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1.
BMJ Paediatr Open ; 8(1)2024 02 05.
Artigo em Inglês | MEDLINE | ID: mdl-38316469

RESUMO

INTRODUCTION: Heterogeneity in reported outcomes of infants with oesophageal atresia (OA) with or without tracheo-oesophageal fistula (TOF) prevents effective data pooling. Core outcome sets (COS) have been developed for many conditions to standardise outcome reporting, facilitate meta-analysis and improve the relevance of research for patients and families. Our aim is to develop an internationally-agreed, comprehensive COS for OA-TOF, relevant from birth through to transition and adulthood. METHODS AND ANALYSIS: A long list of outcomes will be generated using (1) a systematic review of existing studies on OA-TOF and (2) qualitative research with children (patients), adults (patients) and families involving focus groups, semistructured interviews and self-reported outcome activity packs. A two-phase Delphi survey will then be completed by four key stakeholder groups: (1) patients (paediatric and adult); (2) families; (3) healthcare professionals; and (4) researchers. Phase I will include stakeholders individually rating the importance and relevance of each long-listed outcome using a 9-point Likert scale, with the option to suggest additional outcomes not already included. During phase II, stakeholders will review summarised results from phase I relative to their own initial score and then will be asked to rescore the outcome based on this information. Responses from phase II will be summarised using descriptive statistics and a predefined definition of consensus for inclusion or exclusion of outcomes. Following the Delphi process, stakeholder experts will be invited to review data at a consensus meeting and agree on a COS for OA-TOF. ETHICS AND DISSEMINATION: Ethical approval was sought through the Health Research Authority via the Integrated Research Application System, registration no. 297026. However, approval was deemed not to be required, so study sponsorship and oversight were provided by Alder Hey Children's NHS Foundation Trust. The study has been prospectively registered with the COMET Initiative. The study will be published in an open access forum.


Assuntos
Atresia Esofágica , Fístula Esofágica , Fístula Traqueoesofágica , Humanos , Criança , Projetos de Pesquisa , Técnica Delphi , Avaliação de Resultados em Cuidados de Saúde/métodos , Revisões Sistemáticas como Assunto , Metanálise como Assunto
2.
Pediatr Pulmonol ; 59(4): 880-885, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38165151

RESUMO

OBJECTIVE: To determine levels of moral distress in a pediatric unit caring for patients with tracheostomy/ventilator dependence. HYPOTHESIS: Moral distress will be significant in a dedicated pediatric trach/vent unit. METHODS: The Moral Distress Survey-Revised (MDS-R) is a 21-question survey measuring moral distress in pediatrics. The MDS-R was anonymously distributed to medical degree/doctor of osteopathy (MD/DOs), advanced practice practitioners (APPs), registered nurses (RNs), and respiratory therapists (RTs) in a unit caring for tracheostomy/ventilator dependent patients. Descriptive statistics, bivariate and multivariate analysis were performed. RESULTS: Response rate was 48% (61/127). Mean MDS-R score was 83 (range 43-119), which is comparable to reported levels in the pediatric intensive care unit (ICU). APPs had the highest median rate of moral distress (112, interquartile range [IQR], 72-138), while MD/DOs had the lowest median score (49, IQR, 43-77). RNs and RTs had MDS-R scores between these two groups (medians of 91 and 84, respectively). CONCLUSIONS: Moral distress levels in a unit caring for long term tracheostomy and ventilator dependent patients are high, comparable to levels in pediatric ICUs. APPs. APPs had higher levels of distress compared to other groups. This may be attributable to the constant stressors of being the primary provider for complex patients, especially in a high-volume inpatient setting.


