RESUMO
Rationale: Conventionally considered irreversible, bronchiectasis has been demonstrated to be reversible in children in small studies. However, the factors associated with radiographic reversibility of bronchiectasis have yet to be defined. Objectives: In a large cohort of children with bronchiectasis, we aimed to determine: 1) if and to what extent bronchiectasis is reversible and 2) factors associated with radiographic chest high-resolution computed tomography (cHRCT) resolution. Methods: We identified children with bronchiectasis who had a repeat multidetector cHRCT scan between 2010 and 2021. We excluded those with cystic fibrosis, surgical pulmonary resection, traction bronchiectasis only, or lobar opacification. Measurements and Main Results: cHRCT scans were scored using the modified Reiff score (MRS) with a pediatric correction. Resolution was defined as an absence of abnormal bronchoarterial ratio (>0.8) on the second cHRCT scan. We included 142 children (median age, 5 years; IQR, 2.6-7.4). Inter- and intrarater agreement in MRSs was excellent (weighted κ = 0.83-0.86 and 0.95, respectively). There was radiographic resolution in 57 of 142 patients (40.1%), improvement in 56 of 142 (39.4%), and no change or worsening in 29 of 142 (20.4%). Pseudomonas aeruginosa (PsA) was absolutely associated with a lack of resolution. On multivariable regression, in those without PsA cultured, younger age at the time of diagnosis (risk ratio [RR], 0.94; 95% confidence interval [CI], 0.88-0.99), lower MRS (RR, 0.89; 95% CI, 0.82-0.97), and lower annual rate of exacerbations requiring intravenous antibiotic therapy (RR, 0.60; 95% CI, 0.37-0.98) increased the likelihood of radiographic resolution. Conclusions: This first large cohort confirms that bronchiectasis in children is often reversible with appropriate management. Younger children and those with lesser radiographic severity at diagnosis were most likely to exhibit radiographic reversibility, whereas those with PsA infection were least likely.
Assuntos
Bronquiectasia , Humanos , Bronquiectasia/diagnóstico por imagem , Masculino , Feminino , Criança , Pré-Escolar , Estudos Retrospectivos , Tomografia Computadorizada por Raios X/métodos , Estudos de Coortes , Tomografia Computadorizada Multidetectores/métodosRESUMO
BACKGROUND: The parent-proxy paediatric chronic cough quality of life questionnaire (PC-QoL) is a commonly used measure of spillover quality of life in parents of children with chronic cough. To date, spillover health utility in these parents is not routinely estimated largely due to the lack of a suitable instrument. Their perspective is not included in economic evaluations of interventions for their children. We explored developing a health state classification system based on the PC-QoL for measuring health utility spill over in this population. METHODS: This study included PC-QoL 8-item responses of 653 parents participating in a prospective cohort study about paediatric chronic cough. Exploratory factor analysis (EFA) and Rasch analysis were used to examine dimensionality and select potential items and level structure. RESULTS: EFA indicated that the PC-QoL had one underlying domain. Rasch analysis indicated threshold disordering in all items which improved when items were collapsed from seven to four levels. Two demonstrated differential item functioning (DIF) by diagnosis or ethnicity and were excluded from the final scale. This scale satisfied Rasch assumptions of local independence and unidimensionality and demonstrated acceptable fit to the Rasch model. It was presented to and modified by an expert panel and a consumer panel. The resulting classification system had six items, each with four levels. DISCUSSION: The PC-QoL can conform to a Rasch model with minor modifications. It may be a good basis for the classification system of a child cough-specific PBM. A valuation study is required to estimate preference weights for each item and to estimate health utility in parents of children with chronic cough.
