2000年至2022年期间,丹麦患有囊性纤维化症的儿童的早期生长改善
Karlen Bader-Larsen1, Daniel Faurholt-Jepsen2, Thomas Bryrup1
1Cystic Fibrosis Centre Copenhagen, Department of Infectious Diseases, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark.
概括
囊性纤维化 (CF) 护理的进步改善了儿童早期的生长,最近的队列显示出更早的追赶增长. 然而,营养不良的风险仍然存在,需要为患有CF的儿童提供持续的营养支持.
科学领域:
- 儿科 儿科 儿科
- 遗传学和遗传性 遗传学和遗传性
- 公共卫生 公共卫生
背景情况:
- 20多年来,囊性纤维化 (CF) 治疗的进展,包括自2016年以来的新生儿查,可能改善了受影响儿童的生长.
- 这项研究研究了2000年至2022年间出生的丹麦CF儿童的生长变化.
研究的目的:
- 评估不同出生队伍中患有囊性纤维化 (CF) 儿童早期生长模式的变化.
- 评估当代CF护理进步对儿童成长轨迹的影响.
主要方法:
- 使用0-5岁儿童丹麦CF队列数据的观察队列研究.
- 分为四个出生队伍 (2000-2022) 的分层,并使用世卫组织曲线计算体重相对年龄 (WAZ),长度/身高相对年龄 (LAZ/HAZ) 和BMI相对年龄 (BMZ) 的z-score.
- 应用立方线混合效应模型来分析出生队列之间的5年增长趋势.
主要成果:
- 分析包括255名儿童,揭示了随着时间的推移在出生队列中改善了追赶增长.
- 2016-2022年出生队列显示,WAZ,LAZ/HAZ和BMZ的生长参考曲线值最早实现.
- 2016-2022年群体观察到体重不足和发育迟缓的减少,以及超重,低BMZ和高BMZ观察的增加.
结论:
- 改善的儿科CF护理提高了生长结果,最新的出生队列接近最佳生长潜力.
- 尽管有进展,但CF儿童的低年龄BMI (BMZ) 风险仍然存在.
- 在整个幼儿时期进行个性化营养干预对于预防和管理这一群体的营养不良至关重要.
相关概念视频
Cystic Fibrosis: Management
155
Cystic fibrosis (CF) is an autosomal recessive disorder that predominantly affects individuals of Northern European descent, occurring at a rate of 1 in 3500. It is caused by a genetic mutation in a gene on chromosome 7, most commonly the ΔF508 mutation, that codes for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. This results in thicker mucus secretions and obstruction pathologies in multiple organs, including the lungs and sinuses.
Sinus disease and chronic...
Sinus disease and chronic...
155
Cystic Fibrosis: Pathogenesis
218
Cystic fibrosis (CF), an autosomal recessive disorder, significantly affects the function of exocrine glands. This genetically inherited disease is characterized by the production of thick and sticky mucus, which can severely affect various organs and systems in the body.
CF is primarily caused by a genetic mutation in a chromosome 7 gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The most common gene mutation leading to CF is the ΔF508 mutation,...
CF is primarily caused by a genetic mutation in a chromosome 7 gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The most common gene mutation leading to CF is the ΔF508 mutation,...
218


