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1.
Int J Pediatr Otorhinolaryngol ; 127: 109665, 2019 Sep 02.
Artigo em Inglês | MEDLINE | ID: mdl-31526938

RESUMO

INTRODUCTION: acoustic rhinometry (AR) is a non-invasive method measuring the nasal volume (NV) and the nasal minimal cross-sectional area (MCA), reflecting nasal obstruction. The first objective of this study was to measure and compare NV and MCA between 3 groups of children: "achondroplasia", "Down syndrome" and "control". The control group corresponded to children with suspicion of sleep disorder disease and without cranio-facial malformation. The second objective was to correlate AR measurements with the obstructive apnea-hypopnea index (OAHI). METHODS: prospective study between February and July 2017, in a tertiary care center. The following data were collected: demographic characteristics, medical and surgical history, NV, MCA, and OAHI. RESULTS: 83 children were included. The mean NV was lower in achondroplasia group compared to control group: 2.75 cm3 vs 3.60 cm3 (p = 0.02, 95% CI [0.0694, 0.7456]). Negative correlation was found between the NV and the OAHI for children with achondroplasia (T = -0.37; p = 0.02). CONCLUSIONS: AR is an effective tool for assessing nasal obstruction in children. Nasal obstruction was correlated to OAHI in achondroplasia. AR could become a routine tool in the management of nasal obstruction of children with cranio-facial malformations.

2.
J Pediatr ; 2019 Sep 17.
Artigo em Inglês | MEDLINE | ID: mdl-31540763

RESUMO

OBJECTIVES: To evaluate changes in cerebral oxygenation by means of near-infrared spectroscopy during respiratory events in children with sleep-disordered breathing (SDB) and associated disorders. STUDY DESIGN: Sixty-five children suspected of having SDB underwent a respiratory polygraphy with simultaneous recording of cerebral oxygenation indices. Respiratory events were analyzed by type of event, duration, variations of pulse oximetry (oxygen saturation [SpO2]), cerebral tissue oxygenation index (TOI), and heart rate. Data were categorized according to the severity of SDB and age. RESULTS: There were 540 obstructive and mixed apneas, 172 central apneas, and 393 obstructive hypopneas analyzed. The mean decreases in SpO2 and TOI were 4.1 ± 3.1% and 3.4 ± 2.8%, respectively. The mean TOI decrease was significantly smaller for obstructive hypopnea compared with apneas. The TOI decrease was significantly less in children with mild SDB as compared with those with moderate-to-severe SDB and in children >7 years as compared with those <7 years old. TOI decreases correlated significantly with SpO2 decreases, duration of event, and age, regardless of the type of event. In a multivariable regression model, predictive factors of TOI decreases were the type of respiratory event, SpO2 decrease, apnea-hypopnea index, and age. CONCLUSIONS: In children with SDB and associated disorders, cerebral oxygenation variations depend on the type of respiratory event, severity of SDB, and age.

3.
Respir Physiol Neurobiol ; 271: 103308, 2019 Sep 24.
Artigo em Inglês | MEDLINE | ID: mdl-31561013

RESUMO

In spinal muscular atrophy (SMA), respiratory muscles are heterogeneously involved with a weakness of the intercostal muscles, possibly of the abdominal wall muscles, and a relatively spared diaphragm, resulting in cough impairment. An abnormal inspiratory cough phase pattern has been reported in SMA II and III. This short communication analyzed the esogastric pressures during voluntary cough in 49 SMA II and III patients. Four different patterns of coughing, reflecting an increasing degree of respiratory muscle weakness, were identified. The "mild weakness" profile was observed mainly in SMA III, while the "severe weakness" profile, which seems to correspond to the absence of abdominal muscle activity, was observed only in a few patients with SMA II. The cough profiles of 6 patients are presented together with their sniff and maximal static pressures measurements. Different esogastric pressure patterns were observed during these forceful maneuvers, suggesting variable involvement of the intercostal and abdominal muscles, and diaphragm during the evolution of SMA II and III.

