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1.
Pediatr Pulmonol ; 2024 Apr 25.
Artigo em Inglês | MEDLINE | ID: mdl-38661255

RESUMO

Pediatric sleep-related breathing disorders, or sleep-disordered breathing (SDB), cover a range of conditions, including obstructive sleep apnea, central sleep apnea, sleep-related hypoventilation disorders, and sleep-related hypoxemia disorder. Pediatric SDB is often underdiagnosed, potentially due to difficulties associated with performing the gold standard polysomnography in children. This scoping review aims to: (1) provide an overview of the studies reporting on safe, noncontact monitoring of respiration in young children, (2) describe the accuracy of these techniques, and (3) highlight their respective advantages and limitations. PubMed and EMBASE were searched for studies researching techniques in children <12 years old. Both quantitative data and the quality of the studies were analyzed. The evaluation of study quality was conducted using the QUADAS-2 tool. A total of 19 studies were included. Techniques could be grouped into bed-based methods, microwave radar, video, infrared (IR) cameras, and garment-embedded sensors. Most studies either measured respiratory rate (RR) or detected apneas; n = 2 aimed to do both. At present, bed-based approaches are at the forefront of research in noncontact RR monitoring in children, boasting the most sophisticated algorithms in this field. Yet, despite extensive studies, there remains no consensus on a definitive method that outperforms the rest. The accuracies reported by these studies tend to cluster within a similar range, indicating that no single technique has emerged as markedly superior. Notably, all identified methods demonstrate capability in detecting body movements and RR, with reported safety for use in children across the board. Further research into contactless alternatives should focus on cost-effectiveness, ease-of-use, and widespread availability.

2.
Sensors (Basel) ; 23(18)2023 Sep 05.
Artigo em Inglês | MEDLINE | ID: mdl-37765721

RESUMO

Unobtrusive monitoring of children's heart rate (HR) and respiratory rate (RR) can be valuable for promoting the early detection of potential health issues, improving communication with healthcare providers and reducing unnecessary hospital visits. A promising solution for wireless vital sign monitoring is radar technology. This paper presents a novel approach for the simultaneous estimation of children's RR and HR utilizing ultra-wideband (UWB) radar using a deep transfer learning algorithm in a cohort of 55 children. The HR and RR are calculated by processing radar signals via spectrogram from time epochs of 10 s (25 sample length of hamming window with 90% overlap) and then transforming the resultant representation into 2-dimensional images. These images were fed into a pre-trained Visual Geometry Group-16 (VGG-16) model (trained on ImageNet dataset), with weights of five added layers fine-tuned using the proposed data. The prediction on the test data achieved a mean absolute error (MAE) of 7.3 beats per minute (BPM < 6.5% of average HR) and 2.63 breaths per minute (BPM < 7% of average RR). We also achieved a significant Pearson's correlation of 77% and 81% between true and extracted for HR and RR, respectively. HR and RR samples are extracted every 10 s.

3.
Respir Care ; 68(4): 531-546, 2023 04.
Artigo em Inglês | MEDLINE | ID: mdl-36963967

RESUMO

BACKGROUND: Daily application of mechanical insufflation-exsufflation (MI-E) is used increasingly in patients with neuromuscular diseases (NMDs) to prevent pulmonary congestion and thereby respiratory tract infections, although its beneficial effect remains uncertain. We, therefore, conducted a systematic review, registered in PROSPERO (CRD42020158278), to compile available evidence for daily MI-E use in subjects with NMDs and stable respiratory condition. METHODS: We performed a systematic comprehensive search of MEDLINE, Embase, CINAHL, and Web of Science up to December 23, 2021. We excluded articles studying the effect of MI-E in case of acute respiratory failure or infections and studies comparing different MI-E devices and settings. Studied outcomes were prevalence and severity of respiratory infections, lung function, respiratory characteristics, and patient satisfaction. We performed a meta-analysis using DerSimonian-Laird random effects model and assessed methodological quality by using the Alberta Heritage Foundation for Medical Research tool. RESULTS: A total of 3,374 records were screened, of which 25 were included, studying 608 subjects. One randomized controlled trial (RCT) found a trend toward reduced duration of respiratory infections compared to air stacking (AS) that was not statistically significant. Long-term effects on pulmonary function tests (PFT) results were reported in one RCT and one retrospective study, with mixed results regarding vital capacity. Most studies compared PFT results before and immediately after MI-E use. Meta-analysis showed an overall beneficial effect of MI-E on cough peak flow (CPF) compared to unassisted CPF (mean difference 91.6 L/min [95% CI 28.3-155.0], P < .001). Subject satisfaction was high, though possibly influenced by major bias. CONCLUSIONS: There is limited evidence available to support beneficial effects of daily use of MI-E in clinically stable subjects with NMDs, with the possible exception of increased CPF immediately after MI-E application. Lack of longitudinal studies preclude conclusions regarding long-term effects. The very limited data comparing MI-E to AS preclude comparisons.


