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.
Abstract

Related Concept Videos

Bone Disorders01:29

Bone Disorders

Aging and its effect on bone remodeling is the most common cause of bone disorders. In young and healthy people, bone deposition and resorption happen at an equal rate to maintain optimal bone health.
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: Pathogenesis01:23

Cystic Fibrosis: Pathogenesis

Cystic fibrosis (CF), an autosomal recessive disorder, significantly affects the function of exocrine glands. This genetically inherited disease is characterized by the production of thick and sticky mucus, which can severely affect various organs and systems in the body.
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 Age01:09

Changes in the Appendicular Skeleton with Age

The upper and lower limb initially develops as a small bulge called a limb bud, which appears on the lateral side of the early embryo. The upper limb bud appears near the end of the fourth week of development, with the lower limb bud appearing shortly after.
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: Management01:24

Cystic Fibrosis: Management

Cystic fibrosis (CF) is an autosomal recessive disorder that predominantly affects individuals of Northern European descent, occurring at a rate of 1 in 3500. It is caused by a genetic mutation in a gene on chromosome 7, most commonly the ΔF508 mutation, that codes for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. This results in thicker mucus secretions and obstruction pathologies in multiple organs, including the lungs and sinuses.
Sinus disease and chronic sinusitis...
Essential Minerals for Bone Health01:31

Essential Minerals for Bone Health

The minerals contained in all of the food we consume are essential for our organ systems. However, certain essential minerals, such as calcium, phosphorus, magnesium, manganese, and fluoride, largely affect 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 Matrix01:18

The Bone Matrix

Bone contains a relatively small number of cells entrenched in a matrix of collagen fibers that provide an adherent surface for inorganic salt crystals. Both components of the matrix, organic and inorganic, contribute to the unusual properties of bone. Without collagen, bones would be brittle and shatter easily. Without mineral crystals, bones would flex and provide little support. This can be observed by an experiment: when the minerals of a bone are dissolved by soaking the bone in acid or...