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

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


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.


Download data is not yet available.


Rimmer JH, Yamaki K. Obesity and intellectual disability. Ment Retard Dev Disabil Res Rev 2006; 12(1): 22–7. doi: 10.1002/MRDD.20091

Segal M, Eliasziw M, Phillips S, Bandini L, Curtin C, Kral TVE, et al. Intellectual disability is associated with increased risk for obesity in a nationally representative sample of U.S. children. Disabil Health J 2016 Jul 1; 9(3): 392. doi: 10.1016/J.DHJO.2015.12.003

Melville CA, Hamilton S, Hankey CR, Miller S, Boyle S. The prevalence and determinants of obesity in adults with intellectual disabilities. Obes Rev 2007 May; 8(3): 223–30. doi: 10.1111/J.1467-789X.2006.00296.X

Royston R, Howlin P, Waite J, Oliver C. Anxiety disorders in Williams Syndrome contrasted with intellectual disability and the general population: a systematic review and meta-analysis. J Autism Dev Disord 2017 Dec 1; 47(12): 3765–77. doi: 10.1007/S10803-016-2909-Z

Royston R, Oliver C, Howlin P, Waite J. Anxiety characteristics in individuals with Williams syndrome. J Appl Res Intellect Disabil 2021 Jul 1; 34(4): 1098–107. doi: 10.1111/JAR.12864

Thom RP, Pober BR, McDougle CJ. Psychopharmacology of Williams syndrome: safety, tolerability, and effectiveness. Expert Opin Drug Saf 2021; 20(3): 293–306. doi: 10.1080/14740338.2021.1867535

Pitts CH, Klein-Tasman BP, Osborne JW, Mervis CB. Predictors of specific phobia in children with Williams syndrome. J Intellect Disabil Res 2016 Oct 1; 60(10): 1031–42. doi: 10.1111/jir.12327

Pober BR. Williams-Beuren syndrome. N Engl J Med 2010 Jan 21; 362(3): 239–52. doi: 10.1056/nejmra0903074

Lunati ME, Bedeschi MF, Resi V, Grancini V, Palmieri E, Salera S, et al. Impaired glucose metabolism in subjects with the Williams-Beuren syndrome: a five-year follow-up cohort study. PLoS One 2017 Oct 1; 12(10): e0185371. doi: 10.1371/journal.pone.0185371

Masserini B, Bedeschi MF, Bianchi V, Scuvera G, Beck-Peccoz P, Lalatta F, et al. Prevalence of diabetes and pre-diabetes in a cohort of Italian young adults with Williams syndrome. Am J Med Genet A 2013; 161(4): 817–21. doi: 10.1002/ajmg.a.35655

Nordstrøm M, Paus B, Andersen LF, Kolset SO. Dietary aspects related to health and obesity in Williams syndrome, Down syndrome, and Prader-Willi syndrome. Food Nutr Res 2015 Feb 3; 59: 25487. doi: 10.3402/FNR.V59.25487

WHO Consultation on Obesity (1999: Geneva), World Health Organization. Obesity : preventing and managing the global epidemic: report of a WHO consultation. WHO Technical Report Series 894. Geneva: World Health Organization; 2000. Available from: [cited 04 March 2022].

Centers for Disease Control and Prevention (CDC). National Center for Health Statistics (NCHS). National Health and Nutrition Examination Survey Data. Hyattsville, MD: US Department of Health and Human Services, Center for Disease Control and Prevention; 2010. Available from: [cited 04 March 2022].

Thompson FE, Midthune D, Kahle L, Dodd KW. Development and evaluation of the National Cancer Institute’s dietary screener questionnaire scoring algorithms. J Nutr 2017 Jun 1; 147(6): 1226. doi: 10.3945/jn.116.246058

U.S. Department of Agriculture and U.S. Department of Health and Human Services. Dietary guidelines for Americans, 2020–2025. 2020. Available from: [cited 4 March 2022].

Russell H, Oliver C. The assessment of food-related problems in individuals with Prader-Willi syndrome. Br J Clin Psychol 2003 Nov; 42(4): 379–92. doi: 10.1348/014466503322528928

Alaimo JT, Barton LV, Mullegama SV, Wills RD, Foster RH, Elsea SH. Individuals with Smith-Magenis syndrome display profound neurodevelopmental behavioral deficiencies and exhibit food-related behaviors equivalent to Prader-Willi syndrome. Res Dev Disabil 2015 Dec 1; 47: 27–38. doi: 10.1016/J.RIDD.2015.08.011

Welham A, Lau J, Moss J, Cullen J, Higgs S, Warren G, et al. Are Angelman and Prader-Willi syndromes more similar than we thought? Food-related behavior problems in Angelman, Cornelia de Lange, Fragile X, Prader-Willi and 1p36 deletion syndromes. Am J Med Genet A 2015 Mar 1; 167(3): 572–8. doi: 10.1002/ajmg.a.36923

Gandhi AA, Wilson TA, Sisley S, Elsea SH, Foster RH. Relationships between food-related behaviors, obesity, and medication use in individuals with Smith-Magenis syndrome. Res Dev Disabil. 2022 Aug; 127: 104257. doi: 10.1016/j.ridd.2022.104257

Nauta MH, Scholing A, Rapee RM, Abbott M, Spence SH, Waters A. A parent-report measure of children’s anxiety: psychometric properties and comparison with child-report in a clinic and normal sample. Behav Res Ther 2004; 42(7): 813–39. doi: 10.1016/S0005-7967(03)00200-6

