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Paeds Topicsgastroenterology-hepatology-and-nutrition

Paeds · gastroenterology-hepatology-and-nutrition

Nutritional management of chronic disease

Also known as Nutritional management of chronic disease · Nutrition in chronic childhood illness · Chronic disease malnutrition · Disease-related malnutrition in children · Cystic fibrosis nutrition · Chronic kidney disease nutrition · Inflammatory bowel disease nutrition · Congenital heart disease nutrition · Paediatric oncology nutrition · Nutrition support in chronic disease

Fellowship guide to the nutritional management of chronic disease in children: the cystic fibrosis child who needs high calorie, high fat intake with pancreatic enzymes and fat-soluble vitamins, the chronic kidney disease child on a controlled phosphate and potassium diet with careful protein and growth hormone support, the Crohn disease child induced into remission with exclusive enteral nutrition, the cardiac infant with a large shunt who needs one hundred and fifty kilocalories per kilogram to grow, and the oncology child whose chemotherapy strips appetite and muscle, all unified by the four mechanisms of increased demand, decreased intake, nutrient loss and dietary restriction and the assess, screen, support and monitor pathway.

high12 referencesUpdated 15 July 202625 min readVerification in progress

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Red flags

  • A child with cystic fibrosis, inflammatory bowel disease, congenital heart disease, chronic kidney disease or cancer whose weight or body mass index centile falls, whose growth crosses two centile lines downward, or whose mid-upper arm circumference thins, has disease-related malnutrition until proven otherwise and needs formal anthropometry, a dietetic assessment and a nutrition support plan, because undernutrition erodes growth, immunity, treatment tolerance and survival
  • An infant with a large left-to-right cardiac shunt who is tachypnoeic, sweaty on feeds and gaining weight poorly is failing to thrive on the high metabolic demand of the lesion and needs fortified feeds and often a nasogastric tube to reach one hundred and thirty to one hundred and fifty kilocalories per kilogram per day; waiting for spontaneous catch-up while the heart works harder is the wrong strategy
  • A child with newly diagnosed Crohn disease and growth failure may achieve disease remission and nutritional repletion with exclusive enteral nutrition, six to eight weeks of liquid formula as the sole source of nutrition, which induces remission in around four in five children and avoids steroids that stunt growth
  • A child with chronic kidney disease who is restrictively over-dieted for phosphate, potassium and fluid can become protein and energy depleted and stop growing; the kidney diet must protect growth first, with protein at around the recommended dietary allowance, controlled rather than eliminated phosphate, and recombinant growth hormone when nutrition is optimised but stature remains poor

Life stages

infanttoddlerpreschoolschool-ageadolescentyoung-adult-transition

Care settings

preventive-medical-homecommunity-schooloutpatientwarded-acuterural-remotetelehealth

Clinical exam formats

written-only

Board mappings

  • Nutrition in chronic childhood disease and faltering growth
  • Nutritional management of chronic disease: cystic fibrosis, chronic kidney disease, inflammatory bowel disease, congenital heart disease and oncology, with disease-related malnutrition and nutrition support
  • Nutritional management of chronic disease: the cystic fibrosis child on enzymes and fat-soluble vitamins, the cardiac infant needing one hundred and fifty kilocalories per kilogram, and the Crohn disease child on exclusive enteral nutrition
  • Short case: the technology-dependent child with a gastrostomy and faltering growth
  • Long case: the multisystem chronic disease child with disease-related malnutrition and a nutrition support plan
  • Nutrition: nutritional management of chronic disease
  • Nutrition and growth in chronic childhood illness
  • Nutrition and growth: the child with cystic fibrosis or congenital heart disease and faltering growth
  • Nutrition: nutritional management of chronic disease in children
  • Patient Care: nutrition in children with chronic disease
  • Medical Expert: nutritional management of chronic disease in children
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Practise this topic

  • Short-answer question1
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Target exams

