Lucidi V., Papadatou B., et al. Resting energy expenditure in young patients with cystic fibrosis receiving antibiotic therapy for acute respiratory exacerbations. Clin Nutr 2002, 21:141-144.
41. Mc Closkey M., Redmond A.O., Mc Cabe C., Pyper S., Westerterp K.R., Elborn S.J. Energy balance in cystic fibrosis when stable and during a respiratory exacerbation. Clin Nutr 2004, 23:1405-1412.
42. Elborn J.S. How can we prevent multisystem complications of cystic fibrosis?. Semin Respir Crit Care Med 2007, 28:303-311.
43. Morton A.M. Symposium 6: young people, artificial nutrition and transitional care. The nutritional challenges of the young adult with cystic fibrosis: transition. Proc Nutr Soc 2009, 68:430-440.
44. Doring G., Flume P., Heijerman H., Elborn J.S., G. Treatment of lung infection in patients with cystic fibrosis: current and future strategies. J Cyst Fibros 2012, 11:461-479.
45. Greer R., Shepherd R., Cleghorn G., Bowling F.G., Holt T. Evaluation of growth and changes in body composition following neonatal diagnosis of cystic fibrosis. J Pediatr Gastroenterol Nutr 1991, 13:52-58.
46. Konstan M.W., Butler S.M., Wohl M.E., Stoddard M., Matousek R., Wagener J.S., et al. Growth and nutritional indexes in early life predict pulmonary function in cystic fibrosis. J Pediatr 2003, 142:624-630.
47. Koscik R.L., Farrell P.M., Kosorok M.R., Zaremba K.M., Laxova A., Lai H.C., et al. Cognitive function of children with cystic fibrosis: deleterious effect of early malnutrition. Pediatrics 2004, 113:1549-1558.
48. Vieni G., Faraci S., Collura M., Lombardo M., Traverso G., Cristadoro S., et al. Stunting is an independent predictor of mortality in patients with cystic fibrosis. Clin Nutr 2013, 32:382-385.
49. Shoff S.M., Tluczek A., Laxova A., Farrell P.M., Lai H.J. Nutritional status is associated with health-related quality of life in children with cystic fibrosis aged 9-19 years. J Cyst Fibros 2013, 12:746-753.
50. Alicandro G., Frova L., Di Fraia G., Colombo C. Cystic fibrosis mortality trend in Italy from 1970 to 2011. J Cyst Fibros 2015, 14:267-274.
51. Alicandro G., Battezzati P.M., Battezzati A., Speziali C., Claut L., Motta V., et al. Insulin secretion, nutritional status and respiratory function in cystic fibrosis patients with normal glucose tolerance. Clin Nutr 2012, 31:118-123.
52. Lanng S., Thorsteinsson B., Nerup J., Koch C. Influence of the development of diabetes mellitus on clinical status in patients with cystic fibrosis. Eur J Pediatr 1992, 151:684-687.
53. Waugh N., Royle P., Craigie I., Ho V., Pandit L., Ewings P., et al. Screening for cystic fibrosis-related diabetes: a systematic review. Health Technol Assess 2012, 16. iii-iv, 1-179.
54. Lindblad A., Glaumann H., Strandvik B. Natural history of liver disease in cystic fibrosis. Hepatology 1999, 30:1151-1158.
55. Peretti N., Marcil V., Drouin E., Levy E. Mechanisms of lipid malabsorption in Cystic Fibrosis: the impact of essential fatty acids deficiency. Nutr Metab (Lond) 2005, 2:11.
56. Conway S.P., Morton A.M., Oldroyd B., Truscott J.G., White H., Smith A.H., et al. Osteoporosis and osteopenia in adults and adolescents with cystic fibrosis: prevalence and associated factors. Thorax 2000, 55:798-804.
57. Legroux-Gerot I., Leroy S., Prudhomme C., Perez T., Flipo R.M., Wallaert B., et al. Bone loss in adults with cystic fibrosis: prevalence, associated factors, and usefulness of biological markers. Jt Bone Spine 2012, 79:73-77.
58. Javier R.M., Jacquot J. Bone disease in cystic fibrosis: what's new?. Jt Bone Spine 2011, 78:445-450.
59. Lai H.C., Kosorok M.R., Laxova A., Davis L.A., FitzSimmon S.C., Farrell P.M. Nutritional status of patients with cystic fibrosis with meconium ileus: a comparison with patients without meconium ileus and diagnosed early through neonatal screening. Pediatrics 2000, 105:53-61.
60. Yen E.H., 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. 530-535 e531.
61. Giglio L., Candusso M., D'Orazio C., Mastella G., Faraguna D. Failure to thrive: the earliest feature of cystic fibrosis in infants diagnosed by neonatal screening. Acta Paediatr 1997, 86:1162-1165.
62. Borowitz D., Robinson K.A., Rosenfeld M., Davis S.D., Sabadosa K.A., et al. Cystic Fibrosis Foundation evidence-based guidelines for management of infants with cystic fibrosis. J Pediatr 2009, 155:S73-S93. Cystic Fibrosis Foundation.
63. Sermet-Gaudelus I., Mayell S.J., Southern K.W. Guidelines on the early management of infants diagnosed with cystic fibrosis following newborn screening. J Cyst Fibros 2010, 9:323-329. European Cystic Fibrosis Society NSWG.
64. The WHO child growth standards 2006, WHO Multicentre Growth Reference Study Group. http://www.who.int/childgrowth/en/.
65. Cystic Fibrosis Trust Standards for the clinical care of children and adults with cystic fibrosis in the UK December 2011, Cystic Fibrosis Trust, Bromley, UK.
66. Sathe M.N., Patel A.S. Update in pediatrics: focus on fat-soluble vitamins. Nutr Clin Pract 2010, 25:340-346.
67. Wood L.G., Gibson P.G., Garg M.L. Circulating markers to assess nutritional therapy in cystic fibrosis. Clin Chim Acta 2005, 353:13-29.
