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National Network for Pulmonary National Network for Pulmonary Hypertension at Childhood Hypertension at Childhood UMCG, Groningen National Referral Center for Children with Childhood PH Nutritional Implications in Nutritional Implications in


  1. National Network for Pulmonary National Network for Pulmonary Hypertension at Childhood Hypertension at Childhood UMCG, Groningen National Referral Center for Children with Childhood PH Nutritional Implications in Nutritional Implications in Collaboration with 9 Network Centers Pediatric Pulmonary Pediatric Pulmonary University Childrens Hospitals and/or Cardiology Childrens Hospitals Hypertension Hypertension Amsterdam, The Hague, Leiden, Maastricht, Nijmegen, Rotterdam, Utrecht, Veldhoven Standard Diagnostics and Treatment of Pulmonary Hypertension in Children, 2nd Edition, ‘Network for Pulmonary Hypertension Theresia Vissia-Kazemier, RN-MANP in Childhood‘ http://www.kinderph.nl/professionals/ Dutch National Network for Pediatric Pulmonary Hypertension Center for Congenital Heart Diseases Two yearly PVD Conference Department of Pediatric Cardiology Beatrix Children’s Hospital, UMCG Nurses working group Pulmonary Nurses working group Pulmonary Hypertension Hypertension Association for Nursing and Care Netherlands No conflict of interest Task group pulmonary vascular disorders https://longverpleegkundigen.venvn.nl/over-ons/taakgroepen/pulmonaal-vasculaire-aandoeningen Sharing knowledge and exchange experiences Developing brochures, guidelines and protocols Case presentations Collaboration: • Facility companies • Patient association (eg, patient information, patient days) • Pharmaceutical companies 1

  2. Growth is a process Growth is a process Growth is a process Repeated growth measurements are important The most powerful tool in growth assessment is the growth chart Causes of growth impairment:  Malnutrition  Hormonal dysfunction or imbalance  Genetic disorders  Psychosocial factors  Chronic diseases (eg, chronic renal failure, lung disease, chronic heart disease) Malnutrition: Malnutrition: Definition of malnutrition Definition of malnutrition imbalance between nutrient imbalance between nutrient requirement and nutrient intake requirement and nutrient intake Acute malnutrition (‘wasting’): SUPPLY PLY DEMAND • > -2 SD weight for age (WFA) in children > 28 days and < 1 year Growth • > -2 SD weight-for-height (WFH) in children > 1 year or Development • Deflecting growth curve of weight, height or WFH of > 1 SD in 3 months Outcomes: Chronic malnutrition (‘stunting’): Morbidity • Height for age (HFA) ≥ -2 SD Mortality NUTRITION • Deflection of the SD score with 0.5 - 1.0 SD or more in one year Risk factors (children < 4 years of age) • Deflection of 0.25 SD or more in one year (children > 4 years of age) INTAKE INTAK LOSSES LOSSES BODY STORES Overnutrition: • BMI of WFH > 2 SD 2

  3. Retrospective longitudinal study 53 centres in 19 countries What is known ? What is known ? Nutrition & Growth Nutrition & Growth 601 children Median follow up 2-9 years Pilotstudy Pilotstudy During childhood adequate intake is of the utmost importance Mean height for age Z score was significantly lower than the reference (p<0·0001), as was body-mass index for age Z score (p=0·047). Rapid growth A prospective cohort study 2009-2011 Height for age Z score was particularly decreased in young patients (aged ≤ 5 Development years) with idiopathic or hereditary PAH and in all patients with PAH associated with congenital heart disease Prevalent pediatric patients idiopathic PAH or PAH/CHD pts Recent data: low weight , height and body mass index Z-scores increases Multivariable a analysis mortality Associated with height for age Z-scores: • Age Research questions • Cause of PAH • Exprematurity 1. How is the nutritional status in children with PAH? • WHO functional class Associated with BMI Z-scores: Data about Dat about growth growth and nutr and nutrition in in childr children en with with PAH is PAH is lackin lacking 2. Does the severity of the disease and energy intake correlate with the nutritional status ? • Age • Ethnicity 3. Is an energy intake compared with required daily allowances enough to maintain growth or do • Trisomy 21 • Time since diagnosis children with PAH need increased energy intake ? Moledina S, et al. Heart, 2010;96:1401-1406 Associated with increases in height for Z-scores: Barst RJ, et al. Circulation. 2012;125:113-122 Ploegstra MJ, et al. The Lancet. 2016; 4: 241-336 • Favourable WHO FC 4. Does have a structured nutritional advice have a positive impact on the nutritional status Ploegstra et al. 2016 children with PAH ? Measurements an additional Measurements an additional information information Studypopulation Studypopulation • Weight • Height • Weight for height • BMI • Mid-upper arm circumference (MUAC) • Triceps skinfold thickness (TSF) • 3-days dietary assessment (weekdays / 1 weekend day) • Nutritional counseling by a dietician • Patient characteristics: age, gender, etiology of PAH, co-morbidity • Related factors: WHO FC, exercise capacity (6 MWT), NT-pro-BNP, uric acid, norepinephrine Frederiks AM et al. Pediatric Res 2000;47:316-23 Gerver WJM and Bruin R. Universitaires Pers Maastricht, 2001 3

