Body composition and metabolism in adults with molecularly-confirmed Silver-Russell syndrome
Body composition and metabolism in adults with molecularly-confirmed Silver-Russell syndrome
Context: Low birth weight, as seen in Silver-Russell syndrome (SRS), is associated with later cardiometabolic disease. Data on long-term outcomes and adult body composition in SRS are limited. Objective: To evaluate body composition and metabolic health in adults with SRS. Methods: This was an observational study of 25 individuals with molecularly confirmed SRS, aged ≥ 18 years, from research facilities across the UK. Body composition and metabolic health were assessed at a single appointment. Individuals with SRS were compared with unaffected men and women (from the Southampton Women's Survey [SWS]). Fat mass, lean mass, bone mineral density (BMD), blood pressure, lipids, and blood glucose were measured. Results: Twenty-five adults with SRS were included (52% female). The median age was 32.9 years (range, 22.0 to 69.7). Fat percentage was greater in the SRS group than the SWS cohort (44.1% vs 30.3%, P < .001). Fat mass index was similar (9.6 vs 7.8, P = .3). Lean mass percentage (51.8% vs 66.2%, P < .001) and lean mass index (13.5 kg/m2 vs 17.3 kg/m2, P < .001) were lower in the SRS group than the SWS cohort. BMD was lower in the SRS group than the SWS cohort (1.08 vs 1.24, P < .001; all median values). Total cholesterol was ≥ 5 mmol/L in 52.0%. Triglyceride levels were ≥ 1.7 mmol/L in 20.8%. Fasting blood glucose levels were ≥ 6.1 mmol/L in 25.0%. Hypertension was present in 33.3%. Conclusion: Adults with SRS have an unfavorable body composition and predisposition to cardiometabolic disease. These results support the need for a health surveillance strategy to mitigate adverse outcomes.
body composition, metabolic health, body mass index, adults, Silver-Russell syndrome
e2001-e2008
Lokulo-Sodipe, Oluwakemi
d428f857-0e58-4964-b1f0-136af7432805
Inskip, Hazel M.
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Byrne, Christopher D.
1370b997-cead-4229-83a7-53301ed2a43c
Child, Jenny
81b76c9c-eda2-4874-80c6-2d66ae360ca5
Wakeling, Emma L.
88478341-de4a-4b9d-9e35-3a6f392820d6
Mackay, Deborah J.G.
588a653e-9785-4a00-be71-4e547850ee4a
Temple, I. Karen
d63e7c66-9fb0-46c8-855d-ee2607e6c226
Davies, Justin H.
9f18fcad-f488-4c72-ac23-c154995443a9
15 October 2024
Lokulo-Sodipe, Oluwakemi
d428f857-0e58-4964-b1f0-136af7432805
Inskip, Hazel M.
5fb4470a-9379-49b2-a533-9da8e61058b7
Byrne, Christopher D.
1370b997-cead-4229-83a7-53301ed2a43c
Child, Jenny
81b76c9c-eda2-4874-80c6-2d66ae360ca5
Wakeling, Emma L.
88478341-de4a-4b9d-9e35-3a6f392820d6
Mackay, Deborah J.G.
588a653e-9785-4a00-be71-4e547850ee4a
Temple, I. Karen
d63e7c66-9fb0-46c8-855d-ee2607e6c226
Davies, Justin H.
9f18fcad-f488-4c72-ac23-c154995443a9
Lokulo-Sodipe, Oluwakemi, Inskip, Hazel M., Byrne, Christopher D., Child, Jenny, Wakeling, Emma L., Mackay, Deborah J.G., Temple, I. Karen and Davies, Justin H.
(2024)
Body composition and metabolism in adults with molecularly-confirmed Silver-Russell syndrome.
Journal of Clinical Endocrinology & Metabolism, 109 (11), , [dgae074].
(doi:10.1210/clinem/dgae074).
Abstract
Context: Low birth weight, as seen in Silver-Russell syndrome (SRS), is associated with later cardiometabolic disease. Data on long-term outcomes and adult body composition in SRS are limited. Objective: To evaluate body composition and metabolic health in adults with SRS. Methods: This was an observational study of 25 individuals with molecularly confirmed SRS, aged ≥ 18 years, from research facilities across the UK. Body composition and metabolic health were assessed at a single appointment. Individuals with SRS were compared with unaffected men and women (from the Southampton Women's Survey [SWS]). Fat mass, lean mass, bone mineral density (BMD), blood pressure, lipids, and blood glucose were measured. Results: Twenty-five adults with SRS were included (52% female). The median age was 32.9 years (range, 22.0 to 69.7). Fat percentage was greater in the SRS group than the SWS cohort (44.1% vs 30.3%, P < .001). Fat mass index was similar (9.6 vs 7.8, P = .3). Lean mass percentage (51.8% vs 66.2%, P < .001) and lean mass index (13.5 kg/m2 vs 17.3 kg/m2, P < .001) were lower in the SRS group than the SWS cohort. BMD was lower in the SRS group than the SWS cohort (1.08 vs 1.24, P < .001; all median values). Total cholesterol was ≥ 5 mmol/L in 52.0%. Triglyceride levels were ≥ 1.7 mmol/L in 20.8%. Fasting blood glucose levels were ≥ 6.1 mmol/L in 25.0%. Hypertension was present in 33.3%. Conclusion: Adults with SRS have an unfavorable body composition and predisposition to cardiometabolic disease. These results support the need for a health surveillance strategy to mitigate adverse outcomes.
Text
Body composition in SRS revision clean
- Accepted Manuscript
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Accepted/In Press date: 5 February 2024
e-pub ahead of print date: 8 February 2024
Published date: 15 October 2024
Keywords:
body composition, metabolic health, body mass index, adults, Silver-Russell syndrome
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Local EPrints ID: 486835
URI: http://eprints.soton.ac.uk/id/eprint/486835
ISSN: 0021-972X
PURE UUID: 0923bffa-e061-4639-803b-9ed25c2e2392
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Date deposited: 06 Feb 2024 17:52
Last modified: 08 Feb 2025 05:01
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Author:
Oluwakemi Lokulo-Sodipe
Author:
Jenny Child
Author:
Emma L. Wakeling
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