PKD Research Group, GPURE, Department of Development and Regeneration, KU Leuven, Leuven, Belgium.
Department of Pediatrics, ULiège Academic Hospital, Liège, Belgium.
Pediatr Nephrol. 2022 Sep;37(9):1945-1955. doi: 10.1007/s00467-021-05406-z. Epub 2022 Jan 5.
The clinical course of autosomal dominant polycystic kidney disease (ADPKD) starts in childhood. Evidence of the beneficial impact of early nephron-protective strategies and lifestyle modifications on ADPKD prognosis is accumulating. Recent studies have described the association of overweight and obesity with rapid disease progression in adults with ADPKD. Moreover, defective glucose metabolism and metabolic reprogramming have been reported in distinct ADPKD models highlighting these pathways as potential therapeutic targets in ADPKD. Several "metabolic" approaches are currently under evaluation in adults, including ketogenic diet, food restriction, and metformin therapy. No data are available on the impact of these approaches in childhood thus far. Yet, according to World Health Organization (WHO), we are currently facing a childhood obesity crisis with an increased prevalence of overweight/obesity in the pediatric population associated with a cardio-metabolic risk profile. The present review summarizes the knowledge about the role of glucose metabolism in the pathophysiology of ADPKD and underscores the possible harm of overweight and obesity in ADPKD especially in terms of long-term cardiovascular outcomes and renal prognosis.
常染色体显性遗传多囊肾病 (ADPKD) 的临床病程始于儿童期。越来越多的证据表明，早期肾脏保护策略和生活方式改变对 ADPKD 预后有积极影响。最近的研究表明，超重和肥胖与 ADPKD 成人患者的疾病快速进展有关。此外，在不同的 ADPKD 模型中已经报道了葡萄糖代谢缺陷和代谢重编程，这提示这些途径可能是 ADPKD 的潜在治疗靶点。目前正在评估几种“代谢”方法在成人中的应用，包括生酮饮食、饮食限制和二甲双胍治疗。迄今为止，尚无这些方法在儿童期影响的数据。然而，根据世界卫生组织 (WHO) 的说法，我们目前正面临儿童肥胖危机，儿科人群超重/肥胖的流行率增加，与心血管代谢风险特征相关。本综述总结了葡萄糖代谢在 ADPKD 病理生理学中的作用，并强调了超重和肥胖在 ADPKD 中的潜在危害，尤其是在长期心血管结局和肾脏预后方面。
