Nutrition Interventions for Children with Special Health Care Needs

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Chapter 21 - Nutrition Interventions for Metabolic Disorders Adjunct treatment Biochemical features Nutritional treatment Disorder Enzyme: missing or inactive Organic Acid Disorders L-carnitine, vitamin B12 Food: low protein Metabolic acidosis; vomiting; seizures; coma; often death, � organic acid, ammonia levels Methylmalonic aciduria Methylmalonyl-CoA mutase, or similar IV fluids, bicarbonate during acute episodes Specialized formula: lower protein without isoleucine, methionine, threonine, valine L-carnitine, biotin Food: low protein Metabolic acidosis; � ammonia, propionic acid; � methylcitric acid in urine Propionic aciduria Propionyl-CoA carboxylase, or similar IV fluids, bicarbonate during acute episodes Specialized formula: lower protein without isoleucine, methionine, threonine, valine L-carnitine, L-glycine Food: low protein Poor feeding; lethargy; seizures; metabolic ketoacidosis; hyperammonemia Isovaleric acidemia Isovaleryl-CoA dehydrogenase 242 Nutrition Interventions for Children With Special Health Care Needs Specialized formula: without leucine L-carnitine, bicitra Food: low protein Vomiting; dehydration; metabolic ketoacidosis Ketone utilization disorder 2-methylacetoacetyl-CoAthiolase, or similar Specialized formula: without isoleucine Avoid fasting, emphasize high complex carbohydrates Supplemental oral biotin Biotinidase deficiency Biotinidase, or similar In infancy, seizures, hypotonia, rash, stridor apnea; in older children, also alopecia, ataxia, developmental delay, hearing loss
Adjunct treatment Biochemical features Nutritional treatment Disorder Enzyme: missing or inactive L-carnitine, ?thiamin Food: low protein Seizures; acidosis Maple syrup urine disease Branched chain ketoacid dehydrogenase complex Specialized formula: without leucine, isoleucine, valine Plasma leucine, isoleucine, valine 10x normal Fatty Acid Oxidation Disorders MCT oil, ?L-carnitine Low fat, low long chain fatty acids, avoid fasting Vomiting, lethargy, hypoglycemia Long-chain acyl-CoA dehydrogenase Long chain acyl-CoA dehydrogenase (LCAD) deficiency MCT oil, ?L-carnitine Low fat, low long-chain fatty acids; avoid fasting Vomiting, lethargy, hypoglycemia Long-chain 3-hydroxyacyl-CoA dehydrogenase Long chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency Section 3 - Condition-Specific Nutrition Interventions ?L-carnitine Low fat, low medium chain fatty acids, avoid fasting Vomiting, lethargy, hypoglycemia Medium-chain acyl-CoA dehydrogenase Medium chain acyl-CoA dehydrogenase (MCAD) deficiency MCT oil, ?L-carnitine Low fat, low short chain fatty acids, avoid fasting Vomiting, lethargy, hypoglycemia Short-chain acyl-CoA dehydrogenase Short chain acyl-CoA dehydrogenase (SCAD) deficiency Very long chain acyl-CoA dehydrogenase (VLCAD) deficiency Nutrition Interventions for Children With Special Health Care Needs 243 MCT oil, ?L-carnitine Low Fat, low very long chain fatty acids, avoid fasting Vomiting, lethargy, hypoglycemia Very-long-chain acyl-CoA dehydrogenase Carbohydrate Disorders Raw cornstarch, iron supplements Low lactose, low fructose, low sucrose, low fat, high complex carbohydrates, avoid fasting Glucose-6-phosphatase Profound hypoglycemia; hepatomegaly Glycogen storage diseases (Type Ia) No sucrose, fructose Vomiting; hepatomegaly; hypoglycemia, FTT, renal tubular defects after fructose introduction Fructose-1-phosphate aldolase Hereditary fructose intolerance � blood/urine fructose after fructose feeding
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Nutrition Interventions for Childre
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Kay Kopp, OTR/L Occupational Therap
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Appendices Appendix A Sample Screen
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ii Nutrition Interventions for Chil
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Population “Children with special
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vi Nutrition Interventions for Chil
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Chapter 1 - Nutrition Screening and
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Chapter 2 - Anthropometricss needs,
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Chapter 2 - Anthropometricss Statur
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Chapter 2 - Anthropometricss Techni
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Chapter 2 - Anthropometricss paraly
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Chapter 2 - Anthropometricss time a
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Chapter 2 - Anthropometricss measur
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Chapter 2 - Anthropometricss Mild m
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Chapter 2 - Anthropometricss 9. Re-
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Chapter 2 - Anthropometricss 5. Per
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Chapter 2 - Anthropometrics Table 2
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Chapter 2 - Anthropometricss Refere
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Chapter 2 - Anthropometricss 26. Kr
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Chapter 3 - Physical Activity for C
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Chapter 4 - Breastfeeding recommend
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Chapter 4 - Breastfeeding Table 4-1
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Chapter 4 - Breastfeeding Assessmen
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Chapter 4 - Breastfeeding 58 Nutrit
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Chapter 5 - Medication-Nutrient Int
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Chapter 6 - Nutrition Interventions
