Related Experiment Video
Updated: May 12, 2026
05:25
Using Inducible Osteoblastic Lineage-Specific Stat3 Knockout Mice to Study Alveolar Bone Remodeling During Orthodontic Tooth Movement
Published on: July 21, 2023
Osteogenesis imperfecta/lobstein syndrome associated with dentinogenesis imperfecta
Naresh Lingaraju1, P J Nagarathna, R Vijayalakshmi
1Department of Oral Medicine and Radiology, Farooqia Dental College, RGUHS, Mysore, Karnataka, India. nareshlingaraju@gmail.com
The Journal of Contemporary Dental Practice
|April 13, 2013
Summary
Osteogenesis imperfecta, a brittle bone disorder, often presents with dentinogenesis imperfecta, affecting teeth opalescence. This case highlights the oral and skeletal manifestations in a child with Type IV B Osteogenesis imperfecta.
Area of Science:
- Genetics and Molecular Biology
- Orthopedics
- Dentistry
Background:
- Osteogenesis imperfecta (OI) is a group of inherited connective tissue disorders.
- Characterized by bone fragility and low bone mass due to collagen defects.
- Dentinogenesis imperfecta (DI) is a common oral manifestation of OI.
Observation:
- A 7-year-old girl with Type IV B Osteogenesis imperfecta presented with significant clinical features.
- Observed were opalascence primary teeth, severe bone deformity, scoliosis, barrel-shaped rib cage, and short stature.
- Comprehensive clinical, radiographic, and histologic assessments were performed.
Findings:
- The case confirmed the association between Osteogenesis imperfecta Type IV B and Dentinogenesis imperfecta.
- Detailed clinical and radiographic evidence supported the diagnosis.
- Histologic examination provided further insight into the dental anomalies.
Implications:
- Early identification of oral findings like DI is crucial in OI patients.
- Multidisciplinary management is essential for addressing the complex skeletal and dental issues in OI.
- Understanding these associations aids in better patient care and prognosis.
Related Concept Videos
01:29Bone 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 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...
01:29Bone 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...
The process begins when mesenchymal cells in the embryonic skeleton gather together and differentiate into osteogenic cells, which then develop into...
01:27Compact 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...
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...
01:24Bone 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...
01:21Tooth Anatomy
The human tooth enables us to eat a variety of foods, speak clearly, and even aid in shaping our faces. Teeth are composed of various elements that work together. Here's a detailed look at the anatomy of a human tooth.
The Crown, Neck, and Root
The visible part of the tooth is referred to as the crown. It's covered by enamel, the hardest substance in the human body. The crown is uniquely shaped for each type of tooth, allowing for different functions such as cutting, tearing, or grinding food.
The Crown, Neck, and Root
The visible part of the tooth is referred to as the crown. It's covered by enamel, the hardest substance in the human body. The crown is uniquely shaped for each type of tooth, allowing for different functions such as cutting, tearing, or grinding food.
01:31Osteoclasts 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...