Assuntos
Tosse , Psicometria , Qualidade de Vida , Humanos , Inquéritos e Questionários/normas , Tosse/psicologia , Feminino , Masculino , Criança , Doença Crônica , Estudos Prospectivos , Pais/psicologia , Pré-Escolar , Adolescente , Análise Fatorial , Adulto , Nível de SaúdeRESUMO
INTRODUCTION: The Queensland Basic Paediatric Training Network (QBPTN) is responsible for the selection of candidates into paediatric training in Queensland. The COVID-19 pandemic necessitated interviews to be conducted 'virtually' as virtual Multiple-Mini-Interviews (vMMI). The study aimed to describe the demographic characteristics of candidates applying for selection into paediatric training in Queensland, and to explore their perspectives and experiences with the vMMI selection tool. METHODOLOGY: The demographic characteristics of candidates and their vMMI outcomes were collected and analysed with a mixed methods approach. The qualitative component was comprised of seven semi-structured interviews with consenting candidates. RESULTS: Seventy-one shortlisted candidates took part in vMMI and 41 were offered training positions. The demographic characteristics of candidates at various stages of selection were similar. The mean vMMI scores were not statistically different between candidates from the Modified Monash Model 1 (MMM1) location and others [mean (SD): 43.5 (5.1) versus 41.7 (6.7), respectively, p = 0.26]. However, there was a statistically significant difference (p value 0.03) between being offered and not offered a training position for candidates from MMM2 and above. The analysis of the semi-structured interviews suggested that candidate experiences of the vMMI were influenced by the quality of the management of the technology used. Flexibility, convenience, and reduced stress were the main factors that influenced candidates' acceptance of vMMI. Perceptions of the vMMI process focused on the need to build rapport and facilitate communication with the interviewers. DISCUSSION: vMMI is a viable alternative to face-to-face (FTF) MMI. The vMMI experience can be improved by facilitating enhanced interviewer training, by making provision for adequate candidate preparation and by having contingency plans in place for unexpected technical challenges. Given government priorities in Australia, the impact of candidates' geographical location on the vMMI outcome for candidates from MMM >1 location needs to be further explored.
Assuntos
COVID-19 , Pandemias , Humanos , Criança , Queensland , Critérios de Admissão Escolar , DemografiaRESUMO
Individuals with Prader-Willi syndrome (PWS) often have excessive daytime sleepiness and emotional/behavioral disturbances. The objective of this study was to examine whether daytime sleepiness was associated with these emotional/behavioral problems, independent of nighttime sleep-disordered breathing, or the duration of sleep. Caregivers of individuals with PWS (aged 3 to 25 years) completed the Pediatric Sleep Questionnaire (PSQ), Epworth Sleepiness Scale for Children and Adolescents (ESS-CHAD), and the parent version of the Developmental Behavior Checklist (DBC-P). Sleep adequacy was adjusted for age by computing sleep duration against age-specific recommendations. The associations between ESS-CHAD and the total DBC and its subscale scores were evaluated by linear regression, adjusted for sleep-related breathing difficulties, sleep adequacy, and body mass index (BMI). There were 54 responses for individuals with PWS (including 22 males) aged 4.4-24.0 (mean 12.5) years. Daytime sleepiness predicted a substantial proportion of the variance in total DBC-P scores in the unadjusted model (28%; ß = 0.028; p < 0.001) and when adjusted for sleep adequacy, BMI, and sleep-related breathing difficulties (29%; ß = 0.023; p = 0.007). This relationship was not moderated by BMI Z-scores, but the relationship was more prominent for children younger than 12 years than for children older than 12 years.Conclusions: These findings provide preliminary novel evidence that daytime sleepiness may drive the expression of emotional/behavioral disturbances, and should be explored as a potential modifiable risk factor for these disturbances in PWS, particularly pre-adolescent children.
Assuntos
Distúrbios do Sono por Sonolência Excessiva , Síndrome de Prader-Willi , Comportamento Problema , Adolescente , Criança , Distúrbios do Sono por Sonolência Excessiva/complicações , Emoções , Humanos , Masculino , Síndrome de Prader-Willi/complicações , SonoRESUMO
AIM: In children with Prader-Willi syndrome (PWS), growth hormone (GH) improves height and body composition; however, may be associated with worsening sleep-disordered breathing (SDB). Some studies have reported less SDB after GH initiation, but follow-up with polysomnography is still advised in most clinical guidelines. METHODS: This retrospective, multicentre study, included children with PWS treated with GH at seven PWS treatment centres in Australia over the last 18 years. A paired analysis comparing polysomnographic measures of central and obstructive SDB in the same child, before and after GH initiation was performed with Wilcoxon signed-rank test. The proportion of children who developed moderate/severe obstructive sleep apnoea (OSA) was calculated with their binomial confidence intervals. RESULTS: We included 112 patients with available paired data. The median age at start of GH was 1.9 years (range 0.1-13.5 years). Median obstructive apnoea hypopnoea index (AHI) at baseline was 0.43/h (range 0-32.9); 35% had an obstructive AHI above 1.0/h. Follow-up polysomnography within 2 years after the start of GH was available in 94 children who did not receive OSA treatment. After GH initiation, there was no change in central AHI. The median obstructive AHI did not increase significantly (P = 0.13), but 12 children (13%, CI95% 7-21%) developed moderate/severe OSA, with clinical management implications. CONCLUSIONS: Our findings of a worsening of OSA severity in 13% of children with PWS support current advice to perform polysomnography after GH initiation. Early identification of worsening OSA may prevent severe sequelae in a subgroup of children.