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Chapter 8 - Oral-Motor Feeding Prob
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Chapter 9 - Behavior Issues Related
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Chapter 10 - Enteral Feeding Other
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Chapter 10 - Enteral Feeding Table
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Chapter 10 - Enteral Feeding Table
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Chapter 10 - Enteral Feeding Refere
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Chapter 11 - Community Monitoring o
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Chapter 12 - Accommodating Special
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Chapter 13 - Nutrition Intervention
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Page 208 and 209: Chapter 17 - Nutrition Intervention
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Page 292 and 293: Appendix A Please list any other nu
Page 294 and 295: Appendix A Baby formula What kind o
Page 296 and 297: Appendix B Manufacturer Description
Page 298 and 299: Appendix C 282 Nutrition Interventi
Page 300 and 301: Appendix D 284 Nutrition Interventi
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Appendix E 292 Nutrition Interventi
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Appendix E Table 3: Parent-Specific
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Appendix F Percentiles of Upper Arm
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Appendix F 302 Nutrition Interventi
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Appendix G 304 Nutrition Interventi
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Appendix H 312 Nutrition Interventi
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Appendix I 320 Nutrition Interventi
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Appendix J 328 Nutrition Interventi
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Appendix K 336 Nutrition Interventi
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Appendix K 338 Nutrition Interventi
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Appendix L 340 Nutrition Interventi
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Appendix M 342 Nutrition Interventi
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Appendix M 344 Nutrition Interventi
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Appendix N Gastrostomy Tube Feeding
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Appendix N activities. When feeding
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Appendix N Feeding Sets/Extensions
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Appendix N Fluid and Electrolytes W
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Appendix N osmolality of full-stren
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Appendix N Modular Products Modular
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Appendix N his family, and the home
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Appendix N • The feeding tube may
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Appendix N equipment. The Special S
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Appendix N Table N-2: Common Compli
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Appendix N Complication Possible Ca
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Appendix N 368 Nutrition Interventi
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Appendix O Some conditions increase
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Appendix O Table O-3: Recommended P
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Appendix O 374 Nutrition Interventi
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Appendix P Diet Prescription for Me
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Appendix Q Goals Considerations for
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Appendix R Condition Description Nu
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Appendix R Condition Description Nu
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Appendix R References 1. Ekvall SW
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Appendix S Table S-1: Infant Formul
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Appendix S Comments Osmolality (mOs
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Appendix S Table S-2: Pediatric For
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Appendix S Table S-3: Modular Produ
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Appendix S Comments Energy (kcal) P
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Appendix S Contact Information Abbo
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Appendix T Powdered Formulas Accura
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Appendix T Table T-1: Liquid Concen
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Appendix T 402 Nutrition Interventi
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Appendix U Nutrition Strategies for
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Appendix U ADA Pocket Guide to Nutr
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Glossary Autism Spectrum Disorders
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Glossary Early intervention service
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Glossary Length (recumbent length)
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Glossary Stadiometer moveable headb
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Index specialist 98 target 108 Bink
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Index elemental formula 348 elimina
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Index I ICF. See International Clas
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Index risk indicators 4 screening 1
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Index suppositories 72, 77 Surmonti
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