68. Alicandro G., Bisogno A., Battezzati A., Bianchi M.L., Corti F., Colombo C. Recurrent pulmonary exacerbations are associated with low fat free mass and low bone mineral density in young adults with cystic fibrosis. J Cyst Fibros 2014, 13:328-334.
69. Ionescu A.A., Nixon L.S., Luzio S., Lewis-Jenkins V., Evans W.D., Stone M.D., et al. Pulmonary function, body composition, and protein catabolism in adults with cystic fibrosis. Am J Respir Crit Care Med 2002, 165:495-500.
70. Aris R.M., Merkel P.A., Bachrach L.K., Borowitz D.S., Boyle M.P., Elkin S.L., et al. Guide to bone health and disease in cystic fibrosis. J Clin Endocrinol Metab 2005, 90:1888-1896.
71. Sermet-Gaudelus I., Bianchi M.L., Garabedian M., Aris R.M., Morton A., Hardin D.S., et al. European cystic fibrosis bone mineralisation guidelines. J Cyst Fibros 2011, 10(Suppl. 2):S16-S23.
72. DiMagno E.P., Go V.L., Summerskill W.H. Relations between pancreatic enzyme ouputs and malabsorption in severe pancreatic insufficiency. N Engl J Med 1973, 288:813-815.
73. Ledder O., Haller W., Couper R.T., Lewindon P., Oliver M. Cystic fibrosis: an update for clinicians. Part 2: Hepatobiliary and pancreatic manifestations. J Gastroenterol Hepatol 2014, 29:1954-1962.
74. McArdle J.R. Pregnancy in cystic fibrosis. Clin Chest Med 2011, 32. 111-120, ix.
75. Robinson K.A., Saldanha I.J., McKoy N.A. 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. J Pediatr 2009, 155:S94-S105.
76. Colombo C., Costantini D., Zazzeron L., Faelli N., Russo M.C., Ghisleni D., et al. Benefits of breastfeeding in cystic fibrosis: a single-centre follow-up survey. Acta Paediatr 2007, 96:1228-1232.
77. Kerem E., Conway S., Elborn S., Heijerman H., Consensus C. Standards of care for patients with cystic fibrosis: a European consensus. J Cyst Fibros 2005, 4:7-26.
78. Hammons A.J., Fiese B. Mealtime interactions in families of a child with cystic fibrosis: a meta-analysis. J Cyst Fibros 2010, 9:377-384.
79. Crist W., McDonnell P., Beck M., Gillespie C.T., Barrett P., Mathews J. Behavior at mealtimes and the young child with cystic fibrosis. J Dev Behav Pediatr 1994, 15:157-161.
80. Duff A.J., Wolfe S.P., Dickson C., Conway S.P., Brownlee K.G. Feeding behavior problems in children with cystic fibrosis in the UK: prevalence and comparison with healthy controls. J Pediatr Gastroenterol Nutr 2003, 36:443-447.
81. Stark L.J., Jelalian E., Powers S.W., Mulvihill M.M., Opipari L.C., Bowen A., et al. Parent and child mealtime behavior in families of children with cystic fibrosis. J Pediatr 2000, 136:195-200.
82. EFSA Panel on Dietetic Products Nutrition and Allergies. Scientific opinion on dietary reference values for energy. EFSA J 2013, 11. 3005, 112pp.
83. Matel J.L. Nutritional management of cystic fibrosis. JPEN J Parenter Enteral Nutr 2012, 36:60S-67S.
84. Woestenenk J.W., Castelijns S.J., van der Ent C.K., Houwen R.H. Dietary intake in children and adolescents with cystic fibrosis. Clin Nutr 2014, 33:528-532.
85. Arvanitakis S.N., Lobeck C.C. Metabolic alkalosis and salt depletion in cystic fibrosis. J Pediatr 1973, 82:535-536.
86. Coates A.J., Crofton P.M., Marshall T. Evaluation of salt supplementation in CF infants. J Cyst Fibros 2009, 8:382-385.
87. Ozcelik U., Gocmen A., Kiper N., Coskun T., Yilmaz E., Ozguc M. Sodium chloride deficiency in cystic fibrosis patients. Eur J Pediatr 1994, 153:829-831.
88. Commission Directive 2006/141/EC of 22 December 2006 on infant formulae and follow-on formulae and amending Directive 1999/21/EC Text with EEA relevance. Off J Eur Union 2006, L 401/1.
89. Meuller D. Medical nutrition therapy for pulmonoary disease. Krause's food and the nutrition care process 2012, 782-798. Elsevier Saunders, St Louis, MO. L. Mahan, S. Escott-Stump, J. Raymond (Eds.).
90. Cystic Fibrosis Trust Bone Mineralisation Working Group, Bone mineralisation in cystic fibrosis 2007, Cystic Fibrosis Trust, Bromley, UK.
91. Schulze K.J., O'Brien K O., Germain-Lee E.L., Baer D.J., Leonard A.L., Rosenstein B.J. Endogenous fecal losses of calcium compromise calcium balance in pancreatic-insufficient girls with cystic fibrosis. J Pediatr 2003, 143:765-771.
92. EFSA NDA Panel (EFSA Panel on Dietetic Products Nutrition and Allergies) Scientific opinion on dietary reference values for calcium. EFSA J 2015, 13(5):4101-4183. doi:10.2903/j.efsa.2015.4101.
93. Cheng S., Lyytikainen A., Kroger H., Lamberg-Allardt C., Alen M., Koistinen A., et al. Effects of calcium, dairy product, and vitamin D supplementation on bone mass accrual and body composition in 10-12-y-old girls: a 2-y randomized trial. Am J Clin Nutr 2005, 82:1115-1126. quiz 1147-1118.
