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The techniques of frontofacial surgery are most valuable in the clinical management of complex craniofacial deformity to achieve a range of functional and aesthetic gains in children from infancy to maturity. A variety of complex craniofacial osteotomies that can be used to separate the orbits from the skull base have been described. In addition, the combination of circumorbital release and pterygomaxillary disjunction allows advancement of the orbitomaxillary segment for powerful clinical benefit. For the purpose of this article, the principal frontofacial strategies include the monobloc frontofacial advancement by distraction (MBD), frontofacial bipartition advancement by distraction (BpD), orbital box osteotomy (FFBx), and frontofacial bipartition (FFBp). These techniques are broadly used for two purposes: to allow for the translocation of one or both orbits to correct orbitofacial disproportion (hypertelorism, vertical orbital dystopia, or a combination of both), or to advance the orbitomaxillary segment for orbital volume expansion and protection of the eye in syndromes featuring severe exorbitism (oculo-orbital disproportion). Here we describe aspects of our experience of frontofacial surgery in the Craniofacial Centre at Great Ormond Street Hospital for Children, London, with reference to the principles underpinning frontofacial surgical techniques, their challenges, and their impact on function and aesthetics.

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BACKGROUND: Many children experiencing a procedure under general anaesthetic (GA), including those having surgery for cochlear implantation, display behaviours indicative of distress during induction of anaesthesia. It would be useful to ascertain which factors in the pre-operative period are related to the presence of distress at induction in order to target appropriate psychological preparation. METHODS: The families of 84 children aged 4-7 years undergoing a procedure under GA (including insertion of a cochlear implant) completed three questionnaires assessing temperament, behavioural difficulties, and pre-operative worries. Demographic variables were also recorded. The outcome measure was the amount of behavioural distress at induction. RESULTS: Statistically significant relationships with the outcome measure of distress at induction were obtained for three factors; an emotional or sociable temperament, and the number of previous procedures. Further, children receiving inhalation inductions displayed greater distress than those receiving intravenous inductions. CONCLUSION: Undergoing cochlear implantation under GA is a major life event for many recipients. Psychologically preparing children for this has been found to be efficacious, but is not currently available to all children. It is suggested that preparation can be targeted at children, as identified in this study, who have elevated emotionality or sociability scores or who have previous experience of procedures under general anaesthesia. This is of particular current relevance as children are increasingly likely to require re-implantation as they grow older and as unilaterally implanted children are now being offered a sequential bilateral implant, both necessitating further surgery.

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There are few studies that report findings on the speech and language characteristics of Apert syndrome and little is known about the cognitive profile of the syndrome. The current study addresses this gap and explores speech, language, resonance/voice, attention oro-motor and cognitive skills in a group of 10 children (4;1-5;11) with Apert syndrome. The speech and language battery included: the CELF-Pre-school, the PLS-3, the Vocal Profile Analysis, GOS.SP.ASS, PACS; and the Brodsky Drooling Scale. Subscales of the BAS II-Early Years Version were used to assess cognition. Data were also collected on other factors that could influence developmental outcome such as audiological history and management; occlusion/dentition; respiratory problems and management; neuroanatomical abnormalities; the number and nature of cranial surgeries; and the occurrence of raised intracranial pressure. All children for whom a Performance IQ was obtained (n = 8) had abilities within the average range and IQ scores were considerably higher than those reported in previous studies. Eight children had moderate or severe language difficulties and expressive language difficulties were the most frequent. These language difficulties were not associated with a general cognitive deficit. All the children had problems with attention, speech and oro-motor skills. Nine had abnormal voice. In addition, a range of other associated factors that could affect functioning were identified. The discrepancies between the current study and previous investigations are outlined. Parameters for assessment are considered. The implications of these findings for valid assessments of children with Apert syndrome are discussed. Multidisciplinary assessment of children with Apert syndrome across a broad range of dimensions is recommended to obtain a profile of each child's strengths and weaknesses to ensure that appropriate educational placements and early interventions are implemented. Considering patterns of development over time at key ages is also argued to be of central importance.

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The GRAFTER suite of programs provides geometric search and evaluation functions that simplify and automate the process of identifying the best scaffolds for a particular structural motif. Three application of the GRAFTER suite are presented. Potential grafts between lambda repressor and 434 repressor were identified that should change the DNA binding specificity of these repressors. These results are compared with site-directed mutagenesis experiments that have been shown to alter repressor-DNA binding specificity. Next, 26 loops from antibody structures were grouped into families of similar structure. Grafts of antibody loops onto a pre-existing scaffold are an essential component of antibody humanization. Finally, interleukin (IL)-4 was searched as a scaffold that might accept the graft of a five residue epitope from human growth hormone (hGH). The existence of a crystal structure of the hGH-hGH receptor complex, extensive mutagenesis studies of the hGH residues that contribute to the energetics of ligand-receptor interactions and the gross structural homology between hGH and IL-4 make this an appealing computational target. The approach presented here could aid the development of novel enzymes and binding proteins.

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