Growth, Feeding and Nutrition in Rett Syndrome: Retrospective Audit of Twenty Years' Experience From an Australian Multidisciplinary Management Clinic.

AIM This study reviewed the nutritional status, feeding skills, safety, and management of patients with Rett syndrome during childhood and adolescence. METHOD Retrospective chart review of 103 females with classical Rett syndrome, aged ≤ 18 years, attending a Rett syndrome Multidisciplinary Management clinic in a tertiary hospital from 2000 to 2019. RESULTS Over multiple clinic visits the cohort was significantly (p < 0.0001) shorter and of lower weight (p < 0.0001) on standard CDC growth charts but not on Rett syndrome specific growth charts. Body mass index Z score was not significantly different on either chart but at first visit both mean and median were <0 (range -5.39 to +3.44) (p = 0.002) on standard charts. Nutritional issues included inadequate energy, fibre, calcium and iron intake. Participants tolerated food textures ranging from puree to regular diet, and fluids ranging from unmodified to modified. Compensatory strategies for drinking, chewing and swallowing difficulties were frequent. Six percent had a documented episode of aspiration pneumonia. Twenty-four percent had gastrostomy placement with 64% of these continuing oral intake or tastes at last visit. CONCLUSIONS Growth and weight gain in this cohort of females with Rett syndrome supports the pattern observed in other Rett syndrome cohorts. Given the risk of inadequate nutrition, and that optimal nutritional status is undefined in Rett syndrome, there is a need for individualised ongoing clinical assessment of nutritional status and feeding, with a multidisciplinary approach. Oropharyngeal dysphagia and compensatory feeding strategies were common, with feeding safety requiring close monitoring. The burden of assisted feeding was high.

• Publication year

  2025

• Venue

  Journal of Paediatrics and Child Health

• Publication date

  2025-07-13

• Fields of study

  Medicine

• Identifiers
  DOI 10.1111/jpc.70123PMID 40652338
• External record

  Open on Semantic Scholar

• Source metadata

  Semantic Scholar, PubMed

• No claims are published for this paper.

• No concepts are published for this paper.

• Diet and Nutritional Status of Polish Girls with Rett Syndrome—A Case-Control Study

  2023cited by this paper
• Clinical Management of Rett Syndrome in the Real World: A Physician Survey (P12-4.002)

  2023cited by this paper
• Fiberoptic Endoscopic Evaluation of Swallowing Findings in Children With Rett Syndrome

  2023cited by this paper
• Multidisciplinary Management of Rett Syndrome: Twenty Years’ Experience

  2023cited by this paper
• Anthropometric Measures Correspond with Functional Motor Outcomes in Females with Rett Syndrome.

  2022cited by this paper
• Dietary intake and growth deficits in Rett syndrome—A cross‐section study

  2021cited by this paper
• Impact of Gastrostomy Placement on Nutritional Status, Physical Health, and Parental Well‐Being of Females with Rett Syndrome: A Longitudinal Study of an Australian Population

  2018cited by this paper
• Dysphagia in Rett Syndrome: A Descriptive Study

  2017cited by this paper
• Development of International Terminology and Definitions for Texture-Modified Foods and Thickened Fluids Used in Dysphagia Management: The IDDSI Framework

  2016cited by this paper
• Co-occurrence of Dystonic and Dyskinetic Tongue Movements with Oral Apraxia in Post-regression Dysphagia in Classical Rett Syndrome Years of Life 1 Through 5

  2015cited by this paper
• Development and reliability of a system to classify the eating and drinking ability of people with cerebral palsy

  2014cited by this paper
• Gastrointestinal Dysmotility in Rett Syndrome

  2014cited by this paper
• Assessment and Management of Nutrition and Growth in Rett Syndrome

  2013cited by this paper
• Videofluororadiographic Descriptors of Swallowing Physiology in Typical Rett Syndrome: Post-Regression Stabilization During Early Childhood

  2012cited by this paper
• How to perform Subjective Global Nutritional assessment in children.

  2012cited by this paper
• Growth failure and outcome in Rett syndrome

  2012cited by this paper
• Rett syndrome: Revised diagnostic criteria and nomenclature

  2010cited by this paper
• Guided eating or feeding: three girls with Rett syndrome

  2009cited by this paper
• Feeding Experiences and Growth Status in a Rett Syndrome Population

  2007cited by this paper
• Rett syndrome in Australia: a review of the epidemiology.

  2006cited by this paper
• Growth and nutrition in Rett syndrome

  2001cited by this paper

• No citing papers are available for this paper.