94. Uijterschout L., Nuijsink M., Hendriks D., Vos R., Brus F. Iron deficiency occurs frequently in children with cystic fibrosis. Pediatr Pulmonol 2014, 49:458-462.
95. Gifford A.H., Miller S.D., Jackson B.P., Hampton T.H., O'Toole G.A., Stanton B.A., et al. Iron and CF-related anemia: expanding clinical and biochemical relationships. Pediatr Pulmonol 2011, 46:160-165.
96. von Drygalski A., Biller J. Anemia in cystic fibrosis: incidence, mechanisms, and association with pulmonary function and vitamin deficiency. Nutr Clin Pract 2008, 23:557-563.
97. Berry A.J. Pancreatic enzyme replacement therapy during pancreatic insufficiency. Nutr Clin Pract 2014, 29:312-321.
98. Uijterschout L., Swinkels D.W., Akkermans M.D., Zandstra T., Nuijsink M., Hendriks D., et al. The value of soluble transferrin receptor and hepcidin in the assessment of iron status in children with cystic fibrosis. J Cyst Fibros 2014, 13:639-644.
99. Fischer R., Simmerlein R., Huber R.M., Schiffl H., Lang S.M. Lung disease severity, chronic inflammation, iron deficiency, and erythropoietin response in adults with cystic fibrosis. Pediatr Pulmonol 2007, 42:1193-1197.
100. Weiss G., Goodnough L.T. Anemia of chronic disease. N Engl J Med 2005, 352:1011-1023.
101. Akanli L., Lowenthal D.B., Gjonaj S., Dozor A.J. Plasma and red blood cell zinc in cystic fibrosis. Pediatr Pulmonol 2003, 35:2-7.
102. Maqbool A., Schall J.I., Zemel B.S., Garcia-Espana J.F., Stallings V.A. Plasma zinc and growth status in preadolescent children with cystic fibrosis. J Pediatr Gastroenterol Nutr 2006, 43:95-101.
103. Neve J., Van Geffel R., Hanocq M., Molle L. Plasma and erythrocyte zinc, copper and selenium in cystic fibrosis. Acta Paediatr Scand 1983, 72:437-440.
104. Van Biervliet S., Van Biervliet J.-P., Robberecht E., Taylor C. Importance of zinc in cystic fibrosis patients. Curr Pediatr Rev 2009, 5:184-188.
105. Griese M., Kappler M., Eismann C., Ballmann M., Junge S., Rietschel E., et al. Inhalation treatment with glutathione in patients with cystic fibrosis. A randomized clinical trial. Am J Respir Crit Care Med 2013, 188:83-89.
106. Shamseer L., Adams D., Brown N., Johnson J.A., Vohra S. Antioxidant micronutrients for lung disease in cystic fibrosis. Cochrane Database Syst Rev 2010, CD007020.
107. Winklhofer-Roob B.M., Tiran B., Tuchschmid P.E., van't Hof M.A., Shmerling D.H. Effects of pancreatic enzyme preparations on erythrocyte glutathione peroxidase activities and plasma selenium concentrations in cystic fibrosis. Free Radic Biol Med 1998, 25:242-249.
108. Gallagher M. Intake: the nutrients and their metabolism. Krause's food and the nutrition care process 2012, 32-128. Elsevier Saunders, St Louis, MO. L. Mahan, S. Escott-Stump, J. Raymond (Eds.).
109. Dorlochter L., Aksnes L., Fluge G. Faecal elastase-1 and fat-soluble vitamin profiles in patients with cystic fibrosis in Western Norway. Eur J Nutr 2002, 41:148-152.
110. Rana M., Wong-See D., Katz T., Gaskin K., Whitehead B., Jaffe A., et al. Fat-soluble vitamin deficiency in children and adolescents with cystic fibrosis. J Clin Pathol 2014, 67:605-608.
111. Maqbool A., Stallings V.A. Update on fat-soluble vitamins in cystic fibrosis. Curr Opin Pulm Med 2008, 14:574-581.
112. Carr S.B., Dinwiddie R. Annual review or continuous assessment?. J R Soc Med 1996, 89(Suppl. 27):3-7.
113. Feranchak A.P., Sontag M.K., Wagener J.S., Hammond K.B., Accurso F.J., Sokol R.J. Prospective, long-term study of fat-soluble vitamin status in children with cystic fibrosis identified by newborn screen. J Pediatr 1999, 135:601-610.
114. Brei C., Simon A., Krawinkel M.B., Naehrlich L. Individualized vitamin A supplementation for patients with cystic fibrosis. Clin Nutr 2013, 32:805-810.
115. Lancellotti L., D'Orazio C., Mastella G., Mazzi G., Lippi U. Deficiency of vitamins E and A in cystic fibrosis is independent of pancreatic function and current enzyme and vitamin supplementation. Eur J Pediatr 1996, 155:281-285.
116. Hakim F., Kerem E., Rivlin J., Bentur L., Stankiewicz H., Bdolach-Abram T., et al. Vitamins A and E and pulmonary exacerbations in patients with cystic fibrosis. J Pediatr Gastroenterol Nutr 2007, 45:347-353.
117. Cameron C., Lodes M.W., Gershan W.M. Facial nerve palsy associated with a low serum vitamin A level in an infant with cystic fibrosis. J Cyst Fibros 2007, 6:241-243.
118. Ansari E.A., Sahni K., Etherington C., Morton A., Conway S.P., Moya E., et al. Ocular signs and symptoms and vitamin A status in patients with cystic fibrosis treated with daily vitamin A supplements. Br J Ophthalmol 1999, 83:688-691.
119. Scientific Committee on Food Tolerable upper intake levels for vitamins and minerals 2006, European Food Safety Authority.
