Nutrition Interventions for Children with Special Health Care Needs

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Chapter 21 - Nutrition Interventions for Metabolic Disorders Table 21-1: Some Metabolic Disorders Amenable to Nutritional Therapy* Adjunct treatment Biochemical features Nutritional treatment Disorder Enzyme: missing or inactive Amino Acid Disorders Food: low protein Phenylketonuria Phenylalanine hydroxylase �� blood phe Specialized formula: without phenylalanine, supplement tyrosine � phenylketones in urine Progressive, severe MR, which can be prevented by early treatment Nitisinone† Food: low protein Vomiting; acidosis; diarrhea; FTT; hepatomegaly; rickets Tyrosinemia type I Fumaryl-acetoacetate hydrolase Specialized formula: without tyrosine, phenylalanine, and methionine � blood/urine tyrosine, methionine; � urine parahydroxy derivatives of tyrosine; 240 Nutrition Interventions for Children With Special Health Care Needs Liver cancer Betaine, folate, vitamin B12, Food: low protein Detached retinas; thromboembolic and cardiac disease; mild to moderate MR; bone abnormalities; fair hari, skin; � methionine, homocysteine Homocystinuria Cystathionine synthase or similar ?vitamin B6 if folate levels are normal Specialized formula: without methionine, supplement L-cystine Urea Cycle Disorders L-carnitine, phenylbutyrate,‡ L-citrulline, L-arginine Food: low protein Vomiting; seizures; sometimes coma � death. Survivors usually have MR, � plasma ammonia, glutamine Ornithine transcar bamylase Ornithine transcarbamylase deficiency Formula: without nonessential amino acids Hemodialysis or peritoneal dialysis during acute episodes
Adjunct treatment Biochemical features Nutritional treatment Disorder Enzyme: missing or inactive L-carnitine, phenylbutyrate, † L-arginine Food: low protein � plasma citrulline, ammonia, alanine Citrullinemia Argininosuccinate synthetase Formula: without nonessential amino acids Neonatal: vomiting; seizures; coma � death Infantile: vomiting; seizures; progressive developmental delay L-carnitine, phenylbutyrate, † L-citrulline, L-arginine Food: low protein Vomiting; seizures; sometimes coma � death Carbamyl phosphate synthetase Carbamyl phosphate synthetase deficiency Formula: without nonessential amino acids Section 3 - Condition-Specific Nutrition Interventions Survivors usually have MR, � plasma ammonia, glutamine Hemodialysis or peritoneal dialysis during acute episodes L-carnitine, phenylbutyrate, † L-arginine Nutrition Interventions for Children With Special Health Care Needs 241 Food: low protein Argininosuccinic aciduria Argininosuccinate lyase � plasma argininosuccinc acity, citrulline, ammonia Specialized formula: lower protein without nonessential amino acids Neonatal: hypotonia; seizures Subacute: vomiting; FTT, progressive developmental delay L-carnitine, phenylbutyrate † Food: low protein Arginase deficiency Arginase Periodic vomiting; seizures; coma Specialized formula: lower protein without nonessential amino acids Progressive spastic diplegia, developmental delay � arginine, ammonia with protein intake
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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 206 and 207: Chapter 16 - 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
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Page 298 and 299: Appendix C 282 Nutrition Interventi
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Appendix D 290 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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