Wharton S, Raiber L, Serodio KJ, Lee J, Christensen RAG. Medications that cause weight gain and alternatives in Canada: a narrative review. Diabetes Metab Syndr Obes 2018; 11: 427–38. doi: 10.2147/dmso.S171365

Draheim CC, Stanish HI, Williams DP, McCubbin JA. Dietary intake of adults with mental retardation who reside in community settings. Am J Ment Retard 2007 Sep; 112(5): 392–400. doi: 10.1352/0895-8017(2007)112[0392:dioawm]2.0.CO;2

Ptomey L, Goetz J, Lee J, Donnelly J, Sullivan D. Diet quality of overweight and obese adults with intellectual and developmental disabilities as measured by the healthy eating index-2005. J Dev Phys Disabil 2013 Dec; 25(6): 625–36. doi: 10.1007/s10882-013-9339-z

Gast DAA, de Wit GLC, van Hoof A, de Vries JHM, van Hemert B, Didden R, et al. Diet quality among people with intellectual disabilities and borderline intellectual functioning. J Appl Res Intellect Disabil 2022 Mar 1; 35(2): 488. doi: 10.1111/jar.12958

Morris CA, Braddock SR, Chen E, Trotter TL, Berry SA, Burke LW, et al. Health care supervision for children with Williams Syndrome. Pediatrics 2020 Feb; 145(2): e20193761. doi: 10.1542/peds.2019-3761

McRorie JW. Evidence-based approach to fiber supplements and clinically meaningful health benefits, Part 1: what to look for and how to recommend an effective fiber therapy. Nutr Today 2015 Mar 10; 50(2): 82–9. doi: 10.1097/nt.0000000000000082

Dicken SJ, Batterham RL. The role of diet quality in mediating the association between ultra-processed food intake, obesity and health-related outcomes: a review of prospective cohort studies. Nutrients 2021 Dec 22; 14(1): 23. doi: 10.3390/nu14010023

Priano SM, Hong OS, Chen JL. Lifestyles and health-related outcomes of U.S. hospital nurses: a systematic review. Nurs Outlook 2018 Jan 1; 66(1): 66–76. doi: 10.1016/J.outlook.2017.08.013

Clemente-Suárez VJ, Mielgo-Ayuso J, Martín-Rodríguez A, Ramos-Campo DJ, Redondo-Flórez L, Tornero-Aguilera JF. The burden of carbohydrates in health and disease. Nutrients 2022 Sep 15; 14(18): 3809. doi: 10.3390/nu14183809

Miller JL, Tan M. Dietary management for adolescents with Prader–Willi Syndrome. Adolesc Health Med Ther 2020 Aug; 11: 113. doi: 10.2147/ahmt.s214893

Nutrition | National Down Syndrome Society (NDSS) [Internet]. Available from: [cited 24 November 2022].

Crinò A, Fintini D, Bocchini S, Grugni G. Obesity management in Prader–Willi syndrome: current perspectives. Diabetes Metab Syndr Obes 2018; 11: 579. doi: 10.2147/dmso.s141352

Sindhar S, Lugo M, Levin MD, Danback JR, Brink BD, Yu E, et al. Hypercalcemia in Patients with Williams-Beuren Syndrome. J Pediatr 2016 Nov; 178: 254–60.e4. doi: 10.1016/j.jpeds.2016.08.027

Ptomey LT, Walpitage DL, Mohseni M, Dreyer Gillette ML, Davis AM, Forseth B, et al. Weight status and associated comorbidities in children and adults with Down syndrome, autism spectrum disorder and intellectual and developmental disabilities. J Intellect Disabil Res 2020; 64(9): 725–37. doi: 10.1111/jir.12767

Hsieh K, Rimmer JH, Heller T. Obesity and associated factors in adults with intellectual disability. J Intellect Disabil Res 2014 Sep; 58(9): 851–63. doi: 10.1111/jir.12100

Uzogara SG. Underweight, the less discussed type of unhealthy weight and its implications: a review. Am J Food Sci Nutr Res 2016; 3(5): 126–42.

Curtin C, Bandini LG, Must A, Gleason J, Lividini K, Phillips S, et al. Parent support improves weight loss in adolescents and young adults with Down syndrome. J Pediatr 2013; 163(5): 1402–408.e1. doi: 10.1016/J.JPEDS.2013.06.081

Carrillo-Nieto A, Landa-Ramírez E. Cognitive-behavioral therapy for hyperphagia in an adult with Prader-Willi Syndrome: pitfalls and challenges. Revista Latinoamercana de Medicina Conductual 2020; 9(2): 57–64.

Ho AY, Dimitropoulos A. Clinical management of behavioral characteristics of Prader–Willi syndrome. Neuropsychiatr Dis Treat 2010 Apr; 6: 107. doi: 10.2147/ndt.S5560

Thompson F, Subar A. Dietary assessment methodology. In: Nutrition in the prevention and treatment of disease. 2001; p. 3–39. doi: 10.1016/B978-012193155-1/50003-9

Ogata H, Ihara H, Gito M, Sayama M, Murakami N, Ayabe T, et al. Aberrant, autistic, and food-related behaviors in adults with Prader-Willi syndrome. The comparison between young adults and adults. Res Dev Disabil 2018 Feb 1; 73: 126–34. doi: 10.1016/J.ridd.2017.12.020

United States. US Department of Agriculture Economic Research Services. Rural-urban commuting area codes. 2019. Available from: [cited 24 April 2022].
How to Cite
Renzi D., Stanley T., Waxler J., Lee H., Pober B., & Nordstrøm M. (2023). Body mass index variation in adults with Williams syndrome: associations with predicted dietary intake and food behaviors. Food & Nutrition Research, 67.
Original Articles