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INTRODUCTION: Slide tracheoplasty has become the gold standard surgery for congenital tracheal stenosis (CTS). This condition is rare and the surgery can be challenging and is performed by experienced surgeons in tertiary centers. A few reports involving relatively small cohorts have been published. The aim of this review is to evaluate the post-operative mortality and morbidity of pediatric slide tracheoplasty for CTS. METHODS: A systematic literature review was performed according to PRISMA guidelines. The Medline and EMBASE databases were screened using a search strategy defined in collaboration with a librarian. We included articles reporting the post-operative mortality rate of slide tracheoplasties for treatment of CTS in children, when at least 10 patients were included. RESULTS: A total of 932 articles were reviewed, and 15 studies were eligible with a total of 845 patients. The overall post-operative mortality rate was 9.3 %, and most deaths were airway related. The open revision surgery rate after surgery was 2.8 % and the endoscopic revision rate was 27.6 %. DISCUSSION: This study highlights key factors to consider before the surgery and helps anticipate post-operative follow-up considerations for children with CTS. Several factors were identified as predictors of mortality including young age, weight at the time of surgery and association with lung hypoplasia or aplasia. CONCLUSION: Although slide tracheoplasty has gained popularity in recent years due to better outcomes, it remains a major surgery with mortality risk and the need for multidisciplinary management.
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Procedimentos de Cirurgia Plástica , Traqueia , Estenose Traqueal , Humanos , Estenose Traqueal/cirurgia , Estenose Traqueal/congênito , Procedimentos de Cirurgia Plástica/métodos , Traqueia/cirurgia , Traqueia/anormalidades , Resultado do Tratamento , Complicações Pós-Operatórias/epidemiologia , Criança , Reoperação/estatística & dados numéricosRESUMO
HYPOTHESIS: Resonance frequency analysis (RFA) is a reliable, noninvasive method to assess the stability of bone-anchored hearing implants (BAHIs), although surgical-, implant-, and host-related factors can affect its outcome. BACKGROUND: BAHI plays an important role in restoring hearing function. However, implant- and host-related factors contribute to premature implant extrusion. To mitigate this, noninvasive methods to assess implant stability, along with a better understanding of factors contributing to BAHI failure, are needed. METHODS: We evaluated the utility of RFA to quantify implant stability in sawbone (bone mimicking material), 29 human cadaveric samples, and a prospective cohort of 29 pediatric and 27 adult participants, and identified factors associated with implant stability. To validate the use of RFA in BAHI, we compared RFA-derived implant stability quotient (ISQ) estimates to peak loads obtained from mechanical push-out testing. RESULTS: ISQ and peak loads were significantly correlated (Spearman rho = 0.48, p = 0.0088), and ISQ reliably predicted peak load up to 1 kN. We then showed that in cadaveric samples, abutment length, internal table bone volume, and donor age were significantly associated with implant stability. We validated findings in our prospective patient cohort and showed that minimally invasive Ponto surgery (MIPS; versus linear incision), longer implantation durations (>16 wk), older age (>25 yr), and shorter abutment lengths (≤10 mm) were associated with better implant stability. Finally, we characterized the short-term reproducibility of ISQ measurements in sawbone and patient implants. CONCLUSIONS: Together, our findings support the use of ISQ as a measure of implant stability and emphasize important considerations that impact implant stability, including surgical method, implant duration, age, and abutment lengths.
