Most Short Children with Cystic Fibrosis Do Not Catch Up by Adulthood
Margaret P Marks1,2, Sonya L Heltshe3,4, Arthur Baines4
1Department of Pediatrics, University of Alabama, Birmingham, AL 35233, USA.
Insights
Poor linear growth in children with cystic fibrosis (CF) is linked to shorter adult height. Early childhood growth assessment can predict adult height outcomes in CF patients.
Area of Science:
- Pediatrics
- Genetics and Genomics
- Endocrinology
Background:
- Poor linear growth is a common complication in children with cystic fibrosis (CF), often preceding pulmonary decline.
- Growth impairment in CF develops early in life, even with intensive nutritional interventions.
Purpose of the Study:
- To investigate the association between early childhood growth restriction and adult height in individuals with cystic fibrosis.
- To determine if early height for age (HFA) percentiles in CF children predict their adult stature.
Main Methods:
- Utilized the Cystic Fibrosis Foundation (CFF) patient registry to identify 3655 CF adults (ages 18-19) with HFA records from ages 2-4.
- Analyzed the correlation between early childhood HFA and adult height percentiles.
Main Results:
- Only 26% of CF adults were at or above the median HFA, and 25% were below the 10th percentile.
- Children with CF below the 10th percentile HFA at ages 2-4 had a 7.7-fold increased odds of being below the 10th percentile as adults.
- A significant proportion (58%) of CF individuals with HFA between the 10th and 25th percentiles at ages 2-4 remained below the 25th percentile in adulthood.
Conclusions:
- Low early childhood height in cystic fibrosis patients is strongly correlated with reduced adult height.
- Early growth assessment in CF is crucial for predicting adult stature and may inform interventions to improve long-term health outcomes, including pulmonary status.
Abstract:
Poor linear growth is common in children with cystic fibrosis (CF) and predicts pulmonary status and mortality. Growth impairment develops in infancy, prior to pulmonary decline and despite aggressive nutritional measures. We hypothesized that growth restriction during early childhood in CF is associated with reduced adult height. We used the Cystic Fibrosis Foundation (CFF) patient registry to identify CF adults between 2011 and 2015 (ages 18-19 y, n = 3655) and had height for age (HFA) records between ages 2 and 4 y. We found that only 26% CF adults were ≥median HFA and 25% were <10th percentile. Between 2 and 4 years, those with height < 10th percentile had increased odds of being <10th percentile in adulthood compared to children ≥ 10th percentile (OR = 7.7). Of HFA measured between the 10th and 25th percentiles at ages 2-4, 58% were <25th percentile as adults. Only 13% between the 10th and 25th percentile HFA at age 2-4 years were >50th percentile as adults. Maximum height between ages 2 and 4 highly correlated with adult height. These results demonstrate that low early childhood CF height correlates with height in adulthood. Since linear growth correlates with lung growth, identifying both risk factors and interventions for growth failure (nutritional support, confounders of clinical care, and potential endocrine involvement) could lead to improved overall health.
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