Assuntos
Princípios Morais , Traqueostomia , Humanos , Criança , Estudos Transversais , Atitude do Pessoal de Saúde , Inquéritos e Questionários , Ventiladores Mecânicos , Estresse Psicológico
3.
Pediatr Pulmonol ; 59(2): 300-313, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-37937895

RESUMO

BACKGROUND: While bacteria identification on respiratory cultures is associated with poor short-term outcomes in children with bronchopulmonary dysplasia (BPD) and tracheostomies, the influence on longer-term respiratory support needs remains unknown. OBJECTIVE: To determine if respiratory culture growth of pathogenic organisms is associated with ongoing need for respiratory support, decannulation, and death at 3 years posttracheostomy placement in children with BPD and tracheostomies. METHODS: This single center, retrospective cohort study included infants and children with BPD and tracheostomies placed 2010-2018 and ≥1 respiratory culture obtained in 36 months posttracheostomy. Primary predictor was any pathogen identified on respiratory culture. Additional predictors were any Pseudomonas aeruginosa and chronic P. aeruginosa identification. Outcomes included continued use of respiratory support (e.g., oxygen, positive pressure), decannulation, and death at 3 years posttracheostomy. We used Poisson regression models to examine the relationship between respiratory organisms and outcomes, controlling for patient-level covariates and within-patient clustering. RESULTS: Among 170 children, 59.4% had a pathogen identified, 28.8% ever had P. aeruginosa, and 3.5% had chronic P. aeruginosa. At 3 years, 33.1% of alive children required ongoing respiratory support and 24.8% achieved decannulation; 18.9% were deceased. In adjusted analysis, any pathogen and P. aeruginosa were not associated with ongoing respiratory support or mortality. However, P. aeruginosa was associated with decreased decannulation probability (adjusted risk ratio 0.48, 95% CI 0.23-0.98). Chronic P. aeruginosa was associated with lower survival probability. CONCLUSION: Our findings suggest that respiratory pathogens including P. aeruginosa may not promote long-term respiratory dysfunction, but identification of P. aeruginosa may delay decannulation.


Assuntos
Displasia Broncopulmonar , Lactente , Recém-Nascido , Criança , Humanos , Displasia Broncopulmonar/cirurgia , Traqueostomia , Estudos Retrospectivos , Pulmão , Avaliação de Resultados em Cuidados de Saúde
4.
Laryngoscope ; 133(12): 3334-3340, 2023 12.
Artigo em Inglês | MEDLINE | ID: mdl-37159210

RESUMO

OBJECTIVES: Bronchial anomalies are rare but challenging conditions to treat in children, encompassing a variety of structural abnormalities that could compromise airway patency. This includes complete rings, absent cartilage, traumatic avulsions, bronchoesophageal fistulas, and cartilaginous sleeves. The objective of this study is to describe the characteristics and outcomes of a series of pediatric cases of bronchial anomalies that were treated by slide tracheobronchoplasty. METHODS: This is a single-institution retrospective case series of pediatric patients with bronchial anomalies who underwent surgical treatment between February 2004 and April 2020. Data extracted from electronic medical records included patient demographics, comorbidities, and surgical outcomes. RESULTS: There were a total of 29 patients included in the study, of which 14 had complete bronchial rings, 8 had absent bronchial rings, 4 had traumatic bronchial avulsions, 2 had bronchoesophageal fistulas, and one had a cartilaginous sleeve. Median follow-up time was 13 months (with a range of 0.5-213 months). The overall mortality rate was 17.2% (5 patients), all of whom had complete bronchial rings. Patients with complete bronchial rings also had a higher rate of not only cardiac (85.7%) and pulmonary comorbidities (85.7%) but also secondary airway lesions (78.6%). CONCLUSION: This is the largest series to date describing surgical treatment for bronchial anomalies. Complete bronchial rings were the most common anomaly treated, followed by absent rings and trauma. Surgical treatment can be successful but mortality rates are higher in patients with complete bronchial rings, possibly due to higher rates of pulmonary and cardiac comorbidities. LEVEL OF EVIDENCE: 4 Laryngoscope, 133:3334-3340, 2023.