Assuntos
Insuflação , Infarto do Miocárdio , Doenças Neuromusculares , Infecções Respiratórias , Humanos , Insuflação/métodos , Respiração Artificial , Doenças Neuromusculares/complicações , Tosse , Ensaios Clínicos Controlados Aleatórios como Assunto
4.
BMC Neurol ; 23(1): 118, 2023 Mar 23.
Artigo em Inglês | MEDLINE | ID: mdl-36959618

RESUMO

BACKGROUND: Spinal Muscular Atrophy (SMA) is characterized by progressive and predominantly proximal and axial muscle atrophy and weakness. Respiratory muscle weakness results in impaired cough with recurrent respiratory tract infections, nocturnal hypoventilation, and may ultimately lead to fatal respiratory failure in the most severely affected patients. Treatment strategies to either slow down the decline or improve respiratory muscle function are wanting. OBJECTIVE: The aim of this study is to assess the feasibility and efficacy of respiratory muscle training (RMT) in patients with SMA and respiratory muscle weakness. METHODS: The effect of RMT in patients with SMA, aged ≥ 8 years with respiratory muscle weakness (maximum inspiratory mouth pressure [PImax] ≤ 80 Centimeters of Water Column [cmH2O]), will be investigated with a single blinded randomized sham-controlled trial consisting of a 4-month training period followed by an 8-month open label extension phase. INTERVENTION: The RMT program will consist of a home-based, individualized training program involving 30-breathing cycles through an inspiratory and expiratory muscle training device. Patients will be instructed to perform 10 training sessions over 5-7 days per week. In the active training group, the inspiratory and expiratory threshold will be adjusted to perceived exertion (measured on a Borg scale). The sham-control group will initially receive RMT at the same frequency but against a constant, non-therapeutic resistance. After four months the sham-control group will undergo the same intervention as the active training group (i.e., delayed intervention). Individual adherence to the RMT protocol will be reviewed every two weeks by telephone/video call with a physiotherapist. MAIN STUDY PARAMETERS/ENDPOINTS: We hypothesize that the RMT program will be feasible (good adherence and good acceptability) and improve inspiratory muscle strength (primary outcome measure) and expiratory muscle strength (key secondary outcome measure) as well as lung function, patient reported breathing difficulties, respiratory infections, and health related quality of life (additional secondary outcome measures, respectively) in patients with SMA. DISCUSSION: RMT is expected to have positive effects on respiratory muscle strength in patients with SMA. Integrating RMT with recently introduced genetic therapies for SMA may improve respiratory muscle strength in this patient population. TRIAL REGISTRATION: Retrospectively registered at clinicaltrial.gov: NCT05632666.


Assuntos
Atrofia Muscular Espinal , Qualidade de Vida , Humanos , Respiração , Exercícios Respiratórios/métodos , Atrofia Muscular Espinal/terapia , Debilidade Muscular , Força Muscular/fisiologia , Músculos Respiratórios/fisiologia , Ensaios Clínicos Controlados Aleatórios como Assunto
5.
Orphanet J Rare Dis ; 18(1): 40, 2023 02 23.
Artigo em Inglês | MEDLINE | ID: mdl-36823666

RESUMO

BACKGROUND: Progressive lung function decline, resulting in respiratory failure, is an important complication of spinal muscular atrophy (SMA). The ability to predict the need for mechanical ventilation is important. We assessed longitudinal patterns of lung function prior to chronic respiratory failure in a national cohort of treatment-naïve children and adults with SMA, hypothesizing an accelerated decline prior to chronic respiratory failure. METHODS: We included treatment-naïve SMA patients participating in a prospective national cohort study if they required mechanical ventilation because of chronic respiratory failure and if lung function test results were available from the years prior to initiation of ventilation. We analyzed Forced Vital Capacity (FVC), Forced Expiratory Volume in 1 s (FEV1), Peak Expiratory Flow (PEF) and Maximum Expiratory Pressure (PEmax). We studied the longitudinal course using linear mixed-effects models. We compared patients who electively started mechanical ventilation compared to patients who could not be weaned after acute respiratory failure. RESULTS: We analyzed 385 lung function tests from 38 patients with SMA types 1c-3a. At initiation of ventilation median age was 18.8 years (IQR: 13.2-30.1) and median standardized FVC, FEV1 and PEF were 28.8% (95% CI: 23.5; 34.2), 28.8% (95% CI: 24.0; 33.7) and 30.0% (95% CI: 23.4; 36.7), with an average annual decline of 1.75% (95% CI: 0.86; 2.66), 1.72% (95% CI: 1.04; 2.40) and 1.65% (95% CI: 0.71; 2.59), respectively. Our data did not support the hypothesis of an accelerated decline prior to initiation of mechanical ventilation. Median PEmax was 35.3 cmH2O (95% CI: 29.4; 41.2) at initiation of mechanical ventilation and relatively stable in the years preceding ventilation. Median FVC, FEV1, PEF and PEmax were lower in patients who electively started mechanical ventilation (p < 0.001). CONCLUSIONS: Patterns of lung function decline cannot predict impending respiratory failure: SMA is characterized by a gradual decline of lung function. We found no evidence for an accelerated deterioration. In addition, PEmax remains low and stable in the years preceding initiation of ventilation. Patients who electively started mechanical ventilation had more restrictive lung function at initiation of ventilation, compared to patients who could not be weaned after surgery or a respiratory tract infection.


