Perspectives of longitudinal growth in cystic fibrosis from birth to adult age

Abstract

The longitudinal growth in 139 patients with cystic fibrosis (CF) was investigated from birth until the age of 19 years. Already at birth weight and length were reduced (weight: -0.83 +/- 0.13 SDS in girls, -0.44 +/- 0.13 SDS in boys; length: 0.55 +/- 0.13 SDS in girls, -0.39 +/- 0.14 SDS in boys; mean +/- SEM). Both variables showed a further decline until diagnosis was established (weight: -1.57 +/- 0.21 SDS in girls, -1.46 +/- 0.25 SDS in boys; length: -1.15 +/- 0.32 SDS in girls, -1.03 +/- 0.52 SDS in boys; mean +/- SEM). Six to 12 months after diagnosis length improved and reached the 25th percentile in both sexes. Height and weight followed the 25th percentile throughout childhood. Growth velocity was fairly normal during this period. There was a loss in percentiles of both height and weight after the age of 8 years and the pubertal growth spurt was delayed and reduced. However, the 25th percentile was reached again in the adolescent period. At the age of 19 years median height was 161.5 cm in girls and 173 cm in boys, both representing the 25th percentile. Using a sensitive statistical method for analysis of growth data we present CF specific growth curves for height, weight and growth velocity. There was no significant effect of pulmonary colonization with Pseudomonas aeruginosa on growth velocity.

Cystic fibrosis transmembrane conductance regulator with a shortened R domain rescues the intestinal phenotype of CFTR −/− mice

“…2A), similar to observations in CFTR −/− mice (24,28), CFTR −/− pigs (29), and humans with CF (30,31). These results suggest that the growth defect may not be due solely to reduced or absent CFTR in intestinal epithelium, a result consistent with findings in CF pigs and humans with CF (29).…”

Age of menarche in girls with cystic fibrosis.

“…Longitudinal observation of growth and maturation processes in children with CF reveals a delayed and strongly reduced pubertal growth spurt [10,[12][13][14]. Most marked growth delays were observed in patients with F508del/F508del mutation types [12].…”

Annual lung function changes in young patients with chronic lung disease

“…Again, this is unlikely to be due to real improvement of lung disease and may still indicate that ventilatory function is underestimated during the pubertal growth spurt, and overestimated after or near the end of puberty. Thus, the hypothesized artifactual under and overestimation of lung function even plays a role in adolescents with CF, despite the fact that their pubertal growth spurt is delayed and less pronounced [13,14].…”

“…Some of these equations have been used previously in important clinical trials for patients with asthma [10] and in patients with CF [11,12]. The hypothesis was that the choice of reference equations would have a considerable effect on the estimated change of FEV1 and FVC in children and adolescents with chronic lung disease even though their growth spurt is usually delayed and reduced [13,14].…”