Nutrition Interventions for Children with Special Health Care Needs

Condition Description Nutritional Implications/Problems
Possible oral-motor problems, reflux
Fragile X syndrome3 Fragile site on X chromosome, males, mental
retardation, hypotonia, hyperactivity common
Growth problems, oral-motor problems
de Lange syndrome3 Etiology unknown, prenatal poor growth, cardiac
defects, severe mental retardation, short stature,
dysmorphic features
Neurodevelopmental
Growth problems, oral-motor problems, medicationnutrient
interactions seizure disorder, constipation
Cerebral palsy1 Chronic, nonprogressive central nervous system dysfunction
leading to problems with tone and posture
Constipation, urinary tract infections, excessive weight
gain, medication-nutrient interactions
Neural tube defect, immobility, frequently associated with
hydrocephaly
Neural tube defects, e.g.,
myelomeningocele1 Spinal muscular dystrophy 1 Progressive hereditary disorder of muscle Initial growth problems, then obesity, oral-motor problems
Seizure disorders 1 Neurological disorders, e.g., epilepsy Medication-nutrient interactions
Abnormal food-related behaviors, possible nutrient
deficiencies
Autism and autism spectrum disorders3 Abnormal social and communication patterns, stereotypic
behaviors
Oral-motor problems, growth problems, air swallowing
Rett syndrome3 Progressive neurological deterioration in females after normal
early infancy, seizures, microcephaly
Appendix R
Nutrition Interventions for Children With Special Health Care Needs 381
Metabolic
Growth problems, possible cholesterol supplement, oralmotor
problems
Smith-Lemli-Opitz syndrome3 Abnormality in cholesterol metabolism, multiple dysmorphic
features, short stature, abnormal facies, psychomotor
retardation, genital abnormalities in males
Need to restrict phenylalanine, supplement tyrosine
Phenylketonuria (PKU) 3 Deficiency in phenylalanine hydroxylase causes↑
phenylalanine and leads to mental retardation
Defect in organic acid metabolic pathways, acidosis Poor growth, restrict protein and substrate, supplement
product of enzymatic reaction, L-carnitine, bicitra,
compounds to enhance waste ammonia excretion,
possibly biotin, vitamin B6 Organic acidemias, e.g., methylmalonic
acidemia, propionic acidemia3 Growth problems, restrict protein, supplement L-carnitine,
L-citrulline, or L-arginine (except argininemia), and
prescribe compounds to enhance waste ammonia
excretion
Abnormality in urea cycle enzymes,↑ NH 3
Urea cycle disorders, e.g., ornithine
transcarbamylase, carbamyl-phosphate
synthetase deficiency, argininosuccinic
aciduria3 Abnormal fructose metabolism, nausea, vomiting, seizures Restrict/eliminate sucrose, fructose, possible growth
problems
Hereditary fructose intolerance, fructose-
1,6-bisphosphatase deficiency3