Assuntos
Atrofia Muscular Espinal , Insuficiência Respiratória , Criança , Adulto , Humanos , Adolescente , Estudos Prospectivos , Estudos de Coortes , Pulmão , Capacidade Vital , Volume Expiratório Forçado
6.
Brain Commun ; 4(6): fcac269, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-36382221

RESUMO

Nusinersen (Spinraza®) improves survival of infants with hereditary proximal spinal muscular atrophy and motor function in children up to 12 years. Population-based assessments of treatment efficacy are limited and confined to select cohorts of patients. We performed a nationwide, population-based, single-centre cohort study in children with spinal muscular atrophy younger than 9.5 years at start of treatment in line with reimbursement criteria in the Netherlands. We assessed age-relevant motor function scores, the need for tube feeding, hours of ventilatory support and documented adverse events. We used linear mixed modelling to assess treatment effects. We compared motor function during treatment with natural history data and to individual trajectories of muscle strength and motor function before the start of treatment. We included 71 out of 72 Dutch children who were treated (median age 54 months; range 0-117) and followed them for a median of 38 months (range 5-52). We observed improvement of motor function in 72% and stabilization in another 18% of the symptomatic children, which differed from the natural disease course in a matched cohort of which we had previously collected natural history data. Longitudinal analysis showed that motor function improved up to a median of 24 months (range 12-30) of treatment after which it stabilized. Shorter disease duration at start of treatment resulted in better treatment efficacy (P < 0.01). Sixteen children (23%) achieved new motor milestones. Bulbar and respiratory function did not improve significantly during treatment. In 15 patients from whom treatment-naïve data were available, the pre-treatment trajectory of motor function decline changed to stabilization or improvement after the start of treatment. We documented 82 adverse events after 934 injections (9%) in 45 patients. None of the adverse events led to treatment discontinuation. Intrathecal nusinersen treatment is safe and improves or stabilizes motor function in 90% of young children with spinal muscular atrophy types 1c-3a. We did not observe improvement of respiratory and bulbar functions.

7.
Pediatr Pulmonol ; 57(12): 3050-3059, 2022 12.
Artigo em Inglês | MEDLINE | ID: mdl-36039838

RESUMO

BACKGROUND: Respiratory failure is a major cause of morbidity and mortality in patients with Spinal Muscular Atrophy (SMA). Lack of endurance, or "fatigability," is an important symptom of SMA. In addition to respiratory muscle weakness, respiratory function in SMA may be affected by Respiratory Muscle Fatigability (RMF). AIM: The purpose of this study was to explore RMF in patients with SMA. METHODS: We assessed a Respiratory Endurance Test (RET) in 19 children (median age [years]: 11) and 36 adults (median age [years]: 34) with SMA types 2 and 3. Participants were instructed to breath against an inspiratory threshold load at either 20%, 35%, 45%, 55%, or 70% of their individual maximal inspiratory mouth pressure (PImax). RMF was defined as the inability to complete 60 consecutive breaths. Respiratory fatigability response was determined by change in maximal inspiratory mouth pressure (ΔPImax) and perceived fatigue (∆perceived fatigue). RESULTS: The probability of RMF during the RET increased by 59%-69% over 60 breaths with every 10% increase in inspiratory threshold load (%PImax). Fatigability response was characterized by a large variability in ΔPImax (-21% to +16%) and a small increase in perceived fatigue (p = 0.041, range 0 to +3). CONCLUSION AND KEY FINDINGS: Patients with SMA demonstrate a dose-dependent increase in RMF without severe increase in exercise-induced muscle weakness or perceived fatigue. Inspiratory muscle loading in patients with SMA seems feasible and its potential to stabilize or improve respiratory function in patients with SMA needs to be determined in further research.