Assuntos
Síndrome de Prader-Willi , Síndromes da Apneia do Sono , Adolescente , Austrália/epidemiologia , Criança , Pré-Escolar , Hormônio do Crescimento/uso terapêutico , Humanos , Lactente , Síndrome de Prader-Willi/complicações , Síndrome de Prader-Willi/tratamento farmacológico , Estudos Retrospectivos , Síndromes da Apneia do Sono/complicações , Síndromes da Apneia do Sono/tratamento farmacológicoRESUMO
INTRODUCTION: The Queensland Basic Paediatric Training Network (QBPTN) is the centralised pathway for entry into paediatric training in Queensland, Australia. In response to COVID-19 travel and social distancing restrictions imposed in 2020, QBPTN successfully adopted a Virtual Multiple Mini Interviews (vMMIs) model for the selection of candidates for entry into paediatric training. The authors describe the planning, implementation, challenges, and evaluation of candidates' and interviewers' experiences of vMMIs, including the differences between candidates from two geographical areas. METHODS: The contents of six vMMI stations were similar to face-to-face MMI. Implementation required the identification of ZOOMTM as a preferred online platform, securing venues, communication, development of contingency plans and central coordination by the network. Candidates' experiences with vMMI were explored through thematic analysis of the qualitative data from focus groups and free text responses, and descriptive analysis of SurveyMonkey© questionnaire responses. Experiences between 'metropolitan' and 'regional and interstate' candidates were compared. RESULTS: 5-minute stations with 2-minute pre-reading were used. 78 candidates and 14 interviewers participated in the selection process. All candidates attended the focus group. 58.7% of candidates responded to post vMMI questionnaire. 93% of survey responders were happy to undertake vMMI in the future, with 23% feeling they would have performed better in face-to-face. Experiences between 'metropolitan' and 'other' groups were similar. Positive experiences of participants were related to the user-friendly IT platform, successful pre-interview communications, preparation, convenience, time, and cost savings. Stress related IT failures and difficulties establishing rapport with interviewers were reported as the main negative experiences. CONCLUSION: 'vMMI' is a feasible and acceptable method of selection into paediatric training. vMMI has many benefits and can be implemented relatively quickly by addressing key logistical requirements. The model under discussion could be adapted by other centres based on local needs.
Assuntos
COVID-19 , Critérios de Admissão Escolar , Criança , Humanos , Queensland , SARS-CoV-2 , Inquéritos e QuestionáriosRESUMO
Chronic neonatal lung disease (CNLD) is defined as continued need for any form of respiratory support (supplemental oxygen and/or assisted ventilation) beyond 36 weeks PMA. Low-flow supplemental oxygen facilitates discharge from hospital of infants with CNLD who are hypoxic in air and is widely used despite lack of evidence on the most appropriate minimum mean target oxygen saturations. Furthermore, there are minimal data to guide the home monitoring, titration or weaning of supplemental oxygen in these infants. The purpose of this position statement is to provide a guide for the respiratory management of infants with CNLD, with special emphasis on role and logistics of supplemental oxygen therapy beyond the NICU stay. Reflecting a variety of clinical practices and infant comorbidities (presence of pulmonary hypertension, retinopathy of prematurity and adequacy of growth), it is recommended that the minimum mean target range for SpO2 during overnight oximetry to be 93-95% with less than 5% of total recording time to be below 90% SpO2 . Safety of short-term disconnection from supplemental oxygen should be assessed before discharge, with majority of infants with CNLD not ready for discharge until supplemental oxygen requirement is ≤0.5 L/min. Sleep-time assessment of oxygenation with continuous overnight oximetry is recommended when weaning supplemental oxygen. Palivizumab is considered safe and effective for the reduction of hospital admissions with RSV infection in this group. This statement would be useful for paediatricians, neonatologists, respiratory and sleep physicians and general practitioners managing children with CNLD.