4.
Am J Med Genet A ; 179(7): 1196-1204, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-31038846

RESUMO

Mucolipidosis (ML) is a rare lysosomal storage disorder with a wide spectrum of disease severity according to the type. Sleep-disordered breathing is recognized as a characteristic feature of ML but objective data are scarce. The aim of the study was to describe sleep data and medical management in children with ML α/ß. All patients with ML α/ß followed at a national reference center of ML were included. Five patients had ML II, one patient had ML III and one patient had ML II-III. One patient was started on noninvasive ventilation (NIV) to allow extubation after prolonged invasive mechanical ventilation. The six other patients underwent sleep study at a median age of 1.8 years (range 4 months-17.4 years). Obstructive sleep apnea (OSA) was observed in all patients with a median apnea-hypopnea index (AHI) of 36 events/hr (range 5-52) requiring continuous positive airway pressure (CPAP) or NIV. CPAP/NIV resulted in an improvement of nocturnal gas exchange and was continued in all patients with an excellent compliance. Two patients died. Systematic sleep studies are recommended at time of diagnosis in ML. CPAP or NIV are effective treatments of OSA, well tolerated, and may contribute to improve the quality of life of patients and caregivers.

5.
J Pediatr ; 210: 194-200.e2, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-30955791

RESUMO

OBJECTIVE: To describe and compare the lung function decline in patients with Duchenne muscular dystrophy on glucocorticoid therapy in contrast with glucocorticoid-naïve patients, and to define the deciles of pulmonary decline in glucocorticoid-treated patients. STUDY DESIGN: This retrospective study examined lung function of patients with Duchenne muscular dystrophy over 6 years of age followed between 2001 and 2015 at 2 centers-glucocorticoid-treated patients in Cincinnati, Ohio, and glucocorticoid-naïve patients in Paris, France. Forced vital capacity (FVC, FVC%), forced expiratory volume in 1 second, maximal inspiratory pressure, maximal expiratory pressure, and peak expiratory flow data were analyzed. Only FVC data were available for the French cohort. RESULTS: There were 170 glucocorticoid-treated patients (92%), 5 patients (2.7%) with past glucocorticoid use, and 50 French glucocorticoid-naïve patients. The peak absolute FVC was higher and was achieved at earlier ages in glucocorticoid-treated compared with glucocorticoid-naïve patients (peak FVC, 2.4 ± 0.6 L vs 1.9 ± 0.7 L; P < .0001; ages 13.5 ± 3.0 years vs 14.3 ± 2.8 years; P = .03). The peak FVC% was also higher and was achieved at earlier ages in glucocorticoid-treated patients (peak FVC%, 105.1 ± 25.1% vs 56 ± 20.9%; P < .0001; ages 11.9 ± 2.9 years vs 13.6 ± 3.2 years; P = .002). Rates of decline for both groups varied with age. Maximal rates of decline were 5.0 ± 0.26% per year (12-20 years) for glucocorticoid-treated and 5.1 ± 0.39% per year for glucocorticoid-naïve patients (11-20 years; P = .2). Deciles of FVC% decline in glucocorticoid-treated patients show that patients experience accelerated decline at variable ages. CONCLUSIONS: These data describe nonlinear rates of decline of pulmonary function in patients with Duchenne muscular dystrophy, with improved function in glucocorticoid-treated patients. FVC% deciles may be a useful tool for clinical and research use.

6.
Eur Respir J ; 53(6)2019 06.
Artigo em Inglês | MEDLINE | ID: mdl-30956204

RESUMO

Assessing respiratory mechanics and muscle function is critical for both clinical practice and research purposes. Several methodological developments over the past two decades have enhanced our understanding of respiratory muscle function and responses to interventions across the spectrum of health and disease. They are especially useful in diagnosing, phenotyping and assessing treatment efficacy in patients with respiratory symptoms and neuromuscular diseases. Considerable research has been undertaken over the past 17 years, since the publication of the previous American Thoracic Society (ATS)/European Respiratory Society (ERS) statement on respiratory muscle testing in 2002. Key advances have been made in the field of mechanics of breathing, respiratory muscle neurophysiology (electromyography, electroencephalography and transcranial magnetic stimulation) and on respiratory muscle imaging (ultrasound, optoelectronic plethysmography and structured light plethysmography). Accordingly, this ERS task force reviewed the field of respiratory muscle testing in health and disease, with particular reference to data obtained since the previous ATS/ERS statement. It summarises the most recent scientific and methodological developments regarding respiratory mechanics and respiratory muscle assessment by addressing the validity, precision, reproducibility, prognostic value and responsiveness to interventions of various methods. A particular emphasis is placed on assessment during exercise, which is a useful condition to stress the respiratory system.

