2,827 research outputs found

    Reliability of GRBAS evaluation of voice quality in children who have a history of airway reconstruction surgery and how this compares to parental report of voice-related quality of life

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    Voice evaluation includes laryngoscopy, perceptual judgement of voice quality, evaluation of respiratory function, acoustic analysis of the voice signal and patient reported subjective impact of voice on quality of life. This is recommended in adults (Dejonkere et al 2001) and children (Cohen et al 2012). Perceptual evaluation of voice often follows the CAPE-V (ASHA 2002) or GRBAS (Hirano 1981). Children requiring laryngotracheal reconstruction (LTR) surgery tend to have this procedure during infancy, where subglottic stenosis forms following intubation in medically fragile or premature infants. Clinicians require reliable measures, particularly where there is a degree of subjectivity. Aspects of the CAPE-V show a high degree of reliability in rating of severity, pitch, breathiness and roughness (Krival et al 2007, Kelchner et al 2008). UK clinicians favour the GRBAS though there is little published information about reliability in a paediatric population. Comparison of clinician perceptual evaluation with patient report shows weak agreement in adults (Karnell et al 2006) reinforcing the need for both measurements. The extent to which the same is the case in children needs further exploration

    Paediatric voice disorder: who to refer and how to assess? A summary of recent literature

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    Purpose of review The purpose of this review is to update the reader with recent advances and current opinion on the assessment and management of paediatric voice disorders. Recent findings Access to advanced multidisciplinary paediatric voice clinics has increased over the last decade. Often the assessment is combined between speech therapy / pathology and ENT surgery. Vocal fold nodules remain the most prevalent diagnosis at a paediatric voice clinic, but significant pathologies will also present e.g. laryngeal papilloma. Recently more consideration of the psychosocial aspect of voice disorders and evaluation of auditory processing disorders have shown how these can have a negative impact. There also appears to be a lack of parental and teacher awareness of paediatric voice disorders and their significance. Summary We would recommend multiparametric assessment and analysis of all children with a voice disorder. Most paediatric voice conditions will respond to skilled voice therapy. However, the exact techniques and dosage requires further evaluation and research. Paediatric voice conditions are common and should not be dismissed as they may represent significant pathology e.g. papilloma, vagal palsy or have a significant psychosocial impact on the child

    Visual responses in the lateral geniculate evoked by Cx36-independent rod pathways

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    AbstractEmerging evidence indicates rods can communicate with retinal ganglion cells (RGCs) via pathways that do not involve gap-junctions. Here we investigated the significance of such pathways for central visual responses, using mice lacking a key gap junction protein (Cx36−/−) and carrying a mutation that disrupts cone phototransduction (Gnat2cpfl3). Electrophysiological recordings spanning the lateral geniculate revealed rod-mediated ON and OFF visual responses in virtually every cell from all major anatomical sub-compartments of this nucleus. Hence, we demonstrate that one or more classes of RGC receive input from Cx36-independent rod pathways and drive extensive ON and OFF responses across the visual thalamus

    Classification of first branchial cleft anomalies: is it clinically relevant?

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    Background: There are three classification systems for first branchial cleft anomalies currently in use. The Arnot, Work and Olsen classifications describe these lesions on the basis of morphology, tissue of origin and clinical appearance. However, the clinical relevance of these classifications is debated, as they may not be readily applicable in all cases and may provide no additional information on how the lesion should be managed.Objective: We seek to investigate this issue by applying these classification systems to cases from our centre and evaluating the information gained.Patients and methods: A retrospective case note review of all first branchial cleft anomalies excised at our institution between 2004 and 2014 was carried out, recording patient demographics, information on the anomalies and how they were investigated and managed.Results: This search identified eight unilateral cases and one bilateral case of first branchial cleft anomalies. These were a heterogenous group of lesions, which were variably investigated and managed. Categorization of these cases into Arnot, Work and Olsen subtypes did not correlate with the lesion’s relation with the facial nerve or the outcome of excision.Conclusion: The current classification systems used for first branchial cleft anomalies have little clinical relevance apart from providing extensive descriptions to aid in diagnosis. We advise instead that clinicians use imaging techniques to gain as much information as possible about these lesions before excision and be aware of the risk to the facial nerve at the time of excision. A description of the lesion’s relation with the facial nerve at the time of excision may provide more information on the likely outcomes compared with the classifications currently in use.Keywords: branchial cleft, congenital anomaly, facial nerve injury, first branchial cleft anomal

    Proteomic analysis of the postsynaptic density implicates synaptic function and energy pathways in bipolar disorder.

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    The postsynaptic density (PSD) contains a complex set of proteins of known relevance to neuropsychiatric disorders such as schizophrenia and bipolar disorder. We enriched for this anatomical structure in the anterior cingulate cortex of 16 bipolar disorder samples and 20 controls from the Stanley Medical Research Institute. Unbiased shotgun proteomics incorporating label-free quantitation was used to identify differentially expressed proteins. Quantitative investigation of the PSD identified 2033 proteins, among which 288 were found to be differentially expressed. Validation of expression changes of DNM1, DTNA, NDUFV2, SEPT11 and SSBP was performed by western blotting. Bioinformatics analysis of the differentially expressed proteins implicated metabolic pathways including mitochondrial function, the tricarboxylic acid cycle, oxidative phosphorylation, protein translation and calcium signaling. The data implicate PSD-associated proteins, and specifically mitochondrial function in bipolar disorder. They relate synaptic function in bipolar disorder and the energy pathways that underpin it. Overall, our findings add to a growing literature linking the PSD and mitochondrial function in psychiatric disorders generally, and suggest that mitochondrial function associated with the PSD is particularly important in bipolar disorder