Assuntos
Doenças do Recém-Nascido/terapia , Unidades de Terapia Intensiva Neonatal , Pneumopatias/terapia , Respiração , Austrália , Humanos , Recém-Nascido , Doenças do Recém-Nascido/epidemiologia , Doenças do Recém-Nascido/fisiopatologia , Pneumopatias/epidemiologia , Pneumopatias/fisiopatologia , Nova Zelândia , Oximetria , Oxigênio/metabolismo , Oxigenoterapia , Alta do Paciente , Sono , Resultado do TratamentoRESUMO
AIM: Aerodigestive clinics (ADCs) are multidisciplinary programmes for the care of children with complex congenital or acquired conditions affecting breathing, swallowing and growth. Our objective was to describe the demographic, clinical, etiological and investigational profile of children attending the inaugural ADC at a tertiary paediatric centre in Queensland. METHODS: Children referred to the ADC at Queensland Children's Hospital from August 2018 to December 2019 were included. Data on clinical, growth and lung function parameters, bronchoscopy and upper gastrointestinal endoscopy findings, thoracic imaging and comorbidities were retrospectively analysed. RESULTS: Fifty-six children (median (range) age 4 years (3 months-15 years); 18 female) attended the ADC during this 17-month period. Forty-six (82%) children had previous oesophageal atresia with tracheo-oesophageal fistula; 43 of these were type C. Previous isolated oesophageal atresia, congenital diaphragmatic hernia and congenital pulmonary malformation were the underlying disorder in three (5%) children each, with one child having a repaired laryngeal cleft. Vertebral Anal Tracheo Esophageal Renal Limb anomalies (VACTERL)/Vertebral Anal Tracheo Esophageal renal anomalies (VATER) association was seen in 21 (38%) children. Growth was adequate (median weight and body mass index z-score -0.63 and -0.48, respectively). Thirty-four (61%) children reported ongoing wet cough, with 12 (21%) requiring previous hospital admission for lower respiratory tract infection. Fourteen (25%) had bronchiectasis on computed tomography chest and 33 (59%) had clinical tracheomalacia, apparent on bronchoscopic examination in 21 patients. Dysphagia was reported in 15 (27%) children, 11 (20%) were gastrostomy feed-dependent and 5 (9%) had biopsy-proven eosinophilic oesophagitis. CONCLUSION: High proportion of children attending the ADC have ongoing respiratory symptoms resulting in chronic pulmonary suppuration and bronchiectasis. Potential benefits of this model of care need to be studied prospectively to better understand the outcomes.
Assuntos
Atresia Esofágica , Fístula Traqueoesofágica , Criança , Pré-Escolar , Atresia Esofágica/cirurgia , Feminino , Humanos , Queensland/epidemiologia , Estudos Retrospectivos , Traqueia , Fístula Traqueoesofágica/epidemiologia , Fístula Traqueoesofágica/cirurgiaRESUMO
Prader-Willi syndrome (PWS) is a rare genetic condition with multi-system involvement. The literature was reviewed to describe neurodevelopment and the behavioural phenotype, endocrine and metabolic disorders and respiratory and sleep functioning. Implications for child and family quality of life were explored. Challenging behaviours contribute to poorer well-being and quality of life for both the child and caregiver. Recent evidence indicates healthy outcomes of weight and height can be achieved with growth hormone therapy and dietary restriction and should be the current target for all individuals with PWS. Gaps in the literature included therapies to manage challenging behaviours, as well as understanding the effects of growth hormone on respiratory and sleep function. New knowledge regarding the transition of children and families from schooling and paediatric health services to employment, accommodation and adult health services is also needed. Developing a national population-based registry could address these knowledge gaps and inform advocacy for support services that improve the well-being of individuals with PWS and their families.