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Auxiliares de Audição , Análise de Frequência de Ressonância , Humanos , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Criança , Adolescente , Estudos Prospectivos , Prótese Ancorada no Osso , Adulto Jovem , Idoso , Âncoras de Sutura , CadáverRESUMO
COVID-19 accelerated continuing professional development (CPD) delivered online. We aimed to compare the impact of in-person versus online CPD courses on medical specialists' behavioural intentions and subsequent behaviour. In this comparative before-and-after study, medical specialists attended in-person courses on nine clinical topics. A second group attended an adapted online version of these courses. Behavioural intention and its psychosocial determinants were measured before and immediately after the courses. Behaviour change was measured six months later. Generalised estimating equation (GEE) models were used to compare the impact of course formats. A total of 82/206 in-person registrants (mean age: 52±10 years; 50% men) and 318/506 on-line registrants (mean age: 49±12 years; men: 63%) participated. Mean intention before in-person courses was 5.99±1.31 and 6.43±0.80 afterwards (average intention gain 0.44, CI: 0.16-0.74; p=0.003); mean intention before online courses was 5.53±1.62 and 5.98±1.40 afterwards (average intention gain of 0.45, CI: 0.30-0.58; p<0.0001). Difference in intention gain between groups was not statistically significant. Behaviour reported six months later was not significantly associated with post-course intention in either group. However, the intention difference increased significantly among those who said they had adopted the targeted behaviour (paired wilcoxon test: n = 40 and p-value=0.002) while it did not increase significantly in the group of those who had not adopted a targeted behaviour (paired wilcoxon test: n = 16 and p-value=0.223). In conclusion, the increase in intention of specialists after CPD courses was similar whether the course was in-person or online. Also, an increase in intention in both groups signalled more likelihood of adoption.
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BACKGROUND: The onset of the COVID-19 pandemic catalysed a monumental shift in the field of continuing professional development (CPD). Prior to this, the majority of CPD group-learning activities were offered in-person. However, the pandemic forced the field to quickly pivot towards more novel methods of learning and teaching in view of social distancing regulations. The purpose of this study was to obtain the perspectives of CPD leaders on the impact of the pandemic to elucidate trends, innovations, and potential future directions in the field. METHODS: Semi-structured interviews were conducted between April-September 2022 with 23 CPD leaders from Canada and the USA. Interviews were audio-recorded, transcribed, and de-identified. A thematic analysis approach was used to analyse the data and generate themes. RESULTS: Participants characterised COVID-19 as compelling widespread change in the field of CPD. From the interviews, researchers generated six themes pertaining to the impact of the pandemic on CPD: (1) necessity is the mother of innovation, (2) the paradox of flexibility and accessibility, (3) we're not going to unring the bell, (4) reimagining design and delivery, (5) creating an evaluative culture, and (6) a lifeline in times of turmoil. CONCLUSION: This qualitative study discusses the impact of the pandemic on the field of CPD and leaders' vision for the future. Despite innumerable challenges, the pandemic created opportunities to reform design and delivery. Our findings indicate a necessity to maintain an innovative culture to best support learners, to improve the healthcare system, and to prepare for future emergencies.
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COVID-19 , Educação Médica Continuada , Pesquisa Qualitativa , Humanos , COVID-19/epidemiologia , Canadá , Estados Unidos , Pandemias , SARS-CoV-2 , Feminino , Entrevistas como Assunto , Masculino , Liderança , Desenvolvimento de PessoalRESUMO
OBJECTIVES: Recurrent and primary tracheoesophageal fistulas (TEFs) are a challenging surgical pathology to treat, as standard open surgical approaches are associated with high morbidity and mortality. As such, endoscopic modalities have gained interest as an alluring alternative, yet variable success rates have been reported in the literature. The aim of this study was to provide a contemporary update of the literature and describe our institutional experience with the bronchoscopic obliteration of recurrent and primary TEFs. METHODS: Retrospective chart review of all pediatric patients having undergone endoscopic TEF repair at two pediatric academic centers in Montreal, Canada and Lille, France between January 1, 2008 to December 31, 2020. RESULTS: 28 patients with TEFs (20 recurrent, 8 primary) underwent a total of 48 endoscopic procedures. TEF repair was performed under endoscopic guidance using various combinations of techniques, including fistula de-epithelialization (endoscopic brush, thulium laser, trichloroacetic acid-soaked pledgets or electrocautery), tissue adhesives, submucosal augmentation, esophageal clip and stenting. Successful closure was achieved in 16 patients (57 %), while 12 (43 %) required eventual open or thoracoscopic repair. The mean number of endoscopic procedures was 1.7. There were no major treatment-related complications such as pneumothorax, mediastinitis or death (mean follow-up 50.8 months). CONCLUSIONS: Endoscopic repair of recurrent or primary TEFs is a valuable component of our therapeutic armamentarium and may contribute to decreased surgical morbidity in this complex patient population. Families should be counselled that endoscopic results may be more modest than with open or thoracoscopic approaches, and multiple procedures may be required.
