Dominique Turck1, Christian P Braegger2, Carla Colombo3, Dimitri Declercq4, Alison Morton5, Ruzha Pancheva6, Eddy Robberecht7, Martin Stern8, Birgitta Strandvik9, Sue Wolfe10, Stephane M Schneider11, Michael Wilschanski12. 1. Univ. Lille, Inserm U995, LIRIC - Lille Inflammation Research International Center, Division of Gastroenterology, Hepatology and Nutrition, and Cystic Fibrosis Center, Department of Pediatrics, CHU Lille, F-59000 Lille, France. Electronic address: dominique.turck@chru-lille.fr. 2. Division of Gastroenterology and Nutrition and Children's Research Center, University Children's Hospital Zurich, Steinwiesstrasse 75, CH-8032, 10 Zurich, Switzerland. Electronic address: christian.braegger@kispi.uzh.ch. 3. Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico (IRCCS), Università degli Studi di Milano, Milan, Italy. Electronic address: carla.colombo@unimi.it. 4. Department of Pediatrics, CF Centre, Ghent University Hospital, De Pintelaan 185, 9000 Gent, Belgium. Electronic address: dimitri.declercq@uzgent.be. 5. Regional Adult CF Unit, St. James' University Hospital, Beckett Street, Leeds, LS9 7TF, UK. Electronic address: alisonmorton@nhs.net. 6. Department of Hygiene, Faculty of Public Health, Prof. Dr. Paraskev Stoyanov Medical University of Varna, 55 Marin Drinov Str., 9002 Varna, Bulgaria. Electronic address: rouzha_pancheva@yahoo.com. 7. Pediatric Gastroenterology, Hepatology & Nutrition, University Ghent, CF Centre Ghent, Princess Elisabeth Pediatric Hospital, B 9000 Ghent, Belgium. Electronic address: eddy.robberecht@uzgent.be. 8. University Children's Hospital, University of Tübingen, Hoppe-Seyler-Strasse 1, 72076, Tübingen, Germany. Electronic address: martin.stern@med.uni-tuebingen.de. 9. Department of Bioscience and Nutrition, Karolinska Institutet, Novum, Hälsovägen 7-9, 14183 Huddinge, Stockholm, Sweden. Electronic address: birgitta.strandvik@ki.se. 10. Regional Pediatric CF Unit, The Leeds Children's Hospital, Belmont Grove, Leeds, LS2 9NS, UK. Electronic address: sue.wolfe@nhs.net. 11. Gastroenterology and Clinical Nutrition, Archet University Hospital and University of Nice Sophia-Antipolis, Nice, France. Electronic address: stephane.schneider@unice.fr. 12. Pediatric Gastroenterology, Hadassah-Hebrew University Medical Center, PO Box 24035 Jerusalem, 91240, Israel. Electronic address: michaelwil@hadassah.org.il.
Abstract
BACKGROUND: Malnutrition is both a frequent feature and a comorbidity of cystic fibrosis (CF), with nutritional status strongly associated with pulmonary function and survival. Nutritional management is therefore standard of care in CF patients. ESPEN, ESPGHAN and ECFS recommended guidelines to cover nutritional management of patients with CF. METHODS: The guidelines were developed by an international multidisciplinary working group in accordance with officially accepted standards. The GRADE system was used for determining grades of evidence and strength of recommendation. Statements were discussed, submitted to Delphi rounds, reviewed by ESPGHAN and ECFS and accepted in an online survey among ESPEN members. RESULTS: The Working Group recommends that initiation of nutritional management should begin as early as possible after diagnosis, with subsequent regular follow up and patient/family education. Exclusive breast feeding is recommended but if not possible a regular formula is to be used. Energy intake should be adapted to achieve normal weight and height for age. When indicated, pancreatic enzyme and fat soluble vitamin treatment should be introduced early and monitored regularly. Pancreatic sufficient patients should have an annual assessment including fecal pancreatic elastase measurement. Sodium supplementation is recommended and a urinary sodium:creatinine ratio should be measured, corresponding to the fractional excretion of sodium. If iron deficiency is suspected, the underlying inflammation should be addressed. Glucose tolerance testing should be introduced at 10 years of age. Bone mineral density examination should be performed from age 8-10 years. Oral nutritional supplements followed by polymeric enteral tube feeding are recommended when growth or nutritional status is impaired. Zinc supplementation may be considered according to the clinical situation. Further studies are required before essential fatty acids, anti-osteoporotic agents, growth hormone, appetite stimulants and probiotics can be recommended. CONCLUSION: Nutritional care and support should be an integral part of management of CF. Obtaining a normal growth pattern in children and maintaining an adequate nutritional status in adults are major goals of multidisciplinary cystic fibrosis centers.
