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囊性纤维化患儿早期生长轨迹与 6 岁时的肺功能相关。

Early life growth trajectories in cystic fibrosis are associated with lung function at age six.

机构信息

Division of General Pediatrics, Department of Pediatrics, Johns Hopkins University, Baltimore, MD.

Department of Pediatrics, Pulmonary Section, Baylor College of Medicine and Texas Children's Hospital, Houston, Texas.

出版信息

J Cyst Fibros. 2023 May;22(3):395-401. doi: 10.1016/j.jcf.2023.02.008. Epub 2023 Feb 28.

Abstract

BACKGROUND

Higher growth percentiles are associated with more favorable lung function in cystic fibrosis (CF), prompting the creation of CF Foundation (CFF) nutritional guidelines.

OBJECTIVES

To describe early childhood growth trajectories within CF, to determine if growth trajectories are associated with differences in lung function at age six, and to identify factors that differ between trajectory groups.

METHODS

Retrospective cohort study of children diagnosed with CF and born 2000-2011 using the US CFF Patient Registry. Annualized growth parameters prior to age six were included in group-based trajectory modeling to identify unique early life growth trajectories. FEV1 percent predicted (FEV1pp) at age six was compared between trajectory groups using linear regression. Factors associated with group membership were identified using multinomial logistic regression.

RESULTS

6,809 children met inclusion criteria. Six discrete growth trajectories were identified, including three groups that began with growth parameters >50th percentile, termed: "always high", "gradual decliner", "rapid decliner", and three which began with growth parameters <50th percentile, termed: "rapid riser", "gradual riser", "always low". FEV1pp at age six was highest for the Always High trajectory. The Always Low trajectory was nearly 10% lower than the Always High trajectory. Sex, ethnicity, newborn screening and pancreatic function were associated with trajectory class membership.

CONCLUSIONS

Distinct early life growth trajectories were identified within CF. Trajectories that met CFF nutritional guideline recommendations were associated with higher FEV1pp at age six. CF care teams should continue to partner with families to encourage interventions to support optimal growth to improve lung function in CF.

摘要

背景

较高的生长百分位数与囊性纤维化(CF)中更有利的肺功能相关,这促使 CF 基金会(CFF)制定了营养指南。

目的

描述 CF 中幼儿的生长轨迹,确定生长轨迹是否与 6 岁时的肺功能差异相关,并确定轨迹组之间存在差异的因素。

方法

使用美国 CFF 患者登记处,对 2000 年至 2011 年间诊断为 CF 并出生的儿童进行回顾性队列研究。在 6 岁之前,将年度生长参数纳入基于群组的轨迹建模中,以确定独特的早期生命生长轨迹。使用线性回归比较 6 岁时 FEV1 预测百分比(FEV1pp)在轨迹组之间的差异。使用多项逻辑回归确定与组群成员身份相关的因素。

结果

6809 名儿童符合纳入标准。确定了 6 个离散的生长轨迹,包括以生长参数>第 50 百分位数开始的三个组,分别称为:“一直高”、“逐渐下降”、“快速下降”,以及以生长参数<第 50 百分位数开始的三个组,分别称为:“快速上升”、“逐渐上升”、“一直低”。6 岁时的 FEV1pp 最高的是始终高轨迹。始终低轨迹比始终高轨迹低近 10%。性别、种族、新生儿筛查和胰腺功能与轨迹类别成员资格相关。

结论

在 CF 中确定了独特的早期生命生长轨迹。符合 CFF 营养指南建议的轨迹与 6 岁时更高的 FEV1pp 相关。CF 护理团队应继续与家庭合作,鼓励干预措施以支持最佳生长,从而改善 CF 中的肺功能。

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