Assuntos
Fístula , Procedimentos de Cirurgia Plástica , Estenose Traqueal , Criança , Humanos , Lactente , Traqueia/cirurgia , Traqueia/anormalidades , Estenose Traqueal/cirurgia , Estudos Retrospectivos , Fístula/cirurgia , Resultado do Tratamento
5.
Pediatr Pulmonol ; 58(5): 1481-1491, 2023 05.
Artigo em Inglês | MEDLINE | ID: mdl-36751142

RESUMO

BACKGROUND: Among children with tracheostomies, little is known about how respiratory culture results differ between states with and without acute respiratory infections (ARI), or the overall test performance of respiratory cultures. OBJECTIVE: To determine the association of respiratory culture organism isolation with diagnosis of ARI in children with tracheostomies, and assess test characteristics of respiratory cultures in the diagnosis of bacterial ARI (bARI). METHODS: This single-center, retrospective cohort study included respiratory cultures of children with tracheostomies obtained between 2010 and 2018. The primary predictor was ARI diagnosis code at the time of culture; the primary outcomes were respiratory culture organism isolation and species identified. Generalized estimating equations were used to assess for association between ARI diagnosis and isolation of any organism while controlling for potential confounders and accounting for within-patient clustering. A multinomial logistic regression equation assessed for association with specific species. Test characteristics were calculated using bARI diagnosis as the reference standard. RESULTS: Among 3578 respiratory cultures from 533 children (median 4 cultures/child, interquartile range (IQR): 1-9), 25.9% were obtained during ARI and 17.2% had ≥1 organism. Children with ARI diagnosis had higher odds of organism identification (adjusted odds ratio 1.29, 95%  confidence interval [CI] 1.16-1.44). When controlling for covariates, ARI was associated with isolation of Haemophilus influenzae, Moraxella catarrhalis, Streptococcus pneumoniae, and Streptococcus pyogenes. Test characteristics revealed a 24.3% sensitivity, 85.2% specificity, 36.5% positive predictive value, and 76.3% negative predictive value in screening for bARI. CONCLUSION: The utility of respiratory culture testing to screen for, diagnose, and direct treatment of ARI in children with tracheostomies is limited.


Assuntos
Infecções Respiratórias , Traqueostomia , Criança , Humanos , Lactente , Estudos Retrospectivos , Infecções Respiratórias/diagnóstico , Infecções Respiratórias/microbiologia , Streptococcus pneumoniae , Bactérias
6.
Pediatr Pulmonol ; 58(2): 433-440, 2023 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-36226360

RESUMO

BACKGROUND: Sharing data across institutions is critical to improving care for children who are using long-term mechanical ventilation (LTMV). Mechanical ventilation data are complex and poorly standardized. This lack of data standardization is a major barrier to data sharing. OBJECTIVE: We aimed to describe current ventilator data in the electronic health record (EHR) and propose a framework for standardizing these data using a common data model (CDM) across multiple populations and sites. METHODS: We focused on a cohort of patients with LTMV dependence who were weaned from mechanical ventilation (MV). We extracted and described relevant EHR ventilation data. We identified the minimum necessary components, termed "Clinical Ideas," to describe MV from time of initiation to liberation. We then utilized existing resources and partnered with informatics collaborators to develop a framework for incorporating Clinical Ideas into the PEDSnet CDM based on the Observational Medical Outcomes Partnership (OMOP). RESULTS: We identified 78 children with LTMV dependence who weaned from ventilator support. There were 25 unique device names and 28 unique ventilation mode names used in the cohort. We identified multiple Clinical Ideas necessary to describe ventilator support over time: device, interface, ventilation mode, settings, measurements, and duration of ventilation usage per day. We used Concepts from the SNOMED-CT vocabulary and integrated an existing ventilator mode taxonomy to create a framework for CDM and OMOP integration. CONCLUSION: The proposed framework standardizes mechanical ventilation terminology and may facilitate efficient data exchange in a multisite network. Rapid data sharing is necessary to improve research and clinical care for children with LTMV dependence.