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Broncoscopia , Fístula Traqueoesofágica , Fístula Traqueoesofágica/cirurgia , Humanos , Estudos Retrospectivos , Masculino , Feminino , Broncoscopia/métodos , Criança , Pré-Escolar , Lactente , Resultado do Tratamento , Recidiva , AdolescenteRESUMO
Background: ChatGPT is an artificial intelligence tool, which utilizes machine learning to analyze and generate human-like text. The user-friendly accessibility of this tool enables patients conveniently access medical information without intricate terminology challenges. The objective of this study was to assess the accuracy of ChatGPT in providing insights into indications and management of complications after tonsillectomy, a common pediatric otolaryngology procedure. Methods: The responses generated by ChatGPT were compared to the "Clinical practice guidelines: tonsillectomy in children-executive summary" developed by the American Academy of Otolaryngology-Head and Neck Surgery Foundation (AAO-HNSF). An assessment was carried out by presenting predetermined questions regarding indications and complications post tonsillectomy to ChatGPT, followed by a comparison of its responses with the established guideline by 2 otolaryngology experts. The responses of both parties were reviewed by the senior author. Results: A total of 16 responses generated by ChatGPT were assessed. After a comprehensive review, it was concluded that 15 out of 16 (93.8%) responses demonstrated a high degree of reliability and accuracy, closely adhering to the standard established by the AAO-HNSF guideline. Conclusion: The results validate the potential of using ChatGPT to enhance healthcare delivery making guidelines more accessible to patients while also emphasizing the importance of ensuring the provision of accurate and reliable medical advice to patients.
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INTRODUCTION: Sialorrhea, also known as drooling, hypersalivation, or ptyalism, has a significant impact on the medical and psychosocial well-being of children. Onabotulinum toxin A (BoNT-A) is the most commonly used botulinum toxin worldwide for the treatment of sialorrhea in children. OBJECTIVES: To conduct a comprehensive systematic review and meta-analysis to assess the clinical efficacy and potential adverse effects of BoNT-A as a treatment for drooling in children. METHODS: Cochrane, Embase, and Medline databases were systematically searched (up to May 2023). Out of 535 identified publications, 20 were found eligible for inclusion. A systematic review and meta-analysis were performed to determine the efficacy of BoNT-A treatment in children in reducing the frequency and severity of drooling. RESULTS: Out of the 20 studies included, a meta-analysis was conducted on the complete dataset of eight studies involving 131 patients. BoNT-A was found to significantly decrease the severity of drooling in patients with sialorrhea (standardized mean difference [SMD], -2.07; 95% confidence interval [CI], -2.91 to -1.23; p < 0.0001) when compared with the conditions before injections using random-effects models. Six studies out of 20 reported dysphagia as an adverse effect after injection. Other side effects included thickness of saliva and pain at the site of injection. CONCLUSION: BoNT-A is a clinically effective therapy that improves drooling severity in children with sialorrhea. Although there were some adverse side effects reported, they were transient and not severe. Future studies are needed to further evaluate the best techniques and to identify the ideal dosages required to achieve the optimal outcomes. Laryngoscope, 134:3012-3017, 2024.