RACP DWERACP DCEMRCPCH TheoryMRCPCH Clinical

Red flags

  • A child with cystic fibrosis, inflammatory bowel disease, congenital heart disease, chronic kidney disease or cancer whose weight or body mass index centile falls, whose growth crosses two centile lines downward, or whose mid-upper arm circumference thins, has disease-related malnutrition until proven otherwise and needs formal anthropometry, a dietetic assessment and a nutrition support plan, because undernutrition erodes growth, immunity, treatment tolerance and survival
  • An infant with a large left-to-right cardiac shunt who is tachypnoeic, sweaty on feeds and gaining weight poorly is failing to thrive on the high metabolic demand of the lesion and needs fortified feeds and often a nasogastric tube to reach one hundred and thirty to one hundred and fifty kilocalories per kilogram per day; waiting for spontaneous catch-up while the heart works harder is the wrong strategy
  • A child with newly diagnosed Crohn disease and growth failure may achieve disease remission and nutritional repletion with exclusive enteral nutrition, six to eight weeks of liquid formula as the sole source of nutrition, which induces remission in around four in five children and avoids steroids that stunt growth
  • A child with chronic kidney disease who is restrictively over-dieted for phosphate, potassium and fluid can become protein and energy depleted and stop growing; the kidney diet must protect growth first, with protein at around the recommended dietary allowance, controlled rather than eliminated phosphate, and recombinant growth hormone when nutrition is optimised but stature remains poor

Life stages

infanttoddlerpreschoolschool-ageadolescentyoung-adult-transition

Care settings

preventive-medical-homecommunity-schooloutpatientwarded-acuterural-remotetelehealth

Clinical exam formats

written-only

Board mappings

  • Nutrition in chronic childhood disease and faltering growth
  • Nutritional management of chronic disease: cystic fibrosis, chronic kidney disease, inflammatory bowel disease, congenital heart disease and oncology, with disease-related malnutrition and nutrition support
  • Nutritional management of chronic disease: the cystic fibrosis child on enzymes and fat-soluble vitamins, the cardiac infant needing one hundred and fifty kilocalories per kilogram, and the Crohn disease child on exclusive enteral nutrition
  • Short case: the technology-dependent child with a gastrostomy and faltering growth
  • Long case: the multisystem chronic disease child with disease-related malnutrition and a nutrition support plan
  • Nutrition: nutritional management of chronic disease
  • Nutrition and growth in chronic childhood illness
  • Nutrition and growth: the child with cystic fibrosis or congenital heart disease and faltering growth
  • Nutrition: nutritional management of chronic disease in children
  • Patient Care: nutrition in children with chronic disease
  • Medical Expert: nutritional management of chronic disease in children

Overview & Definition

Disease-related malnutrition is the wasting and faltering growth that follows when a chronic illness raises what the body needs, lowers what the child can take in, steals nutrients through loss, or forces a diet so restricted that growth itself is curtailed. The cystic fibrosis child burning energy on the work of breathing and malabsorbing fat, the cardiac infant with a large shunt whose feeding calories barely cover the heart's oxygen bill, the Crohn disease teenager with abdominal pain and anorexia, the dialysis child leaking amino acids and held to a phosphate limit, and the oncology child nauseated and mucositic after chemotherapy are the same child seen through different lenses. The clinician's task is to recognise the faltering centiles and the thinning arm, confirm the degree of malnutrition with formal anthropometry, and build a nutrition support plan that protects growth as an outcome equal to disease control. [1]

The unifying idea is that chronic disease and nutrition move together, and that letting the child dwindle while the disease is treated is a failure of care. Growth is a vital sign in paediatrics, and a falling centile in any chronic illness is a signal that the balance of demand, intake, loss and restriction has tipped against the child. The framework the clinician holds in mind is the four mechanisms by which chronic disease drives malnutrition, the standardised anthropometry that quantifies it, the stepped nutrition support ladder that treats it, and the disease-specific requirements that fine-tune it, all delivered by a multidisciplinary team with the dietitian at its centre. [1] [12]

What is disease-related malnutrition in chronic childhood disease, and why does it matter?

Disease-related malnutrition is the undernutrition caused by a chronic illness rather than by primary food insecurity, operating through four mechanisms that may overlap in one child. Increased metabolic demand comes from the work of breathing in cystic fibrosis and large cardiac shunts, the catabolism and inflammation of active Crohn disease and cancer, and the dialysis process itself. Decreased intake comes from anorexia, nausea, early satiety, pain, taste change and fatigue. Nutrient loss comes from malabsorption in cystic fibrosis and inflammatory bowel disease, proteinuria and dialysis in chronic kidney disease, and gut losses in short bowel syndrome. Dietary restriction comes from the phosphate, potassium, fluid and sodium limits that kidney, cardiac and metabolic disease impose. It matters because undernutrition erodes linear growth, immune competence, wound healing, treatment tolerance and survival, so protecting nutrition is part of treating the disease, not an afterthought to it.

