Oro-dental and cranio-facial characteristics of osteogenesis imperfecta type V

Osteogenesis imperfecta (OI) type V is an ultrarare heritable bone disorder caused by the heterozygous c.-14C > T mutation in IFITM5. The oro-dental and craniofacial phenotype has not been described in detail, which we therefore undertook to evaluate in a multicenter study (Brittle Bone Disease Consortium). Fourteen individuals with OI type V (age 3–50 years; 10 females, 4 males) underwent dental and craniofacial assessment. None of the individuals had dentinogenesis imperfecta. Six of the 9 study participants (66%) for whom panoramic radiographs were obtained had at least one missing tooth (range 1–9). Class II molar occlusion was present in 8 (57%) of the 14 study participants. The facial profile was retrusive and lower face height was decreased in 8 (57%) individuals. Cephalometry, performed in three study participants, revealed a severely retrusive maxilla and mandible, and moderately to severly retroclined incisors in a 14-year old girl, a protrusive maxilla and a retrusive mandible in a 14-year old boy. Cone beam computed tomograpy scans were obtained from two study participants and demonstrated intervertebral disc calcification at the C2-C3 level in one individual. Our study observed that OI type V is associated with missing permanent teeth, especially permanent premolar, but not with dentinogenesis imperfecta. The pattern of craniofacial abnormalities in OI type V thus differs from that in other severe OI types, such as OI type III and IV, and could be described as a bimaxillary retrusive malocclusion with reduced lower face height and multiple missing teeth.     

Dental and craniofacial characteristics caused by the p.Ser40Leu mutation in IFITM5

Severe forms of osteogenesis imperfecta (OI) are usually caused by mutations in genes that code for collagen Type I and frequently are associated with craniofacial abnormalities. However, the dental and craniofacial characteristics of OI caused by the p.Ser40Leu mutation in the IFITM5 gene have not been reported. We investigated a 15‐year‐old girl with severe OI caused by this mutation. She had marked deformations of extremity long bones. There were no clinical or radiological signs of dentinogenesis imperfecta, but one tooth was missing and several teeth were impacted. Cone beam computed tomography revealed a generalized osteopenic appearance of the craniofacial skeleton, bilateral enlargement of mandibular bodies, and areas of cortical erosions. The cranial base and skull showed a generalized granular bone pattern with a mixture of osteosclerosis and osteolysis. Sphenoid and frontal sinuses were congenitally missing. Cephalometric analysis indicated a Class III growth pattern. In this case, the IFITM5 p.Ser40Leu mutation did not affect tooth structure but was associated with deformities in craniofacial bones that resemble those in the other parts of the skeleton.     

Blended learning in orthodontic diagnosis: an interactive approach

Interactive multimedia programs can provide an opportunity for authentic learning both inside and outside the classroom. McGill University designed an interactive Orthodontic Diagnosis program on CD-ROM that has been used successfully in the faculty of dentistry to provide undergraduate students with interactive tutorials and exercises to help them recognize developing malocclusions. Key aspects of this multimedia program are the use of an outside-in approach to diagnosis as well as sound instructional design that provides practice opportunities and feedback to students. The goal is to bridge the gap between theoretical knowledge and the practical skills needed to be a successful dentist.     

Comparison of two-dimensional methods versus three-dimensional scanning systems in the assessment of total body surface area estimation in burn patients

Background

Accurate measurement of percent total body surface area (%TBSA) burn is crucial in the management of burn patients for calculating the estimated fluid resuscitation, determining the need to transfer to a specialized burn unit and probability of mortality. %TBSA can be estimated using many methods, all of which are relatively inaccurate. Three-dimensional (3D) systems have been developed to improve %TBSA calculation and consequently optimize clinical decision-making. The objective of this study was to compare the accuracy of percent total burn surface area calculation by conventional methods against novel 3D methods.

Gianotti-crosti syndrome after childhood vaccination

A 19-month-old boy was evaluated for a skin eruption after recent vaccinations. Clinical and histopathologic findings supported a diagnosis of Gianotti-Crosti syndrome (GCS). This case report examines the link between GCS and vaccinations, particularly the diphtheria, tetanus, and pertussis vaccine and the varicella virus live vaccine.     

Gianotti–Crosti Syndrome Following Childhood Vaccinations

Abstract: A 19‐month‐old boy was evaluated for a skin eruption after recent vaccinations. Clinical and histopathologic findings supported a diagnosis of Gianotti–Crosti syndrome (GCS). This case report examines the link between GCS and vaccinations, particularly the diphtheria, tetanus, and pertussis vaccine and the varicella virus live vaccine.