Assuntos
Família/psicologia , Satisfação Pessoal , Síndrome de Prader-Willi/fisiopatologia , Qualidade de Vida , Adolescente , Criança , Pré-Escolar , Humanos , HiperfagiaRESUMO
BACKGROUND: Bronchiectasis is being increasingly diagnosed and recognised as an important contributor to chronic lung disease in both adults and children in high- and low-income countries. It is characterised by irreversible dilatation of airways and is generally associated with airway inflammation and chronic bacterial infection. Medical management largely aims to reduce morbidity by controlling the symptoms, reduce exacerbation frequency, improve quality of life and prevent the progression of bronchiectasis. This is an update of a review first published in 2000. OBJECTIVES: To evaluate the efficacy and safety of inhaled corticosteroids (ICS) in children and adults with stable state bronchiectasis, specifically to assess whether the use of ICS: (1) reduces the severity and frequency of acute respiratory exacerbations; or (2) affects long-term pulmonary function decline. SEARCH METHODS: We searched the Cochrane Register of Controlled Trials (CENTRAL), the Cochrane Airways Group Register of trials, MEDLINE and Embase databases. We ran the latest literature search in June 2017. SELECTION CRITERIA: All randomised controlled trials (RCTs) comparing ICS with a placebo or no medication. We included children and adults with clinical or radiographic evidence of bronchiectasis, but excluded people with cystic fibrosis. DATA COLLECTION AND ANALYSIS: We reviewed search results against predetermined criteria for inclusion. In this update, two independent review authors assessed methodological quality and risk of bias in trials using established criteria and extracted data using standard pro forma. We analysed treatment as 'treatment received' and performed sensitivity analyses. MAIN RESULTS: The review included seven studies, involving 380 adults. Of the 380 randomised participants, 348 completed the studies.Due to differences in outcomes reported among the seven studies, we could only perform limited meta-analysis for both the short-term ICS use (6 months or less) and the longer-term ICS use (> 6 months).During stable state in the short-term group (ICS for 6 months or less), based on the two studies from which data could be included, there were no significant differences from baseline values in the forced expiratory volume in the first second (FEV1) at the end of the study (mean difference (MD) -0.09, 95% confidence interval (CI) -0.26 to 0.09) and forced vital capacity (FVC) (MD 0.01 L, 95% CI -0.16 to 0.17) in adults on ICS (compared to no ICS). Similarly, we did not find any significant difference in the average exacerbation frequency (MD 0.09, 95% CI -0.61 to 0.79) or health-related quality of life (HRQoL) total scores in adults on ICS when compared with no ICS, though data available were limited. Based on a single non-placebo controlled study from which we could not extract clinical data, there was marginal, though statistically significant improvement in sputum volume and dyspnoea scores on ICS.The single study on long-term outcomes (over 6 months) that examined lung function and other clinical outcomes, showed no significant effect of ICS on any of the outcomes. We could not draw any conclusion on adverse effects due to limited available data.Despite the authors of all seven studies stating they were double-blind, we judged one study (in the short duration ICS) as having a high risk of bias based on blinding, attrition and reporting of outcomes. The GRADE quality of evidence was low for all outcomes (due to non-placebo controlled trial, indirectness and imprecision with small numbers of participants and studies). AUTHORS' CONCLUSIONS: This updated review indicates that there is insufficient evidence to support the routine use of ICS in adults with stable state bronchiectasis. Further, we cannot draw any conclusion for the use of ICS in adults during an acute exacerbation or in children (for any state), as there were no studies.
Assuntos
Corticosteroides/administração & dosagem , Bronquiectasia/tratamento farmacológico , Administração por Inalação , Adulto , Androstadienos/administração & dosagem , Antibacterianos/administração & dosagem , Beclometasona/administração & dosagem , Bronquiectasia/prevenção & controle , Progressão da Doença , Fluticasona , Volume Expiratório Forçado , Humanos , Ensaios Clínicos Controlados Aleatórios como Assunto , Testes de Função Respiratória , Capacidade VitalAssuntos
Relações Pai-Filho , Zeladoria , Núcleo Familiar , Poder Familiar/psicologia , Médicos , Acidentes por Quedas , Humanos , CônjugesRESUMO
STUDY OBJECTIVES: There are limited data on indications and outcomes of home continuous positive airway pressure (CPAP) therapy in the first year of life. We aimed to analyze the clinical, demographic, and polysomnographic characteristics of a cohort of children initiated on home CPAP for treatment of sleep-disordered breathing and as respiratory support in the first year of life. METHODS: Children started on CPAP in the first year of life at the Queensland Children's Hospital were retrospectively evaluated for clinical and demographic parameters, underlying diagnoses, respiratory support, airway surgical intervention, and polysomnography results at baseline and on CPAP. RESULTS: Twenty-nine infants (median age [interquartile range] at CPAP initiation, 182 days [126-265.5 days]) were included. The underlying etiology included Trisomy 21 (n = 6), craniofacial syndromes (n = 5), hypotonia (n = 8; 5 with noncraniofacial syndrome), airway malacia (n = 5), skeletal dysplasia (n = 2), nonsyndromic upper airway obstruction (n = 2), and chronic neonatal lung disease (n = 1). The median (interquartile range) obstructive apnea-hypopnea index was 14 events/h (6.2-31 events/h) at CPAP initiation, which improved on CPAP to 3.4 events/h (1.4-6.4 events/h). The median (interquartile range) transcutaneous CO2 max remained unchanged on CPAP (56.6 mm Hg [49-66.5 mm Hg] pre-CPAP vs 54.9 mm Hg [47-62 mm Hg] on CPAP). Fifteen children needed surgical airway intervention (11 pre-CPAP and 4 post-CPAP). CPAP therapy could be successfully stopped in 9 children, 2 children needed tracheostomy, and 1 child died during the follow-up period. CONCLUSIONS: Home CPAP as respiratory support is an effective long-term therapy in infancy, and these patients can be weaned from CPAP therapy even if it was initiated early. Prospective studies with predefined criteria for CPAP initiation and cessation would help ascertain long-term outcomes in this poorly researched group. CITATION: Joshi SS, Sivapalan D, Leclerc M-J, Kapur N. Home continuous positive airway pressure therapy in infants: a single-center experience. J Clin Sleep Med. 2023;19(3):473-477.
