Clinical burden of hyperphagia, obesity and health outcomes in paediatric individuals with Bardet-Biedl syndrome: A CRIBBS data analysis.

IF 2.7 3区医学 Q1 PEDIATRICS

Pediatric Obesity Pub Date : 2025-06-02 DOI:10.1111/ijpo.70026

Jeremy Pomeroy, Uzoma Okorie, Jesse G Richardson, Ariane Faucher, Raluca Ionescu-Ittu, Francis Vekeman, Caroline Huber, Robert M Haws

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引用次数: 0

Abstract

Background: To date, research on the natural history of hyperphagia, weight gain and health outcomes in individuals with Bardet-Biedl syndrome (BBS) has been limited. Understanding these characteristics is important for disease burden management.

Objectives: The international Clinical Registry Investigating BBS (CRIBBS) is a registry of individuals with BBS to investigate the severity of hyperphagia, the natural history of weight gain and the frequency of obesity-associated health outcomes in paediatric individuals with BBS.

Methods: Paediatric participants (<18 years of age) enrolled in CRIBBS were evaluated to assess weight and weight loss interventions, hyperphagia and the frequency of cardiac, endocrine/diabetic and renal health outcomes.

Results: Of 331 children, 81.0% had obesity at baseline and 22.7% reported use of weight loss diet or medication at baseline or later. Among participants with ≥2 weight measurements ≥2 years apart (n = 186), 17.2% had a higher weight classification from the first to the last assessment. Increasing hyperphagia severity was associated with increasing obesity. The prevalence of cardiac, diabetic, endocrine and renal health outcomes increased with obesity class.

Conclusion: Hyperphagia and obesity are highly prevalent in children with BBS. Many children with BBS also experience adverse health outcomes. Timely diagnosis and targeted treatment of hyperphagia and obesity are needed.

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儿童Bardet-Biedl综合征患者的临床食多、肥胖负担和健康结局：CRIBBS数据分析

背景：迄今为止，关于Bardet-Biedl综合征（BBS）患者的暴饮暴食、体重增加和健康结局的自然史研究还很有限。了解这些特征对疾病负担管理很重要。目的：国际临床登记调查BBS （CRIBBS）是一个BBS患者的登记，以调查小儿BBS患者贪食的严重程度、体重增加的自然史和肥胖相关健康结果的频率。方法：儿童参与者(结果：在331名儿童中，81.0%在基线时患有肥胖症，22.7%报告在基线或之后使用减肥饮食或药物。在两次体重测量间隔≥2年的参与者中（n = 186）， 17.2%的参与者从第一次评估到最后一次评估的体重分类较高。贪食严重程度的增加与肥胖的增加有关。心脏、糖尿病、内分泌和肾脏健康结果的患病率随着肥胖程度的增加而增加。结论：嗜食和肥胖在BBS患儿中非常普遍。许多患有BBS的儿童也会经历不良的健康结果。需要及时诊断和有针对性地治疗贪食和肥胖。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Pediatric Obesity PEDIATRICS-

CiteScore

7.30

自引率

5.30%

发文量

117

审稿时长

6-12 weeks

期刊介绍： Pediatric Obesity is a peer-reviewed, monthly journal devoted to research into obesity during childhood and adolescence. The topic is currently at the centre of intense interest in the scientific community, and is of increasing concern to health policy-makers and the public at large. Pediatric Obesity has established itself as the leading journal for high quality papers in this field, including, but not limited to, the following: Genetic, molecular, biochemical and physiological aspects of obesity – basic, applied and clinical studies relating to mechanisms of the development of obesity throughout the life course and the consequent effects of obesity on health outcomes Metabolic consequences of child and adolescent obesity Epidemiological and population-based studies of child and adolescent overweight and obesity Measurement and diagnostic issues in assessing child and adolescent adiposity, physical activity and nutrition Clinical management of children and adolescents with obesity including studies of treatment and prevention Co-morbidities linked to child and adolescent obesity – mechanisms, assessment, and treatment Life-cycle factors eg familial, intrauterine and developmental aspects of child and adolescent obesity Nutrition security and the "double burden" of obesity and malnutrition Health promotion strategies around the issues of obesity, nutrition and physical activity in children and adolescents Community and public health measures to prevent overweight and obesity in children and adolescents.