Institut de Recerca Sant Joan de Déu, 08950 Barcelona, Spain.
Endocrinology Department, Hospital Sant Joan de Déu, 08950 Barcelona, Spain.
Nutrients. 2020 Apr 11;12(4):1063. doi: 10.3390/nu12041063.
Prader-Willi syndrome is a rare genetic disorder associated with impaired body composition, hyperphagia, and excessive weight gain. Strict dietary restrictions from an early age is crucial to prevent or delay the early onset of obesity, which is the main driver of comorbidities in these patients. The aim of this study was to identify dietary and gut microbiota components closely linked to weight status of these patients. We studied a cohort of children and adolescents with genetic diagnosis of Prader-Willi syndrome ( = 31), in which we determined adiposity by Dual-energy X-ray absorptiometry (DXA) and dietary composition with 4-day food records. Furthermore, we obtained fecal samples to assess microbiota composition by 16S sequencing. Multivariate regression models showed that body mass index standard deviation score (BMI-SDS) and body fat mass were directly associated with saturated fat intake and meat consumption, and inversely associated with fruit consumption. Furthermore, the gut microbiome from normal weight patients was characterized by higher phylogenetic diversity compared to those overweight or obese, with differential abundance of several genera, including , , and . Notably, abundance was inversely correlated to adiposity, lipid and glucose homeostasis parameters, and meat intake. Our results suggest that limiting meat and increasing fruit intake might be beneficial for body weight management in children and adolescents with Prader-Willi syndrome.
普拉德-威利综合征是一种罕见的遗传性疾病,与身体成分受损、食欲过盛和体重过度增加有关。严格的饮食限制从小开始对预防或延迟肥胖的早期发生至关重要,肥胖是这些患者合并症的主要驱动因素。本研究的目的是确定与这些患者体重状况密切相关的饮食和肠道微生物群成分。我们研究了一组经基因诊断患有普拉德-威利综合征的儿童和青少年(n = 31),通过双能 X 射线吸收法(DXA)确定体脂率,并通过 4 天的食物记录确定饮食成分。此外,我们还获得了粪便样本,通过 16S 测序评估微生物群落组成。多变量回归模型显示,体质指数标准差评分(BMI-SDS)和体脂肪量与饱和脂肪摄入量和肉类摄入量呈正相关,与水果摄入量呈负相关。此外,与超重或肥胖患者相比,正常体重患者的肠道微生物组具有更高的系统发育多样性,其中包括 、 和 等几个属的丰度存在差异。值得注意的是, 的丰度与体脂率、脂质和葡萄糖稳态参数以及肉类摄入量呈负相关。我们的研究结果表明,限制肉类摄入和增加水果摄入可能有益于患有普拉德-威利综合征的儿童和青少年的体重管理。