Growth, Body Composition, and Lung Function in Prepubertal Children with Cystic Fibrosis Diagnosed by Newborn Screening

Abstract

Background Children with cystic fibrosis (CF) are at risk of altered body composition (BC). Newborn screening (NBS) may lead to improved BC outcomes. We investigated BC and its relationship with lung function in prepubertal children diagnosed with CF by NBS. Secondary aims explored predictors of fat‐free mass (FFM) and lung function. Methods Thirty‐seven screened (non‐meconium ileus) children with CF (20 boys) born 2007–2012 had a dual‐energy x‐ray absorptiometry scan at 5–8 years to determine whole‐body (WB) and appendicular BC. Anthropometry was performed and routine spirometry recorded. Results were converted to z‐scores, height‐adjusted (fat mass index [FMI] and FFM index [FFMI]) and compared with population mean values. Predictors of forced expiratory volume in 1 second (FEV1) were assessed using linear regression. Results Height, body mass index (BMI), and FEV1 were within normal limits, however, weight and BC were significantly low compared with reference data (weight, P = .03; WB FMI, P = .001; WB FFMI, P = .009). Gender differences were detected, with lower appendicular BC in boys and lower weight, BMI, and BC in girls. The association between FEV1 and WB FFMI (r = 0.38; P = .02) was stronger than with BMI (r = 0.29; P = .08). WB FFMI was the only significant predictor of FEV1 in a multivariable model (95% CI, 0.11–0.99; P = .016). Conclusion In this NBS CF population, gender differences in growth and BC were apparent despite preserved lung function. These results support BC assessment in prepubertal children, particularly girls, with an opportunity to direct interventions to optimize FFM.

The First 4 Years – Outcome of Children Identified by Newborn Screening for CF in Germany

“…Failure to thrive in early childhood is seminal, also in healthy children since early childhood growth impacts lung function development and is considered to positively affect respiratory health at older ages 18 19 ; associations which have also been demonstrated for CF 11 20 . Our findings on improvements of nutrition by NBS are substantiated by results from other countries, such as the US 18 19 20 and Australia 6 , Canada 5 , the UK 21 and Brazil 13 in many of which NBS has been implemented for much longer time periods, permitting longer follow-up periods and larger cohorts than our analyses.…”

Trends in Growth and Maturation in Children with Cystic Fibrosis Throughout Nine Decades

“…They found that 5% of their population had a fat-free mass index (FFMI) z-score of < -1 [near the value of hidden depletion previously published in adult CF studies (69)] despite optimal BMI z-scores. FEV 1 was more strongly associated with whole body FFMI than with BMI (27), supporting the importance of body composition on pulmonary function previously observed in both children and adults (36, 70) (Table 2).…”

“…Owen et al. reported on body composition and its association with pulmonary function in 37 pre-pubertal children ( 27 ). They found that 5% of their population had a fat-free mass index (FFMI) z-score of < -1 [near the value of hidden depletion previously published in adult CF studies ( 69 )] despite optimal BMI z-scores.…”

Growth hormone therapy in short-stature patients with kyphoscoliosis: a literature review

“…Short height can be caused by skeletal dysplasia, sexual precocity, malnourishment, and iatrogenic factors such as chemotherapy, radiation, glucocorticoids, and surgery ( Table 1 ). SGA and intrauterine growth retardation are both associated with growth retardation ( 49 , 50 , 51 , 52 , 53 , 54 , 55 , 56 , 57 ). In addition, toward being an indication of a child's physical health, small stature can have a detrimental effect on a child's mental health.…”