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

Area of Science:

• Biochemistry
• Genetics
• Nutritional Science

Background:

• Zellweger spectrum disorder (ZSD) is a rare genetic condition impacting peroxisome biogenesis.
• ZSD causes multi-organ dysfunction, affecting growth and leading to feeding difficulties.
• No prior studies have evaluated dietary intake in individuals with ZSD.

Purpose of the Study:

• To assess nutrient intake in individuals with ZSD or related peroxisomal disorders.
• To compare two dietary assessment methods: 24-hour recalls and 3-day food records.
• To evaluate the feasibility of dietary assessment using family caregiver input.

Main Methods:

• Multiple 24-hour dietary recalls were conducted with family caregivers.
• Caregivers also completed 3-day food records for their child over six months.
• Data was collected from 21 subjects (1-33 years old), including orally and enterally fed individuals.

Main Results:

• Dietary recall and 3-day food records showed high correlation for nutrient intake (r² = 0.998, p < 0.0001).
• Mean nutrient intakes generally met or exceeded Dietary Reference Intakes (DRIs).
• Fiber intake was approximately 50% of the DRI for most subjects.

Conclusions:

• Dietary assessment in ZSD is feasible using caregiver input, irrespective of feeding modality.
• Consistent dietary intake data can be obtained using different assessment methods.
• Findings support the applicability of these methods for ZSD and similar genetic disorders.