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Growth and nutrition in 10 girls with Rett syndrome
M Thommessen1, B F Kase, A Heiberg
1Institute for Nutrition Research, School of Medicine, University of Oslo, Norway.
Insights
Girls with Rett syndrome experience significant growth deficits and malnutrition due to feeding difficulties. Nutritional interventions are crucial to prevent wasting and improve outcomes for these children.
Area of Science:
- Pediatrics
- Neurology
- Human Growth and Development
Background:
- Rett syndrome is a neurodevelopmental disorder affecting primarily girls.
- Early development is typically normal, followed by regression.
- Growth and nutritional status are significant concerns in this population.
Purpose of the Study:
- To assess growth, anthropometric outcomes, feeding problems, and dietary intake in girls with Rett syndrome.
- To identify nutritional deficiencies and energy intake patterns.
- To highlight the need for nutritional intervention strategies.
Main Methods:
- Cross-sectional and retrospective data collection.
- Analysis of growth parameters (height, weight).
- Evaluation of dietary intake and feeding behaviors in 10 girls aged 3-16 years.
Main Results:
- Most girls exhibited height and/or weight for height below the 2.5th percentile.
- Oral-motor dysfunctions were common, impacting self-feeding abilities.
- Mean energy intake was insufficient based on age recommendations, with low intake of thiamin, vitamin D, calcium, and iron.
Conclusions:
- Girls with Rett syndrome are at high risk for malnutrition and wasting.
- Oral-motor dysfunction significantly affects nutritional status.
- Investigating nutritional interventions is essential to manage and prevent malnutrition in Rett syndrome.
Abstract:
Cross-sectional and retrospective data on growth and anthropometric outcome, feeding problems and dietary intake are presented for 10 girls between three and 16 years of age with Rett syndrome. All girls had birth weight and length within the normal range for gestational age and development was considered normal until six to 24 months of age. The girls presented a fall off in linear growth during the first two years of life and at the time of study, all but one had height and/or weight for height below the 2.5th percentile of healthy children. The girls had good appetite but could not eat by themselves and oral-motor dysfunctions were common. The mean energy intake was 66.9% of the US recommendations according to age and 107.8% of the recommendations according to body weight. The intakes of thiamin, vitamin D, calcium and iron were considered low. None was anaemic. Different nutritional intervention strategies should be investigated to reduce and, if possible, prevent malnutrition and wasting in girls with Rett syndrome.