Assuntos
Fadiga Muscular , Atrofia Muscular Espinal , Músculos Respiratórios , Atrofias Musculares Espinais da Infância , Adulto , Criança , Humanos , Atrofia Muscular Espinal/complicações , Testes de Função Respiratória , Atrofias Musculares Espinais da Infância/complicações
8.
Pediatr Pulmonol ; 57(7): 1618-1624, 2022 07.
Artigo em Inglês | MEDLINE | ID: mdl-35441830

RESUMO

INTRODUCTION: Spirometry plays an important role in the assessment of possible respiratory failure in children with neuromuscular diseases (NMDs). However, obtaining reliable spirometry results is a major challenge. We studied the relation between oscillometry and spirometry results. Oscillometry is an easy, noninvasive method to measure respiratory resistance R and reactance X. We hypothesized an increased R and reduced X in patients with more reduced lung function. METHODS: In this prospective single-center study, we included all children with NMDs able to perform spirometry. We consecutively measured R and X at 5, 11, and 19 Hz and (forced) vital capacity, peak expiratory flow. Spearman correlation coefficients and positive and negative predictive values were calculated. Regression curves were estimated. RESULTS: We included 148 patients, median age 13 years (interquartile range: 8-16). A negative correlation was found between R and spirometry outcomes (Spearman correlation coefficient [ρ]: -0.5 to -0.6, p < 0.001). A positive correlation was found between X (i.e., less negative outcomes) and spirometry outcomes (ρ: 0.4-0.6, p < 0.001). Highest correlation was found at lower frequencies. Regression analysis showed a nonlinear relation. Measurement of inspiratory and expiratory R and X did not provide added value. Positive predictive values of 80%-85% were found for z-scores of R measured at 5 Hz versus (F)VC ≤ 60%. CONCLUSION: We found a nonlinear relation between oscillometry and spirometry results with increased R and reduced X in patients with more restrictive lung function decline. Given the difficulties with performing spirometry, oscillometry may be a promising substitute.


Assuntos
Pulmão , Doenças Neuromusculares , Adolescente , Criança , Volume Expiratório Forçado , Humanos , Doenças Neuromusculares/complicações , Doenças Neuromusculares/diagnóstico , Oscilometria/métodos , Estudos Prospectivos , Espirometria
9.
Chron Respir Dis ; 19: 14799731221094619, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35442817

RESUMO

Air stacking (AS) and mechanical insufflation-exsufflation (MI-E) aim to increase cough efficacy by augmenting inspiratory lung volumes in patients with neuromuscular diseases (NMDs). We studied the short-term effect of AS and MI-E on lung function. We prospectively included NMD patients familiar with daily AS or MI-E use. Studied outcomes were forced vital capacity (FVC), forced expiratory volume in one second (FEV1), and peak expiratory flow (PEF) prior to, immediately after, and up to 2 h after treatment. Paired sample T-test and Wilcoxon signed-rank test was used. Sixty-seven patients participated. We observed increased FVC and FEV1 immediately after AS with a mean difference of respectively 0.090 L (95% CI 0.045; 0.135, p < .001) and 0.073 L (95% CI 0.017; 0.128, p = .012). Increased FVC immediately after MI-E (mean difference 0.059 L (95% CI 0.010; 0.109, p = .021) persisted 1 hour (mean difference 0.079 L (95% CI 0.034; 0.125, p = .003). The effect of treatment was more pronounced in patients diagnosed with Spinal Muscular Atrophy, compared to patients with Duchenne muscular dystrophy. AS and MI-E improved FVC immediately after treatment, which persisted 1 h after MI-E. There is insufficient evidence that short-lasting increases in FVC would explain the possible beneficial effect of AS and MI-E.


Assuntos
Insuflação , Infarto do Miocárdio , Doenças Neuromusculares , Tosse , Humanos , Pulmão , Doenças Neuromusculares/complicações , Doenças Neuromusculares/terapia
10.
Pediatr Pulmonol ; 57(5): 1303-1309, 2022 05.
Artigo em Inglês | MEDLINE | ID: mdl-35174667

