Related Experiment Video

Updated: Aug 18, 2026

Rectal Organoid Morphology Analysis (ROMA): A Diagnostic Assay in Cystic Fibrosis
07:56

Rectal Organoid Morphology Analysis (ROMA): A Diagnostic Assay in Cystic Fibrosis

Published on: June 10, 2022

Bone status in cystic fibrosis

M Sood1, G Hambleton, M Super

  • 1The Cystic Fibrosis Unit, Royal Manchester Children's Hospital, Hospital Road, Manchester M27 4HA, UK.

Archives of Disease in Childhood
|May 23, 2001
PubMed

Insights

Children with cystic fibrosis (CF) show normal bone mineral density overall, but reduced bone mineral apparent density (BMAD) at the lumbar spine, indicating potential cortical thinning. This suggests bone health monitoring is crucial even in mild CF cases.

Area of Science:

  • Pediatric Endocrinology
  • Bone Metabolism
  • Cystic Fibrosis Research

Background:

  • Cystic Fibrosis (CF) is a genetic disorder affecting multiple organs, including bones.
  • Bone mineral status in children with CF requires further investigation, particularly in those with milder disease phenotypes.

Purpose of the Study:

  • To assess the bone mineral status in children diagnosed with cystic fibrosis.
  • To compare bone parameters between CF patients and age- and sex-matched healthy controls.

Main Methods:

  • Dual-energy X-ray absorptiometry (DXA) was used to measure bone mineral content (BMC), bone area (BA), and areal bone mineral density (BMD).
  • Quantitative computed tomography (QCT) assessed vertebral trabecular bone mineral density (vTBMD), and quantitative ultrasound (QUS) measured calcaneal broadband ultrasound attenuation (BUA).
  • Bone mineral apparent density (BMAD) was calculated to assess volumetric density, and disease severity was evaluated using the Shwachman-Kulczycki (SK) score.

Main Results:

  • No significant differences were observed in BUA, whole body/regional BA, adjusted BMC, or areal BMD between CF children and controls.
  • CF patients exhibited reduced lumbar spine bone mineral apparent density (BMAD) compared to controls.
  • Vertebral trabecular bone mineral density (vTBMD) standard deviation scores were significantly higher in CF patients, despite a relatively mild disease severity (median SK score 81).

Conclusions:

  • The findings suggest potential reductions in vertebral cortical thickness or density in CF subjects, indicated by normal vertebral BMC but decreased BMAD.
  • Overall bone mineral status in children with mild CF is comparable to size-matched controls.
  • These results highlight the importance of evaluating volumetric bone density measures in pediatric CF populations.
Abstract

More Related Videos

Cystic Fibrosis Aggregate Biofilm Model to Study Infection-relevant Gene Expression08:58

Cystic Fibrosis Aggregate Biofilm Model to Study Infection-relevant Gene Expression

Published on: April 18, 2025

Implementation of Non-invasive Point of Care Transient Elastography for Evaluation of Liver Disease in Pediatric Populations with Cystic Fibrosis05:56

Implementation of Non-invasive Point of Care Transient Elastography for Evaluation of Liver Disease in Pediatric Populations with Cystic Fibrosis

Published on: August 29, 2025

Related Concept Videos

The Functions of the Skeletal System01:22

The Functions of the Skeletal System

The most apparent functions of the skeletal system are support, protection, and movement. However, bone tissue also performs several other critical metabolic functions. For one, the bone matrix acts as a reservoir for a number of minerals important to the functioning of the body, especially calcium and phosphorus. These minerals, present in the bone tissue, can be released back into the bloodstream when required. Calcium ions, for example, are essential for muscle contractions and controlling...
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...
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...