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Late adult-onset Niemann Pick type C (NPC): An "atypical" typical presentation at the age of 62.
Get PDFTrans-mastoid plugging of superior semicircular canal dehiscence: long-term follow-up.
No full textOBJECTIVE
To evaluate the long-term outcomes of trans-mastoid plugging of superior semicircular canal dehiscence (SSCD), focusing on complicated cases.
METHODS
In this cohort study, we included all patients who underwent trans-mastoid plugging of SSCD between 2009 and 2019. We evaluated the symptoms (autophony, sound-/pressure-induced vertigo, disequilibrium, aural fullness and pulsatile tinnitus) before and 1 year after surgery in the medical records. We systematically assessed the current symptoms 6.2 ± 3 years postoperative (range 2.2-12.3 years) using questionnaires sent by post and validated by telephone interviews. We also documented any complications and the need for further procedures. We compared pure tone and speech audiometry before and 1 year after surgery. Finally, the degree of mastoid pneumatisation and mastoid tegmen anatomy were reviewed on preoperative CT scans.
RESULTS
We included 24 ears in 23 patients. No complications were recorded, and none required a second procedure for SSCD. Following surgery, oscillopsia and Tullio phenomena resolved in all patients. Hyperacusis, autophony, and aural fullness were also settled in all patients except one. Balance impairment persisted to some degree in 35% of patients. No deterioration over the years was reported regarding the above symptoms. On average, bone conduction pure tone average pre- and 1 year postoperative were 13.7 ± 17 and 20.5 ± 18 dB, respectively (P = 0.002). Air bone gaps were reduced from 12.7 ± 8 to 5.9 ± 6 (P = 0.001). Two patients had a significant sclerotic mastoid, three had a prominent low-lying mastoid tegmen, and two had both. Anatomy had no effect on outcome.
CONCLUSION
Trans-mastoid plugging of SSCD is a reliable and effective technique which achieves long-lasting symptom control, even in cases with sclerotic mastoid or low-lying mastoid tegmen
Effectiveness of bedside investigations to diagnose peripheral artery disease among people with diabetes mellitus: A systematic review.
Get PDFAs a progressive disease process, early diagnosis and ongoing monitoring and treatment of lower limb peripheral artery disease (PAD) is critical to reduce the risk of diabetes-related foot ulcer (DFU) development, non-healing of wounds, infection and amputation, in addition to cardiovascular complications. There are a variety of non-invasive tests available to diagnose PAD at the bedside, but there is no consensus as to the most diagnostically accurate of these bedside investigations or their reliability for use as a method of ongoing monitoring. Therefore, the aim of this systematic review was to first determine the diagnostic accuracy of non-invasive bedside tests for identifying PAD compared to an imaging reference test and second to determine the intra- and inter-rater reliability of non-invasive bedside tests in adults with diabetes. A database search of Medline and Embase was conducted from 1980 to 30 November 2022. Prospective and retrospective investigations of the diagnostic accuracy of bedside testing in people with diabetes using an imaging reference standard and reliability studies of bedside testing techniques conducted in people with diabetes were eligible. Included studies of diagnostic accuracy were required to report adequate data to calculate the positive likelihood ratio (PLR) and negative likelihood ratio (NLR) which were the primary endpoints. The quality appraisal was conducted using the Quality Assessment of Diagnostic Accuracy Studies and Quality Appraisal of Reliability quality appraisal tools. From a total of 8517 abstracts retrieved, 40 studies met the inclusion criteria for the diagnostic accuracy component of the review and seven studies met the inclusion criteria for the reliability component of the review. Most studies investigated the diagnostic accuracy of ankle -brachial index (ABI) (N = 38). In people with and without DFU, PLRs ranged from 1.69 to 19.9 and NLRs from 0.29 to 0.84 indicating an ABI 1.3, TBI of <0.70, and absent or monophasic pedal Doppler waveforms are useful to identify the presence of disease. The ability of the tests to exclude disease is variable and although reliability may be acceptable, evidence of error in the measurements means test results that are within normal limits should be considered with caution and in the context of other vascular assessment findings (e.g., pedal pulse palpation and clinical signs) and progress of DFU healing
Organization of Hannover Skin Bank: Sterile culture and procurement protocols for viable cryopreserved allogeneic skin grafts of living donors.