Assuntos
Registros Eletrônicos de Saúde , Respiração Artificial , Criança , Humanos , Ventiladores Mecânicos , Fenômenos Fisiológicos Respiratórios
7.
Paediatr Respir Rev ; 44: 3-10, 2022 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-36411238

RESUMO

The current available literature evaluating pediatric multidisciplinary aerodigestive programs for the management of aerodigestive disorders in infants was reviewed. Multidisciplinary aerodigestive programs have emerged to provide coordinated and comprehensive care for the growing population of children with aerodigestive conditions, including complex airway, pulmonary, gastrointestinal, and feeding disorders, which are prevalent among infants discharged from the neonatal intensive care unit (NICU). The team approach central to aerodigestive clinics offers a comprehensive diagnostic workup and unified management plan through consolidated interdisciplinary clinics, combined endoscopic procedures, and regular team discussions, leading to improved resource utilization and health care outcomes. We review common conditions presenting in the NICU that benefit from the aerodigestive model of care, including esophageal atresia, prematurity, bronchopulmonary dysplasia with or without tracheostomy or ventilator dependence, and dysphagia.


Assuntos
Transtornos de Deglutição , Atresia Esofágica , Doenças Respiratórias , Lactente , Recém-Nascido , Criança , Humanos , Transtornos de Deglutição/etiologia , Transtornos de Deglutição/terapia , Unidades de Terapia Intensiva Neonatal , Sistema Respiratório
8.
Pediatrics ; 149(6)2022 06 01.
Artigo em Inglês | MEDLINE | ID: mdl-35641471

RESUMO

BACKGROUND AND OBJECTIVES: Children requiring long-term mechanical ventilation are at high risk of mortality. Setting ventilator alarms may improve safety, but best practices for setting ventilator alarms have not been established. Our objective was to increase the mean proportion of critical ventilator alarms set for those children requiring chronic mechanical ventilation followed in our pulmonary clinic from 63% to >90%. METHODS: Using the Institute for Healthcare Improvement Model for Improvement, we developed, tested, and implemented a series of interventions using Plan-Do-Study-Act cycles. We followed our progress using statistical process control methods. Our primary interventions were: (1) standardization of the clinic workflow, (2) development of an algorithm to guide physicians in selecting and setting ventilator alarms, (3) updating that algorithm based on review of failures and inpatient testing, and (4) enhancing staff engagement to change the culture surrounding ventilator alarms. RESULTS: We collected baseline data from May 1 to July 13, 2017 on 130 consecutive patients seen in the pulmonary medicine clinic. We found that 63% of critical ventilator alarms were set. Observation of the process, standardization of workflow, and adaptation of an alarm algorithm led to an increase to 85.7% of critical alarms set. Through revising our algorithm to include an apnea alarm, and maximizing provider engagement, more than 95% of critical ventilator alarms were set, exceeding our goal. We sustained this improvement through January 2021. CONCLUSIONS: Our stepwise approach, including process standardization, staff engagement, and integration of an alarm algorithm, improved the use of ventilator alarms in chronically ventilated pediatric patients.


Assuntos
Respiração Artificial , Ventiladores Mecânicos , Algoritmos , Criança , Falha de Equipamento , Humanos , Padrões de Referência
9.
Pediatr Pulmonol ; 57(2): 560-566, 2022 02.
Artigo em Inglês | MEDLINE | ID: mdl-34816625

RESUMO

OBJECTIVE: With advances in technology, pediatric patients are sent home with chronic invasive mechanical ventilation (CIMV). Ventilation optimization and preparation for weaning are essential processes, however, there is no standard approach to weaning and titrating ventilator settings in children. There is little information in the literature on the utility of polysomnography (PSG) in weaning and titration of CIMV. Our objective is to review the role of PSG on titration and weaning of CIMV in children. METHODS: A retrospective review of medical records and polysomnograms was performed on CIMV dependent patients at Cincinnati Children's Hospital Medical Center from January 2011 to October 2017. Patients underwent overnight ventilator weaning or titration PSG. Subjects with less than 3 h of total sleep time were excluded. RESULTS: About 163 PSGs were divided into 97 PSGs for ventilator titration and 66 PSGs for ventilator weaning. Of the 97 ventilator titration PSGs, 59 (60.8%) had inadequate ventilation, 10 (10.3%) had inadequate oxygenation, 22 (22.7%) had significant tracheostomy leak, 13 (13.4%) had autocycling, 3 (3.1%) had failure to trigger, 4 (4.1%) had central sleep apnea, and 24 (25.8%) had adequate ventilation on current ventilator settings. Of the 66 ventilator weaning PSGs, 48 (72.7%) weaned to lower ventilator settings or off ventilator support and 18 (27.3%) did not wean. DISCUSSION: Our results indicate that PSG is useful in titration and weaning of ventilator settings in children with CIMV. Larger studies are needed to evaluate the subgroup of this population who would benefit most from PSG for assessment of adequate ventilator support.


