Nutrition Interventions for Children with Special Health Care Needs

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Appendix R Condition Description Nutritional Implications/Problems Restrict galactose, possible growth problems, use soy formula Galactosemia3 Abnormal galactose-1-phosphate uridyl transferase, possible cataracts, liver disease, developmental delay Growth problems, restrict branched chain amino acids, supplement L-carnitine Maple syrup urine disease3 Abnormal oxidative decarboxylation of branched chain keto acids, can lead to mental retardation, seizures, and death Restrict methionine, protein, supplement cystine, folate, betaine, possibly vitamin B6 Homocystinuria3 Abnormal cystathionine-β-synthase, possible mental retardation, detached retinas, thromboembolic and cardiac disease Tyrosinemia, type 13 Abnormal fumarylacetoacetate hydrolase causes liver disease Restrict tyrosine, phenylalanine, prescribe nitisinone (Orfadin®) Nephrogenic Diabetes Insipidus3 Hereditary non-responsiveness to antidiuretic hormone Restrict sodium, protein; increased water requirements, provide chlorothiazide, delayed puberty Restrict protein; supplement bicitra, L-carnitine, avoid fasting Ketone Utilization Disorders3 Lack of enzyme(s) necessary to process ketones when catabolic, vomiting, dehydration, ↑ ketones in urine Cerebral degeneration and storage of mucopolysaccarides Oral-motor problems, constipation Mucopolysaccharidoses, e.g., Hunter, Hurler, San filippo, Morquio syndromes3 Possible oral-motor problems, avoid fasting, need for ↑ CHO, L-carnitine, ↓ fat, avoid non-metabolized fatty acids, supplement with MCT oil for VLCAD, LCAD 382 Nutrition Interventions for Children With Special Health Care Needs Disorder of β-oxidation of fatty acids of specific chain lengths, vomiting, lethargy, hypoglycemia Fatty Acid Oxidation Disorders (VLCAD, LCAD, MCAD, SCAD) 3* Supplement raw cornstarch, restrict fat, increase complex CHO, avoid lactose, supplement iron, calcium use soy formula, prescribe granulocyte colony stimulating factor(GCSF) (for type Ib) Defect in one of several enzymes that affect the use and storage of glycogen, ↑ liver size, severe hypoglycemia, ↑ cholesterol, triglycerides, infections in some types Glycogen Storage Diseases (Ia, Ib, III, IV) 3 Storage of GM 2 gangliosides, usually cerebral degeneration Oral-motor problems, constipation Sphingolipidoses, e.g., Gaucher, Nieman- Pick, Krabbe, Tay-Sachs diseases3 Oral-motor problems, restrict copper intake Wilson’s disease1,3 Abnormal storage of copper leads to renal, cardiac, pancreatic and liver disease and/or central nervous system manifestations Growth problems, oral-motor problems, air swallowing Mitochondrial disorders 3 A heterogenous group of disorders, result of dysfunction of the mitochondrial respiratory chain, muscle, neurological, GI, cardiac problems, poor growth, developmental delay, seizures
Condition Description Nutritional Implications/Problems Possible growth problems, oral-motor problems Environmental/ Teratogenic Fetal alcohol syndrome Excessive fetal alcohol exposure, possible developmental delays, short stature, microcephaly, hyperactivity Possible growth problems, oral-motor problems Drug affected, e.g., heroin, cocaine2,3 Excessive fetal drug exposure, possible developmental delays and behavior problems Possible growth problems, oral-motor problems Maternal PKU 2,3 Excessive fetal phenylalanine exposure, possible developmental delays Other Growth problems, requires pancreatic enzyme replacement therapy, vitamin therapy, CF-related diabetes Cystic fibrosis2 Chloride channel abnormalities, chronic and progressive lung disease, pancreatic insufficiency, liver disease Hirschsprung’s anomaly 1,3 Partial or total intestinal obstruction, enterocolitis Vomiting, distention, constipation alternating with diarrhea Central diabetes insipidus 1 Lack of antidiuretic hormone, polyuria, polydipsia Possible growth problems, dehydration *VLCAD Deficiency: Very Long Chain Acyl-Coenzyme A Dehydrogenase Deficiency LCAD Deficiency: Long-Chain Acyl-CoA Dehydrogenase Deficiency LCHAD Deficiency:Long Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency MCAD Deficiency:Medium Chain Acyl-Coenzyme A Dehydrogenase Deficiency SCAD Deficiency: Short Chain Acyl-CoA Dehydrogenase Deficiency Appendix R Nutrition Interventions for Children With Special Health Care Needs 383
