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Growth failure in cystic fibrosis
S A McNaughton1, D A Stormont, R W Shepherd
1Department of Paediatrics and Child Health, University of Queensland, Australia. sm@nutprog.herston.uq.edu.au
Insights
Children with cystic fibrosis (CF) showed worsening nutritional status and growth failure in 1996 compared to 1986. This trend is linked to increased survival rates in CF patients with more severe symptoms.
Area of Science:
- Pediatrics
- Pulmonology
- Nutritional Science
Background:
- Cystic Fibrosis (CF) is a genetic disorder affecting multiple organs, notably the lungs and digestive system.
- Nutritional status and growth are critical indicators of health and prognosis in children with CF.
- Previous studies have indicated challenges in maintaining adequate nutrition and growth in pediatric CF populations.
Purpose of the Study:
- To compare the nutritional status of pediatric cystic fibrosis patients between 1986 and 1996.
- To analyze trends in height, weight, and pulmonary function over a decade in this cohort.
- To identify potential factors influencing nutritional outcomes in CF patients.
Main Methods:
- Retrospective analysis of outpatient data from a cystic fibrosis clinic (1986: n=97; 1996: n=227).
- Evaluation of height, weight (z-scores: HAZ, WAZ, WHZ), and pulmonary function (FEV1, FVC, FEF) data.
- Statistical comparison of anthropometric and pulmonary function parameters between the two study periods.
Main Results:
- In 1996, males exhibited significantly poorer weight-for-age (WAZ) and weight-for-height (WHZ) scores compared to 1986.
- Females in 1996 were shorter and lighter, with reduced FEV1 and FVC compared to 1986.
- Despite apparent declines, no significant differences in nutritional status (z-scores) were observed when patients were matched for gender and FEV1 severity.
Conclusions:
- The observed worsening nutritional status in 1996 CF patients is likely an artifact of improved survival.
- Increased survival allows patients with more severe CF symptoms to persist, contributing to poorer nutritional indicators.
- Growth failure remains a significant and potentially worsening challenge in the management of children with cystic fibrosis.
Objective:
The aim of this study was to describe and compare the nutritional status of children aged 0-18 years attending the cystic fibrosis (CF) clinic at the Royal Children's Hospital, Brisbane, Australia, as outpatients in 1986 and 1996.
Methodology:
The heights, weights and pulmonary function of children attending the CF clinic as outpatients in 1986 (n = 97) and 1996 (n = 227) were retrospectively analysed using a computerized database maintained by the CF clinic. The heights and weights were analysed in terms of z scores for height for age (HAZ), weight for age (WAZ) and weight for height (WHZ). Pulmonary function data is not available for all children. Forced expiratory volume in one second (FEV1), forced vital capacity (FVC) and forced mid expiratory flows (FEF) were expressed as a percentage of predicted and are presented here.
Results:
The 1986 sample consisted of 41 males (age range 0.18-14.59 years, mean age 6.52 (4.33)) and 56 females (age range 0.15-14.97 years, mean age 7.75 (3.70)). The 1996 sample consisted of 111 males (age range 0.09-17.97 years, mean age 8.80 (5.49)) and 114 females (age range 0.12-17.98 years, mean age 8.49 (5.26)). In 1986, males were shorter than females (P = 0.0096) and females had a lower mean FVC than males (P = 0.0438). In 1996, males were shorter, lighter and more wasted than females (P = 0.0357, P = 0.0034 and P = 0.0273, respectively) and females had a lower mean FEV1 and mean FVC than males (P = 0.0176 and P = 0.0079, respectively). Males in 1996 were lighter and more wasted than males in 1986 (P = 0.0023 and P = 0.0139, respectively) and had a lower mean FEV1, mean FVC and mean FEF (P < 0.0001, P = 0.0012 and P = 0.0069, respectively). Females in 1996 were shorter and lighter than females in 1986 (P = 0.0273 and P = 0.0405, respectively) and had a lower mean FEV1, mean FVC and mean FEF (P < 0.0001, P < 0.0001 and P < 0.0001, respectively). When subjects were classified according to FEV1 (FEV1 > or = 75% or FEV1 < 75%), there were no significant differences in z scores between the 1986 group and 1996 group. Similarly, when the 1986 group were matched for gender and FEV1 with the 1996 group, there were no significant differences in z scores for males or females.
Conclusions:
It is suggested that the apparent worsening of nutritional status among the 1996 group of CF patients is in fact due to an effect of increased survival of patients with more severe clinical symptoms. The findings from this study highlight the continuing, and in fact, worsening problem of growth failure in children with CF.
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