The inclusion of preoperative education in Eras spinal surgery pathways: A systematic review

Objectives: Enhanced Recovery after Surgery (ERAS) principles are starting to be adopted in major spinal procedures, and the high volume of surgeries, and wide variations in length of stay and complications rates suggest that improvements are both desirable and possible. Psychosocial factors related to different degrees of clinical impairment and quality of life in the preoperative period may influence outcomes, and patients have expressed a need for individualised information given in sufficient quantities and at the appropriate time to help attenuate preoperative anxiety. Preoperative education is a core component of ERAS that aims to empower patients to undertake positive health actions and support autonomous decision making. This review aims to summarise the current evidence for inclusion of a preoperative education session into ERAS spinal surgery pathways

Victorian index of estuary condition : recommended themes and measures

Deakin University was engaged by the Department of Sustainability and Environment to develop an Index of Estuary Condition (IEC) for evaluating the environmental condition of Victorian estuaries. The Index will ultimately complement the existing Index of Stream Condition (ISC) by providing a consistent statewide assessment of the environmental condition of estuaries. This will better enable:&bull; Estuarine condition to be reported at regional, state and national levels.&bull; Prioritisation of resource allocation.&bull; Strategic evaluation of management interventions in estuaries.Workshops involving participants with expertise in a variety of disciplines were convened to integrate learnings from assessment programs currently being developed interstate and overseas. This report synthesises and builds on the output from those workshops which: &bull; Identified key components (themes) of estuaries that contribute to estuarine condition. &bull; Contributed to development of a broad conceptual model for Victorian estuaries &bull; Identified possible measures of each themeIn keeping with the sub-indices of the ISC, six themes were identified for use in the IEC: Physical form, Hydrology, Water quality, Sediment, Flora and Fauna. Several measures within each theme are recommended to assess estuary condition.Implementation of particular measures in the IEC partly depends on the investment required to both collect and interpret the required data. With regard to data collection, each measure was assessed according to whether there is an established sampling procedure, how frequently data need to be collected and the level of expertise required for collection and processing. For interpreting the data, measures were scored on whether baseline condition is established and whether descriptions and scores are developed which reflect the extent of deviation from that condition. These scores were used to indicate which measures are feasible to implement immediately and which require further investigation. A trial of the recommended IEC measures in a selection of estuaries is recommended as it would provide an opportunity to test the measures and their suitability for application statewide. It is suggested that the trial is conducted in estuaries subject to various levels of threats within each of the four estuary classes described by Barton et al. (2008)<br /

Osteogenesis imperfecta: Ultrastructural and histological findings on examination of skin revealing novel insights into genotype-phenotype correlation

© 2016 Taylor & Francis. Osteogenesis imperfecta (OI) is a heterogeneous group of inherited disorders of bone formation, resulting in low bone mass and an increased propensity to fracture. Over 90% of patients with OI have a mutation in COL1A1/COL1A2, which shows an autosomal dominant pattern of inheritance. In-depth phenotyping and in particular, studies involving manifestations in the skin connective tissue have not previously been undertaken in OI. The aims of the study were to perform histological and ultrastructural examination of skin biopsies in a cohort of patients with OI; to identify common and distinguishing features in order to inform genotype-phenotype correlation; and to identify common and distinguishing features between the different subtypes of OI. As part of the RUDY (Rare Diseases in Bone, Joints and/or Blood Vessels) study, in collaboration with the NIHR Rare Diseases Translational Research Collaboration, we undertook a national study of skin biopsies in patients with OI. We studied the manifestations in the skin connective tissue and undertook in-depth clinical and molecular phenotyping of 16 patients with OI. We recruited 16 patients: analyses have shown that in type 1 collagen mutation positive patients (COL1A1/ COL1A2) (n-4/16) consistent findings included: variable collagen fibril diameter (CFD) and presence of collagen flowers. Histological examination in these patients showed an increase in elastic fibers that are frequently fragmented and clumped. These observations provide evidence that collagen flowers and CFD variability are consistent features in OI due to type 1 collagen defects and reinforce the need for accurate phenotyping in conjunction with genomic analyses

Expanding the phenotype of SPARC-related osteogenesis imperfecta: clinical findings in two patients with pathogenic variants in SPARC and literature review

BACKGROUND: Secreted protein, acidic, cysteine rich (SPARC)-related osteogenesis imperfecta (OI), also referred to as OI type XVII, was first described in 2015, since then there has been only one further report of this form of OI. SPARC is located on chromosome 5 between bands q31 and q33. The encoded protein is necessary for calcification of the collagen in bone, synthesis of extracellular matrix and the promotion of changes to cell shape. METHODS: We describe a further two patients with previously unreported homozygous SPARC variants with OI: one splice site; one nonsense pathogenic variant. We present detailed information on the clinical and radiological phenotype and correlate this with their genotype. There are only two previous reports by Mendozo-Londono et al and Hayat et al with clinical descriptions of patients with SPARC variants. RESULTS: From the data we have obtained, common clinical features in individuals with OI type XVII caused by SPARC variants include scoliosis (5/5), vertebral compression fractures (5/5), multiple long bone fractures (5/5) and delayed motor development (3/3). Interestingly, 2/4 patients also had abnormal brain MRI, including high subcortical white matter changes, abnormal fluid-attenuated inversion in the para-atrial white matter and a large spinal canal from T10 to L1. Of significance, both patients reported here presented with significant neuromuscular weakness prompting early workup. CONCLUSION: Common phenotypic expressions include delayed motor development with neuromuscular weakness, scoliosis and multiple fractures. The data presented here broaden the phenotypic spectrum establishing similar patterns of neuromuscular presentation with a presumed diagnosis of 'myopathy'