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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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Chapter 22 - Ketogenic Diet for Sei
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Appendix A Please list any other nu
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Appendix A Baby formula What kind o
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Appendix B Manufacturer Description
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Appendix C 282 Nutrition Interventi
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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 J 330 Nutrition Interventi
Page 348 and 349: Appendix J 332 Nutrition Interventi
Page 350 and 351: Appendix J 334 Nutrition Interventi
Page 352 and 353: Appendix K 336 Nutrition Interventi
Page 354 and 355: Appendix K 338 Nutrition Interventi
Page 356 and 357: Appendix L 340 Nutrition Interventi
Page 358 and 359: Appendix M 342 Nutrition Interventi
Page 360 and 361: Appendix M 344 Nutrition Interventi
Page 362 and 363: Appendix N Gastrostomy Tube Feeding
Page 364 and 365: Appendix N activities. When feeding
Page 366 and 367: Appendix N Feeding Sets/Extensions
Page 368 and 369: Appendix N Fluid and Electrolytes W
Page 370 and 371: Appendix N osmolality of full-stren
Page 372 and 373: Appendix N Modular Products Modular
Page 374 and 375: Appendix N his family, and the home
Page 376 and 377: Appendix N • The feeding tube may
Page 378 and 379: Appendix N equipment. The Special S
Page 380 and 381: Appendix N Table N-2: Common Compli
Page 382 and 383: Appendix N Complication Possible Ca
Page 384 and 385: Appendix N 368 Nutrition Interventi
Page 386 and 387: Appendix O Some conditions increase
Page 388 and 389: Appendix O Table O-3: Recommended P
Page 390 and 391: Appendix O 374 Nutrition Interventi
Page 392 and 393: Appendix P Diet Prescription for Me
Page 394 and 395: Appendix Q Goals Considerations for
Page 396 and 397: Appendix R Condition Description Nu
Page 400 and 401: Appendix R References 1. Ekvall SW
Page 402 and 403: Appendix S Table S-1: Infant Formul
Page 404 and 405: Appendix S Comments Osmolality (mOs
Page 406 and 407: Appendix S Table S-2: Pediatric For
Page 408 and 409: Appendix S Table S-3: Modular Produ
Page 410 and 411: Appendix S Comments Energy (kcal) P
Page 412 and 413: Appendix S Contact Information Abbo
Page 414 and 415: Appendix T Powdered Formulas Accura
Page 416 and 417: Appendix T Table T-1: Liquid Concen
Page 418 and 419: Appendix T 402 Nutrition Interventi
Page 420 and 421: Appendix U Nutrition Strategies for
Page 422 and 423: Appendix U ADA Pocket Guide to Nutr
Page 424 and 425: Glossary Autism Spectrum Disorders
Page 426 and 427: Glossary Early intervention service
Page 428 and 429: Glossary Length (recumbent length)
Page 430 and 431: Glossary Stadiometer moveable headb
Page 432 and 433: Index specialist 98 target 108 Bink
Page 434 and 435: Index elemental formula 348 elimina
Page 436 and 437: Index I ICF. See International Clas
Page 438 and 439: Index risk indicators 4 screening 1
Page 440 and 441: Index suppositories 72, 77 Surmonti
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Nutrition Interventions for Children with Special Health Care Needs

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