7.
Respir Care ; 64(5): 509-518, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-30538160

RESUMO

BACKGROUND: The present study aimed to characterize the behavior of 3 components of respiratory muscle function during mechanical ventilation weaning in children to better understand the respective impact of a spontaneous breathing trial on ventilatory mechanical action (esophageal pressure [Pes], ventilatory demand (electrical activity of the diaphragm [EAdi]), and oxygen consumption. METHODS: This was a prospective single-center study. All children > 1 months and <18 y old who were intubated and on mechanical ventilation, and who were hospitalized in the pediatric ICU were eligible. Subjects considered as ready to extubate were included. Simultaneous recordings of oxygen consumption, Pes, and EAdi were performed during 3 steps: before, during, and after the spontaneous breathing test. RESULTS: Twenty subjects (median age, 5.5 mo) were included. Half of them were admitted for a respiratory cause. The increase in Pes swings and esophageal pressure-time product during the spontaneous breathing trial was not significant (P = .33 and P = .75, respectively), and a similar trend was observed with peak EAdi (P = .06). Oxygen consumption obtained by indirect calorimetry was stable in the 3 conditions (P = .98). CONCLUSIONS: In these children who were critically ill, a spontaneous breathing trial induced a moderate and nonsignificant increase in work of breathing, as reflected by the respiratory drive with EAdi and respiratory mechanics with Pes. However, indirect calorimetry did not seem to be a sensitive tool to assess respiratory muscle function during the weaning phase in children who were on mechanical ventilation, especially when work of breathing was slightly increased.

8.
Front Pediatr ; 6: 351, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30525013

RESUMO

Pierre Robin sequence (PRS) may lead to life-threatening respiratory and feeding disorders. With the aim to analyse the association of the severities of retrognathia and glossoptosis with those of respiratory and feeding disorders, we retrospectively studied a series of 50 infants with retrognathia, glossoptosis, cleft palate, and airway obstruction. The patients were managed from birth to at least 6 years of age by a single pediatric team at the Armand Trousseau Hospital in Paris within a 12 years period (2000-2012). Retrognathia and glossoptosis were graded in the neonatal period according to a specific clinical examination. Ventilation assistance was required for 32/50 (64%) patients, and enteral feeding for 41/50 (82%). The grades of retrognathia and glossoptosis and the severity of respiratory disorders did not differ between patients with isolated PRS and syndromic PRS. Severe respiratory disorders were more common and long-lasting feeding (>12 months) was more frequently required in patients with syndromic PRS compared with isolated PRS (42 vs. 13%, p = 0.04 and 42 vs. 4%, p < 0.01 respectively). Using univariate analysis, neurological impairments and laryngomalacia were associated with severe respiratory disorders [Odds ratio (OR) 5.0, 95% CI 1.3-19.6; and OR 14.6, 95% CI 1.3-161.4; p < 0.05] as well as with long-lasting feeding (>12 months) disorders (OR 18.6, 95% CI 3.9-89.2 and OR 20.4, 95% CI 3,4-122.8; p < 10-2). Syndromic SPR status was also associated with severe respiratory disorders (OR 4.9, 95% CI 1-32.5; p < 0.05). Using multivariate analysis, only syndromic PRS status was predictive for severe respiratory disorders (adjusted OR 8, 95% CI 1.47-44.57; p < 0.05); and only neurological impairments remained a significant risk for long lasting feeding disorders (>12 months) (adjusted OR 21.72, 95% CI 3.4-138.63; p < 10-2). The grades of retrognathia and glossoptosis were not predictive factors for the severity of respiratory and feeding disorders. Conclusion: In children with PRS, the severity of clinical conditions may not correlate with anatomic variables but rather with laryngeal abnormalities, neurological impairement and syndromic PRS status.

