Dimitri Declercq

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

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.

Relation between Fatty Acid Composition and Clinical Status or Genotype in Cystic Fibrosis Patients

Objective: To evaluate the relation of clinical parameters and genotype with the serum phospholipid fatty acid (FA) composition in cystic fibrosis (CF) patients. Methods: A blood sample was taken from CF patients with stable pulmonary disease for the determination of phospholipid FA composition and vitamin E concentration who had been followed for at least 6 months at our Cystic Fibrosis Centre. Genotype, age, pancreatic function, nutritional status, caloric intake, pulmonary function and presence of Pseudomonas colonization, liver disease or diabetes mellitus were recorded. Patients were divided into two groups according to their genotype (group A: mutation class I, II, or III, group B: mutation class IV, V). Results: CF patients (group A and B together) have significantly lower docosahexaenoic acid (DHA) (p < 0.007) and linoleic acid (LA) (p < 0.0001) and higher dihomogammalinolenic acid (DHGLA) (p < 0.0001), oleic acid (OA) (p < 0.0001) and Mead acid (MA) (p < 0.0001), resulting in an increased ratio of arachidonic acid (AA)/DHA (p < 0.004), MA/AA (p < 0.0001) and OA/LA (p < 0.0001). Compared to group B, group A had a lower LA (p < 0.002) and a higher DHGLA (p < 0.002), 22:4ω–6 (p < 0.03), 22:5ω–6 (p < 0.03) and 20:3ω–9 (p < 0.04). There was however no significant difference between the groups for age, pulmonary function, nutritional status and vitamin E concentration. There was no relation of serum FA composition with nutritional status, caloric intake, pancreatic function, gender, pulmonary function, Pseudomonas colonization or diabetes mellitus. In CF with liver disease the DHA was lower than in the patients of the same genotype. Conclusion: FA disturbances are more pronounced in the severe CF genotypes and the presence of CF-related liver disease. Future studies on supplementation should take these parameters into account.

Nutrition and pancreatic enzyme intake in patients with cystic fibrosis with distal intestinal obstruction syndrome

BACKGROUND The etiology of distal intestinal obstruction syndrome (DIOS) remains unclear. Food intake and pancreatic enzyme replacement therapy (PERT) are often blamed for its occurrence. This study evaluates the nutrition intake and PERT of patients with cystic fibrosis (CF) at a first episode of DIOS. METHODS All patients with CF perform annually a 3-day intake diary to evaluate their caloric, protein, fat, dietary fiber, liquid, and PERT intake. Patients diagnosed with a first episode of DIOS (n = 12) retrospectively completed an intake diary of the 3 days preceding the DIOS episode supervised by an expert dietitian. RESULTS were compared with those of 1 year before and also with 36 CF controls matched for age, sex, genotype, and disease severity. All were pancreatic insufficient. RESULTS A first DIOS episode was diagnosed in 12 patients with CF. Only the absolute median fat intake (P = .015) and pancreatic enzyme intake (P = .035) were higher at the time of the DIOS attack in comparison to the preceding year. This could result from the difference in data collection or from the recommendations to increase fat intake and concomitant enzyme intake, since this trend was also found in the control group. The significant difference disappears when enzyme intake is expressed as units of lipase/g of fat. No other significant dietary differences were found. CONCLUSIONS This study provides no indications for a potential role of nutrition factors or pancreatic enzymes in the first occurrence of DIOS.