Assuntos
Pneumopatias , Síndromes da Apneia do Sono , Criança , Recém-Nascido , Humanos , Lactente , Pressão Positiva Contínua nas Vias Aéreas/métodos , Estudos Retrospectivos , Estudos Prospectivos , Síndromes da Apneia do Sono/terapiaRESUMO
BACKGROUND: Bronchiectasis, a previously neglected condition, now has renewed research interest. There are a few systematic reviews that have reported on the economic and societal burden of bronchiectasis in adults, but none have reported on children. We undertook this systematic review to estimate the economic burden of bronchiectasis in children and adults. RESEARCH QUESTION: What is the health care resource utilization and economic burden of bronchiectasis in adults and children? STUDY DESIGN AND METHODS: We performed a systematic review identifying publications from Embase, PubMed, Web of Science, Cochrane (trials, reviews, and editorials), and EconLit about the economic burden and health care utilization in adults and children with bronchiectasis between January 1, 2001, and October 10, 2022. We used a narrative synthesis approach and estimated aggregate costs for several countries. RESULTS: We identified 53 publications reporting on the economic burden and/or health care utilization of people with bronchiectasis. Total annual health care costs per adult patient ranged from 2021 $3,579 to $82,545 USD and were predominantly driven by hospitalization costs. Annual indirect costs including lost income because of illness (reported in only five studies) ranged from $1,311 to $2,898 USD. Total health care costs in children with bronchiectasis were $23,687 USD annually in the one study that estimated them. Additionally, one publication found that children with bronchiectasis missed 12 school days per year. We estimated aggregate annual health care costs for nine countries, ranging from $101.6 million per year in Singapore to $14.68 billion per year in the United States. We also estimated the aggregate cost of bronchiectasis in Australian children to be $17.77 million per year. INTERPRETATION: This review highlights the substantial economic burden of bronchiectasis for patients and health systems. To our knowledge, it is the first systematic review to include the costs for children with bronchiectasis and their families. Future research to examine the economic impact of bronchiectasis in children and economically disadvantaged communities, and to further understand the indirect burden of bronchiectasis on individuals and the community, is needed.
