Peer-reviewed article
Comparison of Caregiver-Reported Dietary Intake Methods in Zellweger Spectrum Disorder.
Mousumi Bose, Nancy L von Thun, Adrian L Kerrihard, Melisa L Lopez, Chelsea I Donlon et al. ·
Summary
Written by AI, not yet AICS's view · claude-haiku-4-5-20251001This is a methodological study about how to assess dietary intake in children and young adults with Zellweger spectrum disorder, a rare genetic disease. The researchers compared two ways of measuring what children ate (dietary recall interviews and food records) using information from family caregivers. The study found that both methods produced similar results and that dietary assessment is practical for this population, though most children did not get enough fiber.
Key points
- 21 individuals aged 1-33 years with Zellweger spectrum disorder or related peroxisomal disorders participated
- Both 24-hour dietary recall and 3-day food records produced highly correlated nutrient intake data (r² = 0.998)
- Mean nutrient intakes generally met or exceeded dietary reference intake (DRI) requirements except for fiber (about 50% of DRI)
- Dietary assessment was feasible for both orally fed (11) and enterally fed (10) individuals
- No previous published studies had assessed dietary intake in this population before this work
Limitations
This is a methodological study focused on comparing dietary assessment tools rather than evaluating a health intervention, so it does not measure whether any treatment improved child outcomes. The study is also limited to a small sample of individuals with a very rare genetic disorder, which limits generalizability.
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.