239 Intake of pancreatic enzymes: Consistent with the guidelines?

Objectives Pancreatic enzyme (PE) replacement therapy is required in pancreatic insufficient CF patients to prevent malnutrition, nutrient deficiency and control abdominal maldigestion symptoms. This study evaluates and compares the actual PE intake with current expert recommendations (ER). Methods The dietary intake, measured by a 3-day dietary diary with PE intake (Creon® 10000, 25000 and 40000) for each meal was reviewed during a dietary consult. Intake is compared to the ER expressed as lipase units (IU)/g fat, /kg body-weight (BW)/day and /kgBW/meal. Results are presented as median (range). Results 54 patients [age 12.8 y (1, 50); 27 males] with BMI z-score –0.3 (–1.9, 1.5) and FEV1% 85.7 (31.8,114.1) (n = 43) were included. Results are presented in the table. TableComparison of PE intake with expert recommendationsGuidelineNumber of patientsTotalIntake greater than advised intakeIn accordance with advised intaken (%)Intake, median (range)n (%)Intake, median (range)Child: 400–800 IU/g fat2424 (100%)5,885 (1,106–13,875)–Adolescent-adult: 4,000 IU/g fat3026 (87%)7,316 (3,220–16,888)4 (13%)2,983 (2,857–3,135)10,000 IU/kgBW/d5440 (74%)18,753 (10,110–36,194)14 (26%)6,996 (2,455–9,711)2,000 IU/kgBW/meal5442 (78%)4,445 (2,210–8,691)12 (22%)1,398 (1,052–1,973) Conclusion The majority of our patients has a pancreatic enzyme intake above the current recommendations without known side effects. Research is needed to formulate recommendations based on achieving optimal digestion rather than using a protective dosing strategy.

The effect of enteral tube feeding in cystic fibrosis: A registry based study

BACKGROUND Long-term effect of enteral tube feeding (ETF) in cystic fibrosis (CF) remains equivocal. METHODS A Belgian CF registry based, retrospective, longitudinal study, evaluated the pre- and post- ETF (n = 113) clinical evolution and compared each patient with 2 age, gender, pancreatic status and genotype class-matched controls. RESULTS At baseline ETF had a worse BMI z-score (p < 0.0001) and FEV1% (p < 0.0001) compared to controls. Patients eventually receiving ETF, had already a significant worse nutritional status and pulmonary function at first entry in the registry. Both parameters displayed a significant decline before ETF-introduction. ETF had more hospitalization and intravenous antibiotic (IVAB) treatment days (p < 0.0001). After ETF introduction hospitalizations and IVAB decreased significantly. After ETF-introduction BMI z-score recuperated towards the original curve before the decline, but remained below the controls. Starting ETF had no effect on rate of height gain in children. The pre-index FEV1 decline (-1.52%/year (p = 0.002)) stabilized to +0.39%/year afterwards. Controls displayed decline of -0.48%/year (p < 0.0001). CONCLUSION ETF introduction improved BMI z-score and stabilized FEV1, associated with less hospitalizations and IVAB treatments. Higher mortality and transplantation in the ETF cases, leading to drop-outs, made determination of the effect size difficult.

238 How are pancreatic enzymes distributed over mealtimes and meal types

Objectives According to the current CF guidelines, body weight (BW) and fat content are used to express the PE intake and define the PE dose. In clinical practice it is not clear to what extent patients adjust their PE dose. This study explores the PE intake in relation to the fat content of different meal times (MT) and meal types (MTy). Methods The dietary intake, measured by a 3-day dietary diary with PE intake (Creon® 10000, 25000 and 40000) for each meal (M) was reviewed during a dietary consult. PE intake was compared between different MT [breakfast (B), lunch (L), dinner (D) and snacks (S)] and MTy [B, cold meal (CM) and hot meal (HM)]. Results 54 patients [aged 12.3 y (1; 50 y), 27 males] with BMI z-score –0.3 (–1.9; 1.5) and FEV1% 85 (31.8; 114.1) (n = 43) were included. No significant difference was found for PE intake (IU/g fat) between the different MT. Corrected for BW (IU/kgBW/M) a significant difference was found between main Ms and S (p = 0.001). The median (range) PE intake was for B: 3,686 IU/kgBW/M (921; 10,262), L: 4,420 IU/kgBW/M (921; 11,979), D: 3,971 IU/kgBW/M (1,052; 11,979) and S: 2,612 IU/kgBW/M (480; 9,882). No significant difference was found for PE intake (IU/g fat) between the different MTys. Expressed as IU/kgBW/M a significant difference (p = 0.001) was found between CM (n = 140) 4,036 IU/kgBW/M (921; 11,367), HM (n = 180) 4,328 IU/kgBW/M (921; 13,187) and B (n = 157) 3,538 IU/kgBW/M (661; 12,542). Conclusion Patients seem to adjust their PE dose to the fat content of a meal. When corrected for BW the PE intake is lower with snacks and higher at breakfast. However the importance of this remains unclear. To evaluate the PE intake a reconsideration of the denominator may be needed.