120. Lindblad A., Diczfalusy U., Hultcrantz R., Thorell A., Strandvik B. Vitamin A concentration in the liver decreases with age in patients with cystic fibrosis. J Pediatr Gastroenterol Nutr 1997, 24:264-270.
121. Maqbool A., Graham-Maar R.C., Schall J.I., Zemel B.S., Stallings V.A. Vitamin A intake and elevated serum retinol levels in children and young adults with cystic fibrosis. J Cyst Fibros 2008, 7:137-141.
122. Rust P., Eichler I., Renner S., Elmadfa I. Long-term oral beta-carotene supplementation in patients with cystic fibrosis - effects on antioxidative status and pulmonary function. Ann Nutr Metab 2000, 44:30-37.
123. Shenkin A. Physiological function and deficiency states of vitamins. Basics in clinical nutrition 2011, 145-153. Galén, Prague. 4th ed. L. Sobotka (Ed.).
124. Graham-Maar R.C., Schall J.I., Stettler N., Zemel B.S., Stallings V.A. Elevated vitamin A intake and serum retinol in preadolescent children with cystic fibrosis. Am J Clin Nutr 2006, 84:174-182.
125. Myhre A.M., Carlsen M.H., Bohn S.K., Wold H.L., Laake P., Blomhoff R. Water-miscible, emulsified, and solid forms of retinol supplements are more toxic than oil-based preparations. Am J Clin Nutr 2003, 78:1152-1159.
126. IOM (Institute of Medicine) Dietary reference intakes: vitamin A, K, iron, zinc, and other elements 2001, 773. National Academies Press, Washington, DC.
127. Smets K.J., Barlow T., Vanhaesebrouck P. Maternal vitamin A deficiency and neonatal microphthalmia: complications of biliopancreatic diversion?. Eur J Pediatr 2006, 165:502-504.
128. Green D., Carson K., Leonard A., Davis J.E., Rosenstein B., Zeitlin P., et al. Current treatment recommendations for correcting vitamin D deficiency in pediatric patients with cystic fibrosis are inadequate. J Pediatr 2008, 153:554-559.
129. Hall W.B., Sparks A.A., Aris R.M. Vitamin d deficiency in cystic fibrosis. Int J Endocrinol 2010, 2010:218691.
130. Robberecht E., Vandewalle S., Wehlou C., Kaufman J.M., De Schepper J. Sunlight is an important determinant of vitamin D serum concentrations in cystic fibrosis. Eur J Clin Nutr 2011, 65:574-579.
131. Tangpricha V., Kelly A., Stephenson A., Maguiness K., Enders J., Robinson K.A., et al. 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, 97:1082-1093.
132. EFSA panel on dietetic products nutrition and allergies. Scientific opinion on the tolerable upper intake level of vitamin D. EFSA J 2012, 10. 2813, 45pp.
133. Braegger C., Campoy C., Colomb V., Decsi T., Domellof M., Fewtrell M., et al. Vitamin D in the healthy European paediatric population. J Pediatr Gastroenterol Nutr 2013, 56:692-701.
134. IOM (Institute of Medicine) Dietary reference intakes for calcium and vitamin D 2011, The National Academies Press, Washington, D.C.
135. Tripkovic L., Lambert H., Hart K., Smith C.P., Bucca G., Penson S., et al. Comparison of vitamin D2 and vitamin D3 supplementation in raising serum 25-hydroxyvitamin D status: a systematic review and meta-analysis. Am J Clin Nutr 2012, 95:1357-1364.
136. Shepherd D., Belessis Y., Katz T., Morton J., Field P., Jaffe A. Single high-dose oral vitamin D(3) (stoss) therapy - a solution to vitamin D deficiency in children with cystic fibrosis?. J Cyst Fibros 2013, 12:177-182.
137. Mailhot G. Vitamin D bioavailability in cystic fibrosis: a cause for concern?. Nutr Rev 2012, 70:280-293.
138. Lagrange-Puget M., Durieu I., Ecochard R., Abbas-Chorfa F., Drai J., Steghens J.P., et al. Longitudinal study of oxidative status in 312 cystic fibrosis patients in stable state and during bronchial exacerbation. Pediatr Pulmonol 2004, 38:43-49.
139. Cantin A.M., Bilodeau G., Ouellet C., Liao J., Hanrahan J.W. Oxidant stress suppresses CFTR expression. Am J Physiol Cell Physiol 2006, 290:C262-C270.
140. Huang S.H., Schall J.I., Zemel B.S., Stallings V.A. Vitamin E status in children with cystic fibrosis and pancreatic insufficiency. J Pediatr 2006, 148:556-559.
141. Winklhofer-Roob B.M., van't Hof M.A., Shmerling D.H. Long-term oral vitamin E supplementation in cystic fibrosis patients: RRR-alpha-tocopherol compared with all-rac-alpha-tocopheryl acetate preparations. Am J Clin Nutr 1996, 63:722-728.
142. Rhodes B., Nash E.F., Tullis E., Pencharz P.B., Brotherwood M., Dupuis A., et al. Prevalence of dyslipidemia in adults with cystic fibrosis. J Cyst Fibros 2010, 9:24-28.
143. Nast D., Paniagua C., Anderson P. Cystic fibrosis: a clinician's tool for management of care advancing into the adult population. J Am Acad Nurse Pract 2012, 24:625-632.
144. Dougherty K.A., Schall J.I., Stallings V.A. Suboptimal vitamin K status despite supplementation in children and young adults with cystic fibrosis. Am J Clin Nutr 2010, 92:660-667.
145. Rashid M., Durie P., Andrew M., Kalnins D., Shin J., Corey M., et al. Prevalence of vitamin K deficiency in cystic fibrosis. Am J Clin Nutr 1999, 70:378-382.