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Toxinas Botulínicas Tipo A , Sialorreia , Humanos , Sialorreia/tratamento farmacológico , Sialorreia/etiologia , Toxinas Botulínicas Tipo A/administração & dosagem , Toxinas Botulínicas Tipo A/uso terapêutico , Criança , Fármacos Neuromusculares/administração & dosagem , Fármacos Neuromusculares/uso terapêutico , Fármacos Neuromusculares/efeitos adversos , Resultado do Tratamento , Pré-Escolar , Adolescente , Masculino , FemininoRESUMO
The MOSIA is a novel minimally invasive endoscopic-assisted technique that has been shown to reduce postoperative morbidity and improve aesthetics. This is the first series to include children under the age of 12, as the FDA in the United States only approved OSIA for children 12 and older. Laryngoscope, 134:1901-1906, 2024.
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Condução Óssea , Auxiliares de Audição , Criança , Humanos , Estudos Retrospectivos , Próteses e Implantes , Perda Auditiva Condutiva/cirurgiaRESUMO
OBJECTIVE: To provide recommendations for a comprehensive management approach for infants and children presenting with symptoms or signs of aspiration. METHODS: Three rounds of surveys were sent to authors from 23 institutions worldwide. The threshold for the critical level of agreement among respondents was set at 80 %. To develop the definition of "intractable aspiration," each author was first asked to define the condition. Second, each author was asked to complete a 5-point Likert scale to specify the level of agreement with the definition derived in the first step. RESULTS: Recommendations by the authors regarding the clinical presentation, diagnostic considerations, and medical and surgical management options for aspiration in children. CONCLUSION: Approach to pediatric aspiration is best achieved by implementing a multidisciplinary approach with a comprehensive investigation strategy and different treatment options.
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Otolaringologia , Lactente , Criança , Humanos , Consenso , Inquéritos e Questionários , Técnica DelphiRESUMO
BACKGROUND: The emergence of ChatGPT, a state-of-the-art language model developed by OpenAI, has introduced a novel avenue for patients to seek medically related information. This technology holds significant promise in terms of accessibility and convenience. However, the use of ChatGPT as a source of accurate information enhancing patient education and engagement requires careful consideration. The objective of this study was to assess the accuracy and reliability of ChatGPT in providing information on the indications and management of complications post-tympanostomy, the most common pediatric procedure in otolaryngology. METHODS: We prompted ChatGPT-3.5 with questions and compared its generated responses with the recommendations provided by the latest American Academy of Otolaryngology-Head and Neck Surgery Foundation (AAO-HNSF) "Clinical Practice Guideline: Tympanostomy Tubes in Children (Update)". RESULTS: A total of 23 responses were generated by ChatGPT against the AAO-HNSF guidelines. Following a thorough review, it was determined that 22/23 (95.7%) responses exhibited a high level of reliability and accuracy, closely aligning with the gold standard. CONCLUSION: Our research study indicates that ChatGPT may be of assistance to parents in search of information regarding tympanostomy tube insertion and its clinical implications.
