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

## TL;DR

This study compares two dietary assessment methods in individuals with Zellweger spectrum disorder, showing that caregiver reports are reliable for evaluating nutrient intake.

## Contribution

The first study to assess dietary intake in Zellweger spectrum disorder using caregiver-reported methods.

## Key findings

- Energy and nutrient intake measured by dietary recall and 3-day food records were highly correlated.
- Mean nutrient intakes met or exceeded dietary reference intakes, except for fiber.
- Dietary assessment is feasible in Zellweger spectrum disorder using caregiver input, regardless of feeding method.

## Abstract

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.