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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...
Bone Formation by Intramembranous Ossification01:29

Bone Formation by Intramembranous Ossification

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.
The process begins when mesenchymal cells in the embryonic skeleton gather together and differentiate into osteogenic cells, which then develop into...
Bone Formation by Endochondral Ossification01:24

Bone Formation by Endochondral Ossification

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...
Compact Bone01:27

Compact Bone

Most bones contain compact and spongy osseous tissue, but their distribution and concentration vary based on the bone's overall function.
Compact bone, also called cortical bone, is the denser, stronger of the two types of bone tissue. It is found under the periosteum and in the diaphyses of long bones, where it provides support and protection. The microscopic structural unit of compact bone is called an osteon, or haversian system. Each osteon is composed of concentric rings of calcified...
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...
Osteoclasts in Bone Remodeling01:31

Osteoclasts in Bone Remodeling

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...

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Culture of Murine Embryonic Metatarsals: A Physiological Model of Endochondral Ossification
07:23

Culture of Murine Embryonic Metatarsals: A Physiological Model of Endochondral Ossification

Published on: December 3, 2016

Osteogenesis imperfecta.

S K Bhadada1, R Santosh, A Bhansali

  • 1Department of Endocrinology, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh.

The Journal of the Association of Physicians of India
|September 17, 2009
PubMed
Summary
This summary is machine-generated.

Osteogenesis imperfecta (OI) patients often present late with fractures and deformities. Pamidronate therapy significantly reduced fracture frequency and pain in OI patients.

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Area of Science:

  • Medical Research
  • Genetics
  • Metabolic Disorders

Background:

  • Osteogenesis imperfecta (OI) is a rare genetic bone disorder causing fragile bones and recurrent fractures.
  • Many OI patients remain undiagnosed, especially in developed nations.
  • Bisphosphonate therapy has emerged as a significant advancement in OI management.

Purpose of the Study:

  • To analyze clinical features, radiological findings, and treatment outcomes in OI patients.
  • To evaluate the effectiveness of pamidronate in managing OI symptoms.

Main Methods:

  • A cohort of twenty consecutive OI patients was studied over four years.
  • Clinical data, radiological assessments, and biochemical parameters were analyzed.
  • Treatment outcomes, including fracture frequency and pain, were documented.

Main Results:

  • The study included 20 OI patients (80% male, mean age 20.8 years) with an average of 1-20 fractures per person.
  • Long bone fractures and deformities were common (65%), along with dentinogenesis imperfecta (55%) and blue sclerae (50%).
  • Nine patients receiving pamidronate showed remarkable decreases in fracture frequency and pain.

Conclusions:

  • OI patients frequently present late with long bone fractures, deformities, and blue sclerae.
  • Pamidronate therapy demonstrated significant efficacy in reducing fractures and pain in OI patients.