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BACKGROUND: Continuing professional development (CPD) for health professionals includes educational activities to maintain or improve skills. We evaluated the impact of a series of CPD courses by identifying factors influencing physicians' intention to adopt targeted behaviors and assessing self-reported behavior adoption six months later. METHODS: In this pre-post study, eligible participants attended at least one in-person course at the Fédération des Médecins Spécialistes du Québec annual meeting in November 2019. Before and afterwards, participants completed CPD-REACTION, a validated questionnaire based on Godin's integrated model for health professional behavior change that measures intention and psychosocial factors influencing intention. We used Wilcoxon signed-rank test to compare pre- and post-course intention scores and linear regression analyses to identify factors influencing intention. We also compared the post-course intention scores of participants reporting a behavior change six months later with the scores of those reporting no behavior change six months later. Qualitative data was collected only six months after courses and responses to open-ended questions were analyzed using the Theoretical Domains Framework. RESULTS: A total of 205/329 course attendees completed CPD-REACTION (response rate 62.3%). Among these participants, 158/329 (48%) completed the questionnaire before CPD courses, 129/329 (39.2%) only after courses and 47/329 (14.3%) at 6 months. Study population included 192 physicians of whom 78/192(40.6%) were female; 59/192(30.7%) were between 50 and 59 years old; and 72/192 (37.5%) were surgical specialist physicians. Mean intention scores before (n = 158) and after (n = 129) courses were 5.74(SD = 1.52) and 6.35(SD = 0.93) respectively. Differences in mean (DM) intention before and afterwards ranged from - 0.31(p = 0.17) to 2.25(p = 0.50). Multivariate analysis showed that beliefs about capabilities (ß = 0.15, p = 0.001), moral norm (ß = 0.75, p < 0.0001), and beliefs about consequences (ß = 0.11, p = 0.04) influenced post-course intention. Post-course intention was correlated with behavior six months later (DM = 0.63; p = 0.02). Qualitative analysis showed that facilitators to behavior adoption after six months were most often related to the TDF domains of beliefs about capabilities. Most frequent barriers to adoption related to lack of resources. CONCLUSIONS: Overall, scores for intention to adopt targeted behaviors increased after the courses. CPD providers could increase participants' intention by including interventions that emphasize beliefs about capabilities, moral norm and beliefs about consequences.
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Competência Clínica , Educação Médica Continuada , Intenção , Médicos , Médicos/psicologia , Humanos , Autorrelato , Quebeque , Masculino , Feminino , Pessoa de Meia-Idade , ComportamentoRESUMO
INTRODUCTION: The COVID-19 pandemic positioned healthcare systems in North America at the epicentre of the crisis, placing inordinate stress on clinicians. Concurrently, discussions about structural racism, social justice and health inequities permeated the field of medicine, and society more broadly. The confluence of these phenomena required rapid action from continuing professional development (CPD) leaders to respond to emerging needs and challenges. METHODS: In this qualitative study, researchers conducted 23 virtual semistructured interviews with CPD leaders in Canada and the USA. Interview audiorecordings were transcribed, deidentified and thematically analysed. RESULTS: This study revealed that the CPD leaders attributed the pandemic as illuminating and exacerbating problems related to clinician wellness; equity, diversity and inclusion; and health inequities already prevalent in the healthcare system and within CPD. Analysis generated two themes: (1) From heroes to humans: the shifting view of clinicians and (2) Melding of crises: an opportunity for systemic change in CPD. DISCUSSION: The COVID-19 pandemic increased recognition of burn-out and health inequities creating momentum in the field to prioritise and restrategise to address these converging public health crises. There is an urgent need for CPD to move beyond mere discourse on these topics towards holistic and sustainable actionable measures.
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OBJECTIVE: N-acetylcysteine (NAC) is an anti-oxidant and mucolytic effective against bacterial biofilms, making it useful in the treatment of chronically discharging ears that are unresponsive to traditional treatment methods. The objective of this study was to evaluate the otologic safety of intratympanic NAC combined with Ciprodex® in an animal model. METHODS: Baseline distortion product otoacoustic emissions (DPOAE) and auditory brainstem response (ABR) measurements were performed for both ears on thirteen guinea pigs from the animal care research facilities of the McGill University Health Center. This was followed by intratympanic administration of control solution (Ciprofloxacin 0.3%/Dexamethasone 0.1%) to the left ear and experimental solution (1.25% NAC/Ciprofloxacin 0.3%/Dexamethasone 0.1%) to the right ear. Three additional intratympanic injections were performed over the next fourteen days. DPOAE and ABR measurements were repeated 3-4 weeks after the initial procedure. Outcome measures included differences in DPOAE and ABR thresholds after intervention, clinical evidence of vestibular dysfunction and histological evidence of ototoxicity. RESULTS: There were no significant differences in the ABR thresholds and DPOAE results of the control and experimental ears at baseline and after intervention. There was neither clinical manifestation of vestibular dysfunction nor histological evidence of ototoxicity. CONCLUSION: Our study suggests that intratympanic 1.25% NAC with ciprofloxacin and dexamethasone is safe in guinea pigs and support its potential use in the treatment of chronically discharging ears. Further studies in humans are required to analyze its efficacy relative to conventional treatments. LEVEL OF EVIDENCE: Animal Research.
