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Pubertal Height Growth and Adult Height in Cystic Fibrosis After Newborn Screening

Zhumin Zhang1, Mary J Lindstrom2, Philip M Farrell3

  • 1Departments of Nutritional Sciences.

Pediatrics
|June 1, 2016
PubMed

Insights

Newborn screening (NBS) for cystic fibrosis improves long-term growth, leading to better adult height. Early diagnosis via NBS benefits children, particularly those without meconium ileus, by enhancing pubertal growth and final stature.

Area of Science:

  • Pediatric Endocrinology
  • Genetics and Genomics
  • Public Health Screening

Background:

  • Newborn screening (NBS) allows for early diagnosis of conditions like cystic fibrosis (CF).
  • Long-term growth outcomes in CF patients diagnosed via NBS have not been fully elucidated.
  • Assessing the impact of NBS on pubertal growth and adult height is crucial for understanding its benefits.

Purpose of the Study:

  • To compare adolescent peak height velocity (PHV) and adult height in children with CF diagnosed through NBS versus those diagnosed clinically.
  • To determine if early diagnosis via NBS leads to improved linear growth trajectories in CF patients.
  • To identify subgroups of CF patients who may benefit most from NBS regarding growth outcomes.

Main Methods:

  • Analysis of data from 107 children in the Wisconsin Randomized Clinical Trial, born 1985-1994 and followed until 2012.
  • Estimation of PHV using a semiparametric growth curve model and comparison with Tanner references.
  • Comparison of growth parameters and adult height between screened (early diagnosis via NBS) and control (clinical diagnosis) groups.

Main Results:

  • Children with meconium ileus (MI) showed the poorest growth, while those with pancreatic sufficiency had normal growth.
  • In CF patients with pancreatic insufficiency but without MI, screened children exhibited similar PHV but significantly better adult height than controls.
  • Screened boys had comparable PHV, while screened girls had slightly later and lower PHV, but adult height was significantly greater in screened individuals (50th vs. 29th percentile).

Conclusions:

  • Early linear growth benefits observed from NBS in cystic fibrosis are sustained through puberty.
  • Newborn screening for CF leads to improved adult height, primarily due to better prepubertal growth and early intervention.
  • NBS demonstrates a significant long-term positive impact on the growth and final stature of individuals with cystic fibrosis.
Abstract

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