Assuntos
Respiração Artificial , Desmame do Respirador , Criança , Humanos , Polissonografia , Traqueostomia , Desmame do Respirador/métodos , Ventiladores Mecânicos
10.
J Pediatr Nurs ; 61: 96-101, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33813374

RESUMO

PURPOSE: To describe the proportion of children with an index hospitalization in 2014 who had established long-term invasive ventilator dependence (LTVD), and determine regional variation in hospital length of stay, charges, and readmissions. DESIGN AND METHODS: Multicenter, longitudinal, retrospective cohort study using a recently established algorithm to identify children with LTVD from the Pediatric Health Information System database with an index hospitalization at least once during 2014, excluding normal newborn care or chemotherapy, and the subset with established LTVD. Hospitals were grouped by geographic regions. Analysis included descriptive statistics and multi-variable mixed modeling for length of stay, charges, and readmissions. RESULTS: Of the 615,883 unique children discharged from 45 children's hospitals in 2014, 2235 (0.4%) had established LTVD. Of these, 342 (15%) were hospitalized in the Northeast, 677 (30%) Midwest, 733 (32%) South and 481 (22%) West. Most had at least two complex chronic conditions (97%) and used a medical device for at least two body systems (71%). No statistically significant regional variation was found for length of stay, charges, or readmissions after adjustment for child demographics, admission type, disposition, primary diagnosis, ICU stay, and number of chronic conditions. CONCLUSIONS: This study characterized the population of children with LTVD hospitalized in 2014. No regional variation was found for length of stay, charges, or readmissions. PRACTICE IMPLICATIONS: Children with established LTVD make up a small subset of all children admitted to children's hospitals however, they require substantial, costly, multifaceted care as most have additional complex chronic conditions and require multiple medical devices.


Assuntos
Hospitalização , Traqueostomia , Criança , Hospitais Pediátricos , Humanos , Lactente , Recém-Nascido , Tempo de Internação , Estudos Retrospectivos
11.
Pediatr Pulmonol ; 56(6): 1635-1643, 2021 06.
Artigo em Inglês | MEDLINE | ID: mdl-33647193

RESUMO

BACKGROUND: The care of mechanically ventilated pediatric patients is complex and burdensome. It is essential to adequately support the family caregivers of these children to optimize outcomes; however, there is no literature describing interventions for caregivers in this population. RESEARCH QUESTION: This study described a novel behavioral health program and examined its impact on family caregiver engagement and psychological distress on a pediatric inpatient chronic ventilator unit. STUDY DESIGN AND METHODS: Electronic chart review was completed with retrospective and prospective participant enrollment for the purposes of longitudinal evaluation of caregiver engagement. For analytic purposes, participants were grouped into three categories: (1) preprogram, (2) postprogram, and (3) postprogram with completion of psychotherapy. RESULTS: The behavioral health program was associated with increased caregiver participation in rounds, t = 7.76, p = < .001. Parents who completed a course of psychotherapy within the behavioral health program demonstrated reduced time to training completion (F = 5.89; p < .01), higher staff-rated caregiver engagement (F = 3.69; p < .05), and significantly reduced levels of caregiver distress (t = 2.09; p < .05).


