Evidence map›Paper›PMID 41414930›Full record

ArticleJournal of intellectual disability research : JIDR2026

Resting Energy Expenditure in Adults With Williams Syndrome: Comparative Accuracy of Predictive Equations.

Danielle B Renzi, Jamie Garry, Jane Hubbard, Marianne Nordstrøm, Kathleen V Fitch, Takara L Stanley, Barbara R Pober

Abstract readComparative Study
In one paragraph

Article in Journal of intellectual disability research : JIDR, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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1 · What the graph read from it

What it found

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2 · The registry

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3 · Its place in the literature

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4 · The record

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5 · Who and what money

Authors and funding

7 authors.

Danielle B RenziDivision of Medical Genetics and Metabolism, Department of Pediatrics, Massachusetts General Hospital, Boston, Massachusetts, USA.ORCID 0009-0001-8189-8531
Jamie GarryTranslational & Clinical Research Centers, Massachusetts General Hospital, Boston, Massachusetts, USA.
Jane HubbardTranslational & Clinical Research Centers, Massachusetts General Hospital, Boston, Massachusetts, USA.
Marianne NordstrømFrambu Resource Centre for Rare Disorders, Siggerud, Norway.
Kathleen V FitchMetabolism Unit, Department of Medicine, Endocrine Division, Massachusetts General Hospital, Boston, Massachusetts, USA.
Takara L StanleyHarvard Medical School, Boston, Massachusetts, USA.
Barbara R PoberDivision of Medical Genetics and Metabolism, Department of Pediatrics, Massachusetts General Hospital, Boston, Massachusetts, USA.

Funding

The Harvard Clinical and Translational Science CenterUL1TR002541 · NCATS · HARVARD MEDICAL SCHOOL · PI NADLER, LEE MARSHALL · 2018 to 2022
$93.0M
ROLE OF DIETARY CONSTITUENTS ON GENE EXPRESSION IN INTESTINAL EPITHELIUMP30DK040561 · NIDDK · MASSACHUSETTS GENERAL HOSPITAL · PI Elizabeth Austen Lawson, Takara Leah Stanley · 1994 to 2026
$31.6M
NCATS NIH HHS UL1 TR002541NIDDK NIH HHS P30 DK040561Nutrition Obesity Research Center at Harvard (NORCH) NIH P30 DK040561The Bernon FamilyThe Harvard Clinical and Translational Science Center 1UL1TR002541-01Williams Syndrome Association
6 · The paper itself

Abstract

backgroundWilliams syndrome (WS) is a rare neurodevelopmental disorder caused by a microdeletion on chromosome 7q. Previous research indicates that adults with WS are prone to having overweight and obesity and also have decreased fat-free mass (FFM) on body composition analysis. To date, no explorations of factors associated with measured resting energy expenditure (mREE) or with the accuracy of predictive resting energy expenditure (pREE) equations have been performed. This study aimed to (1) obtain mREE in adults with WS and examine contributing factors; (2) compare mREE between adults with WS and matched controls; and (3) assess the accuracy of widely used pREE equations.

methodsA convenience sample of 41 adults with WS (mean age = 31.5 ± 10.2 years, mean body mass index [BMI] = 28.6 ± 7.7 kg/m

resultsWithin the WS cohort, mREE was significantly lower in females (1183.1 ± 186.6 kcal/day) than in males (1366.4 ± 217.8 kcal/day) and in individuals with a BMI < 30 kg/m

conclusionsThese findings indicate that (i) the amount of FFM explains most of the mREE reduction observed when comparing females and males with WS, and when comparing WS and matched controls, and (ii) few predictive equations provide clinically useful REE estimates in adults with WS. More accurate predictions tended to originate from equations developed in cohorts with a high prevalence of overweight and obesity, or with older adults. These findings highlight the need for cautious application of pREE equation values in the clinical care of adults with WS and underscore the importance of continued investigation into energy requirements and their determinants.

Indexed as

Energy MetabolismWilliams SyndromeAbsorptiometry, PhotonAdultBody CompositionCalorimetry, IndirectFemaleHumansMaleMiddle AgedYoung Adultagingenergy expenditure predictionsfat‐free massindirect calorimetryobesityWilliams syndrome

Identifiers

PMID41414930
PMCPMC13075980

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