دورية أكاديمية
Body composition and metabolism in adults with molecularly-confirmed Silver-Russell syndrome.
| العنوان: | Body composition and metabolism in adults with molecularly-confirmed Silver-Russell syndrome. |
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| المؤلفون: | Lokulo-Sodipe O; Department of Human Development and Health, Faculty of Medicine, University of Southampton.; Regional Paediatric Endocrinology Service, University Southampton Hospitals NHS Foundation Trust, Southampton., Inskip HM; Department of Human Development and Health, Faculty of Medicine, University of Southampton.; MRC Epidemiology Unit, Faculty of Medicine, University of Southampton, Southampton.; NIHR Southampton Biomedical Research Centre, University of Southampton and University Hospital Southampton NHS Foundation Trust., Byrne CD; Department of Human Development and Health, Faculty of Medicine, University of Southampton.; NIHR Southampton Biomedical Research Centre, University of Southampton and University Hospital Southampton NHS Foundation Trust., Child J; Child Growth Foundation, c/o Kinnair Associates Limited, Aston House, Redburn Road, Newcastle, NE5 1NB, UK., Wakeling EL; North East Thames Regional Genetic Service, Great Ormond Street Hospital for Children NHS Foundation Trust, Great Ormond Street London WC1N 3JH., Mackay DJG; Department of Human Development and Health, Faculty of Medicine, University of Southampton.; Wessex Regional Genetics Laboratory, Salisbury Health Care Trust, Salisbury., Temple IK; Department of Human Development and Health, Faculty of Medicine, University of Southampton.; The Wessex Clinical Genetics Service, University Hospitals Southampton NHS Foundation Trust, Southampton., Davies JH; Department of Human Development and Health, Faculty of Medicine, University of Southampton.; Regional Paediatric Endocrinology Service, University Southampton Hospitals NHS Foundation Trust, Southampton. |
| المصدر: | The Journal of clinical endocrinology and metabolism [J Clin Endocrinol Metab] 2024 Feb 08. Date of Electronic Publication: 2024 Feb 08. |
| Publication Model: | Ahead of Print |
| نوع المنشور: | Journal Article |
| اللغة: | English |
| بيانات الدورية: | Publisher: Oxford University Press Country of Publication: United States NLM ID: 0375362 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1945-7197 (Electronic) Linking ISSN: 0021972X NLM ISO Abbreviation: J Clin Endocrinol Metab Subsets: MEDLINE |
| أسماء مطبوعة: | Publication: 2017- : New York : Oxford University Press Original Publication: Springfield, Ill. : Charles C. Thomas |
| مستخلص: | 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. Design: This was an observational study. 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)). Setting: Clinical research facilities across the UK. Participants: 25 individuals with molecularly-confirmed SRS aged ≥18 years. Main Outcome Measures: Fat mass, lean mass, bone mineral density (BMD), blood pressure, lipids, and blood glucose were measured. Results: 25 adults with SRS were included (52% female). The median age was 32.9 years (range 22.0-69.7). Fat percentage was greater in the SRS group than the SWS cohort (44.1% vs 30.3%, p<0.001). Fat mass index was similar (9.6 vs 7.8, p=0.3). Lean mass percentage (51.8% vs 66.2%, p<0.001) and lean mass index (13.5 kg/m2 vs 17.3 kg/m2, p<0.001) 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<0.001) (all median values). Total cholesterol was ≥5mmol/L in 52.0%. Triglyceride levels were ≥1.7mmol/L in 20.8%. Fasting blood glucose levels were ≥6.1mmol/L in 25.0%. Hypertension was present in 33.3%. Conclusions: Adults with SRS have an unfavourable body composition and predisposition to cardiometabolic disease. These results support the need for a health surveillance strategy to mitigate adverse outcomes. (© The Author(s) 2024. Published by Oxford University Press on behalf of the Endocrine Society.) |
| فهرسة مساهمة: | Keywords: Silver-Russell syndrome; adults; body composition; body mass index; metabolic health |
| تواريخ الأحداث: | Date Created: 20240208 Latest Revision: 20240208 |
| رمز التحديث: | 20240209 |
| DOI: | 10.1210/clinem/dgae074 |
| PMID: | 38330234 |
| قاعدة البيانات: | MEDLINE |
| تدمد: | 1945-7197 |
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| DOI: | 10.1210/clinem/dgae074 |