Assuntos
Cuidadores , Serviços de Assistência Domiciliar , Criança , Humanos , Estudos Prospectivos , Estudos Retrospectivos , Ventiladores Mecânicos
13.
Otolaryngol Head Neck Surg ; 165(6): 876-880, 2021 12.
Artigo em Inglês | MEDLINE | ID: mdl-33528307

RESUMO

OBJECTIVE: To determine if time to tracheostomy decannulation differs among children by socioeconomic status. STUDY DESIGN: Case series with chart review. SETTING: Tertiary pediatric medical center. METHODS: Patients (≤21 years old) who underwent tracheostomy from January 1, 2011, to December 31, 2016. Patients were divided into 2 groups based on their socioeconomic status (SES), low SES and high SES. Principal components analysis was used to create an index for SES using census data obtained by the US Census Bureau's American Community Survey 5 year data profile from 2013 to 2017. Statistical analysis was performed using a χ2 for categorical variables and Wilcoxon rank-sum test for continuous variables. A general linear model was constructed to control for clinical factors to understand the independent effect of SES on time to decannulation. RESULTS: In total, 215 patients were included; of these patients, 111 patients (52%) were included in the high-SES group and 104 patients (48%) were included in the low-SES group. There was a significant difference in the time to decannulation for children based on SES status, with those children in the low-SES group taking on average 10 months longer to decannulate (38.7 vs 28.0 months, P = .0007). Median follow-up was 44.1 months (interquartile range, 29.6-61.3 months). CONCLUSION: Health care disparities appear to exist among children undergoing decannulation of their tracheostomy tube. Patients with lower SES had a significantly longer time to decannulation than those with higher SES.


Assuntos
Remoção de Dispositivo , Disparidades em Assistência à Saúde , Classe Social , Traqueostomia , Pré-Escolar , Feminino , Humanos , Lactente , Modelos Lineares , Masculino , Análise de Componente Principal , Estatísticas não Paramétricas , Tempo para o Tratamento
14.
Laryngoscope ; 131(1): 201-204, 2021 01.
Artigo em Inglês | MEDLINE | ID: mdl-32282085

RESUMO

OBJECTIVES/HYPOTHESIS: Both flexible and rigid bronchoscopy can be used to assess tracheomalacia; however, there is limited evidence comparing the two techniques. The objective of this study was to compare flexible and rigid bronchoscopy for evaluating the location and severity of tracheomalacia in children. STUDY DESIGN: Retrospective case series. METHODS: This was a retrospective study of children with both flexible and rigid bronchoscopy under the same sedation. All bronchoscopies were reviewed by three bronchoscopists for the location and severity of tracheomalacia. The location of collapse was defined as upper, middle, or lower trachea, and the severity of collapse was defined as none (0%-25% collapse), mild/moderate (26%-75% collapse), and severe (>75% collapse). RESULTS: Twenty-one patients were recruited for this study with a variety of neonatal respiratory diseases. There was 94% agreement (κ = 0.64) for assessment of tracheomalacia in the upper trachea. However, agreement was only 75% (κ = 0.50) in the middle trachea and 76% (κ = 0.52) in the lower trachea. In the subset of patients without tracheostomy, agreement improved to 100%, 88%, and 82% for the upper, middle, and lower trachea, respectively. There was poor correlation for tracheomalacia severity in the middle trachea (ρ = 0.30, P = .2) and moderate in the lower trachea (ρ = 0.63, P = .002). CONCLUSIONS: Although there is moderate agreement between flexible and rigid bronchoscopy for evaluating the presence of tracheomalacia, there can be differences in the two techniques, particularly when assessing severity of airway collapse. Future studies will be needed to understand factors that result in the discordance of flexible and rigid bronchoscopy for assessing airway dynamics. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:201-204, 2021.


Assuntos
Broncoscópios , Broncoscopia , Traqueomalácia/patologia , Desenho de Equipamento , Feminino , Humanos , Lactente , Masculino , Estudos Retrospectivos , Índice de Gravidade de Doença
16.
Curr Opin Otolaryngol Head Neck Surg ; 28(6): 425-429, 2020 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-33027138

RESUMO

PURPOSE OF REVIEW: The aim of this review is to provide an update on the management of pediatric tracheostomies. RECENT FINDINGS: Recent literature has focused on optimization of care for children with tracheostomies including prevention and management of skin breakdown, timing of tracheostomy tube changes, the role of multidisciplinary and team-based approaches to education and management of tracheostomy patients, ideal timing of surveillance endoscopy, and the emerging role of telemedicine in the care of tracheostomy patients. SUMMARY: A focus on quality improvement and a systematic, team-based approach to care has the potential to improve the quality of care for pediatric tracheostomy patients.


