ESPEN-ESPGHAN-ECFS指南：婴儿、儿童和成人囊性纤维化营养诊疗

　　囊性纤维化是一种遗传性外分泌腺疾病，主要影响胃肠道和呼吸系统，通常具有慢性梗阻性肺部病变、胰腺外分泌功能不良和汗液电解质异常升高的特征。

　　欧洲肠外肠内营养学会（ESPEN）官方期刊《临床营养》2016年6月第3期正式发表ESPEN、欧洲儿科胃肠肝病营养学会（ESPGHAN）、欧洲囊性纤维化学会（ECFS）指南：婴儿、儿童和成人囊性纤维化营养诊疗。营养诊疗和支持是囊性纤维化管理不可分割的部分，指南工作组建议营养管理应在疾病诊断之后尽早开始，并且需要规律随访以及进行患者或者家庭教育。

Clin Nutr. 2016 Jun;35(3):557-77.

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

Turck D, Braegger CP, Colombo C, Declercq D, Morton A, Pancheva R, Robberecht E, Stern M, Strandvik B, Wolfe S, Schneider SM, Wilschanski M.

  • 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.

  • Division of Gastroenterology and Nutrition and Children's Research Center, University Children's Hospital Zurich, Steinwiesstrasse 75, CH-8032, 10 Zurich, Switzerland.

  • Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico (IRCCS), Università degli Studi di Milano, Milan, Italy.

  • Department of Pediatrics, CF Centre, Ghent University Hospital, De Pintelaan 185, 9000 Gent, Belgium.

  • Regional Adult CF Unit, St. James' University Hospital, Beckett Street, Leeds, LS9 7TF, UK.

  • Department of Hygiene, Faculty of Public Health, Prof. Dr. Paraskev Stoyanov Medical University of Varna, 55 Marin Drinov Str., 9002 Varna, Bulgaria.

  • Pediatric Gastroenterology, Hepatology & Nutrition, University Ghent, CF Centre Ghent, Princess Elisabeth Pediatric Hospital, B 9000 Ghent, Belgium.

  • University Children's Hospital, University of Tübingen, Hoppe-Seyler-Strasse 1, 72076, Tübingen, Germany.

  • Department of Bioscience and Nutrition, Karolinska Institutet, Novum, Halsovagen 7-9, 14183 Huddinge, Stockholm, Sweden.

  • Regional Pediatric CF Unit, The Leeds Children's Hospital, Belmont Grove, Leeds, LS2 9NS, UK.

  • Gastroenterology and Clinical Nutrition, Archet University Hospital and University of Nice Sophia-Antipolis, Nice, France.

  • Pediatric Gastroenterology, Hadassah-Hebrew University Medical Center, PO Box 24035 Jerusalem, 91240, Israel.

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.

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

PMID: 27068495

DOI: 10.1016/j.clnu.2016.03.004

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