9.
Am J Med Genet A ; 2018 Nov 18.
Artigo em Inglês | MEDLINE | ID: mdl-30450712

RESUMO

Respiratory problems are common in Morquio-A syndrome (MPS IVA) but objective data on sleep-disordered breathing are scarce. The aim of our study was to review polygraphic (PG) findings and the need for noninvasive continuous positive airway pressure (CPAP) or noninvasive ventilation (NIV) in children with MPS IVA. A retrospective review of the clinical charts and PG of 16 consecutive children (7 boys, mean age 10.5 ± 4.2 years) with MPS IVA seen over a period of 3 years was performed. The prevalence of obstructive sleep apnea (OSA) was 69% with only five patients, all younger than 10 years old, having a normal PG. Four patients had mild OSA (apnea-hypopnea index [AHI] ≥1.5 and <5 events/hr), three patients had moderate OSA (AHI ≥5 and <10 events/hr), and three patients had severe OSA (AHI ≥ 10 events/hr). Among the 10 patients with OSA, 3 had prior adenoidectomy ± tonsillectomy and 6 were on enzyme replacement therapy. Only one patient had a central apnea index >5 events/hr despite prior cervico-occipital decompression. Six patients, all older than 11 years old, were started on CPAP or NIV because of severe OSA (n = 4), nocturnal hypoventilation (n = 1), or impossibility to be weaned from NIV after an acute respiratory failure (n = 1). Prevalence of OSA is high in patients with MPS IVA, underlying the importance of a systematic screening for sleep-disordered breathing. CPAP and NIV are efficient and well accepted for treating sleep-disordered breathing.

10.
Artigo em Inglês | MEDLINE | ID: mdl-30262236

RESUMO

INTRODUCTION: Rett syndrome (RS) is a severe neurodevelopment disorder associated with abnormal breathing during wakefulness and disturbed nocturnal behaviour. Breathing abnormalities during daytime have been extensively reported but polysomnographic (PSG) findings have been poorly studied. MATERIALS AND METHODS: Consecutive patients with RS carrying distinct mutations in MECP2 gene, who underwent a PSG between October 2014 and January 2018, were included in the study. Clinical and PSG data were collected. RESULTS: Seventeen RS girls, mean age 9.5 ± 2.8 years, were included in the study. Mean total sleep time was 366 ± 102 min. Mean sleep efficiency was reduced (66 ± 19%) with only 3 girls presenting a sleep efficiency above 80%. Wake after sleep onset was increased (33 ± 20%) with an arousal index of 7 ± 6 events/hour. Sleep stages were altered with a normal N1 (2 ± 3%), a decreased N2 (34 ± 20%), an increase of N3 (51 ± 23%) and a decrease of REM sleep (12 ± 9%). Mean apnea hypopnea index (AHI) was increased at 19 ± 37 events/hour, with a predominance of obstructive events. Thirteen patients had an AHI > 1.5 event/hour. Four patients had an obstructive AHI >10 events/hour with one patient having associated tonsillar hypertrophy. Two patients had predominant severe central apneas (central AHI 53 and 132 events/hour) which resolved with noninvasive ventilation and nocturnal oxygen therapy respectively. CONCLUSION: Girls with RS have poor sleep quality with alterations in slow wave and REM sleep stages. Obstructive respiratory events are uncommon in patients without adenotonsillar hypertrophy. Central respiratory events are rare. Longitudinal studies should help understanding the natural history of sleep disturbances in RS and their relationship with the neurocognitive decline.