Get PDFPreserved allogeneic donor skin still represents one of the gold standard therapies in temporary wound coverage in severely burned patients or chronic wounds. Allogeneic skin grafts are currently commercially available as cryo- or glycerol-preserved allografts through skin tissue banks all over the world. Most of the skin tissue banks rely on human cadaveric skin donations. Due to the chronic shortage of human allogeneic transplants, such as skin, and increasing costs in the procurement of allografts from other skin tissue banks, Hannover Medical School has been building up its own skin tissue bank based on allogeneic skin grafts from living donors who underwent surgical treatment (i.e., body-contouring procedures, such as abdominioplasties). This article presents procedures and protocols for the procurement and processing of allogeneic skin grafts according to national legislation and European regulations and guidelines. Beside protocols, initial microbiological data regarding the sterility of the harvested grafts are presented. The results currently form the basis for further investigations as well as clinical applications. In summary, a microbiological testing and acceptance procedure is presented that ensures adequate patient safety and skin viability
Twenty-five years of recombinant human growth factors rhPDGF-BB and rhBMP-2 in oral hard and soft tissue regeneration.
Get PDFContemporary oral tissue engineering strategies involve recombinant human growth factor approaches to stimulate diverse cellular processes including cell differentiation, migration, recruitment, and proliferation at grafted areas. Recombinant human growth factor applications in oral hard and soft tissue regeneration have been progressively researched over the last 25 years. Growth factor-mediated surgical approaches aim to accelerate healing, tissue reconstruction, and patient recovery. Thus, regenerative approaches involving growth factors such as recombinant human platelet-derived growth factor-BB (rhPDGF-BB) and recombinant human bone morphogenetic proteins (rhBMPs) have shown certain advantages over invasive traditional surgical approaches in severe hard and soft tissue defects. Several clinical studies assessed the outcomes of rhBMP-2 in diverse clinical applications for implant site development and bone augmentation. Current evidence regarding the clinical benefits of rhBMP-2 compared to conventional therapies is inconclusive. Nevertheless, it seems that rhBMP-2 can promote faster wound healing processes and enhance de novo bone formation, which may be particularly favorable in patients with compromised bone healing capacity or limited donor sites. rhPDGF-BB has been extensively applied for periodontal regenerative procedures and for the treatment of gingival recessions, showing consistent and positive outcomes. Nevertheless, current evidence regarding its benefits at implant and edentulous sites is limited. The present review explores and depicts the current applications, outcomes, and evidence-based clinical recommendations of rhPDGF-BB and rhBMPs for oral tissue regeneration
MICROSTATELAB: The EEGLAB Toolbox for Resting-State Microstate Analysis.
Get PDFMicrostate analysis is a multivariate method that enables investigations of the temporal dynamics of large-scale neural networks in EEG recordings of human brain activity. To meet the enormously increasing interest in this approach, we provide a thoroughly updated version of the first open source EEGLAB toolbox for the standardized identification, visualization, and quantification of microstates in resting-state EEG data. The toolbox allows scientists to (i) identify individual, mean, and grand mean microstate maps using topographical clustering approaches, (ii) check data quality and detect outlier maps, (iii) visualize, sort, and label individual, mean, and grand mean microstate maps according to published maps, (iv) compare topographical similarities of group and grand mean microstate maps and quantify shared variances, (v) obtain the temporal dynamics of the microstate classes in individual EEGs, (vi) export quantifications of these temporal dynamics of the microstates for statistical tests, and finally, (vii) test for topographical differences between groups and conditions using topographic analysis of variance (TANOVA). Here, we introduce the toolbox in a step-by-step tutorial, using a sample dataset of 34 resting-state EEG recordings that are publicly available to follow along with this tutorial. The goals of this manuscript are (a) to provide a standardized, freely available toolbox for resting-state microstate analysis to the scientific community, (b) to allow researchers to use best practices for microstate analysis by following a step-by-step tutorial, and (c) to improve the methodological standards of microstate research by providing previously unavailable functions and recommendations on critical decisions required in microstate analyses
QRS complex and T wave planarity for the efficacy prediction of automatic implantable defibrillators.
Get PDFOBJECTIVE
To test the hypothesis that in recipients of primary prophylactic implantable cardioverter-defibrillators (ICDs), the non-planarity of ECG vector loops predicts (a) deaths despite ICD protection and (b) appropriate ICD shocks.