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Acetilcisteína , Ototoxicidade , Humanos , Animais , Cobaias , Acetilcisteína/farmacologia , Modelos Animais , Ciprofloxacina , DexametasonaRESUMO
OBJECTIVE: To evaluate and compare perioperative outcomes of percutaneous bone-anchored hearing implant (BAHI) surgery in syndromic and nonsyndromic pediatric patients. STUDY DESIGN: Retrospective cohort study. SETTING: McGill University Health Centre in Montreal, Quebec, Canada. PATIENTS: Forty-one pediatric patients (22 syndromic, 19 nonsyndromic) who underwent percutaneous BAHI surgery between March 2008 and April 2021. INTERVENTION: Percutaneous BAHI surgery. MAIN OUTCOME MEASURES: Patient demographics (age at surgery, gender, implant laterality), operative information (American Society of Anesthesia [ASA] score, anesthesia type, surgical technique, implant/abutment characteristics), and postoperative outcomes (implant stability, soft tissue integrity, surgical revisions, implant failure). RESULTS: The most frequent syndromes among implanted patients were Treacher Collins (27.3%), Goldenhar (13.6%), Trisomy 21 (13.6%), and Nager (9.1%). Syndromic patients were more frequently given higher ASA scores: 2 ( p = 0.003) and 3 ( p = 0.014). All cases of implant extrusion were in syndromic patients: two posttraumatic and two failures to osseointegrate. Nine (40.9%) syndromic patients experienced a Holgers Grade 4 skin reaction at one of their postoperative follow-up visits as compared to 0% of nonsyndromic patients ( p < 0.001). Implant stability was similar between cohorts at all postoperative time-points, except for significantly greater nonsyndromic implant stability quotient scores at 16 weeks ( p = 0.027) and 31+ weeks ( p = 0.016). CONCLUSIONS: Percutaneous BAHI surgery is a successful rehabilitation option in syndromic patients. However, it presents a relatively higher incidence of implant extrusion and severe postoperative skin reactions as compared to nonsyndromic patients. In light of these findings, syndromic patients may be great candidates for novel transcutaneous bone conduction implants.
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Prótese Ancorada no Osso , Auxiliares de Audição , Perda Auditiva , Humanos , Criança , Perda Auditiva/cirurgia , Perda Auditiva/etiologia , Auxiliares de Audição/efeitos adversos , Estudos Retrospectivos , Audição , Prótese Ancorada no Osso/efeitos adversos , Resultado do Tratamento , Âncoras de SuturaRESUMO
OBJECTIVE: To provide guidance for the comprehensive management of children referred for anterior drooling. The mission of the International Pediatric Otolaryngology Group (IPOG) is to develop expertise-based recommendations for the management of pediatric otolaryngologic disorders with the goal of improving patient care. METHODS: Survey of expert opinion by the members of the International Pediatric Otolaryngology Group (IPOG). The recommendations are derived from current expert consensus and critical review of the literature. RESULTS: Consensus recommendations include initial care and approach recommendations for health care providers who commonly evaluate children with drooling. This includes evaluation and treatment considerations for commonly debated issues in drooling management, initial work-up of children referred for anterior drooling, treatment recommendations, indications and contra-indications for rehabilitation, medical, and surgical management, as well as pros and cons of different surgical procedures in the hands of drooling management experts. CONCLUSION: Anterior drooling consensus recommendations are aimed at improving patient-centered care in children referred for sialorrhea.
