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Related Concept Videos

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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.
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Treatment for a fracture is based on the type of break, the bone affected, and the patient's age.
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Bone Formation by Endochondral Ossification01:24

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Bone formation, or ossification, begins around the sixth to seventh week of embryonic development. Most bones develop from a cartilaginous template through the process of endochondral ossification. Cartilage formation begins when clusters of mesenchymal cells differentiate into chondrocytes. These chondrocytes proliferate rapidly and secrete an extracellular matrix that becomes encased in a membrane called the perichondrium. The resulting cartilage model provides a template that resembles the...
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Osteoclasts in Bone Remodeling01:31

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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...
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Bone Formation by Intramembranous Ossification01:29

Bone Formation by Intramembranous Ossification

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Intramembranous ossification is one of the two processes involved in the development of bones within an embryo. The flat bones of the face, most of the cranial bones, and the clavicles are formed via this process. During intramembranous ossification, the bones develop directly from sheets of undifferentiated mesenchymal connective tissue.
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Compact Bone01:27

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Most bones contain compact and spongy osseous tissue, but their distribution and concentration vary based on the bone's overall function.
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Creating Rigidly Stabilized Fractures for Assessing Intramembranous Ossification, Distraction Osteogenesis, or Healing of Critical Sized Defects
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Osteogenesis imperfecta - A clinical update.

Symeon Tournis1, Anastasia D Dede2

  • 1Laboratory for Research of the Musculoskeletal System 'Th. Garofalidis', KAT Hospital, University of Athens, Athens, Greece.

Metabolism: Clinical and Experimental
|June 20, 2017
PubMed
Summary
This summary is machine-generated.

Osteogenesis imperfecta (OI), a genetic bone fragility disorder, often stems from type 1 collagen defects. Current treatments like bisphosphonates improve bone density, but new therapies are emerging for this complex condition.

Keywords:
Atypical fractureBlue scleraeBone fragilityCollagenZebra sign

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Area of Science:

  • Genetics and Molecular Biology
  • Orthopedics
  • Pharmacology

Background:

  • Osteogenesis imperfecta (OI) is a common inherited bone fragility disorder.
  • Most cases result from COL1A1/COL1A2 gene mutations affecting type 1 collagen.
  • Other protein defects and genetic advances are refining OI classification.

Purpose of the Study:

  • To review the current understanding of Osteogenesis Imperfecta.
  • To discuss existing and emerging therapeutic strategies for OI.
  • To highlight the need for further research in OI treatment.

Main Methods:

  • Literature review of genetic defects in Osteogenesis Imperfecta.
  • Analysis of current therapeutic interventions, including bisphosphonates.
  • Exploration of novel and investigational treatments for OI.

Main Results:

  • Bisphosphonates improve bone mineral density and vertebral reshaping in children.
  • Bisphosphonates have limited effects on long bone fractures and adult bone density.
  • Promising results are seen with denosumab, teriparatide, and other novel agents.

Conclusions:

  • Osteogenesis Imperfecta is a heterogeneous genetic disorder primarily affecting type 1 collagen.
  • Intravenous bisphosphonates are a cornerstone treatment, particularly in pediatric OI.
  • Further research is essential to optimize therapeutic approaches for this complex bone fragility condition.