RESUMO

INTRODUCTION: Understanding the impact of scoliosis surgery on lung function is important for counseling patients about risks and benefits of surgery. We prospectively compared the trends in lung function test (LFT) results before and after scoliosis surgery in children with neuromuscular diseases or dysmorphic syndromes. We hypothesized a stabilization. METHODS: We prospectively included children with neuromuscular or syndromic scoliosis able to perform LFTs. We studied (forced) vital capacity ([F]VC), ratio of forced expiratory volume in 1 s (FEV1 ) and FVC, and peak expiratory flow (PEF). Preoperative LFT results were compared with results 3-4 months after surgery. The mean monthly change in LFT results up to 2 years after surgery was compared with the preoperative natural history using linear mixed-effects models. RESULTS: We included 43 patients. No significant change was observed in absolute values of (F)VC, FEV1 /FVC, and PEF before and after surgery. In 23 neuromuscular patients median standardized VC, FVC, and PEF decreased significantly after surgery from 43% to 33%, 42% to 31%, and 51% to 40%, respectively. In 20 syndromic patients, median FVC decreased from 68% to 65%. The monthly rate of change in FVC did not change significantly in both groups with a mean difference of 0.18% (95% CI: -0.27, -0.61) and -0.44% (95% CI: -1.05, 0.16). CONCLUSION: No stabilization of lung function 3-4 months after scoliosis surgery was observed in children with neuromuscular and syndromic scoliosis with restrictive lung function disease. The effect on the rate of lung function decline remains inconclusive.


Assuntos
Pneumopatias , Escoliose , Criança , Volume Expiratório Forçado , Humanos , Pulmão/cirurgia , Testes de Função Respiratória , Escoliose/cirurgia , Capacidade Vital
11.
Orphanet J Rare Dis ; 17(1): 70, 2022 02 21.
Artigo em Inglês | MEDLINE | ID: mdl-35189949

RESUMO

BACKGROUND: Respiratory complications are the most important cause of morbidity and mortality in spinal muscular atrophy (SMA). Respiratory muscle weakness results in impaired cough, recurrent respiratory tract infections and eventually can cause respiratory failure. We assessed longitudinal patterns of respiratory muscle strength in a national cohort of treatment-naïve children and adults with SMA, hypothesizing a continued decline throughout life. METHODS: We measured maximal expiratory and inspiratory pressure (PEmax and PImax), Sniff Nasal inspiratory pressure (SNIP), peak expiratory flow (PEF), and peak cough flow (PCF) in treatment-naïve patients with SMA. We used mixed-models to analyze natural history patterns. RESULTS: We included 2172 measurements of respiratory muscle function from 80 treatment-naïve patients with SMA types 1c-3b. All outcomes were lower in the more severe phenotypes. Significant differences in PEF were present between SMA types from early ages onwards. PEF decline was linear (1-2%/year). PEF reached values below 80% during early childhood in types 1c-2, and during adolescence in type 3a. PEmax and PImax were severely lowered in most patients throughout life, with PEmax values abnormally low (i.e. < 80 cmH2O) in virtually all patients. The PEmax/PImax ratio was < 1 throughout life in all SMA types, indicating that expiratory muscles were most affected. All but SMA type 3b patients had a lowered PCF. Patients with types 2b and 3a had PCF levels between 160 and 270 L/min, those with type 2a around 160 L/min and patients with type 1c well below 160 L/min. Finally, SNIP was low in nearly all patients, most pronounced in more severely affected patients. CONCLUSIONS: There are clear differences in respiratory muscle strength and its progressive decline between SMA types. We observed lower outcomes in more severe SMA types. Particularly PEF may be a suitable outcome measure for the follow-up of respiratory strength in patients with SMA. PEF declines in a rather linear pattern in all SMA types, with clear differences at baseline. These natural history data may serve as a reference for longer-term treatment efficacy assessments.


Assuntos
Atrofia Muscular Espinal , Insuficiência Respiratória , Pré-Escolar , Estudos de Coortes , Tosse , Humanos , Força Muscular/fisiologia , Estudos Prospectivos , Músculos Respiratórios
13.
Orphanet J Rare Dis ; 15(1): 88, 2020 04 10.
Artigo em Inglês | MEDLINE | ID: mdl-32276635

RESUMO

BACKGROUND: Respiratory muscle weakness is an important feature of spinal muscular atrophy (SMA). Progressive lung function decline is the most important cause of mortality and morbidity in patients. The natural history of lung function in SMA has, however, not been studied in much detail. RESULTS: We analysed 2098 measurements of lung function from 170 treatment-naïve patients with SMA types 1c-4, aged 4-74 years. All patients are participating in an ongoing population-based prevalence cohort study. We measured Forced Expiratory Volume in 1 s (FEV1), Forced Vital Capacity (FVC), and Vital Capacity (VC). Longitudinal patterns of lung function were analysed using linear mixed-effects and non-linear models. Additionally, we also assessed postural effects on results of FEV1 and FVC tests. In early-onset SMA types (1c-3a), we observed a progressive decline of lung function at younger ages with relative stabilisation during adulthood. Estimated baseline values were significantly lower in more severely affected patients: %FEV1 ranged from 42% in SMA type 1c to 100% in type 3b, %FVC 50 to 109%, and %VC 44 to 96%. Average annual decline rates also differed significantly between SMA types, ranging from - 0.1% to - 1.4% for FEV1, - 0.2% to - 1.4% for FVC, and + 0.2% to - 1.7% for VC. In contrast to SMA types 1c-3a, we found normal values for all outcomes in later-onset SMA types 3b and 4 throughout life, although with some exceptions and based on limited available data. Finally, we found no important differences in FVC or FEV1 values measured in either sitting or supine position. CONCLUSIONS: Our data illustrate the longitudinal course of lung function in patients with SMA, which is characterised by a progressive decline in childhood and stabilisation in early adulthood. The data do not support an additional benefit of measuring FEV1 or FVC in both sitting and supine position. These data may serve as a reference to assess longer-term outcomes in clinical trials.


