Elsevier

The Journal of Pediatrics

Volume 163, Issue 2, August 2013, Pages 376-382.e1
The Journal of Pediatrics

Original Article
Pubertal Height Velocity and Associations with Prepubertal and Adult Heights in Cystic Fibrosis

https://doi.org/10.1016/j.jpeds.2013.02.026Get rights and content

Objectives

To test the hypothesis that pubertal peak height velocity (PHV) in cystic fibrosis (CF) has improved and is influenced by prepubertal growth and genetic potential.

Study design

PHV from 1862 children born in 1984-87 and documented in the 1986-2008 US CF Foundation Registry was determined by statistical modeling and classified into normal, delayed (2-SD > average age), attenuated (magnitude <5th percentile), or both delayed and attenuated (D&A). Genetic potential for height was estimated by parental stature.

Results

PHV averaged 8.4 cm/year at age 14.0 years in boys and 7.0 cm/year at age 12.1 years in girls, ∼6-month delay and ∼15% reduction compared with healthy children. PHV was normal in 60%, delayed in 9%, attenuated in 21%, and D&A in 5%. Patients with delayed PHV reached similar adult height percentile (boys: 34th, girls: 46th) to those with normal PHV (boys: 33rd, girls: 34th); both were significantly taller than the attenuated (boys: 11th, girls: 19th) and D&A PHV subgroups (boys: 8th, girls: 14th). Pancreatic-sufficient patients had taller prepubertal and adult heights but similar PHV compared with pancreatic-insufficient or meconium ileus patients. Adjusting for genetic potential reduced adult height percentiles more in boys (from 25th to 16th) than girls (from 28th to 24th). Height at age 7 years, PHV age and magnitude, and parental stature significantly predicted adult height.

Conclusions

Pubertal PHV has improved in children with CF born after mid-1980s compared with older cohorts but remains below normal. Suboptimal prepubertal and pubertal growth led to adult height below genetic potential in CF.

Section snippets

Methods

The CFF Registry documents the diagnosis and follow-up evaluations of patients with CF seen at accredited centers in the US.23 Height data were reported annually before 1993 and quarterly after 1994. Therefore, patients born in 1984-1987 would have quarterly height data from 1994 (at age 7-10 years) and reached adulthood by 2008, the most recent year of CFF Registry data available for this study. Of the 4198 born in 1984-1987, 309 died, 951 were lost to follow-up before age 18 years, and 1076

HV Curve and Pubertal PHV Pattern

Figure 1 shows the height and HV curve of a child with CF fitted by the shape invariant model.24 This method yields more accurate PHV magnitude because it uses a smooth estimate from the fitted curve rather than that calculated from 2 adjacent heights (point “•”, the largest Y-axis value of all open circles).

Figure 2 shows the overall common shape of HV curves. Compared with healthy children,25 PHV in children with CF occurred later (0.5 years later in boys and 0.6 years later in girls) with

Discussion

With the use of a novel semi-parametric growth curve model,24 which can accommodate missing data, irregular measurement intervals, and patterns of growth that may not be well fitted by parametric models29, 30 used in previous studies,5, 10 our study generated new and important findings on pubertal growth in the CF population. First, we demonstrated that children with CF born in the mid-1980s still experienced D&A pubertal PHV compared with healthy children, but the delay and attenuation were

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    Supported by The National Institutes of Health (R01DK072126). The authors declare no conflicts of interest.

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