Comparison of Caregiver-Reported Dietary Intake Methods in Zellweger Spectrum Disorder.

Background/

Objectives

Zellweger spectrum disorder (ZSD), a rare genetic disease characterized by defects in peroxisome biogenesis, results in dysfunction of all organ systems, including feeding difficulties, gastrointestinal bleeding, and reduced overall growth. Despite this nutritional impact, no published studies have assessed dietary intake in ZSD. The purpose of this study was to determine nutrient intake in individuals with ZSD or a related peroxisomal disorder using two methods of dietary assessment as provided by family caregivers.

Methods

Family caregivers participated in multiple 24 h dietary recall interviews and completed 3-day food records for their child with ZSD or a related single-enzyme peroxisomal disorder over a 6-month period.

Results

Twenty-one subjects (eleven orally fed and ten enterally fed), ranging from 1 to 33 years of age, were included in the study. Energy and nutrient intake as reported by dietary recall vs. 3-day food record were highly correlated for all nutrients (r2 = 0.998, p < 0.0001). Mean nutrient intakes for subjects generally achieved or exceeded DRI requirements, except for fiber (about 50% of DRI).

Conclusions

These results show that dietary assessment is feasible in individuals with ZSD using caregiver input, regardless of feed modality, and that dietary intake is consistent across different methods of assessment. These findings may be applicable in dietary assessments for individuals with ZSD and similar genetic disorders and a methodological consideration in clinical interventions.