Bone Health Index (BHI): a Fracture Risk Assessment tool in Children with Osteogenesis Imperfecta

Ruggero Lanzafame1, Alistair D Calder2, Belinda Crowe3

  • 1Paediatric Endocrinology Department, Great Ormond Street Hospital for Children NHS Foundation Trust, London WC1N 3JH, UK.

Insights

The Bone Health Index (BHI) shows promise for assessing fracture risk in children with Osteogenesis Imperfecta (OI). This method is more effective than bone mineral density (BMD) scans, especially for infants.

Area of Science:

  • Pediatric Orthopedics
  • Medical Imaging
  • Skeletal Dysplasias

Background:

  • Fracture risk assessment in children with Osteogenesis Imperfecta (OI) is challenging due to limitations of current tools like bone mineral density (BMD).
  • BMD is often unavailable or technically difficult for children under 5 years old.
  • The Bone Health Index (BHI), measuring metacarpal bone characteristics, is proposed as a potential alternative for fracture risk evaluation.

Purpose of the Study:

  • To evaluate the efficacy of the Bone Health Index (BHI) in assessing fracture risk in pediatric patients with Osteogenesis Imperfecta (OI).
  • To compare the performance of BHI with traditional BMD measurements in predicting fractures in young OI patients.

Main Methods:

  • A cohort of 277 children with classical OI was studied, analyzing their Bone Health Index (BHI) using BoneXpert® software.
  • Fracture history over two years and lateral spine X-rays were collected.
  • Lumbar BMD measurements were obtained on the same day as BHI analysis where available for comparison.

Main Results:

  • A 1-SD decrease in BHI SDS was significantly associated with a 5.3-fold increased odds of fracture within two years (p < 0.001).
  • BHI SDS demonstrated superior discriminatory performance (AUC 0.84) compared to DXA BMD Z-score (AUC 0.60) and BMAD Z-score (AUC 0.51).
  • BMD and BMAD Z-scores were not found to be independent predictors of fracture in multivariable analysis.

Conclusions:

  • Lower BHI SDS values correlate with higher fracture risk in children with classical OI.
  • BHI shows stronger predictive performance than DXA-derived measures in the studied subgroup.
  • BHI is a potentially valuable tool for fracture risk stratification in young OI patients, particularly when DXA is not feasible, such as in infants.
Abstract

Related Concept Videos

Fractures: Bone Repair01:27

Fractures: Bone Repair

Treatment for a fracture is based on the type of break, the bone affected, and the patient's age.
Minor fractures with no bone displacement are treated by immobilizing the fractured bone using a cast or splint. However, in the case of fractures with displaced bones, the broken bones are repositioned before immobilization to ensure successful healing without deformation and loss of function. The realignment of fractured bone ends is performed through a process called reduction. If the procedure...
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...
Bone Remodeling and Repair01:31

Bone Remodeling and Repair

Osteoclasts are cells responsible for bone resorption and remodeling. They originate from hematopoietic progenitor cells present in the bone marrow. Numerous progenitor cells fuse to form multinucleated cells, each with 10-20 nuclei. A single osteoclast has a diameter of 150 to 200 µM. These cells have ruffled borders that break down the underlying bone tissue and release minerals such as calcium into the blood in bone resorption. Osteoclasts cling to bones with their ruffled edges during bone...
Role of Vitamins in Maintaining Bone Health01:25

Role of Vitamins in Maintaining Bone Health

The growth and maintenance of bone are regulated by a combination of nutritional factors, including vitamins, such as vitamin A, B12, C, D, and K.
Vitamin A
Vitamin A is involved in the process of bone remodeling. Retinoic acid, the active metabolite of Vitamin A, has nuclear receptors in osteoblasts and osteoclasts, which are involved in bone remodeling.
Vitamin B12
Vitamin B12 acts as a cofactor during the formation of osteoblast-related proteins, such as osteocalcin. Vitamin B12 plays a role...
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...