Assuntos
Complicações Pós-Operatórias/prevenção & controle , Traqueostomia , Criança , Humanos , Equipe de Assistência ao Paciente , Melhoria de Qualidade
17.
Orphanet J Rare Dis ; 15(1): 204, 2020 08 06.
Artigo em Inglês | MEDLINE | ID: mdl-32762706

RESUMO

BACKGROUND: Perinatal and infantile hypophosphatasia (HPP) are associated with respiratory failure and respiratory complications. Effective management of such complications is of key clinical importance. In some infants with HPP, severe tracheobronchomalacia (TBM) contributes to respiratory difficulties. The objective of this study is to characterize the clinical features, investigations and management in these patients. METHODS: We report a case series of five infants with perinatal HPP, with confirmed TBM, who were treated with asfotase alfa and observed for 3-7 years. Additionally, we reviewed respiratory function data in a subgroup of patients with perinatal and infantile HPP included in the clinical trials of asfotase alfa, who required high-pressure respiratory support (positive end-expiratory pressure [PEEP] ≥6 cm H2O and/or peak inspiratory pressure ≥18 cm H2O) during the studies. RESULTS: The case series showed that TBM contributed significantly to respiratory morbidity, and prolonged respiratory support with high PEEP was required. However, TBM improved over time, allowing weaning of all patients from ventilator use. The review of clinical trial data included 20 patients and found a high degree of heterogeneity in PEEP requirements across the cohort; median PEEP was 8 cm H2O at any time and some patients presented with high PEEP (≥8 cm H2O) over periods of more than 6 months. CONCLUSION: In infants with HPP presenting with persistent respiratory complications, it is important to screen for TBM and initiate appropriate respiratory support and treatment with asfotase alfa at an early stage. TRIAL REGISTRATION: ClinicalTrials.gov numbers: NCT00744042 , registered 27 August 2008; NCT01205152 , registered 17 September 2010; NCT01176266 , registered 29 July 2010.


Assuntos
Hipofosfatasia , Insuficiência Respiratória , Traqueobroncomalácia , Terapia de Reposição de Enzimas , Humanos , Hipofosfatasia/tratamento farmacológico , Lactente , Testes de Função Respiratória
18.
Int J Pediatr Otorhinolaryngol ; 136: 110138, 2020 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-32544639

RESUMO

OBJECTIVES: SUBJECTS/METHODS: Moral distress is defined as "when one knows the right thing to do, but institutional constraints make it nearly impossible to pursue the right course of action". The Moral Distress Survey-Revised (MDS-R) is a validated 21-question survey measuring moral distress in pediatrics. The MDS-R was anonymously distributed to pediatric otolaryngology faculty and fellows at a tertiary institution. Descriptive statistics, bivariate and multivariate analysis were performed. RESULTS: Response rate was 89% (16/18). Overall MDS-R score was 40 (range 14-94), which is lower than that found in the literature for pediatric surgeons (reported mean 72), pediatric intensivists (reported means 57-86), and similar to pediatric oncologists (reported means 42-52). Fellows had a significantly higher level of moral distress than faculty (mean 69 vs. 26, p < 0.05). Factors leading to higher degrees of distress involved communication breakdowns and pressure from administration/insurance companies to reduce costs. CONCLUSION: Pediatric Otolaryngologists at our institution have lower degrees of moral distress compared to other pediatric subspecialists. Fellows had higher levels of distress compared to faculty. Further research is necessary to determine degrees of distress across institutions and to determine its impact on the wellness of pediatric otolaryngologists.


