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Bone mineral status in prepubertal children with cystic fibrosis
L A Mortensen1, G M Chan, S C Alder
1Departments of Pediatrics, Intermountain CF Center, Family & Preventive Medicine and Internal Medicine, and Salt Lake VA Medical Center, University of Utah School of Medicine, Salt Lake City, UT 84132, USA.
Insights
Prepubertal children with cystic fibrosis (CF) show no bone mineral deficit. However, lower vitamin D levels in CF patients may indicate future bone mineralization issues.
Area of Science:
- Pediatric Endocrinology
- Bone Metabolism
- Cystic Fibrosis Research
Background:
- Cystic Fibrosis (CF) is a genetic disorder affecting multiple organs, including bones.
- Osteopenia, a precursor to osteoporosis, is a concern in chronic childhood diseases.
- Understanding bone health in prepubertal children with CF is crucial for long-term outcomes.
Purpose of the Study:
- To investigate the presence of osteopenia in prepubertal children with CF.
- To determine if bone formation deficiency or increased bone resorption contributes to potential osteopenia.
- To assess bone mineral density and related biochemical markers in this population.
Main Methods:
- A prospective case-control study involving 11 prepubertal children with CF (ages 8-12) and matched controls.
- Measurements included bone density (radius, ulna, trochanter, femoral neck, lumbar spine), calcium, vitamin D metabolites, and parathyroid hormone.
- Statistical analysis used Wilcoxon matched pairs and Fisher exact tests for group comparisons.
Main Results:
- Children with CF had significantly lower serum 25(OH)vitamin D levels compared to controls (median 22 vs. 39 ng/mL).
- Borderline or low 1,25(OH)(2) vitamin D and elevated intact parathyroid hormone levels were observed more frequently in the CF group.
- Despite biochemical differences, no evidence of bone mineral deficiency was found in prepubertal children with CF.
Conclusions:
- Prepubertal children with CF do not exhibit bone mineral deficits compared to healthy peers.
- Lower vitamin D levels in CF patients are a significant finding.
- These lower vitamin D levels may pose a risk for impaired bone mineralization in later life (adolescence and adulthood).
Objectives:
To determine whether osteopenia is evident in prepubertal children with cystic fibrosis (CF) and, if so, whether it is caused by a deficiency in bone formation or increased bone resorption.
Study Design:
With the use of a prospective case control study design, we investigated 11 prepubertal children with CF between the ages of 8 and 12 years old and a non-CF control group matched by weight and sex. Bone density at the radius, ulnar, trochanter, femoral neck, and lumbar spine, biochemical markers of bone metabolism, calcium, vitamin D metabolites, and intact parathyroid hormone were measured in all subjects. Comparisons between the 2 groups were performed with Wilcoxon matched pairs and Fisher exact tests.
Results:
Intake of total calories, calcium, phosphorus, and vitamin D was significantly greater in the CF group than in the control group. Serum 25(OH)vitamin D levels were significantly lower in the CF group: median 22 ng/mL for the CF group and 39 ng/mL for the control group (P =.02). 1,25(OH)(2) vitamin D levels were borderline or low in 7 subjects in the CF group and 2 members of the control group (P =.08, Fisher exact test). Intact parathyroid hormone levels were higher than the upper limit of normal in 4 subjects of the CF group and 1 member of the control group. Despite these biochemical abnormalities, we found no evidence of bone mineral deficiency in the CF group.
Conclusions:
Prepubertal children with CF do not have bone mineral deficit compared with a weight- and sex-matched control group; however, their lower vitamin D levels may portend problems with bone mineralization during adolescence and adulthood.