152 Influencing factors of skeletal muscle weakness in adults with cystic fibrosis

Background Muscle weakness in cystic fibrosis (CF) is reported to be reduced. Influencing factors are still not well defined. Aim This study correlates muscle strength measurements: handgrip force (HGF), quadriceps force (QF) and 6 minute walking distance (6MWD) in adult CF patients with physical activity (PA), pulmonary function and decline, colonization status, and number of hospitalization over the past year. Methods A handheld dynamometer (HHD) was used for HGF, a knee shuttle with HHD for the isometric voluntary QF and a standard 6MWD was performed. Results are expressed as % of normal. PA is measured using Sensewear Pro® armband [Moderate PA (mPA) = 4.8–7.2 metabolic equivalents (MET), vigorous PA (vPA) MET >7.2). Pulmonary function (FVC%, FEV1%) and number of hospitalization were retrieved from the medical file. Results are given as median (interquartile range). Results 31 patients (aged 25 (22–33)years) (12 female(38.7%))(12 chronically colonized(38.7%)) with a FEV1% of 75.4(57–84.7)% participated. Muscle strength was not related to pulmonary function or pulmonary function decline or pseudomonas colonization. 21 patients measured PA. They had an average MET of 1.7 (1.55–2.1), a mPA of 26′/day (19–68.5) and a m-vPA of 40′/day (22–98). The 6MWD was 72.7% (66.9–77.8), the QF% was 46% (40.2–57.4) and the HGF 97.4% (79.8–106.5). Pseudomonas negative patients were more PA (p = 0.034). None of the muscle strength measurements correlated with the PA. The number of hospitalizations correlated with the QF (p = 0.001), the HGF (p = 0.0001) and 6MWD (p = 0.007). Conclusion Muscle weakness in CF was only influenced by the number of hospitalizations over the past year.

WS21.2 Skeletal muscle strength measurements in adult CF patients compared to controls

Background In chronic illnesses, such as cystic fibrosis (CF), important muscle weakness has been reported. Aim To study the interaction between handgrip force (HGF), quadriceps force (QF) and six minute walking distance (6MWD) measurements and body composition factors influencing these muscle strengths. Methods A handheld dynamometer was used for HGF, a knee shuttle bank for the isometric QF and a standard 6MWD was performed in CF and controls. Age and body mass index (BMI) were noted for all. Lean body mass %, body fat mass index and fat free mass index (FFMI) of CF patients was assessed by a BIA. Results are median (interquartile range [IQR]). Results 10 age matched controls and 31 CF patients measured muscle strength (Table 1). CF patients display an asymmetric muscle weakness with a relative preservation of the HGF. There was a mutually significant relation of the different strength measurements. All strength measurements were significantly correlated with BMI. Finally, HGF correlated with FFMI (p = 0.011). Table 1ParameterControlsP-valueCF patientsMalesFemalesMalesFemalesAge (yrs)23 (22.3, 25.3)29.5 (22.5, 38.5)n.s.25 (22–31)26 (22.5–33)BMI (kg/m 2 )23.5 (21.3, 24.8)22.5 (21.9, 27.5)0.03120.5 (18.4, 23.3)20.9 (20.1, 21.8)QF (%)103.7 (95.3, 152.2)124.6 (94.8, 156.2)0.000163.5 (52.2, 72.9)55.6 (42.4, 75.8)HGF R (%)164.2 (115.9, 176.6)95.6 (91.4, 108.6)0.000190.9 (72.6, 97.7)87.5 (60.2, 98.5)HGF L (%)183.1 (123.2, 191.8)106.0 (94.7, 119.6)0.000188.1 (73.3, 93.4)83.6 (73.1, 96.8)6MWD (%)84.8 (78.1, 100.1)83.0 (79.0, 88.4)0.000173.2 (63.2, 77.8)72.7 (70.7, 83.4)FFMI17.9 (15.4, 19.1)15.8 (14.4, 16.6)Values are median (IQR). Conclusion Strength parameters are influenced by body composition. Maintaining a healthy BMI is essential in maintaining muscle reserves and as a consequence muscle strength.