11.
Sleep Breath ; 2018 Sep 05.
Artigo em Inglês | MEDLINE | ID: mdl-30187367

RESUMO

PURPOSE: Co-sleeping is common in children with co-morbid conditions. The aim of the study was to analyze the prevalence and determinants of parent-child co-sleeping in children with co-morbid conditions and sleep-disordered breathing and the impact on parental sleep. METHODS: Parents of consecutive children undergoing a sleep study filled in a questionnaire on co-sleeping. RESULTS: The parents of 166 children (80 boys, median age 5.7 years (0.5-21) participated in this study. The most common co-morbid conditions of the children were Down syndrome (17%), achondroplasia (11%), and Chiari malformation (8%). The prevalence of parent-child co-sleeping was 46%. Reasons for co-sleeping were mainly reactive and included child's demand (39%), crying (19%), nightmares (13%), medical reason (34%), parental reassuring or comforting (27%), and/or over-crowding (21%). Sixty-eight percent of parents reported that co-sleeping improved their sleep quality because of reassurance/comforting (67%), reduced nocturnal awakening (23%), and child supervision (44%). Forty percent of parents reported that co-sleeping decreased their sleep quality because of nocturnal awakenings or early wake up, or difficulties initiating sleep (by 77% and 52% of parents, respectively), whereas both positive and negative associations were reported by 29% of the parents. Co-sleeping was more common with children < 2 years of age as compared to older children (p < 0.001). CONCLUSIONS: Parent-child co-sleeping is common in children with co-morbid conditions and sleep-disordered breathing. Co-sleeping was mainly reactive and had both positive and negative associations with parental sleep quality. Co-sleeping should be discussed on an individual basis with the parents in order to improve the sleep quality of the family.

12.
Pediatr Pulmonol ; 53(10): 1422-1428, 2018 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-30070059

RESUMO

INTRODUCTION: Current guidelines recommend initiating continuous positive airway pressure (CPAP) in children during an overnight in-hospital titration study. Due to a shortage of hospital beds and economic constraints, we started a program for outpatient initiation of CPAP in selected children with obstructive sleep apnea (OSA). METHODS: Objective CPAP compliance and efficacy were evaluated in consecutive children enrolled in an outpatient CPAP program when they fulfilled the following criteria: persistent OSA, age >6 months, stable condition, family living in the Parisian area and agreeing with a regular follow-up. RESULTS: Thirty-one children, median (range) age 8.9 years (0.8-17.5), were included in the program. The most common diagnosis were Down syndrome (n = 7), achondroplasia (n = 3), and obesity (n = 3). Median baseline obstructive apnea-hypopnea index (OAHI) was 12.5 events/h (5-100). Median duration of follow-up was 12.3 months (2.2-25.2). Four subjects (three adolescents with Down syndrome) were not compliant at 2-month follow-up with a compliance <4 h/night. In the other 27 subjects, median compliance was 08:21 h:min/night (05:45-12:20), with a median number of night use per month of 25 (18-30). CPAP efficacy at the end of follow-up was excellent in the compliant subjects with a median AHI of two events/h (0-4) and normal gas exchange with CPAP therapy. Three subjects were successfully weaned from CPAP treatment during the study period. CONCLUSION: Initiating CPAP in an outpatient setting in children is feasible and effective in selected subjects. A high rate of compliance can be achieved as well as a correction of OSA.

13.
Neuromuscul Disord ; 28(9): 731-740, 2018 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-30097248

RESUMO

Congenital muscular dystrophies and congenital myopathies represent a heterogeneous group of disorders of the muscle characterized by an early onset of hypotonia and muscle weakness and consequently, a high respiratory morbidity and mortality. The diagnosis and characterization of the weakness of the respiratory muscles is crucial for clinical management of patients and the evaluation of innovative therapies. Routine respiratory evaluation is based on noninvasive volitional tests, such as the measurement of lung volumes, spirometry, and maximal static pressures, which may be difficult or impossible to obtain in young children. Tests using natural maneuvers such as a sniff, a cough or a whistle, are easier to perform and may be more informative in young children. The combination of multiple tests of respiratory muscle function is essential and both increases diagnosis accuracy and the strength of the data in case of clinical trials assessing new therapies for these diseases.

