TOPICS IN CLINICAL NUTRITION

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1 Convegno Nazionale della Rete delle Strutture di Dietetica e Nutrizione Clinica della Regione Piemonte TOPICS IN CLINICAL NUTRITION Stresa Grand Hotel Bristol Settembre/01 Ottobre 2016 LA NUTRIZIONE CLINICA NELLA FIBROSI CISTICA DELL ADULTO Esperienza della AOU San Luigi Gonzaga P a o l o Av a g n i n a M a r c o Ti n i ve l l a S S D D i e t e t i c a e N u t r i z i o n e C l i n i c a

2 Good nutrition is crucial for people with cystic fibrosis (CF). For all ages, there appears to be a connection between good lung function and a higher body weight. A balanced, high-calorie diet with salt, fat, and protein gives the body what it needs to grow normally and live well. Normal gains in weight and height help build strong lungs and preserve lung function. Good nutrition also helps to build and keep a strong immune system to fight infections. Less infections and stronger lungs mean a longer, healthier life Cystic Fibrosis Foundation

3 Getting Off To A Good Start Good nutrition starts with teaching infants and children good eating skills. Malabsorption And Pancreatic Enzyme Replacement Most people with CF have a pancreas that does not work as well as it should. This is called pancreatic insufficiency. Poor Growth Or Poor Weight Gain Normal height and weight and good nutrition are vital in the care of CF. Tube Feedings Some people still do not gain weight or grow normally even after adding extra calories. Cystic Fibrosis Related Diabetes (CFRD) Diabetes related to CF occurs in 10 20% of people with CF Osteoporosis People with CF are at risk for poor bone health. CF And Pregnancy Women with CF can have healthy pregnancies Cystic Fibrosis Foundation

4 STATO DI NUTRIZIONE FABBISOGNI NUTRIZIONALI FUNZIONI COMPOSIZIONE CORPOREEE CORPOREA SPESA ENERGETICA STATO DI SALUTE

5 Factors determining energy requirements in C.F. Expenditure CF Energy requirements Intake Losses W Haller et al.: J Gastroenterol Hepatol 29 (2014)

6 Background on undernutrition in people with CF HIGH ENERGY NEEDS High resting energy expenditure Work of breathing Growth, puberty, repair Female gender Persistent lung inflammation and associated infections CFTR mutations (CF Transmembrane conductance Regulator Gene) MALNUTRITION IN CYSTIC FIBROSIS ENERGY LOSSES Malabsorption, Maldigestion Esophagitis Intestinal Inflammation and SIBO Low bicarbonate output Impaired insulin Secretion Impaired liver function Expectoration INADEGUATE NUTRIENT INTAKE W Haller et al.: J Gastroenterol Hepatol 29 (2014) Inadequate diet and micronutrient deficiency Pain, salt Psychosocial issue and anorexia Decrease appetite (gastro-esophageal reflux, constipation, distal intestinal obstructive syndrome, bacterial overgrowth and the side-effects of medications)

7 Cystic fibrosis GI phenotype multisystem disease manifestations within the gastrointestinal tract W Haller et al.: J Gastroenterol Hepatol 29 (2014)

8 Grade of evidence High: Further research is unlikely to change our confidence in the estimate of effect. Low: Further research is very likely to have an important impact on our confidence in the estimate of effect and is likely to change the estimate. Clinical Nutrition 35 (2016)

9 Grade of evidence Nutrition education and dietary counseling HIGH We recommend nutrition education and behavioral counseling for patients with CF and their families to achieve and maintain healthy feeding/eating behaviors. Energy intake We suggest adjusting energy intake upward to achieve normal growth and nutritional status while avoiding obesity. We recommend energy intake targets by age We recommend consideration of macronutrient balance goals, especially protein and fat HIGH LOW for increased energy intake for macronutrient balance Clinical Nutrition 35 (2016)

10 Nutrition intervention Grade of evidence HIGH HIGH LOW We recommend nutrition intervention based on a full review of nutrition status, including a detailed review of pancreatic enzyme replacement therapy (PERT), and correction of any underlying medical conditions. We recommend using age-appropriate BMI-related thresholds for deciding when to advance nutrition intervention. We recommend a progressive approach to intensification of nutrition interventions as needs increase: preventive nutritional counseling, dietary modification and/or oral nutrition supplements, and enteral tube feeding. Clinical Nutrition 35 (2016)

11 Grade of evidence Oral nutrition supplements LOW We recommend clinicians consider the use of oral nutritional supplements for treating children and adults who fail to achieve optimal growth rates and nutritional status with oral dietary intake and pancreatic enzyme replacement therapy (PERT) alone. HIGH We recommend clinicians regularly review and re-evaluate patients who are taking oral nutritional supplements to determine whether the patient should continue taking them. Clinical Nutrition 35 (2016)

12 Enteral nutrition Grade of evidence HIGH We recommend that clinicians consider the use of polymeric enteral tube feeding when oral interventions have failed to achieve acceptable rates of growth and nutritional status. LOW We recommend basing route, formula, and timing for enteral feeding selection on individual needs and preferences. Parenteral nutrition LOW We recommend the use of parenteral nutrition be reserved for exceptional cases when enteral feeding is not possible. Clinical Nutrition 35 (2016)

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