Body mass index variation in adults with Williams syndrome: associations with predicted dietary intake and food behaviors

Danielle Renzi; Takara  Stanley; Jessica  Waxler; Hang  Lee; Barbara  Pober; Marianne Nordstrøm

doi:10.29219/fnr.v67.9321

Danielle Renzi Division of Genetics, Department of Pediatrics, Massachusetts General Hospital, Boston, MA, USA
Takara Stanley Metabolism Unit, Department of Medicine, and Pediatric Endocrine Unit, Department of Pediatrics, Massachusetts General Hospital, Boston, MA; and Harvard Medical School, Boston, MA, USA
Jessica Waxler Division of Genetics, Department of Pediatrics, Massachusetts General Hospital, Boston, MA, USA
Hang Lee Harvard Medical School, Boston, MA, USA; and Biostatistics Center, Massachusetts General Hospital, Boston, MA, USA
Barbara Pober Division of Genetics, Department of Pediatrics, Massachusetts General Hospital, Boston, MA, USA; and Metabolism Unit, Department of Medicine, and Pediatric Endocrine Unit, Department of Pediatrics, Massachusetts General Hospital, Boston, MA, USA
Marianne Nordstrøm Department of Nutrition, Institute of Basic Medical Sciences, University of Oslo, Oslo, Norway; Frambu Resource Centre for Rare Disorders, Siggerud, Norway; and Unit for Rare Neuromuscular Disorders, Movement, Muscle and Neurodegeneration, Department of Neurology, Oslo University Hospital, Oslo, Norway

DOI: https://doi.org/10.29219/fnr.v67.9321

Keywords: Williams syndrome, body weight, Food frequency questionnaire, Food behavior, Weight trajectory

Abstract

Background: Dietary intake and body weight are important predictors of long-term health. However, few studies have focused on these topics in adults with genetic syndromes that have associated intellectual disability, such as Williams syndrome (WS).

Objective: In adults with WS, describe predicted dietary intake, food-related problems, and associations between body mass index (BMI) and possible factors contributing to differences in weight status.

Design: In this study of 82 participants (median age of 30 years, range 18–69), we cross sectionally investigated associations between BMI, predicted dietary intakes (Dietary Screener Questionnaire), food-related behaviors (Food-Related Problem Questionnaire), and anxiety (Spence Children’s Anxiety Scale). Longitudinal patterns of weight change were further studied in a subset (n = 41).

Results: BMI variation was observed with median BMI of 27.3 kg/m² (range 16.7–55.5 kg/m²). Several components of dietary intake deviated from recommendations in the WS cohort. When compared with WS participants with either normal or overweight BMI, WS participants with obesity had reduced daily intake of fruits and vegetables of 0.15 cup equivalents (P = 0.049), while participants with underweight BMI had reduced daily intake of fruits and vegetables of 0.44 cup equivalents (P = 0.026) and additionally had reduced intake of dietary fiber of 2.12 grams per day (P = 0.019). A one-point increase in the ‘preoccupation with food’ sub-score was associated with a 0.57 unit increase in BMI (P = 0.16), while a one-point increase in the ‘takes and stores food’ sub-score was associated with a 0.72 unit increase in BMI. In the longitudinal weight subset, a weight gain group and a weight stable group were identified. The former was associated with increased ‘takes and stores food’ sub-score but not with dietary intakes.

Conclusion: We observed considerable BMI variability. While few dietary intakes were associated with BMI, increased BMI and weight gain were associated with ‘preoccupation with food’ and with ‘takes and stores food’ behavior sub-scores.

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