146. Conway S.P., Wolfe S.P., Brownlee K.G., White H., Oldroyd B., Truscott J.G., et al. Vitamin K status among children with cystic fibrosis and its relationship to bone mineral density and bone turnover. Pediatrics 2005, 115:1325-1331.
147. Drury D., Grey V.L., Ferland G., Gundberg C., Lands L.C. Efficacy of high dose phylloquinone in correcting vitamin K deficiency in cystic fibrosis. J Cyst Fibros 2008, 7:457-459.
148. Jagannath V.A., Fedorowicz Z., Thaker V., Chang A.B. Vitamin K supplementation for cystic fibrosis. Cochrane Database Syst Rev 2011, CD008482.
149. Borowitz D., Konstan M.W., O'Rourke A., Cohen M., Hendeles L., Murray F.T. Coefficients of fat and nitrogen absorption in healthy subjects and individuals with cystic fibrosis. J Pediatr Pharmacol Ther 2007, 12:47-52.
150. Giuliano C.A., Dehoorne-Smith M.L., Kale-Pradhan P.B. Pancreatic enzyme products: digesting the changes. Ann Pharmacother 2011, 45:658-666.
151. Trapnell B.C., Maguiness K., Graff G.R., Boyd D., Beckmann K., Caras S. Efficacy and safety of Creon 24,000 in subjects with exocrine pancreatic insufficiency due to cystic fibrosis. J Cyst Fibros 2009, 8:370-377.
152. Wier H.A., Kuhn R.J. Pancreatic enzyme supplementation. Curr Opin Pediatr 2011, 23:541-544.
153. Wooldridge J.L., Heubi J.E., Amaro-Galvez R., Boas S.R., Blake K.V., Nasr S.Z., et al. EUR-1008 pancreatic enzyme replacement is safe and effective in patients with cystic fibrosis and pancreatic insufficiency. J Cyst Fibros 2009, 8:405-417.
154. Haupt M.E., Kwasny M.J., Schechter M.S., McColley S.A. Pancreatic enzyme replacement therapy dosing and nutritional outcomes in children with cystic fibrosis. J Pediatr 2014, 164. 1110-1115 e1111.
155. Dominguez-Munoz J.E. Pancreatic enzyme therapy for pancreatic exocrine insufficiency. Curr Gastroenterol Rep 2007, 9:116-122.
156. Brady M.S., Garson J.L., Krug S.K., Kaul A., Rickard K.A., Caffrey H.H., et al. An enteric-coated high-buffered pancrelipase reduces steatorrhea in patients with cystic fibrosis: a prospective, randomized study. J Am Diet Assoc 2006, 106:1181-1186.
157. Colombo C., Fredella C., Russo M.C., 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, 38:693-699.
158. Kashirskaya N.Y., Kapranov N.I., Sander-Struckmeier S., Kovalev V. Safety and efficacy of Creon(R) Micro in children with exocrine pancreatic insufficiency due to cystic fibrosis. J Cyst Fibros 2014, 14:275-281.
159. Munck A., Duhamel J.F., Lamireau T., Le Luyer B., Le Tallec C., Bellon G., et al. Pancreatic enzyme replacement therapy for young cystic fibrosis patients. J Cyst Fibros 2009, 8:14-18.
160. Graff G.R., Maguiness K., McNamara J., Morton R., Boyd D., Beckmann K., et al. Efficacy and tolerability of a new formulation of pancrelipase delayed-release capsules in children aged 7 to 11 years with exocrine pancreatic insufficiency and cystic fibrosis: a multicenter, randomized, double-blind, placebo-controlled, two-period crossover, superiority study. Clin Ther 2010, 32:89-103.
161. Graff G.R., McNamara J., Royall J., Caras S., Forssmann K. Safety and tolerability of a new formulation of pancrelipase delayed-release capsules (CREON) in children under seven years of age with exocrine pancreatic insufficiency due to cystic fibrosis: an open-label, multicentre, single-treatment-arm study. Clin Drug Investig 2010, 30:351-364.
162. Maqbool A., Schall J.I., Garcia-Espana J.F., Zemel B.S., Strandvik B., Stallings V.A. Serum linoleic acid status as a clinical indicator of essential fatty acid status in children with cystic fibrosis. J Pediatr Gastroenterol Nutr 2008, 47:635-644.
163. Steinkamp G., Demmelmair H., Ruhl-Bagheri I., von der Hardt H., Koletzko B. Energy supplements rich in linoleic acid improve body weight and essential fatty acid status of cystic fibrosis patients. J Pediatr Gastroenterol Nutr 2000, 31:418-423.
164. van Egmond A.W., Kosorok M.R., Koscik R., Laxova A., Farrell P.M. Effect of linoleic acid intake on growth of infants with cystic fibrosis. Am J Clin Nutr 1996, 63:746-752.
165. Conway S., Morton A., Wolfe S. Enteral tube feeding for cystic fibrosis. Cochrane Database Syst Rev 2012, 12:CD001198.
166. Olsen E.M., Petersen J., Skovgaard A.M., Weile B., Jorgensen T., Wright C.M. Failure to thrive: the prevalence and concurrence of anthropometric criteria in a general infant population. Arch Dis Child 2007, 92:109-114.
167. Lai H.J., Shoff S.M. Classification of malnutrition in cystic fibrosis: implications for evaluating and benchmarking clinical practice performance. Am J Clin Nutr 2008, 88:161-166.
168. Kindstedt-Arfwidson K., Strandvik B. Food intake in patients with cystic fibrosis on an ordinary diet. Scand J Gastroenterol Suppl 1988, 143:160-162.
169. Roulet M., Frascarolo P., Rappaz I., Pilet M. Essential fatty acid deficiency in well nourished young cystic fibrosis patients. Eur J Pediatr 1997, 156:952-956.
170. Jelalian E., Stark L.J., Reynolds L., Seifer R. Nutrition intervention for weight gain in cystic fibrosis: a meta analysis. J Pediatr 1998, 132:486-492.