Assuntos
Bronquiectasia , Efeitos Psicossociais da Doença , Adulto , Criança , Humanos , Estados Unidos , Estresse Financeiro , Austrália , Custos de Cuidados de Saúde , Bronquiectasia/epidemiologiaRESUMO
STUDY OBJECTIVES: Sleep-disordered breathing is a major cause of morbidity and mortality among pediatric patients with severe neurological disabilities such as cerebral palsy. Despite increasing use of noninvasive ventilation (NIV) in this group, there remains a lack of consensus about its role and indications. We aim to explore the indications, acceptability, and outcomes of a cohort of children with severe, complex neurological disability and sleep-disordered breathing, managed with NIV. METHODS: Data were retrospectively extracted on children with severe neurological disabilities (Gross Motor Function Classification System V equivalent) initiated on NIV in Queensland over a 5-year period. Demographic, clinical, hospitalization, and polysomnography data were collected, as well as caregiver-reported side effects and NIV adherence. RESULTS: Fourteen (median age 9.1 years; 6 female) children were included, 8 with cerebral palsy and 6 with other complex neurological disabilities. Obstructive sleep apnea was the most common indication for NIV (n = 12). The median (interquartile range) apnea-hypopnea index improved on NIV [pre-NIV 21.3 (interquartile range 10.0-28.2) vs post-NIV 12.2 (interquartile range 2.8-15.2)], although this was not statistically significant. There was significant improvement in proportion of time spent with SpO2 < 95% (22.2% pre-NIV vs 7.85% post-NIV; P < .05). Reported side effects were minimal. There was no reduction in hospital admissions in the 12 months post-NIV initiation. CONCLUSIONS: Our findings suggest that NIV improves polysomnography parameters among children with severe neurological disability. Long-term outcomes and overall impact on quality of life remain unclear. Ethical issues and overall benefit must be considered before embarking on this mode of therapy. CITATION: Morrison L, Suresh S, Leclerc MJ, Kapur N. Symptom care approach to noninvasive ventilatory support in children with complex neural disability. J Clin Sleep Med. 2022;18(4):1145-1151.
Assuntos
Ventilação não Invasiva , Síndromes da Apneia do Sono , Criança , Feminino , Humanos , Ventilação não Invasiva/efeitos adversos , Polissonografia , Qualidade de Vida , Estudos Retrospectivos , Síndromes da Apneia do Sono/terapiaRESUMO
STUDY OBJECTIVES: To describe the outcomes of central sleep apnea requiring home supplemental oxygen therapy in otherwise healthy term infants. METHODS: All children < 1 year of age undergoing polysomnography between 2015 and 2020 at the Queensland Children's Hospital were retrospectively studied. Children with gestational age < 37 weeks, underlying syndrome, cleft palate, those with obstructive apnea-hypopnea index > 50% of total apnea-hypopnea index, or with underlying cardiac or pulmonary parenchymal pathology were excluded. Polysomnography parameters were extracted for periods both on and off supplemental oxygenation. RESULTS: Fifty-two (mean [standard deviation] age at polysomnography 32.6 [34.7] days; 21 females) term infants were included. There was a statistically significant improvement in apnea-hypopnea index on supplemental oxygen (mean [standard deviation] in room air 50.2 [36.3] vs 11.6 [9], P < .001 on supplemental oxygen), in both rapid eye movement and nonrapid eye movement sleep, as well as in mean oxygen saturations (96.6% in room air to 98.9% on oxygen; P < .001). There was no statistically significant change in transcutaneous carbon dioxide levels or sleep duration. Oxygenation was prescribed for a median (interquartile range) age of 197 (127) days. CONCLUSIONS: Central sleep apnea in term infants who are otherwise healthy generally has a good prognosis, with oxygen therapy prescribed for around 6 months. Oxygen therapy was associated with improved saturations and decrease in apnea-hypopnea index when assessed with polysomnography. CITATION: Hayashi A, Suresh S, Kevat A, Robinson J, Kapur N. Central sleep apnea in otherwise healthy term infants. J Clin Sleep Med. 2022; 18(12):2813-2817.
Assuntos
Apneia do Sono Tipo Central , Apneia Obstrutiva do Sono , Lactente , Criança , Feminino , Humanos , Apneia do Sono Tipo Central/diagnóstico , Apneia do Sono Tipo Central/terapia , Apneia Obstrutiva do Sono/terapia , Estudos Retrospectivos , Polissonografia , OxigênioRESUMO
We report a child with persistently low oxygen saturations (SpO2 90%-92%) [normal SpO2 > 98%], with delayed diagnosis due to the co-existing congenital pulmonary airway malformation with possible arterio-venous malformation. The diagnosis was only achieved after low oxygen saturations incidentally discovered from the child's father. The eventual cause was Hemoglobin I-Toulouse, making both patients the first reported cases with low oxygen saturations.
RESUMO
We describe a 6-year-old girl with homozygous p.Phe508del cystic fibrosis with severe multi-lobar bronchiectasis and obstructive lung disease who was found to have prominent parenchymal calcifications in the right middle lobe on a computed tomography scan of the chest. Histopathology from the calcified area of lung biopsy showed fibrous tissue with chronic inflammation with CD3+ T-lymphocytes and macrophages with no granulomas. Dystrophic calcification was seen within this necrotic debris.