15.
Breathe (Sheff) ; 14(2): 91-92, 2018 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-29875827

RESUMO

Faculty and delegates of an @ERStalk course on paediatric long-term NIV describe their experiences http://ow.ly/n3gi30iYYuM.

16.
Acta Paediatr ; 107(7): 1253-1261, 2018 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-29617049

RESUMO

AIM: We evaluated the impact that having a child with sleep-disordered breathing had on their parents, including their own sleep quality. METHODS: Questionnaires were completed by 96 parents of 86 children referred for a sleep study or control of continuous positive airway pressure (CPAP) or noninvasive ventilation (NIV) at the sleep laboratory of the Necker Hospital, Paris, France, between October 2015 and January 2016. The questionnaires evaluated anxiety and depression, family functioning, the parents' quality of life, daytime sleepiness and sleep quality. RESULTS: The children had a mean age of seven ±five years and most of the responses (79%) came from their mothers. These showed that 26% of parents showed moderate-to-severe anxiety, 8% moderate-to-severe depression, 6% complex family cohesion, 59% moderate-to-severe daytime sleepiness and 54% poor sleep quality. Anxiety was higher in mothers than in fathers (p < 0.001). The questionnaire scores did not differ according to the child's age, the results of the sleep studies or the CPAP or NIV treatment. The symptoms seem to be more commonly related to the child's underlying disease than their sleep-disordered breathing. CONCLUSION: The parents of children referred to a sleep laboratory reported frequent anxiety, daytime sleepiness and poor sleep quality.

17.
Respir Med ; 136: 98-110, 2018 03.
Artigo em Inglês | MEDLINE | ID: mdl-29501255

RESUMO

This is a unique state of the art review written by a group of 21 international recognized experts in the field that gathered during a meeting organized by the European Neuromuscular Centre (ENMC) in Naarden, March 2017. It systematically reports the entire evidence base for airway clearance techniques (ACTs) in both adults and children with neuromuscular disorders (NMD). We not only report randomised controlled trials, which in other systematic reviews conclude that there is a lack of evidence base to give an opinion, but also include case series and retrospective reviews of practice. For this review, we have classified ACTs as either proximal (cough augmentation) or peripheral (secretion mobilization). The review presents descriptions; standard definitions; the supporting evidence for and limitations of proximal and peripheral ACTs that are used in patients with NMD; as well as providing recommendations for objective measurements of efficacy, specifically for proximal ACTs. This state of the art review also highlights how ACTs may be adapted or modified for specific contexts (e.g. in people with bulbar insufficiency; children and infants) and recommends when and how each technique should be applied.

18.
Infect Dis Ther ; 7(1): 87-120, 2018 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-29470837

RESUMO

INTRODUCTION: The REGAL (RSV Evidence - A Geographical Archive of the Literature) series has provided a comprehensive review of the published evidence in the field of respiratory syncytial virus (RSV) in Western countries over the last 20 years. This seventh and final publication covers the past, present and future approaches to the prevention and treatment of RSV infection among infants and children. METHODS: A systematic review was undertaken of publications between January 1, 1995 and December 31, 2017 across PubMed, Embase and The Cochrane Library. Studies reporting data on the effectiveness and tolerability of prophylactic and therapeutic agents for RSV infection were included. Study quality and strength of evidence (SOE) were graded using recognized criteria. A further nonsystematic search of the published literature and Clinicaltrials.gov on antiviral therapies and RSV vaccines currently in development was also undertaken. RESULTS: The systematic review identified 1441 studies of which 161 were included. Management of RSV remains centered around prophylaxis with the monoclonal antibody palivizumab, which has proven effective in reducing RSV hospitalization (RSVH) in preterm infants < 36 weeks' gestational age (72% reduction), children with bronchopulmonary dysplasia (65% reduction), and infants with hemodynamically significant congenital heart disease (53% reduction) (high SOE). Palivizumab has also shown to be effective in reducing recurrent wheezing following RSVH (high SOE). Treatment of RSV with ribavirin has conflicting success (moderate SOE). Antibodies with increased potency and extended half-life are currently entering phase 3 trials. There are approximately 15 RSV vaccines in clinical development targeting the infant directly or indirectly via the mother. CONCLUSION: Palivizumab remains the only product licensed for RSV prophylaxis, and only available for high-risk infants. For the general population, there are several promising vaccines and monoclonal antibodies in various stages of clinical development, with the aim to significantly reduce the global healthcare impact of this common viral infection. FUNDING: AbbVie.

