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Comparative Study
. 2010 Mar;9(2):135-42.
doi: 10.1016/j.jcf.2010.01.003.

Incorporating genetic potential when evaluating stature in children with cystic fibrosis

Zhumin Zhang  1 , Suzanne M Shoff, Huichuan J Lai
Affiliations
Comparative Study

Incorporating genetic potential when evaluating stature in children with cystic fibrosis

Zhumin Zhang et al. J Cyst Fibros. 2010 Mar.

Abstract

Objective: The 2002 Cystic Fibrosis Foundation (CFF) practice guidelines recommend adjusting for genetic potential when evaluating height status in children with CF. However, there is paucity of data to support this recommendation. We compared three methods of classifying short stature: unadjusted height percentile <10th, Himes adjusted height percentile <10th, and unadjusted height below the CFF target height lower bound.

Patients and methods: Data from 3306 children with parental heights documented in the 1986-2005 CFF Patient Registry were analyzed.

Results: Mean height percentile of CF children (33rd) was lower than their parents' (mothers' 53rd, fathers' 57th), and 80% of CF children were below the average of their parental height percentiles. In children with short parents, Himes adjusted height percentile was significantly higher than unadjusted height percentile (27th vs. 8th), whereas the opposite was found in children with tall parents (Himes adjusted at 18th vs. unadjusted at 49th). Consequently, the prevalence of short stature decreased from 52% to 22% in children with short parents and increased from 8% to 34% in children with tall parents after Himes adjustment. In children with discrepant classification on short stature before and after Himes adjustment, percent predicted forced expiratory volume in one second was negatively associated with unadjusted height percentile but positively associated with Himes adjusted height percentile. In children with short parents, the CFF method underestimated the prevalence of short stature (9%) compared to the Himes method (22%).

Conclusion: Without adjustment of genetic potential, the prevalence of short stature is underestimated and the association between height and lung function is biased.

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Conflict of interest statement

ZZ contributed to the design of the study, the analysis of data, the interpretation of results, and writing of the manuscript. SMS contributed to the interpretation of results and writing of the manuscript. HJL contributed to the design of the study, the interpretation of results, and the writing of the manuscript. The authors had no conflicts of interest.

Figures

Figure 1
Figure 1
An example illustrating unadjusted height, Himes adjusted height, and the CFF target height and lower bound for a 15 year-old boy with unadjusted height of 157 cm (= 5th percentile) and mid-parental height of 160 cm.
Figure 2
Figure 2
Comparison of unadjusted height percentiles and Himes adjusted height percentiles in children with CF. Short parents: average parental height percentiles ≤ 25th; average parents: average parental height percentiles between 26th and 74th; tall parents: average parental height percentiles ≥ 75th.
Figure 3
Figure 3
Pair-wise comparisons of the discrepancy among four methods of determining the prevalence of short stature in CF children. Short parents: average parental height percentiles ≤ 25th; average parents: average parental height percentiles between 26th and 74th; tall parents: average parental height percentiles ≥ 75th.
Figure 4
Figure 4
Comparison between unadjusted height percentile (panel A) and Himes adjusted height percentile (panel B) on their associations to lung function parameter percent predicted forced expiratory volume in one second (%FEV1) in CF children with discrepant classification on short stature at age 15 (n = 217).
See this image and copyright information in PMC

References

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