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Toxinas Botulínicas Tipo A , Otolaringologia , Sialorreia , Criança , Humanos , Sialorreia/cirurgia , ConsensoRESUMO
INTRODUCTION: Non-tuberculous mycobacterial (NTM) infection commonly manifests as subacute or chronic cervicofacial lymphadenitis in immunocompetent children. The optimal management of this pathology remains controversial. OBJECTIVES: This international consensus guideline aims to understand the practice patterns for NTM cervicofacial lymphadenitis and to address the primary diagnostic and management challenges. METHODS: A modified three-iterative Delphi method was used to establish expert recommendations on the diagnostic considerations, expectant or medical management, and operative considerations. The recommendations herein are derived from current expert consensus and critical review of the literature. SETTING: Multinational, multi-institutional, tertiary pediatric hospitals. RESULTS: Consensus recommendations include diagnostic work-up, goals of treatment and management options including surgery, prolonged antibiotic therapy and observation. CONCLUSION: The recommendations formulated in this International Pediatric Otolaryngology Group (IPOG) consensus statement on the diagnosis and management of patients with NTM lymphadenitis are aimed at improving patient care and promoting future hypothesis generation.
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Linfadenite , Infecções por Mycobacterium não Tuberculosas , Otolaringologia , Criança , Humanos , Micobactérias não Tuberculosas , Linfadenite/microbiologia , Antibacterianos/uso terapêutico , Excisão de Linfonodo , Infecções por Mycobacterium não Tuberculosas/diagnósticoRESUMO
In the skeleton, osteoblasts and osteoclasts synchronize their activities to maintain bone homeostasis and integrity. Investigating the molecular mechanisms governing bone remodeling is critical and helps understand the underlying biology of bone disorders. Initially, we have identified the ubiquitin-specific peptidase gene (Usp53) as a target of the parathyroid hormone in osteoblasts and a regulator of mesenchymal stem cell differentiation. Mutations in USP53 have been linked to a constellation of developmental pathologies. However, the role of Usp53 in bone has never been visited. Here we show that Usp53 null mice have a low bone mass phenotype in vivo. Usp53 null mice exhibit a pronounced decrease in trabecular bone indices including trabecular bone volume (36%) and trabecular number (26%) along with an increase in trabecular separation (13%). Cortical bone parameters are also impacted, showing a reduction in cortical bone volume (12%) and cortical bone thickness (15%). As a result, the strength and mechanical bone properties of Usp53 null mice have been compromised. At the cellular level, the ablation of Usp53 perturbs bone remodeling, augments osteoblast-dependent osteoclastogenesis, and increases osteoclast numbers. Bone marrow adipose tissue volume increased significantly with age in Usp53-deficient mice. Usp53 null mice displayed increased serum receptor activator of NF-κB ligand (RANKL) levels, and Usp53-deficient osteoblasts and bone marrow adipocytes have increased expression of Rankl. Mechanistically, USP53 regulates Rankl expression by enhancing the interaction between VDR and SMAD3. This is the first report describing the function of Usp53 during skeletal development. Our results put Usp53 in display as a novel regulator of osteoblast-osteoclast coupling and open the door for investigating the involvement of USP53 in pathologies. © 2023 The Authors. Journal of Bone and Mineral Research published by Wiley Periodicals LLC on behalf of American Society for Bone and Mineral Research (ASBMR).
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Medula Óssea , Osteoblastos , Ligante RANK , Proteases Específicas de Ubiquitina , Animais , Camundongos , Adipócitos/metabolismo , Osso e Ossos/metabolismo , Medula Óssea/metabolismo , Diferenciação Celular/fisiologia , Homeostase , Osteoblastos/metabolismo , Osteoclastos/metabolismo , Osteogênese , Ligante RANK/metabolismo , Proteases Específicas de Ubiquitina/metabolismoRESUMO
We hereby present the first-reported pediatric case of a hard palate neurovascular hamartoma in a male newborn, in which the diagnosis was established following an initial nondiagnostic biopsy, extensive radiological investigations, and eventual wide local excision. These benign lesions can easily be mistaken for malignant diagnoses, leading to increased parental and child anxiety as well as avoidable diagnostic and therapeutic interventions.