  4. Calculated energy: 1750 kcal Calculation of Individual Intake Calculation of Individual Intake Childs’ intake: 1500 kcal Calculations and comparisons Calculations and comparisons 1500 x 100 = 85,7% requirements (EIRc) requirements (EIRc) 1750 Percentage Energy intake of Individual Requirement (%EIRc) Using WHO (weight) equation of (measu (measured energy energy inta intake/EIR IR x x 100) 100) resting metabolic rate (RMR) Additional factors Individual protein requirements (PIRc) • Physical activity level (PAL) (multiplying tiplying childs’ w ds’ weight w ht with th r recommended pr ended protein ein intake) take) (consulting patient activity level parents opinion /set on normal activity 1.5) Calculated Energy intake and intake of protein, carbohydrates and fat • Ilnessfactor (IF) from 3-days dietary assessment (yet no evidence for increased energy requirement /set on 1.0) • Growth factor (GF) Compared with recommended daily allowances (RDA) (range from 1.03-1.20 depending on age/growth spurt) and data of Dutch Food Survey (DFS) • Energy absorption coefficient (EAC) EIRc = RMR x (PAL+IF) x G Dutch Malnutrition Steering Group. Energy requirements in children. 2010 (age dependent range 0,85-0.98) Dutch Malnutrition Steering Group. Protein requirements in children. 2010 EAC Health Council of The Netherlands. Dietary Reference Intake; energy, proteins, fat and digestible carbohydrates. Health Council of The Netherlands: The Hague, 2001 National institute for Public Health and Environment. Dutch National Food Consumption Survey young Children 2006/2006; 350070001 National institute for Public Health and the Environment. Dutch National Food Consumption Survey 2007-2010; Diet of children and adults aged 7 to 69 years. 2011; 350050006 Dutch Malnutrition Steering Group. Energy requirements in children.2010 Results nutritional status Results nutritional status Normal nutritional status in 5 children Acute malnutrition ‘wasting’ (WFH or BMI <- 2 SD) n= 2 (1 syndrome group and 1 non-syndrome group) Chronic malnutrition height for age ‘stunting’ (HFA) of ≥ -2 SD: n=4 (3 syndrome/1 non-syndrome) Two patients had both acute and chronic malnutrition (n=1 syndrome, n=1 non-syndrome patient) 4

  5. Energy intake: kcal and % Energy intake: kcal and % Nutritional Advice Nutritional Advice calculated energy intake (%Eic) calculated energy intake (%Eic) After dietary assessment and this first measure point the dietician offered individual advice to promote growth n= 7 advice intake according recommended daily allowances (RDA) n=1 more calories to anticipate on growth spurt (adaptation of WHO equation) n= 1 extra calories because of individual severely low SD scores (adaptation of WHO equation to achieve catch up growth) Energy intake (kcal) compared with Energy intake (kcal) compared with Energy intake (%EIc) Energy intake (%EIc) recommended daily allowances recommended daily allowances (RDA) (RDA) %EIc Baseline Follow up Mean 102% (range 67 ‐ 129% Mean 105% ( range 70 ‐ 139%) n= 3 > 110% n=4 > 110% n= 5 > 90 ‐ 110% n=3 > 90 ‐ 110% n=1 < 90% n=2 < 90% Patients with normal nutritional status Both in normal and inpaired group 5

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