Assuntos
Atrofia Muscular Espinal , Adulto , Estudos de Coortes , Volume Expiratório Forçado , Humanos , Pulmão , Capacidade Vital
14.
Neurology ; 94(15): e1634-e1644, 2020 04 14.
Artigo em Inglês | MEDLINE | ID: mdl-32217777

RESUMO

OBJECTIVE: To investigate probabilities of survival and its surrogate, that is, mechanical ventilation, in patients with spinal muscular atrophy (SMA). METHODS: We studied survival in a population-based cohort on clinical prevalence of genetically confirmed, treatment-naive patients with SMA, stratified for best acquired motor milestone (i.e., none: type 1a/b; head control in supine position or rolling: type 1c; sitting independently: type 2a; standing: type 2b; walking: type 3a/b; adult onset: type 4). We also assessed the need for mechanical ventilation as a surrogate endpoint for survival. RESULTS: We included 307 patients with a total follow-up of 7,141 person-years. Median survival was 9 days in SMA type 1a, 7.7 months in type 1b, and 17.0 years in type 1c. Patients with type 2a had endpoint-free survival probabilities of 74.2% and 61.5% at ages 40 and 60 years, respectively. Endpoint-free survival of SMA types 2b, 3, and 4 was relatively normal, at least within the first 60 years of life. Patients with SMA types 1c and 2a required mechanical ventilation more frequently and from younger ages compared to patients with milder SMA types. In our cohort, patients ventilated up to 12 h/d progressed not gradually, but abruptly, to ≥16 h/d. CONCLUSIONS: Shortened endpoint-free survival is an important characteristic of SMA types 1 and 2a, but not types 2b, 3, and 4. For SMA types 1c and 2a, the age at which initiation of mechanical ventilation is necessary may be a more suitable endpoint than the arbitrarily set 16 h/d.


Assuntos
Fatores Etários , Atrofia Muscular Espinal/mortalidade , Respiração Artificial/mortalidade , Atrofias Musculares Espinais da Infância/mortalidade , Adulto , Estudos de Coortes , Progressão da Doença , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Caminhada/fisiologia
15.
Pediatr Pulmonol ; 55(2): 510-513, 2020 02.
Artigo em Inglês | MEDLINE | ID: mdl-31909568

RESUMO

INTRODUCTION: Children with neuromuscular diseases develop cough impairment. Airway clearance techniques (ACTs) may help to prevent recurrent respiratory tract infections (RTIs). A commonly used ACT is mechanical insufflation-exsufflation (MI-E), but evidence for efficacy is limited. We hypothesize that MI-E has beneficial effect on RTI related hospital admission rate. METHODS: In this single-center retrospective study, we reviewed all children who used daily MI-E between 2005 till June 2019. Primary outcome studied was the number of RTIs requiring hospital admission. Patient satisfaction and burden experienced by MI-E use were explored by questionnaires using a Likert scale. The relative number of RTIs requiring admission and the number of admission days per eligible period before and after the introduction of MI-E were compared using the Friedman test and the Wilcoxon signed-rank test. RESULTS: Thirty-seven children were included. The median number of RTI related hospital admissions per 1000 eligible days after the introduction of MI-E was 0.9 (interquartile range [IQR] 0.0-3.1) compared to the 3 preceding years (median 3.7; IQR 1.4-5.9; P = .006). The median number of RTI related admission days per 1000 eligible days after the introduction of MI-E was significantly lower with a median of 2.7 (IQR 0.0-17.4) compared to the 3 preceding years (median 33.6; IQR 15.0-51.1; P = .001). Patient satisfaction was high with low burden, even in patients who discontinued treatment. CONCLUSION: A significantly lower number of RTIs requiring hospital admission and shorter admission duration after the introduction of MI-E was found, with high patient satisfaction and low burden.