171. Powers S.W., Mitchell M.J., Patton S.R., Byars K.C., Jelalian E., Mulvihill M.M., et al. Mealtime behaviors in families of infants and toddlers with cystic fibrosis. J Cyst Fibros 2005, 4:175-182.
172. Stark L.J., Quittner A.L., Powers S.W., Opipari-Arrigan L., Bean J.A., Duggan C., 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, 163:915-921.
173. Stark L.J., Opipari-Arrigan L., Quittner A.L., Bean J., Powers S.W. The effects of an intensive behavior and nutrition intervention compared to standard of care on weight outcomes in CF. Pediatr Pulmonol 2011, 46:31-35.
174. Abbott J., Morton A.M., Musson H., Conway S.P., Etherington C., Gee L., et al. Nutritional status, perceived body image and eating behaviours in adults with cystic fibrosis. Clin Nutr 2007, 26:91-99.
175. Shearer J.E., Bryon M. The nature and prevalence of eating disorders and eating disturbance in adolescents with cystic fibrosis. J R Soc Med 2004, 97(Suppl. 44):36-42.
176. Gunnell S., Christensen N.K., McDonald C., Jackson D. Attitudes toward percutaneous endoscopic gastrostomy placement in cystic fibrosis patients. J Pediatr Gastroenterol Nutr 2005, 40:334-338.
177. Rettammel A.L., Marcus M.S., Farrell P.M., Sondel S.A., Koscik R.E., Mischler E.H. Oral supplementation with a high-fat, high-energy product improves nutritional status and alters serum lipids in patients with cystic fibrosis. J Am Diet Assoc 1995, 95:454-459.
178. Shepherd R.W., Holt T.L., Cleghorn G., Ward L.C., Isles A., Francis P. Short-term nutritional supplementation during management of pulmonary exacerbations in cystic fibrosis: a controlled study, including effects of protein turnover. Am J Clin Nutr 1988, 48:235-239.
179. Caramia G., Cocchi M., Gagliardini R., Malavolta M., Mozzon M., Frega N.G. Fatty acids composition of plasma phospholipids and triglycerides in children with cystic fibrosis. The effect of dietary supplementation with an olive and soybean oils mixture. Pediatr Med Chir 2003, 25:42-49.
180. Olveira G., Olveira C., Acosta E., Espildora F., Garrido-Sanchez L., Garcia-Escobar E., et al. Fatty acid supplements improve respiratory, inflammatory and nutritional parameters in adults with cystic fibrosis. Arch Bronconeumol 2010, 46:70-77.
181. Best C., Brearley A., Gaillard P., Regelmann W., Billings J., Dunitz J., et al. A pre-post retrospective study of patients with cystic fibrosis and gastrostomy tubes. J Pediatr Gastroenterol Nutr 2011, 53:453-458.
182. Bradley G.M., Blackman S.M., Watson C.P., Doshi V.K., Cutting G.R. Genetic modifiers of nutritional status in cystic fibrosis. Am J Clin Nutr 2012, 96:1299-1308.
183. Efrati O., Mei-Zahav M., Rivlin J., Kerem E., Blau H., Barak A., 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, 42:222-228.
184. Steinkamp G., von der Hardt H. Improvement of nutritional status and lung function after long-term nocturnal gastrostomy feedings in cystic fibrosis. J Pediatr 1994, 124:244-249.
185. Truby H., Cowlishaw P., O'Neil C., Wainwright C. The long term efficacy of gastrostomy feeding in children with cystic fibrosis on anthropometric markers of nutritonal status and pulmonary function. Open Respir Med J 2009, 3:112-115.
186. Van Biervliet S., De Waele K., Van Winckel M., Robberecht E. Percutaneous endoscopic gastrostomy in cystic fibrosis: patient acceptance and effect of overnight tube feeding on nutritional status. Acta Gastroenterol Belg 2004, 67:241-244.
187. Walker S.A., Gozal D. Pulmonary function correlates in the prediction of long-term weight gain in cystic fibrosis patients with gastrostomy tube feedings. J Pediatr Gastroenterol Nutr 1998, 27:53-56.
188. Houwen R.H., van der Doef H.P., Sermet I., Munck A., Hauser B., Walkowiak J., et al. Defining DIOS and constipation in cystic fibrosis with a multicentre study on the incidence, characteristics, and treatment of DIOS. J Pediatr Gastroenterol Nutr 2010, 50:38-42.
189. Colombo C., Ellemunter H., Houwen R., Munck A., Taylor C., Wilschanski M., et al. Guidelines for the diagnosis and management of distal intestinal obstruction syndrome in cystic fibrosis patients. J Cyst Fibros 2011, 10(Suppl. 2):S24-S28.
190. Haworth C.S. Impact of cystic fibrosis on bone health. Curr Opin Pulm Med 2010, 16:616-622.
191. Buntain H.M., Schluter P.J., Bell S.C., Greer R.M., Wong J.C., Batch J., et al. Controlled longitudinal study of bone mass accrual in children and adolescents with cystic fibrosis. Thorax 2006, 61:146-154.
192. Lucidi V., Bizzarri C., Alghisi F., Bella S., Russo B., Ubertini G., et al. Bone and body composition analyzed by Dual-energy X-ray Absorptiometry (DXA) in clinical and nutritional evaluation of young patients with Cystic Fibrosis: a cross-sectional study. BMC Pediatr 2009, 9:61.
193. Gronowitz E., Pitkanen S., Kjellmer I., Heikinheimo M., Strandvik B. Association between serum oncofetal antigens CA 19-9 and CA 125 and clinical status in patients with cystic fibrosis. Acta Paediatr 2003, 92:1267-1271.