BACKGROUND: Malnutrition is both a frequent feature and a comorbidity of cystic fibrosis (CF), with nutritional status strongly associated with pulmonary function and survival. Nutritional management is therefore standard of care in CF patients. ESPEN, ESPGHAN and ECFS recommended guidelines to cover nutritional management of patients with CF. METHODS: The guidelines were developed by an international multidisciplinary working group in accordance with officially accepted standards. The GRADE system was used for determining grades of evidence and strength of recommendation. Statements were discussed, submitted to Delphi rounds, reviewed by ESPGHAN and ECFS and accepted in an online survey among ESPEN members. RESULTS: The Working Group recommends that initiation of nutritional management should begin as early as possible after diagnosis, with subsequent regular follow up and patient/family education. Exclusive breast feeding is recommended but if not possible a regular formula is to be used. Energy intake should be adapted to achieve normal weight and height for age. When indicated, pancreatic enzyme and fat soluble vitamin treatment should be introduced early and monitored regularly. Pancreatic sufficientpatients should have an annual assessment including fecal pancreatic elastase measurement. Sodium supplementation is recommended and a urinary sodium:creatinine ratio should be measured, corresponding to the fractional excretion of sodium. If iron deficiency is suspected, the underlying inflammation should be addressed. Glucose tolerance testing should be introduced at 10 years of age. Bone mineral density examination should be performed from age 8-10 years. Oral nutritional supplements followed by polymeric enteral tube feeding are recommended when growth or nutritional status is impaired. Zinc supplementation may be considered according to the clinical situation. Further studies are required before essential fatty acids, anti-osteoporotic agents, growth hormone, appetite stimulants and probiotics can be recommended. CONCLUSION: Nutritional care and support should be an integral part of management of CF. Obtaining a normal growth pattern in children and maintaining an adequate nutritional status in adults are major goals of multidisciplinary cystic fibrosis centers.
Authors: Stephanie S Filigno; Shannon M Robson; Rhonda D Szczesniak; Leigh A Chamberlin; Meredith A Baker; Stephanie M Sullivan; John Kroner; Scott W Powers Journal: J Cyst Fibros Date: 2017-02-06 Impact factor: 5.482
Authors: Marianne S Muhlebach; Joseph E Hatch; Gisli G Einarsson; Stef J McGrath; Deirdre F Gilipin; Gillian Lavelle; Bojana Mirkovic; Michelle A Murray; Paul McNally; Nathan Gotman; Sonia Davis Thomas; Matthew C Wolfgang; Peter H Gilligan; Noel G McElvaney; J Stuart Elborn; Richard C Boucher; Michael M Tunney Journal: Eur Respir J Date: 2018-07-11 Impact factor: 16.671
Authors: Moriah P Bellissimo; Ivana Zhang; Elizabeth A Ivie; Phong H Tran; Vin Tangpricha; William R Hunt; Arlene A Stecenko; Thomas R Ziegler; Jessica A Alvarez Journal: J Cyst Fibros Date: 2019-01-18 Impact factor: 5.482