Assuntos
Insuflação , Doenças Neuromusculares , Respiração Artificial , Adolescente , Criança , Pré-Escolar , Tosse/terapia , Feminino , Humanos , Masculino , Infecções Respiratórias , Estudos Retrospectivos
16.
Paediatr Anaesth ; 27(11): 1091-1097, 2017 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-28940868

RESUMO

BACKGROUND: Hydrostatic or pneumatic reduction of intussusception is an invasive procedure that is stressful and may be painful for a child. Resistance of the child may increase the duration of the procedure and decrease success rate of reduction. Analgesia can help to reduce pain, but not necessarily resistance. General anesthesia increases success rate of reduction. However, it requires the presence of an anesthesiologist, and may lead to anesthesia-related complications. Procedural sedation with esketamine could be a safe alternative. AIM: The aim of this study was to compare hydrostatic reduction using morphine analgesia compared to procedural sedation with esketamine in terms of success rate, adverse events, and duration of reduction. METHODS: A retrospective case-cohort comparison study was performed with two groups of patients who had undergone hydrostatic reduction for ileocolic intussusception and received morphine analgesia (n = 37) or esketamine sedation (n = 20). Until July 2013, reduction was performed after intravenously administered morphine. Hereafter, a new protocol for procedural sedation was implemented and reduction was performed after administration of esketamine. Cases were matched for age and duration of symptoms. RESULTS: No adverse events requiring intervention other than administration of oxygen were reported for either group. Success rate of reduction using esketamine sedation was 90% vs 70% using morphine analgesia, risk ratio (RR) 1.29, 95% CI[0.93-1.77]. Recurrence rate using esketamine sedation was 10% vs 15% using morphine analgesia, RR 0.67, 95% CI[0.12-3.57]. Reduction time was shorter using esketamine sedation (Median 5 minutes, IQR 9 minutes) vs morphine analgesia (Median 8 minutes, IQR 16 minutes, P = .04, Median difference 3, 95% CI[-1.50-8.75]). Median hospital stay in the esketamine group was 1.5 days (IQR 1.8) vs 2 days (IQR 5.3) in the morphine group. CONCLUSION: No serious adverse events were recorded. In comparison to morphine analgesia, with esketamine there was weak evidence for a higher success rate, lower recurrence rate, shorter duration, and shorter length of hospital stay.


Assuntos
Analgesia/métodos , Doenças do Íleo/cirurgia , Intussuscepção/cirurgia , Ketamina/uso terapêutico , Morfina/uso terapêutico , Analgésicos/uso terapêutico , Analgésicos Opioides/uso terapêutico , Estudos de Casos e Controles , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Estudos Retrospectivos , Resultado do Tratamento
17.
Pediatr Nephrol ; 32(10): 1971-1978, 2017 10.
Artigo em Inglês | MEDLINE | ID: mdl-28578542

RESUMO

BACKGROUND: Citrate is preferred over heparin as an anticoagulant in adult continuous renal replacement therapy (CRRT). However, its potential adverse effects and data on use in CRRT in infants and toddlers is limited. We conducted a prospective study on using citrate in CRRT in critically ill small children. METHODS: Children who underwent CRRT with the smallest filter in our PICU between November 2011 and November 2016 were included. Both heparin and citrate were applied according to a strict protocol. Our primary outcome was circuit survival time. Secondary outcomes were alkalosis, citrate toxicity, and number of red blood cell transfusions. RESULTS: Heparin was used in six patients (121 circuits, total CRRT time 3723 h). Citrate was used in 14 patients (105 circuits, total CRRT time 4530 h). Median circuit survival time with heparin was 21 h (IQR 14.5-27.5) compared to 45.2 h (IQR 37.5-52.8) with citrate (p < 0.001). Actual administered effluent dose compared to prescribed dose was 85% (IQR 69-98%) with heparin compared to 92% (IQR 88-98%) with citrate (p = 0.31). No patient treated with citrate developed citrate toxicity. No other differences in electrolytes were found between the two CRRT regimes. In the heparin group, a median of 6.5 units of red blood cells (IQR 1.5-23.8) were given during CRRT, compared to three in the citrate group (IQR 2.0-5.0, p = 0.12). CONCLUSIONS: Use of regional citrate significantly prolongs circuit survival time and thereby should increase CRRT efficiency when compared to heparin. In addition, citrate appears safe for CRRT in critically ill small children.


