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Published on: April 11, 2018
Pubertal growth and development in cystic fibrosis: a retrospective review
N Aswani1, C J Taylor, J McGaw
1Academic Unit of Child Health, University of Sheffield Division of Clinical Sciences (South), Sheffield, UK.
Insights
Children with cystic fibrosis (CF) experience delayed pubertal growth spurts, but ultimately reach normal final heights. Disease severity impacts growth timing and magnitude in these CF patients.
Area of Science:
- Pediatrics
- Endocrinology
- Genetics
Background:
- Children with cystic fibrosis (CF) typically exhibit normal growth during the first decade of life.
- Growth in the second decade for CF patients is often suboptimal, potentially indicating pubertal delay.
Purpose of the Study:
- To assess the extent of pubertal delay in children with CF.
- To identify factors influencing the timing and magnitude of the pubertal growth spurt.
- To determine if final height in CF patients significantly differs from the general population.
Main Methods:
- A study of 30 pediatric CF patients (16 males) was conducted.
- Peak height velocity (PHV), final height, and age of achievement were compared to population norms.
- Disease severity was assessed using Shwachman and Chrispin-Norman scores and forced expiratory volume in 1 second (FEV1).
Main Results:
- Peak height velocity (PHV) was significantly delayed in both genders compared to established standards (boys: 14.6 years, girls: 12.6 years).
- Mean PHV was lower in both genders (boys: 7.7 cm/year, girls: 6.4 cm/year).
- Final heights did not significantly differ from population standards, with 52% meeting or exceeding mid-parental height.
Conclusions:
- Cystic fibrosis patients experience suboptimal peak height velocities and delayed pubertal growth spurts.
- Disease severity influences the timing and magnitude of the pubertal growth spurt in CF.
- Despite pubertal delays, CF patients achieve normal final heights.
Aim:
Normal growth patterns are seen throughout the first decade in children with cystic fibrosis (CF). Growth in the second decade is, however, less satisfactory and may reflect pubertal delay. This study was performed to assess the extent of pubertal delay, to examine factors that influence the timing and magnitude of the pubertal growth spurt, and to establish whether the final height for most CF patients differed significantly from the normal population.
Methods:
Thirty subjects (16 male) attending a single centre were studied. Peak height velocity (PHV), final height and ages when achieved were compared with population norms. Outcome data were correlated with disease severity using Shwachman and Chrispin-Norman scores and forced expiratory volume in 1 s.
Results:
PHV was significantly later in both genders in this CF population compared with Tanner and Whitehouse standards: boys 14.6 y (95% confidence interval (95% CI) 12.4-16.8, p < 0.01) and girls 12.6 y (95% CI 10.5-14.7, p < 0.01). Mean PHV was also lower in both genders (boys 7.7 cm y(-1) and girls 6.4 cm y(-1), both p<0.001). However, final heights did not differ significantly from Freeman standards (height standard deviation scores: males--1.2, females--0.1); 52% of final heights equalled or exceeded the mid-parental centile.
Conclusion:
CF patients showed suboptimal PHVs with a later pubertal growth spurt influenced by disease severity, but eventually achieved a normal final height.
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