A case of an extremely large accessory bone with unusual sutures and foramina parietalia permagna in multiple premature craniosynostoses

BackgroundWithin the entity of craniofacial malformations premature craniosynostoses represent the majority of cases. They originate from disturbances in the ossification process, resulting in an osseous closure of cranial sutures ahead of time. Depending on severity and number of the affected sutures, a local or general growth inhibition of the skull follows. In the rare instance of accessory bones along these affected sutures, they may interfere with diagnostics and therapy.PatientThis clinical report describes the case of a seven-month-old male infant with multiple craniosynostoses, an extraordinary large accessory median calvarial bone, two foramina parietalia permagna and a submucosal cleft palate. Chromosomal and genetic analysis did not reveal potential mutations.ResultsThe osseous abnormalities were diagnosed and displayed by three-dimensional computed tomography. The sequential surgical treatment consisted of occipital remodelling at seven months of age and frontoorbital advancement at fourteen months.ConclusionThis rare anatomical variation of the cranial bones – superimposed by multiple premature craniosynostoses – demonstrates the necessity of accurately conducted preoperative diagnostics for appropriate surgical planning. Knowledge, and precise medical examination of potential anatomical variations facilitate the planning and secure performance of surgery as well as its outcome.     

Improvdent: Improving dentures for patient benefit. A crossover randomised clinical trial comparing impression materials for complete dentures

ABSTRACT: BACKGROUND: According to the UK Adult Dental Health Survey (2009) 15% of adults aged 65-74, 30% aged 75-84 and 47% aged >85 years are edentulous and require complete dentures. Patients' quality of life and nutrition status are affected by poor dentures. The quality of the dental impression is the most important issue for improving the fit and comfort of new dentures. There is paucity of RCT evidence for which impression material is best for complete dentures construction. This study aims to compare two impression materials for effectiveness and cost effectiveness. METHODS: IMPROVDENT is a double-blind crossover trial comparing the use of alginate and silicone, two commonly used denture impression materials, in terms of patient preference and cost-effectiveness. Eighty five edentulous patients will be recruited and provided with two sets of dentures, similar in all aspects except for the impression material used (alginate or silicone). Patients will try both sets of dentures for a two-week period, unadjusted, to become accustomed to the feel of the new dentures (habituation period). Patients will then wear each set of dentures for a period of 8 weeks (in random order) during which time the dentures will be adjusted for optimum comfort. Finally, patients will be given both sets of dentures for a further two weeks to wear whichever denture they prefer (confirmation period). Patients will be asked about quality of life and to rate dentures on function and comfort at the end of each trial period and asked which set they prefer at the end of the habituation period (unadjusted denture preference) and confirmation period (adjusted denture preference). A health economic evaluation will estimate incremental cost-effectiveness ratios of producing dentures from the two materials. A qualitative study will investigate the impact of dentures on behaviour and quality of life. Funding: IMPROVDENT is funded by NIHR RfPB (PB-PG-0408-16300). DISCUSSION: This trial aims to provide evidence on the costs and quality of dentures cast from two different commonly used impression materials; the intention is to significantly impact on the quality of denture production within NHS dentistry. Trial Registration ISRCTN Register: ISRCTN01528038 UKCRN Portfolio ID: 8305.     

The mucopolysaccaridoses

The mucopolysaccaridoses are a collection of rare genetic conditions where there is a defect in lysosomal storage causing an accumulation of glycosaminoglycans. There are seven different forms of mucopolysaccaridosis (MPS), each with a different enzymatic mutation and thus each form has similar but separate clinical features. There are multiple effects of glycosaminoglycan deposition including musculoskeletal manifestations such as joint problems and growth arrests. Common treatments of the orthopaedic complications include hip arthroplasty, cervical spine surgery and epiphyseal stapling to correct genu valgum. Presently, the only curative treatment for MPS is haematopoietic stem cell transplantation. Enzyme replacement therapy is a future target for all forms of MPS and a range of therapies are currently in development.     

Geographic variation in the relationship between human Lyme disease incidence and density of infected host-seeking Ixodes scapularis nymphs in the Eastern United States

Prevention and control of Lyme disease is difficult because of the complex biology of the pathogen's (Borrelia burgdorferi) vector (Ixodes scapularis) and multiple reservoir hosts with varying degrees of competence. Cost-effective implementation of tick- and host-targeted control methods requires an understanding of the relationship between pathogen prevalence in nymphs, nymph abundance, and incidence of human cases of Lyme disease. We quantified the relationship between estimated acarological risk and human incidence using county-level human case data and nymphal prevalence data from field-derived estimates in 36 eastern states. The estimated density of infected nymphs (mDIN) was significantly correlated with human incidence (r = 0.69). The relationship was strongest in high-prevalence areas, but it varied by region and state, partly because of the distribution of B. burgdorferi genotypes. More information is needed in several high-prevalence states before DIN can be used for cost-effectiveness analyses.