Assuntos
Injúria Renal Aguda/terapia , Anticoagulantes/uso terapêutico , Ácido Cítrico/uso terapêutico , Estado Terminal/terapia , Heparina/uso terapêutico , Terapia de Substituição Renal/efeitos adversos , Anticoagulantes/farmacologia , Coagulação Sanguínea/efeitos dos fármacos , Pré-Escolar , Ácido Cítrico/farmacologia , Feminino , Heparina/farmacologia , Humanos , Lactente , Unidades de Terapia Intensiva/estatística & dados numéricos , Masculino , Estudos Prospectivos , Terapia de Substituição Renal/métodos , Terapia de Substituição Renal/estatística & dados numéricos , Fatores de Tempo , Resultado do Tratamento , Equilíbrio Hidroeletrolítico
18.
PLoS One ; 12(2): e0172096, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-28207796

RESUMO

AIM: The purpose of this study was to determine the incidence of reintubation due to upper airway obstruction in a homogeneous group of ventilated infants with Respiratory Syncytial Virus bronchiolitis. Our secondary objective was to determine whether prophylactic administration of dexamethasone prior to extubation was associated with decreased risk of reintubation. METHODS: This retrospective observational study in two Pediatric Intensive Care Units in 2 university hospitals in The Netherlands included two hundred patients younger than 13 months admitted with respiratory insufficiency caused by Respiratory Syncytial Virus bronchiolitis, requiring invasive mechanical ventilation. A logistic regression analysis with propensity score method was used to adjust for possible confounding. RESULTS: Reintubation due to post-extubation stridor occurred in 17 (8.5%) of 200 patients. After propensity score matching, administration of dexamethasone prior to extubation was associated with a significantly (p = 0.0011) decreased risk of reintubation due to post-extubation stridor compared to patients not receiving prophylactic dexamethasone (absolute risk reduction 13%, 95% CI 5.3-21%). CONCLUSION: Reintubation due to post-extubation stridor is an important complication of ventilation for Respiratory Syncytial Virus bronchiolitis. Dexamethasone administered prior to extubation probably reduces the risk of post-extubation stridor necessitating reintubation in these infants. The results of this study support initiation of a placebo-controlled trial to confirm the beneficial effect of prophylactic dexamethasone.


Assuntos
Extubação/efeitos adversos , Bronquiolite/tratamento farmacológico , Dexametasona/uso terapêutico , Transtornos Respiratórios/tratamento farmacológico , Respiração Artificial/efeitos adversos , Sons Respiratórios/efeitos dos fármacos , Infecções por Vírus Respiratório Sincicial/tratamento farmacológico , Anti-Inflamatórios/uso terapêutico , Bronquiolite/complicações , Bronquiolite/virologia , Feminino , Humanos , Lactente , Recém-Nascido , Unidades de Terapia Intensiva Pediátrica , Masculino , Transtornos Respiratórios/etiologia , Sons Respiratórios/etiologia , Infecções por Vírus Respiratório Sincicial/complicações , Infecções por Vírus Respiratório Sincicial/virologia , Vírus Sinciciais Respiratórios/isolamento & purificação , Estudos Retrospectivos
19.
Clin Oral Investig ; 21(6): 2063-2076, 2017 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-27868158

RESUMO

OBJECTIVES: In addition to breathing problems, patients with Robin sequence (RS) often encounter feeding difficulties (FD). Data regarding the occurrence of FD and possible influencing factors are scarce. The study aim was to elucidate these factors to improve treatment strategies. MATERIAL AND METHODS: A retrospective comparative cohort study was conducted, consisting of 69 infants diagnosed with both RS and a cleft palate and 64 isolated cleft palate only (iCPO) infants. Data regarding FD, growth, and airway intervention were collected during the first 2 years of life. A systematic review of the literature was conducted to identify reported FD in RS patients. RESULTS: RS patients had more FD (91 %) than iCPO patients (72 %; p = 0.004). Also, nasogastric (NG)-tube feeding was necessary more frequently and for a longer period (both p < 0.001). Growth was lower in RS than iCPO infants (p = 0.008) and was not affected by the kind of airway management (conservative/surgical; p = 0.178), cleft palate grade (p = 0.308), or associated disorders (p = 0.785). By contrast, surgical intervention subtype did significantly affect growth. Mean reported FD for RS in the literature is 80 % (range = 47-100 %), and 55 % (range = 11-100 %) of infants need NG-tube feeding. CONCLUSIONS: FD is present in a large proportion of infants with RS, which indicates the need for early recognition and proper treatment to ensure optimal growth. Growth during the first 2 years of life is significantly lower in RS patients than iCPO patients, which indicates the need for careful attention and long-term follow-up. CLINICAL RELEVANCE: This study indicates the need for early recognition and proper treatment of FD in RS to ensure optimal growth. In addition, growth needs careful attention and long-term follow-up.


Assuntos
Desenvolvimento Infantil , Transtornos da Alimentação e da Ingestão de Alimentos/fisiopatologia , Síndrome de Pierre Robin/fisiopatologia , Fenda Labial/fisiopatologia , Fissura Palatina/fisiopatologia , Feminino , Humanos , Lactente , Masculino , Estudos Retrospectivos
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