194. Gronowitz E., Lorentzon M., Ohlsson C., Mellstrom D., Strandvik B. Docosahexaenoic acid is associated with endosteal circumference in long bones in young males with cystic fibrosis. Br J Nutr 2008, 99:160-167.
195. Gronowitz E., Mellstrom D., Strandvik B. Serum phospholipid fatty acid pattern is associated with bone mineral density in children, but not adults, with cystic fibrosis. Br J Nutr 2006, 95:1159-1165.
196. Conway S.P., Oldroyd B., Brownlee K.G., Wolfe S.P., Truscott J.G. A cross-sectional study of bone mineral density in children and adolescents attending a Cystic Fibrosis Centre. J Cyst Fibros 2008, 7:469-476.
197. Gronowitz E., Garemo M., Lindblad A., Mellstrom D., Strandvik B. Decreased bone mineral density in normal-growing patients with cystic fibrosis. Acta Paediatr 2003, 92:688-693.
198. Fewtrell M.S., Benden C., Williams J.E., Chomtho S., Ginty F., Nigdikar S.V., et al. Undercarboxylated osteocalcin and bone mass in 8-12 year old children with cystic fibrosis. J Cyst Fibros 2008, 7:307-312.
199. Moran A., Dunitz J., Nathan B., Saeed A., Holme B., Thomas W. Cystic fibrosis-related diabetes: current trends in prevalence, incidence, and mortality. Diabetes Care 2009, 32:1626-1631.
200. Moran A., Pillay K., Becker D.J., Acerini C.L. Management of cystic fibrosis-related diabetes in children and adolescents. Pediatr Diabetes 2014, 15(Suppl. 20):65-76.
201. Moran A., Brunzell C., Cohen R.C., Katz M., Marshall B.C., Onady G., et al. Clinical care guidelines for cystic fibrosis-related diabetes: a position statement of the American Diabetes Association and a clinical practice guideline of the Cystic Fibrosis Foundation, endorsed by the Pediatric Endocrine Society. Diabetes Care 2010, 33:2697-2708.
202. Colombo C., Battezzati P.M., Crosignani A., Morabito A., Costantini D., Padoan R., et al. Liver disease in cystic fibrosis: a prospective study on incidence, risk factors, and outcome. Hepatology 2002, 36:1374-1382.
203. Van Biervliet S., Van Biervliet J.P., Robberecht E., Christophe A. Fatty acid composition of serum phospholipids in cystic fibrosis (CF) patients with or without CF related liver disease. Clin Chem Lab Med 2010, 48:1751-1755.
204. Colombo C., Costantini D., Rocchi A., Romano G., Rossi G., Bianchi M.L., et al. Effects of liver transplantation on the nutritional status of patients with cystic fibrosis. Transpl Int 2005, 18:246-255.
205. Christophe A., Robberecht E. Current knowledge on fatty acids in cystic fibrosis. Prostagl Leukot Essent Fat Acids 1996, 55:129-138.
206. Freedman S.D., Blanco P.G., Zaman M.M., Shea J.C., Ollero M., Hopper I.K., et al. Association of cystic fibrosis with abnormalities in fatty acid metabolism. N Engl J Med 2004, 350:560-569.
207. Strandvik B., Gronowitz E., Enlund F., Martinsson T., Wahlstrom J. Essential fatty acid deficiency in relation to genotype in patients with cystic fibrosis. J Pediatr 2001, 139:650-655.
208. Carlstedt-Duke J., Bronnegard M., Strandvik B. Pathological regulation of arachidonic acid release in cystic fibrosis: the putative basic defect. Proc Natl Acad Sci U. S. A 1986, 83:9202-9206.
209. Kuo P.T., Huang N.N., Bassett D.R. The fatty acid composition of the serum chylomicrons and adipose tissue of children with cystic fibrosis of the pancreas. J Pediatr 1962, 60:394-403.
210. Coste T.C., Armand M., Lebacq J., Lebecque P., Wallemacq P., Leal T. An overview of monitoring and supplementation of omega 3 fatty acids in cystic fibrosis. Clin Biochem 2007, 40:511-520.
211. Jorgensen M.H., Ott P., Michaelsen K.F., Porsgaard T., Jensen F., Lanng S. Long-chain PUFA in granulocytes, mononuclear cells, and RBC in patients with cystic fibrosis: relation to liver disease. J Pediatr Gastroenterol Nutr 2012, 55:76-81.
212. Njoroge S.W., Seegmiller A.C., Katrangi W., Laposata M. Increased Delta5- and Delta6-desaturase, cyclooxygenase-2, and lipoxygenase-5 expression and activity are associated with fatty acid and eicosanoid changes in cystic fibrosis. Biochim Biophys Acta 2011, 1811:431-440.
213. Wouthuyzen-Bakker M., Bodewes F.A., Verkade H.J. Persistent fat malabsorption in cystic fibrosis; lessons from patients and mice. J Cyst Fibros 2011, 10:150-158.
214. Lloyd-Still J.D., Bibus D.M., Powers C.A., Johnson S.B., Holman R.T. Essential fatty acid deficiency and predisposition to lung disease in cystic fibrosis. Acta Paediatr 1996, 85:1426-1432.
215. Walkowiak J., Lisowska A., Blaszczynski M., Przyslawski J., Walczak M. Polyunsaturated fatty acids in cystic fibrosis are related to nutrition and clinical expression of the disease. J Pediatr Gastroenterol Nutr 2007, 45:488-489. author reply 489.
216. Panchaud A., Sauty A., Kernen Y., Decosterd L.A., Buclin T., Boulat O., et al. Biological effects of a dietary omega-3 polyunsaturated fatty acids supplementation in cystic fibrosis patients: a randomized, crossover placebo-controlled trial. Clin Nutr 2006, 25:418-427.
