Related Experiment Video
Updated: Jul 17, 2026

Scanning Skeletal Remains for Bone Mineral Density in Forensic Contexts
Published on: January 29, 2018
Low bone mineral density in young children with cystic fibrosis
Isabelle Sermet-Gaudelus1, Jean Claude Souberbielle, Jean Charles Ruiz
1Service de Pédiatrie Générale, 149 rue de Sévres, Paris, France. isabelle.sermet@nck.aphp.fr
Insights
Children with cystic fibrosis (CF) under six years old have low bone mineral density (BMD). This bone disease may start early and be unrelated to nutrition or disease severity in young CF patients.
Area of Science:
- Pediatric Endocrinology
- Bone Metabolism
- Cystic Fibrosis Research
Background:
- Low bone mineral density (BMD) is a common issue in adults with cystic fibrosis (CF).
- Limited data exists regarding BMD in very young CF patients.
- Early assessment of bone health in pediatric CF is crucial.
Purpose of the Study:
- To evaluate bone mineral density (BMD) in children with cystic fibrosis (CF) under the age of six.
- To compare BMD in this young cohort with older pediatric CF groups.
- To identify potential factors influencing BMD in pediatric CF.
Main Methods:
- Bone mineral density (BMD) was measured at the lumbar spine (LS) in 25 children with CF younger than 6 years.
- BMD was also assessed in 53 prepubertal children (6-10 years) and 36 adolescents (11-18 years).
- Nutritional status, body composition, disease severity, corticosteroid use, and nutrient intake were evaluated as potential correlates.
Main Results:
- The mean LS z score in children under six was significantly lower than normal (-0.96).
- LS z scores did not significantly differ between the youngest group and older pediatric CF groups.
- LS z score positively correlated with fat-free mass; 34% of patients with mild disease and normal nutrition had LS z scores below -1.
Conclusions:
- Bone disease in early childhood cystic fibrosis (CF) may originate independently of nutritional status or disease severity.
- Early detection and intervention for bone health in pediatric CF are warranted.
- Further research into the early pathogenesis of CF bone disease is needed.
Rationale:
Low bone mineral density (BMD) is a frequent problem for adult patients with cystic fibrosis (CF). Only limited information is available for young patients.
Objectives:
The aim of this study was to evaluate BMD of children with CF younger than 6 years.
Methods:
BMD was measured at the lumbar spine (LS) after adjustment for height, sex, and pubertal status in 25 children with CF younger than 6 years, 53 prepubertal children aged 6 to 10 years, and 36 adolescents aged 11 to 18 years. Nutritional status, body composition, pulmonary disease severity, corticosteroid usage, dietary calcium, caloric intake, and vitamin D status were evaluated as potential correlates of BMD.
Measurements And Main Results:
The mean LS z score in the youngest group was significantly lower than normal (-0.96; SEM, 0.3). It did not differ significantly from that of children aged 6 to 10 years (-0.91; SEM, 0.2) or adolescents (-1.4; SEM, 0.2). LS z score was positively correlated with fat-free mass in multiple regression analysis. LS z score was less than -1 in 34% of the patients with mild pulmonary disease and normal nutritional status.
Conclusions:
These data suggest that the origin of CF bone disease in early childhood may be independent of nutritional status or disease severity.
Related Concept Videos
Bone Disorders
Bone deposition is also affected by the levels of sex hormones like estrogen and testosterone that promote osteoblast activity and bone matrix synthesis. When the level of these hormones decreases due to aging, it causes a reduction in bone deposition. As a result, bone resorption by osteoclasts...
Cystic Fibrosis: Pathogenesis
CF is primarily caused by a genetic mutation in a chromosome 7 gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The most common gene mutation leading to CF is the ΔF508 mutation, but...
Changes in the Appendicular Skeleton with Age
Initially, the limb buds consist of a core of mesenchyme covered by a layer of ectoderm. The ectoderm at the end of the limb bud thickens to form a narrow crest called the apical ectodermal ridge. This ridge stimulates the underlying...
Cystic Fibrosis: Management
Sinus disease and chronic sinusitis...
Essential Minerals for Bone Health
Calcium and Phosphorus
Calcium is a critical component of bones, especially in the form of calcium phosphate and calcium carbonate. Since the body cannot make calcium, it must be obtained from the diet. However, calcium cannot be absorbed from the small intestine without...
The Bone Matrix
