关键词: Silver-Russell syndrome adults body composition body mass index metabolic health

来  源:   DOI:10.1210/clinem/dgae074

Abstract:
BACKGROUND: 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. 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)).
METHODS: Clinical research facilities across the UK.
METHODS: 25 individuals with molecularly-confirmed SRS aged ≥18 years.
METHODS: 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.
摘要:
背景:低出生体重,如银-罗素综合征(SRS)所示,与后来的心脏代谢疾病相关。SRS中有关长期结果和成人身体组成的数据有限。
目的:评估患有SRS的成年人的身体成分和代谢健康。
方法:这是一项观察性研究。在单次预约时评估身体成分和代谢健康。将SRS患者与未受影响的男性和女性进行比较(来自南安普敦女性调查(SWS))。
方法:英国各地的临床研究机构。
方法:25名年龄≥18岁的分子确认SRS患者。
方法:脂肪质量,瘦质量,骨矿物质密度(BMD),血压,脂质,并测量血糖。
结果:包括25名患有SRS的成年人(52%为女性)。中位年龄为32.9岁(范围22.0-69.7)。SRS组的脂肪百分比高于SWS组(44.1%vs30.3%,p<0.001)。脂肪质量指数相似(9.6vs7.8,p=0.3)。贫质量百分比(51.8%vs66.2%,SRS组p<0.001)和瘦体重指数(13.5kg/m2vs17.3kg/m2,p<0.001)低于SWS队列。SRS组的BMD低于SWS组(1.08vs1.24,p<0.001)(所有中值)。总胆固醇≥5mmol/L占52.0%。甘油三酯水平≥1.7mmol/L,占20.8%。空腹血糖≥6.1mmol/L的占25.0%。高血压占33.3%。
结论:患有SRS的成年人具有不利的身体组成和心脏代谢疾病的易感性。这些结果支持需要一种健康监测策略来减轻不良后果。
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