METHODS
Digital pre-implant ECGs were collected in 1948 ICD recipients: 21.4% females, median age 65 years, 61.5% ischaemic heart disease (IHD). QRS and T wave three-dimensional loops were constructed using singular value decomposition that allowed to measure the vector loop planarity. The non-planarity, that is, the twist of the three-dimensional loops out of a single plane, was related to all-cause mortality (n=294; 15.3% females; 68.7% IHD) and appropriate ICD shocks (n=162; 10.5% females; 87.7% IHD) during 5-year follow-up after device implantation. Using multivariable Cox regression, the predictive power of QRS and T wave non-planarity was compared with that of age, heart rate, left ventricular ejection fraction, QRS duration, spatial QRS-T angle, QTc interval and T-peak to T-end interval.
RESULTS
QRS non-planarity was significantly (p<0.001) associated with follow-up deaths despite ICD protection with HR of 1.339 (95% CI 1.165 to 1.540) but was only univariably associated with appropriate ICD shocks. Non-planarity of the T wave loop was the only ECG-derived index significantly (p<0.001) associated with appropriate ICD shocks with multivariable Cox regression HR of 1.364 (1.180 to 1.576) but was not associated with follow-up mortality.
CONCLUSIONS
The analysed data suggest that QRS and T wave non-planarity might offer distinction between patients who are at greater risk of death despite ICD protection and those who are likely to use the defibrillator protection
Towards an operational definition of oral frailty: A e-Delphi study.
Get PDFOBJECTIVES
Building upon our recently developed conceptual definition of oral frailty (the age-related functional decline of orofacial structures), this e-Delphi study aims to develop an operational definition of oral frailty by identifying its components.
METHODS
We used a modified e-Delphi study to reach a consensus among international experts on the components of oral frailty. Twelve out of fifteen invited experts in the field of gerodontology participated. Experts responded to three rounds of an online 5-point scale questionnaire of components to be included or excluded from the operational definition of oral frailty. After each round, scores and rationales were shared with all experts, after which they could revise their position. A consensus was reached when at least 70% of the experts agreed on whether or not a component should be included in the operational definition of oral frailty.
RESULTS
The experts achieved a high level of agreement (80 - 100%) on including eight components of oral frailty and excluding nineteen. The operational definition of oral frailty should include the following components: 1) difficulty eating hard or tough foods, 2) inability to chew all types of foods, 3) decreased ability to swallow solid foods, 4) decreased ability to swallow liquids, 5) overall poor swallowing function, 6) impaired tongue movement, 7) speech or phonatory disorders, and 8) hyposalivation or xerostomia.
CONCLUSION
This e-Delphi study provided eight components that make up the operational definition of oral frailty. These components are the foundation for the next stage, which involves developing an oral frailty assessment tool
Type 2 T cell responses against distinct epitopes of the desmoglein 3 ectodomain in pemphigus vulgaris.
Get PDFPemphigus vulgaris (PV) is an autoimmune blistering disorder of the skin and/or mucous membranes caused by IgG autoantibodies which predominantly target two transmembrane desmosomal cadherins, desmoglein (Dsg)1 and Dsg3. Dsg-specific T cells play a central role in PV pathogenesis as they provide help to autoreactive B cells for autoantibody production. We here characterized Dsg3-specific peripheral T cells in a cohort of 52 PV patients and 41 healthy controls (HC) with regard to cytokine profile and epitope specificity. By ELISpot analysis, type 2 T cells reactive with the Dsg3 ectodomain were significantly increased in PV patients compared to HC. By dextramer analysis, CD4+ T cells specific for an epitope within the extracellular domain of Dsg3, Dsg3(206-220), were found at significantly higher frequencies in PV patients than in HLA-matched HC. T cell recognition of two distinct Dsg3 epitopes, i.e. Dsg3(206-220) and Dsg3(378-392), correlated significantly suggesting a synergistic effect in B cell help. Immunization of HLA-DRB1*04:02-transgenic PV mice with the same set of Dsg3 peptides induced pathogenic Dsg3-specific IgG antibodies which induced loss of keratinocyte adhesion in vitro. Thus, Dsg3 peptide-specific T cells are of particular interest as surrogate marker of disease activity and potential therapeutic targets in PV
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