Body Composition and Metabolism in Adults With Molecularly Confirmed Silver-Russell Syndrome

J Clin Endocrinol Metab. 2024 Oct 15;109(11):e2001-e2008. 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.

Keywords: Silver-Russell syndrome; adults; body composition; body mass index; metabolic health.

Publication types

  • Observational Study

MeSH terms

  • Adult
  • Aged
  • Blood Glucose / analysis
  • Blood Glucose / metabolism
  • Blood Pressure / physiology
  • Body Composition*
  • Bone Density
  • Female
  • Humans
  • Male
  • Middle Aged
  • Silver-Russell Syndrome* / genetics
  • Young Adult

Substances

  • Blood Glucose