Nutrition- Focused Physical Assessment in Pediatric Chronic Disease

Ellen McCloy, MS, RD, CNSC Esther Berenhaut, RD, CSP, CSR, CNSC Objectives

• Identify common physical assessment indicators in pediatric chronic disease.

• Identify physical signs of micronutrient deficiencies common in pediatric chronic disease.

Chronic Kidney Disease

Cerebral Palsy

Inborn Errors of Metabolism CHRONIC KIDNEY DISEASE Pediatric CKD Overview

• Congenital vs Glomerular – Congenital disorders have earlier onset • Growth failure is most frequent and severe with congenital renal anomalies and when the onset of CKD is during the first two years of life – 50% of a child’s growth occurs during this time and is mostly dependent on nutritional status – Glomerular • Focal segmental glomerular sclerosis is the most prevalent acquired renal disease in children – Increasing in pediatrics due to obesity

Chronic Malnutrition

• Disease related – Uremia, metabolic acidosis – Polyuria – Altered mineral metabolism – Growth hormone – Reflux – Anemia – Frequent catabolic illness • Non-disease related – Feeds held for surgeries – Late placement of Gtube Common Physical Findings

• Short stature • Pallor • Body mass/habitus • Bowed legs, poor posture • Large abdomen, thin limbs

Other Nutrient Concerns

• Wounds

• Micronutrients – Carnitine – Zinc

Nutrition Interventions

• Uremia -> decrease protein load, if appropriate • Metabolic acidosis -> correct acidosis with alkalizing agent • Polyuria -> replace renal losses (sodium, sometimes potassium, etc) • Altered mineral metabolism -> DRI for Ca and Phos unless 2’ hyperparathyroidism (80% DRI for phos), active vitamin D

Nutrition Interventions, cont’d

• Growth hormone deficiency/resistence -> rGH (screen for appropriateness; height z- score <-1.88; all other factors must be corrected first) • Reflux -> small, frequent feeds, continuous overnight Gtube feeds, MD prescribed meds (antacid, gut motility stimulator) • Anemia -> iron supplement, MD prescribed erythropoietin • Carnitine and Zinc -> replacement

CEREBRAL PALSY Cerebral Palsy Overview

• Heterogeneous group of conditions involving permanent nonprogressive central motor dysfunction that affect muscle tone, posture, and movement • Often classified according to the predominant neurological signs – Muscle tone – Extrapyramidal function Chronic Malnutrition

• Disease related – High/low kcal needs • Athetoid movements • Ataxia – Short stature • Chronic malnutrition • May also be due to brain injury – Poor feeding skills • Tongue thrusting • Swallowing Problems • Sialorrhea

• Non-disease related – Parents averse to place G-tube Other Nutrition Concerns

• Wounds – Related to level of mobility • Osteopenia – Seizure meds – Non-weight bearing • Carnitine deficiency – Valproic acid • Gastrointestinal complications – Chronic constipation, reflux, abdominal pain

Common Physical Findings

• Temporal wasting, buccal pads

• Bony prominences – Clavicle, scapula, acromion process

• Lack of energy stores (lean vs adipose)

Additional Considerations

• Tone • Movement • Mobility

Nutrition Interventions

• Calories – Different ways to estimate • Poor feeding skills – OT – Texture modifications, adaptive utensils – Nutrition support (enteral) • Wounds – Adequate protein – Treat with vitamin C & A, and zinc, and arginine

Nutrition Interventions Cont’d

• Osteopenia – Monitoring and supplementation of calcium, phosphorus and vitamin D • Carnitine deficiency – Supplementation • Gastrointestinal complications – Small, frequent feeds, slow feeds, medication – Fiber, fluid for constipation INBORNS ERRORS OF METABOLISM (IEM) INBORNS ERRORS OF METABOLISM (IEM) OVERVIEW

• Aminoacidopathies/Energy(mitochondrial) disorders

• Hyperphenylalaninemia (PKU)

• Maple Syrup Urine Disease (MSUD)

• Mitochondrial disorders (MD)

INBORNS ERRORS OF METABOLISM (IEM) OVERVIEW OVERVIEW • Inherited conditions (AR, maternal)

• Untreated/late treated conditions can cause

• mental retardation (PKU) • feeding problems, altered mental status, neurologic damage (MSUD) • Poor overall growth, multiorgan issues, progressive neurological decline (mDNA) Hyperphenylalaninemia (PKU) Maple Syrup Urine Disease (MSUD Mitochondrial Disease (MD) Mitochondrial Disease (MD) Tissues With High Energy Demands

Potential for Malnutrition • Diets for AA disorders are “synthetic” • Medical foods (formulas) and low protein foods devoid of offending metabolites • Fruit (no limit), vegetables and grains (with limits) • Possible risk for nutritional deficiencies on full treatment • Greater risk for deficiencies in later childhood/adolescent patients (compliance)

• Diets for MD • Need substrate alteration (higher CHO) • May need semi/elemental diets – tube feedings

What to look for • Poor growth/weight gain/head circumference

• Hair • Sparse • Colour (PKU – tyrosine deficiency)

• Skin/Eyes • Decreased pigmentation (PKU)

• Odour • Musty (PKU) • Sweet (MSUD)

What to look for

• Tone • Low/high (All)

• GI (MD) • Constipation, • vomiting (MD)

• Neurologic/mental status • Delayed/loss of motor skills (MD) • “Fog” (PKU) • Ataxia (MSUD) • Tremors (PKU)

Energy (Mitochondrial ) Disorders Nutrition Interventions

• Adequate protein/kcal/amino acid supplementation • Tyrosine (PKU) • Branched chain AA (excess leucine, inadequate isoleucine and valine)

• Vitamin/mineral status (MD) • Riboflavin • Carnitine • CoQ10

Nutrition Interventions (cont.)

• Vitamin/Minerals

• B vitamins (thiamine, folate, B6, B12) • Vitamin D – osteopenia • Iron • Trace minerals (Se, Cu, Zn) • Carnitine? (PKU) In Summary

• Common threads in pediatric chronic disease related malnutrition. – Growth – Weight – Bone health – Protein/amino acids – Micronutrients • Questions? References

• Corkin et al. ASPEN Pediatric Nutrition Support Core Curriculum. 2015. • Helal, Imed & Brosnahan, Godela & Gitomer, Berenice & Schrier, Robert. (2012). Glomerular hyperfiltration: Definitions, mechanisms and clinical implications. Nature reviews. Nephrology. 8. 293-300. 10.1038/nrneph.2012.19. • Kidney Disease Outcomes Quality Initiative. Clinical Practice Guideline for Nutrition in Children with CKD: 2008 Update. American Journal of Kidney Diseases. • Maruja Navarro Díaz; Consequences of morbid obesity on the kidney. Where are we going?, Clinical Kidney Journal, Volume 9, Issue 6, 1 December 2016, Pages 782–787 • Ramezani & Raj. The Gut Microbiome, Kidney Disease, and Targeted Interventions. JASN April 2014 vol. 25 no. 4657-670 • Southeast Regional Genetics Network. PKU Nutrition Management Guidelines. Nutrition Assessment. March 2015. v 1.12 • Southeast Regional Genetics Network. MSUD Nutrition Management Guidelines. Nutrition Assessment. August 2016 v 1.54