BACKGROUND AND OBJECTIVES Osteogenesis Imperfecta is an uncommon genetic connective tissue disease with prevalence of 1/10000, primarily involving endochondral ossification, resulting in brittle bones, multiple fractures and skeletal deformities. This article aimed at reporting a case of Osteogenesis Imperfecta patient submitted to total intravenous anesthesia for fractured femur surgical repai...

OBJECTIVE Osteogenesis imperfecta is a hereditary disease resulting from mutation in type I procollagen genes. One of the extra skeletal manifestations of this disease is cardiac involvement. The prevalence of cardiac involvement is still unknown in the children with osteogenesis imperfecta. The present study aimed to investigate the prevalence of cardiovascular abnormalities in these patients....

AIM The aim of this case report is to present the clinical and radiographic findings of hereditary opalescent dentin to facilitate an early diagnosis. BACKGROUND Hereditary opalescent dentin (or dentinogenesis imperfecta) may manifest itself in three variations: i.e., Shields type I, Shields type II, and Shields type III. Dentinogenesis imperfecta occurs as an autosomal dominant trait with va...

We describe two patients with osteogenesis imperfecta who developed transient osteoporosis in both hips sequentially.

ABSTRACT Background: Osteogenesis imperfecta (OI) comprises a heterogeneous group of diseases characterized by susceptibility to bone fractures with variable severity and, in most cases, presumed or proven defects collagen type I biosynthesis and an estimated prevalence 1/15,000 births. Management is multidisciplinary involving mainly surgery, physiotherapy, rehabilitation Case Presentation: A ...

Keywords Disease name and synonyms Definition/Diagnosis criteria Differential diagnosis Frequency Clinical description Etiology Diagnostic methods Management Treatment Genetic counselling References Abstract Osteogenesis imperfecta (OI) is a group of inherited diseases responsible for varying degrees of skeletal fragility. Minimal trauma is sufficient to cause fractures and bone deformities. A ...