19.
Eur J Cardiothorac Surg ; 53(4): 740-747, 2018 04 01.
Artigo em Inglês | MEDLINE | ID: mdl-29165681

RESUMO

OBJECTIVES: Few studies have evaluated long-term diaphragmatic function in congenital diaphragmatic hernia (CDH). The aim of our cross-sectional study was to assess diaphragmatic function in infants and young children with CDH after surgical repair. METHODS: All the patients with CDH repair followed in our centre between February 2014 and January 2016 were enrolled. Patients with a postnatal diagnosis after 1 month of life were excluded. Breathing pattern and diaphragmatic function were assessed using esophageal and gastric (Pgas) pressure recording after surgery, or at 1 or 5 years of age. RESULTS: Twenty-eight patients (24 left-sided CDH, 6 with diaphragmatic patch) were included. Twelve patients were assessed before hospital discharge (Y0), 6 around the age of 1 year (Y1) and 10 around the age of 5 years (Y5). Mean antenatal estimated pulmonary volume (VLA) was 42 ± 10% (n = 23). Diaphragmatic strength, assessed by transdiaphragmatic pressure during crying/sniff, was low at Y0 (47 ± 18 cmH2O, n = 12) and within normality at Y5 (81 ± 15 cmH2O, n = 7). Diaphragmatic dysfunction, assessed by Pgas during crying/sniff, was present at Y0 (-58 ± 22 cmH2O, n = 12) and Y1 (-53 ± 36 cmH2O, n = 5) and still present at Y5 (3 ± 9 cmH2O, n = 7) but to a lesser extent. The diaphragmatic tension time index (TTdi), which estimates diaphragmatic endurance, was high at Y0 (0.10 ± 0.04, n = 11) and within normality at Y5 (0.03 ± 0.01, n = 6). VLA correlated with neonatal TTdi (r = -0.961, P < 0.001). CONCLUSIONS: Infants with CDH have diaphragmatic dysfunction in the neonatal period, which correlates with VLa and normalizes with age. Future longitudinal studies should assess the role of CDH side, size of diaphragmatic defect and patch repair.

20.
Sleep Breath ; 22(2): 311-316, 2018 05.
Artigo em Inglês | MEDLINE | ID: mdl-28281031

RESUMO

PURPOSE: The characterization of apneas during polysomnography (PSG) as obstructive or central is a key element of a sleep study. Pulse transit time (PTT) has demonstrated its potential as a noninvasive surrogate marker for inspiratory efforts. The aim of the study was to assess the ability of PTT to classify apneas as central or obstructive, as compared to respiratory inductance plethysmography (RIP) in children. METHODS: Overnight PSG with simultaneous PTT recording was performed on 11 consecutive children (mean age 8.9 years, range 1-18.2 years). The same observer scored the apneas using two blinded configurations: (1) the RIP scoring used the nasal pressure, thermistors, thoracic and abdominal movements, and pulse oximetry signals: (2) the PTT scoring used PTT in combination with all the other signals without the thoracic and abdominal movements. RESULTS: One hundred fourteen apneas out of a total of 520 respiratory events were analyzed. With RIP, 58 (51%) apneas were scored as obstructive and 56 (49%) as central. Using PTT, 77 (68%) of the apneas were scored as obstructive and 37 (32%) as central. When using PTT, 30 apneas scored as central by RIP were scored as obstructive. PTT was highly sensitive (81%) but poorly specific (46%) in scoring 58 apneas as obstructive. PTT was less sensitive (46%) but highly specific (81%) to score 56 apneas as central. CONCLUSION: PTT may be used as an additional tool to RIP to improve the scoring of apneas as obstructive or central in children. The high percentage of artifact is a limitation of PTT.

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