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Nutrition Interventions for Children with Special Health Care Needs

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Chapter 21 - <strong>Nutrition</strong> <strong>Interventions</strong> <strong>for</strong> Metabolic Disorders<br />

Adjunct<br />

treatment<br />

Biochemical features <strong>Nutrition</strong>al<br />

treatment<br />

Disorder Enzyme: missing<br />

or inactive<br />

Eliminate lactose, low<br />

galactose, use soy protein<br />

isolate <strong>for</strong>mula<br />

Vomiting; hepatomegaly; FTT;<br />

cataracts; MR; often, early<br />

sepsis<br />

Galactosemia Galactose-1-phosphate<br />

uridyl transferase<br />

� urine/blood galactose<br />

*Table adapted from reference 6<br />

† 2-(2-nitro-4-trifluoro-methyl-benzoyl)-1,3-cyclohexanedione which is an inhibitor of 4-hydroxy-phenylpyruvate dioxygenase<br />

‡ Sodium phenylbutyrate and phenylacetate are chemicals administered to enhance waste ammonia excretion; other compounds producing the same<br />

effect are also used.<br />

244 <strong>Nutrition</strong> <strong>Interventions</strong> <strong>for</strong> <strong>Children</strong> With <strong>Special</strong> <strong>Health</strong> <strong>Care</strong> <strong>Needs</strong>

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