217. Strandvik B., Berg U., Kallner A., Kusoffsky E. Effect on renal function of essential fatty acid supplementation in cystic fibrosis. J Pediatr 1989, 115:242-250.
218. Alicandro G., Faelli N., Gagliardini R., Santini B., Magazzu G., Biffi A., et al. A randomized placebo-controlled study on high-dose oral algal docosahexaenoic acid supplementation in children with cystic fibrosis. Prostagl Leukot Essent Fat Acids 2013, 88:163-169.
219. De Vizia B., Raia V., Spano C., Pavlidis C., Coruzzo A., Alessio M. Effect of an 8-month treatment with omega-3 fatty acids (eicosapentaenoic and docosahexaenoic) in patients with cystic fibrosis. JPEN J Parenter Enteral Nutr 2003, 27:52-57.
220. Keen C., Olin A.C., Eriksson S., Ekman A., Lindblad A., Basu S., et al. Supplementation with fatty acids influences the airway nitric oxide and inflammatory markers in patients with cystic fibrosis. J Pediatr Gastroenterol Nutr 2010, 50:537-544.
221. Oliver C., Watson H. Omega-3 fatty acids for cystic fibrosis. Cochrane Database Syst Rev 2013, 11:CD002201.
222. Bianchi M.L., Colombo C., Assael B.M., Dubini A., Lombardo M., Quattrucci S., et al. Treatment of low bone density in young people with cystic fibrosis: a multicentre, prospective, open-label observational study of calcium and calcifediol followed by a randomised placebo-controlled trial of alendronate. Lancet Respir Med 2013, 1:377-385.
223. Stalvey M.S., Clines G.A. Cystic fibrosis-related bone disease: insights into a growing problem. Curr Opin Endocrinol Diabetes Obes 2013, 20:547-552.
224. Whyte M.P., McAlister W.H., Novack D.V., Clements K.L., Schoenecker P.L., Wenkert D. Bisphosphonate-induced osteopetrosis: novel bone modeling defects, metaphyseal osteopenia, and osteosclerosis fractures after drug exposure ceases. J Bone Min Res 2008, 23:1698-1707.
225. Hardin D.S. A review of the management of two common clinical problems found in patients with cystic fibrosis: cystic fibrosis-related diabetes and poor growth. Horm Res 2007, 68(Suppl. 5):113-116.
226. Bianchi M.L., Romano G., Saraifoger S., Costantini D., Limonta C., Colombo C. BMD and body composition in children and young patients affected by cystic fibrosis. J Bone Min Res 2006, 21:388-396.
227. Phung O.J., Coleman C.I., Baker E.L., Scholle J.M., Girotto J.E., Makanji S.S., et al. Recombinant human growth hormone in the treatment of patients with cystic fibrosis. Pediatrics 2010, 126:e1211-e1226.
228. Stalvey M.S., Anbar R.D., Konstan M.W., Jacobs J.R., Bakker B., Lippe B., et al. A multi-center controlled trial of growth hormone treatment in children with cystic fibrosis. Pediatr Pulmonol 2012, 47:252-263.
229. Thaker V., Haagensen A.L., Carter B., Fedorowicz Z., Houston B.W. Recombinant growth hormone therapy for cystic fibrosis in children and young adults. Cochrane Database Syst Rev 2013, 6:CD008901.
230. Marchand V., Baker S.S., Stark T.J., Baker R.D. Randomized, double-blind, placebo-controlled pilot trial of megestrol acetate in malnourished children with cystic fibrosis. J Pediatr Gastroenterol Nutr 2000, 31:264-269.
231. Eubanks V., Koppersmith N., Wooldridge N., Clancy J.P., Lyrene R., Arani R.B., et al. Effects of megestrol acetate on weight gain, body composition, and pulmonary function in patients with cystic fibrosis. J Pediatr 2002, 140:439-444.
232. Homnick D.N., Homnick B.D., Reeves A.J., Marks J.H., Pimentel R.S., Bonnema S.K. Cyproheptadine is an effective appetite stimulant in cystic fibrosis. Pediatr Pulmonol 2004, 38:129-134.
233. Epifanio M., Marostica P.C., Mattiello R., Feix L., Nejedlo R., Fischer G.B., et al. A randomized, double-blind, placebo-controlled trial of cyproheptadine for appetite stimulation in cystic fibrosis. J Pediatr (Rio J) 2012, 88:155-160.
234. Butel M.J. Probiotics, gut microbiota and health. Med Mal Infect 2014, 44:1-8.
235. Chiu Y.H., Lin S.L., Tsai J.J., Lin M.Y. Probiotic actions on diseases: implications for therapeutic treatments. Food Funct 2014, 5:625-634.
236. Bruzzese E., Raia V., Gaudiello G., Polito G., Buccigrossi V., Formicola V., et al. Intestinal inflammation is a frequent feature of cystic fibrosis and is reduced by probiotic administration. Aliment Pharmacol Ther 2004, 20:813-819.
237. Bruzzese E., Raia V., Spagnuolo M.I., Volpicelli M., De Marco G., Maiuri L., et al. Effect of Lactobacillus GG supplementation on pulmonary exacerbations in patients with cystic fibrosis: a pilot study. Clin Nutr 2007, 26:322-328.
238. Weiss B., Bujanover Y., Yahav Y., Vilozni D., Fireman E., Efrati O. Probiotic supplementation affects pulmonary exacerbations in patients with cystic fibrosis: a pilot study. Pediatr Pulmonol 2010, 45:536-540.
239. Kerem B., Rommens J.M., Buchanan J.A., Markiewicz D., Cox T.K., Chakravarti A., et al. Identification of the cystic fibrosis gene: genetic analysis. Science 1989, 245:1073-1080.
240. Riordan J.R., Rommens J.M., Kerem B., Alon N., Rozmahel R., Grzelczak Z., et al. Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA. Science 1989, 245:1066-1073.