scholarly journals Osteogenesis Imperfecta (Type IV) with Dental Findings in Siblings

2011 ◽  
Vol 2011 ◽  
pp. 1-4 ◽  
Author(s):  
Shishir Ram Shetty ◽  
Deepa Dsouza ◽  
Subhas Babu ◽  
Preethi Balan
Keyword(s):  
Osteogenesis Imperfecta ◽  
Bone Fractures ◽  
Age Groups ◽  
Bone Fragility ◽  
Osteogenesis Imperfecta Type ◽  
Recessive Factor ◽  
Radiological Features ◽  
Type Iv ◽  
History Of ◽  
Blue Sclera

Osteogenesis imperfecta (OI) is a hereditary disorder characterized by increased tendency for bone fractures due to high fragility. The clinical and radiological features of OI manifest in different age groups, although the disease is congenital in nature. Besides bone fragility, features like laxity of the ligaments, blue sclera, growth retardation, and scoliosis are also observed. In severe cases, respiratory distress and death have been reported. The most important oral finding in OI is the presence of yellowish-brown-coloured brittle teeth characteristic of dentinogenesis imperfecta. Genetic factors play a very important role in the pathogenesis of OI either as a dominant or recessive factor. When a child has OI, there is a 25% chance of the sibling to have the same disorder. We report two cases of OI in siblings born to parents with a history of consanguineous marriage. The clinical and radiological features of the two cases are described in detail.

scholarly journals Dental behavior management of a patient with osteogenesis imperfecta type V: case report

2020 ◽  
Vol 28 (87) ◽  
Author(s):  
Danilo Cassiano Ferraz ◽  
Ana Luiza Rodrigues Ribeiro ◽  
Ronan Machado de Alcântra ◽  
Alessandra Maia de Castro ◽  
Fabiana Sodré de Oliveira ◽  
...  
Keyword(s):  
Osteogenesis Imperfecta ◽  
Behavior Management ◽  
Parental Education ◽  
Bone Fractures ◽  
Pediatric Dentistry ◽  
Bone Fragility ◽  
Osteogenesis Imperfecta Type ◽  
Dental Biofilm ◽  
Stabilization Technique ◽  
Type V

Aim: the aim of this study is to report a case of a four-year-old male patient diagnosed with Osteogenesis Imperfecta (OI) type V and the dental care performed.  Material and method: the patient has been monitored by a multidisciplinary team composed by Pediatric Dentistry, Nurse and Nutritionist at a Special Patients Center in Dentistry Hospital on the Federal University of Uberlândia since one month of life. It was reported that a child had already suffered several bone fractures and currently he has been using calcium carbonate, D vitamin and pamidronate. Results: the proposed intervention was dental preventive strategies by parental education, caries risk assessment and controlling the dental biofilm with professional prophylaxis. But, due to the uncooperative behavior, it was used the active protective stabilization technique and mouth opener. Conclusion: it was concluded that due to the bone fragility and the risk of fractures, it is important a careful placement of the patient on the dental chair. Also, the correct use of basic auxiliary devices and advanced techniques of behavior management were relevant.

scholarly journals Osteogenesis Imperfecta/Lobstein Syndrome associated with Dentinogenesis Imperfecta

2013 ◽  
Vol 14 (1) ◽  
pp. 140-142
Author(s):  
Naresh Lingaraju ◽  
PJ Nagarathna ◽  
R Vijayalakshmi ◽  
P Sheshadri
Keyword(s):  
Bone Mass ◽  
Osteogenesis Imperfecta ◽  
Primary Teeth ◽  
Bone Fragility ◽  
Osteogenesis Imperfecta Type ◽  
Dentinogenesis Imperfecta ◽  
Low Bone Mass ◽  
Bone Deformity ◽  
Related Disorder ◽  
Type Iv

ABSTRACT Osteogenesis imperfecta is a collagen related disorder characterized by increased bone fragility and low bone mass. The important oral finding in osteogenesis imperfect is the presence of dentinogenesis imperfecta. This article presents a case of osteogenesis imperfecta (type IV B) with dentinogenesis imperfecta where a 7-year-old girl had opalacent primary teeth associated with severe bone deformity, scoliosis, barrel shaped rib cage, and short stature. The clinical, radiographic ad histologic features are reviewed along with management aspects. How to cite this article Lingaraju N, Nagarathna PJ, Vijayalakshmi R, Sheshadri P. Osteogenesis Imperfecta/ Lobstein Syndrome associated with Dentinogenesis Imperfecta. J Contemp Dent Pract 2013;14(1):140-142.

Skeletal Dysplasias

2019 ◽  
pp. 249-254
Author(s):  
Robin D. Clark ◽  
Cynthia J. Curry
Keyword(s):  
Differential Diagnosis ◽  
Osteogenesis Imperfecta ◽  
Bone Fragility ◽  
Osteogenesis Imperfecta Type ◽  
Accidental Injury ◽  
Type Iv ◽  
Gene Panels ◽  
Non Accidental Injury ◽  
Recessive Disorders

This chapter reviews the incidence, genetics, and classification of osteogenesis imperfecta (OI) and other bone fragility syndromes that can present with fractures in the newborn. A few rare recessive disorders including Stuve Wiedemann syndrome are discussed briefly. Distinquishing clinical features are highlighted. The utility of gene panels in the differential diagnosis of these disorders is presented. Therapeutic advances including the use of bisphosphonates and asfotase alfa in disorders with increased fracture risk can be expected to increase. Non-accidental injury, is discussed briefly as is the differential diagnosis of rickets, including that associated with prematurity. The clinical case presentation features an infant with osteogenesis imperfecta type IV.

Sign in / Sign up

Export Citation Format

Share Document