[1][12]
The features that put disease-related malnutrition at the top of the differential

A weight or body mass index centile falling in any chronic illness, a growth curve crossing two centile lines downward, a thinning mid-upper arm circumference, a child who has stopped gaining height, or one who is losing muscle while accumulating oedema, all signal disease-related malnutrition. The cardiac infant who is tachypnoeic and sweaty on feeds, the cystic fibrosis child with steatorrhoea and a fallen body mass index, the Crohn disease child with growth failure preceding the bowel symptoms, the dialysis child on an ever-tighter phosphate restriction, and the oncology child losing weight through a transplant cycle are the high-risk patterns. Formal anthropometry, a dietetic assessment and a stepped nutrition plan are needed, not reassurance and watchful waiting.

[1][9]
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References12ShowHide
  1. [1]Becker P; Carney LN; Corkins MR; Monczka J; Smith E; Smith SE; Spear BA; White JV Consensus statement of the Academy of Nutrition and Dietetics/American Society for Parenteral and Enteral Nutrition: indicators recommended for the identification and documentation of pediatric malnutrition (undernutrition). Nutr Clin Pract, 2015.PMID 25422273
  2. [2]Stallings VA; Stark LJ; Robinson KA; Feranchak AP; Quinton H Evidence-based practice recommendations for nutrition-related management of children and adults with cystic fibrosis and pancreatic insufficiency. J Am Diet Assoc, 2008.PMID 18442507
  3. [3]Turck D; Braegger CP; Colombo C; Declercq D; Morton A; Pancheva R; Robberecht E; Stern M; Toddenroth D; Van Dyck P ESPEN-ESPGHAN-ECFS guidelines on nutrition care for infants, children, and adults with cystic fibrosis. Clin Nutr, 2016.PMID 27068495
  4. [4]KDOQI Work Group KDOQI Clinical Practice Guideline for Nutrition in Children with CKD: 2008 update. Executive summary. Am J Kidney Dis, 2009.PMID 19231749
  5. [5]Mak RH; Iyengar A; Lai WM Nutrition in Children With Chronic Kidney Disease: How to Thrive? J Ren Nutr, 2023.PMID 37558172
  6. [6]Ruemmele FM; Veres G; Kolho KL; Griffiths A; Levine A; Escher JC; Amil Dias J; Barabino A; Braegger CP; Bronsky J; et al Consensus guidelines of ECCO/ESPGHAN on the medical management of pediatric Crohn's disease. J Crohns Colitis, 2014.PMID 24909831
  7. [7]Narula N; Dhillon A; Zhang D; Sherlock ME; Tondeur M; Zachos M Enteral nutritional therapy for induction of remission in Crohn's disease. Cochrane Database Syst Rev, 2018.PMID 29607496
  8. [8]Levine A; Wine E; Assa A; Sigall Boneh R; Shaoul R; Kori M; Cohen S; Peleg S; Shamaly H; On A; et al Crohn's Disease Exclusion Diet Plus Partial Enteral Nutrition Induces Sustained Remission in a Randomized Controlled Trial. Gastroenterology, 2019.PMID 31170412
  9. [9]Mills KI; Kim JH; Fogg K; Vricella L; McFadden D; Tobias JD Nutritional Considerations for the Neonate With Congenital Heart Disease. Pediatrics, 2022.PMID 36317972
  10. [10]Tsintoni A; Dimitriou G; Karatza AA Nutrition of neonates with congenital heart disease: existing evidence, conflicts and concerns. J Matern Fetal Neonatal Med, 2020.PMID 30608033
  11. [11]Liu K; Sharma P; Bartle J; Yap J; Fraser C; Newbould E; Sgouropoulou C; Foo J; Ha Y; Kennedy B; et al Protein intake and requirements in children and adolescents undergoing Hematopoietic Stem Cell Transplant (HSCT): An international benchmarking survey and a scoping review. Clin Nutr ESPEN, 2024.PMID 38972038
  12. [12]Joosten K; Embleton N; Yan W; Senterre T; Braegger CP; Bronsky J; Cai W; Campoy C; Colomb V; Domellof M; et al ESPGHAN/ESPEN/ESPR/CSPEN guidelines on pediatric parenteral nutrition: Energy. Clin Nutr, 2018.PMID 30078715

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