    Cross-sectional follow up of voice outcomes in children who have a history of airway reconstruction surgery

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    Objectives - This study reports vocal function in a cross-section of children with subglottic stenosis. Each child had a history of laryngotracheal reconstruction and/or cricotracheal resection surgery. Vocal function was measured using laryngoscopy, acoustic analysis, perceptual evaluation and impact of voice on quality of life.  Design - All patients aged >5 years with history of laryngotracheal reconstruction and/or cricotracheal resection surgery at the Scottish National Complex Airways service were invited to participate.  Setting - Data was gathered in the Royal Hospital for Children in Glasgow in a single out-patient appointment. Participants - Twelve out of fifty-six former patients (aged 5 – 27) provided a voice sample and eleven consented to awake laryngoscopy. All consented for detailed evaluation of their medical records.  Main outcome measures - Acoustic analysis of fundamental frequency and pitch perturbation was conducted on sustained vowel [a]. Perceptual evaluation was conducted by four trained listeners on a series of spoken sentences. Impact on quality of life was measured using the Paediatric Voice Related Quality of Life questionnaire. Laryngeal function was descriptively evaluated.  Results - Four children had normal voice acoustically, perceptually and in relation to voice related quality of life. One of these had vocal fold nodules unrelated to surgical history. Two other children had ‘near normal’ vocal function, defined where most voice measurements fell within the normal range.  Conclusions - Normal or ‘near normal’ voice is a possible outcome for children who have had this surgery. Where there is an ongoing complex medical condition, voice outcome may be poorer

    Perceptual evaluation of voice disorder in children who have had laryngotracheal reconstruction surgery and the relationship between clinician perceptual rating of voice quality and parent proxy/child self-report of voice related quality of life

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    Reliability of the GRBAS tool for perceptual evaluation of paediatric voice disorder is measured in this study of children with a history of laryngotracheal reconstruction surgery (LTR). Additionally, the relationship between parent proxy/child self-report of voice related quality of life with clinician perceptual rating of voice quality is analysed. Eleven children with a history of LTR provided voice recordings following the stimuli set by the CAPE-V protocol. Subjective impact of voice quality on life was measured using the Pediatric Voice-Related Quality of Life Questionnaire. Four trained judges rated the sound files according to both the GRBAS and CAPE-V protocol. Intraclass correlation coefficients were high for both intrarater and interrater judgements across all parameters of the GRBAS protocol, and a strong correlation was found between the Grade rating of the GRBAS and the Overall Severity rating of the CAPE-V. Some elements of parent proxy reporting of VRQOL were significantly negatively correlated with clinician perceptual rating of voice quality, while there was no significant relationship between child self-report and clinician perceptual rating

    Evaluation of changes in sleep breathing patterns after primary palatoplasty in cleft children

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    Introduction: There is a need to more clearly understand the characteristics of breathing patterns in children with cleft palate in the first year of life, as there is little data available to guide current practice. Pierre Robin patients are known to have a higher incidence, however we hypothesised sleep breathing disturbance is not confined to this sub-group of cleft patient. Methods: We conducted a prospective observational study of sleep disordered breathing patterns in a cohort of infants with oro-nasal clefts (cleft palate with or without cleft lip) to describe the spectrum of sleep breathing patterns both pre and post palate repair. Sleep breathing studies were performed pre- and post-operatively in sequential infants referred to a regional cleft lip and palate unit. Results of sleep breathing studies were analysed according to American Academy of Sleep Medicine scoring guidelines and correlated with clinical history and details of peri-operative respiratory compromise. The degree of sleep disordered breathing was characterised using desaturation indices (number of desaturations from baseline SpO2 of >=4%, per hour). Results: Thirty-nine infants were included in this study, twenty-five female and fourteen male. Twelve had isolated Cleft Palate as part of an associated syndrome. Patients were categorised into Isolated Cleft Palate, Isolated Cleft Palate in the context of Pierre Robin Sequence, and those with Cleft Lip and Palate. All groups demonstrated some degree of sleep breathing abnormality. Not unsurprisingly the eight infants with Pierre Robin Sequence had a significantly higher desaturation index before surgical intervention (p=0.043), and were more likely to require a pre-operative airway intervention (p=0.009). Palate repair in this group did not alter the relative distribution of patients in each severity category of sleep disorder breathing. Surgical repair of the secondary palate in the remaining children was associated with some improvement but by no means complete resolution of their sleep disordered breathing patterns. Conclusions: We conclude that sleep breathing disturbance is not confined to Pierre Robin patients alone and all cleft palate patients should undergo pre-operative and post-operative sleep breathing analysis

    Phase-Control of Photoabsorption in Optically Dense Media

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    We present a self-consistent theory, as well as an illustrative application to a realistic system, of phase control of photoabsorption in an optically dense medium. We demonstrate that, when propagation effects are taken into consideration, the impact on phase control is significant. Independently of the value of the initial phase difference between the two fields, over a short scaled distance of propagation, the medium tends to settle the relative phase so that it cancels the atomic excitation. In addition, we find some rather unusual behavior for an optically thin layer.Comment: 5 pages, 3 figures, submitted to PR
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