WS12.01Final height in cystic fibrosis: what are the predictive factors?

Background

Growth and nutrition are indexes of health status in people with cystic Fibrosis (pwCF). In this study we evaluated final height of pwCF and compared it to known final adult height in the general population in Israel, and to identify factors potentially affecting final height.

Methods

We retrieved data from files of 147 pwCF that were followed at Safra Children's Hospital since its establishment until present day and reached final height. Final height was compared to norms of the general healthy population in Israel. Potential factor that may affect final height were assessed including CFTR mutation severity, gender, weight, date of birth, glucocorticoid treatment, pancreatic insufficiency (PI), diabetes, renal and liver diseases, number of hospitalizations, microbial colonization, and FEV1 %predicted. Height data during growth was retrieved to describe growth patterns.

Results

Adult final height in CF is significantly lower in comparison to the healthy population in Israel (p<0.001). When analyzed by gender, the difference in final height was significant in males with CF (172.4±7.6 cm) compared to healthy males in Israel (174.8±6.6 cm) (p<0.001) but not lower in females with CF (161.2±6.5 cm vs 162.9±5.9 cm) (p=0.078). Contributing factors to lower final height in CF are Male sex (p=0.045) and minimal function mutation (p=0.009).

When examining growth during specific periods, we observed changes compatible with precocious puberty. People with CF had a lower height Z-score to begin with, however with time there was a gradual decrease in height Z-score, and finally, from adolescence to final height there was an increase, reaching final height Z-score similar to values measured during childhood.

Conclusions

People with CF had lower final height in comparison to the general healthy population in Israel. Predicting factors were male sex and minimal function CFTR mutations. Growth pattern in both sex were compatible with precious puberty.