Assuntos
Princípios Morais , Estresse Ocupacional/psicologia , Otorrinolaringologistas/ética , Otorrinolaringologistas/psicologia , Pediatras/ética , Pediatras/psicologia , Angústia Psicológica , Adulto , Feminino , Inquéritos Epidemiológicos , Hospitais Pediátricos/ética , Humanos , Relações Interprofissionais/ética , Masculino , Pessoa de Meia-Idade , Estresse Ocupacional/diagnóstico , Ohio , Projetos Piloto
19.
Pediatr Pulmonol ; 55(8): 2150-2155, 2020 08.
Artigo em Inglês | MEDLINE | ID: mdl-32492284

RESUMO

BACKGROUND: Children who require chronic positive pressure ventilation (CPPV) are frequently hospitalized with acute respiratory infections. Although respiratory viral testing is often performed, it is unclear how positive results impact antibiotic use. We sought to assess the impact of respiratory viral testing on antibiotic use in hospitalized children on CPPV. METHODS: This retrospective cohort study included hospitalized children on CPPV who had respiratory viral polymerase chain reaction (RVP) testing on admission. Primary exposure was a positive RVP result; primary outcome was antibiotic de-escalation, defined as discontinuation of antibiotics or narrowing of antimicrobial spectra. To determine the independent association of positive RVP and antibiotic de-escalation, a generalized linear mixed effect model was used to account for within patient clustering and confounders defined a priori (blood and respiratory cultures, leukocytosis, bandemia, chest radiograph findings, aspiration risk, and recent admission). RESULTS: A total of 200 admissions representing 118 patients were included. A viral pathogen was identified in 46.5% (93/200) of admissions; rhinovirus was most frequently identified (61.5% of positive RVPs). Antibiotic de-escalation occurred in 33% of admissions (35.5% of RVP-positive admissions vs 30.8% of RVP-negative admissions; P = .49). In adjusted analysis, there was no association between positive RVP and antibiotics de-escalation (adjusted OR: 0.86; 95% confidence interval: 0.32-2.26). CONCLUSION: This single center cohort study suggests that respiratory viral testing may not impact antibiotic prescribing for hospitalized children on CPPV. There is need for improved stewardship of both diagnostic testing and antimicrobial use in this population.


Assuntos
Antibacterianos/uso terapêutico , Reação em Cadeia da Polimerase , Respiração com Pressão Positiva , Infecções Respiratórias/diagnóstico , Viroses/diagnóstico , Criança , Pré-Escolar , Feminino , Hospitalização , Humanos , Masculino , Padrões de Prática Médica , Infecções Respiratórias/terapia , Infecções Respiratórias/virologia , Estudos Retrospectivos , Viroses/terapia , Viroses/virologia , Vírus/genética
20.
Otolaryngol Head Neck Surg ; 162(3): 362-366, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-31959060

RESUMO

OBJECTIVE: To determine the optimal timing of the first posttracheostomy microlaryngoscopy and bronchoscopy (MLB). STUDY DESIGN: Case series with chart review. SETTING: Tertiary pediatric medical center. SUBJECTS AND METHODS: Patients (<21 years of age) who underwent tracheostomy placement from January 1, 2011, to December 31, 2016. Patients were divided into early and late posttracheostomy surveillance groups (<6 weeks vs 6 to 14 weeks, respectively) based on the timing of their first posttracheostomy MLB. The primary outcome was to ascertain the clinical yield of the initial posttracheostomy MLB by documenting whether a medical or surgical treatment decision was made based on MLB findings. RESULTS: In total, 202 patients were included; of these patients, 162 met criteria for placement in the early group and 40 met criteria for the late group. There was no significant difference between the early and late groups regarding whether a medical or surgical decision was made at the time of the first MLB (21.5% vs 19%, respectively; P = .49). Multiple logistic regression identified that the presence of tracheostomy-related symptoms prior to MLB (odds ratio, 6.75; 95% confidence interval, 2.78-16.39) was the sole predictor of a medical or surgical decision being made at the first posttracheostomy MLB. CONCLUSION: The presence of tracheostomy-related symptoms was predictive of a medical or surgical decision being made using information obtained at the time of the first posttracheostomy MLB. We thus recommend that surveillance endoscopy be initiated when tracheotomized children start to develop tracheostomy-related symptoms.


Assuntos
Broncoscopia , Laringoscopia , Vigilância da População , Cuidados Pós-Operatórios , Traqueostomia , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Fatores de Tempo
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