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Early childhood growth in patients with cystic fibrosis
1Department of Pediatrics, Case Western Reserve University, Cleveland, Ohio 44106.
Insights
Children with cystic fibrosis (CF) experience normal growth in the first year, but growth slows later. This pattern suggests cumulative health issues, not a basic metabolic defect, cause growth retardation in CF patients.
Area of Science:
- Pediatric Endocrinology
- Growth and Development Studies
- Biostatistics
Background:
- Cystic Fibrosis (CF) is a genetic disorder affecting multiple organs.
- Growth patterns in children with CF are not fully understood.
- Previous studies suggest potential growth deficits in CF patients.
Purpose of the Study:
- To analyze longitudinal growth data in young children with CF.
- To compare CF growth patterns with normative data.
- To identify factors contributing to growth retardation in CF.
Main Methods:
- Fitting mathematical models (Reed and Jenss) to recumbent length data from 65 CF patients (3 months to 6 years).
- Comparing CF patient data with published data from the Harvard Longitudinal Studies of Child Health and Development.
- Analyzing growth curve parameters and variability between patient subgroups.
Main Results:
- CF girls showed normal first-year growth; CF boys were slightly smaller than normal.
- Both sexes exhibited declining length relative to normal children in later years.
- Growth curve parameters significantly deviated from normal, with higher variability in CF patients.
Conclusions:
- Early childhood growth in CF is characterized by near-normal initial growth followed by an earlier asymptotic approach.
- Growth retardation in CF appears primarily post-infancy, suggesting cumulative effects of chronic illness.
- No significant growth differences were observed between infants diagnosed symptomatically versus those diagnosed via family history.
Abstract:
Longitudinal recumbent length data on 65 cystic fibrosis (CF) patients from 3 months to 6 years of age were used to fit two mathematical models of early childhood growth. The results were compared to published data from the Longitudinal Studies of Child Health and Development at the Harvard School of Public Health. The median values for girls with CF indicate essentially normal growth in the first year of life, whereas CF boys are slightly smaller than normal boys. Both sexes show declines in length relative to normal children in later years. This pattern is reflected in significant deviations from normal values in growth curve parameters for CF patients. While the linear Reed model gave a slightly better fit to the data, the non linear Jenss model allowed easier interpretation of parameters and comparisons with the Boston children. Early childhood growth in cystic fibrosis is characterized by fairly normal exponential growth in the first year of life followed by an earlier approach to a linear asymptote than is found in normal children. Although both the raw data and curve parameters were much more variable in CF patients than in the Boston sample, there were no significant differences in growth between patients diagnosed in infancy because of disease symptoms as opposed to those diagnosed because of family history alone. The appearance of growth retardation in CF primarily after infancy suggests that the cumulative effect of chronic digestive or pulmonary malfunctions, rather than a basic metabolic defect, is responsible for much of the growth retardation seen in these children.