Acknowledgement to the 2007 Reviewers

T. Decsi, Pécs, Hungary K.A. Dewaal, Zwolle, The Netherlands I. Dostalova, Prague, Czeck Republic C.P. Earthman, St. Paul, USA P. Eckl, Salzburg, Austria K. Eder, Halle/Saale, Germany I. Elmadfa, Vienna, Austria G. Emil, Bratislava, Republic of Slovakia M. Faber, Tygerberg, South Africa E. Fabian, Vienna, Austria L. Ferguson, Auckland, New Zealand A. Flynn, Cork, Ireland S.D. Freedman, Boston, USA J. Freeland-Graves, Austin, USA H. Freisling, Vienna, Austria J. Frohlich, Vancouver, Canada D. Fuchs, Innsbruck, Austria N. Fuller, London, UK P. Galassetti, Calif., USA K. Gedrich, Freising-Weihenstephan, Germany D. Genser, Vienna, Austria H. Goldenberg, Vienna, Austria R. Grossklaus, Berlin, Germany F. Guillon, Nantes, France P. Haber, Vienna, Austria J.E. Haddow, Providence, USA M.H. Hamdaoui, Tunis, Tunisia J.C. Hansen, Aarhus, Denmark H. Hauner, Munich, Germany S. Hercberg, Bobigny, France M. Hershfinkel, Be’er-Sheva, Israel H. Heseker, Paderborn, Germany M. Hiesmayr, Vienna, Austria E.C. Hinton, Cambridge, UK M. Hohenegger, Vienna, Austria B.J. Holub, Guelph, Canada N. Houalla, Beirut, Lebanon G. Hu, Helsinki, Finland M. Huettinger, Vienna, Austria T. Inagawa, Izumo, Japan C. Invitti, Milan, Italy S. Jackson, London, UK G. Jahreis, Jena, Germany A. Kafatos, Heraklion, Greece H. Karlic, Vienna, Austria C.L. Keen, Davis, USA M.J. Keenan, Baton Rouge, USA R. Kelishadi, Isfahan, Iran D. Kelley, California, USA E.T. Kennedy, Boston, USA V.G. Athyros, Thessaloniki, Greece S. Lee, Seoul, Korea W. Aberer, Graz, Austria F.B. Aksungar, Istanbul, Turkey L. Allen, Davis, USA S.B. Astley, Colney, UK P. Athias, Dijon, France K.T. Augusti, Thalassery, India F. Azizi, Tehran, Iran P. Balagopal, Jacksonville, USA D.E. Barre, Sydney, Canada R.L. Batterham, London, UK I. Bautista, Las Palmas de Gran Canaria, Spain H. Bays, Louisville, USA R. Benamouzig, Bobigny, France A. Berg, Freiburg, Germany J. Berger, Vienna, Austria E. Oerich Berghofer, Vienna, Austria R. Bergmann, Berlin, Germany L.M. Bermejo López, Madrid, Spain V. Boehm, Jena, Germany H. Boeing, Nuthetal, Germany A. Bordoni, Bologna, Italy C.L. Bowlus, Sacramento, USA J. Bradbury, London, UK M.A. Brehm, Amsterdam, The Netherlands J. Briedé, Maastricht, The Netherlands T.L. Broderick, Glendale, USA C. Brombach, Wädenswil, Switzerland D.L. Brown, Ithaca, USA R.S. Bruno, Storrs, USA W. Bursch, Vienna, Austria P.C. Calder, Southampton, UK J. Campion, Pamplona, Spain M. Caroli, Brindisi, Italy R. Cermak, Leipzig, Germany N. Chang, Seoul, Korea J.-M. Chardigny, Clermont-Ferrand, France M.-F. Chen, Taipei, Taiwan S. Claeyssens, Rouen, France F. Correia, Porto, Portugal T. Crowe, Melbourne, Australia E. Curis, Paris, France S. Czernichow, Bobigny, France H. Dabadie, Pessac, France S. Dalton, New York, USA U. Das, Willemstad, Curacao, Neerlandaises Antilles M. Daniel Vaz de Almeida, Porto, Portugal J. de la Osada, Zaragoza, Spain D. de Luis, Simancas, Spain