Helmut Teschler

Long­term noninvasive ventilation in children and adolescents with neuromuscular disorders

The aim of the current study was to investigate the longterm impact of nocturnal noninvasive (positivepressure) ventilation (NIV) on sleep, sleepdisordered breathing (SDB) and respiratory function in children and adolescents with progressive neuromuscular disorders (NMD). Thirty patients (12.3±4.1u2005yrs) with various inherited NMD were treated with NIV for ventilatory insufficiency (n=14) or symptomatic SDB (n=16). Patients were prospectively followed with sleep studies, spirometry and peak inspiratory muscle pressure. Ten patients were studied before and after 3 nights withdrawal from NIV. NIV normalised nocturnal gas exchange in all patients and diurnal gas exchange in patients with ventilatory insufficiency. The effects persisted over 25.3±12.7 months. Nocturnal transcutaneous partial pressure of carbon dioxide improved from (baseline versus latest control) 7.1±1.3 to 5.5±0.6u2005kPa (53.7±9.9 to 41.6±4.8u2005mmHg), diurnalcarbon dioxide arterial tension from 6.3±1.6 to 5.4±0.5u2005kPa (47.5±11.9 to 40.6±3.6u2005mmHg). NIV improved respiratory disturbance index, arousals from sleep, nocturnal heart rate and sleep architecture. Vital capacity decreased in five adolescents with Duchenne muscular dystrophy −183±111u2005mL·yr−1 but remained stable in 25 children with other conditions (8±78u2005mL·yr−1). Three nights withdrawal of NIV in 10 previously stable patients resulted in prompt deterioration of SDB and gas exchange back to baseline but could be instantly normalised by resumption of NIV. Noninvasive (positivepressure) ventilation has favourable longterm impact on nocturnal and diurnal gas exchange and sleep and in patients with nonDuchenne neuromuscular disorders on vital capacity as well. It is indicated in children and adolescents with symptomatic sleepdisordered breathing or ventilatory insufficiency due to neuromuscular disorders.

Daytime predictors of sleep disordered breathing in children and adolescents with neuromuscular disorders

Sleep disordered breathing with or without nocturnal hypercapnic hypoventilation is a common complication of respiratory muscle weakness in childhood neuromuscular disorders. Nocturnal hypercapnic hypoventilation as a sign of respiratory muscle fatigue, portends a particularly poor prognosis. We aimed at identifying daytime predictors of sleep disordered breathing at its onset and sleep disordered breathing with nocturnal hypercapnic hypoventilation. Forty-nine children and adolescents (11.3+/-4.4 years) with progressive neuromuscular disorders were studied with inspiratory vital capacity, peak inspiratory pressure, arterial blood gases, polysomnography, and a ten-item symptoms questionnaire. Daytime respiratory function was prospectively compared with polysomnographic variables. Sleep disordered breathing was found in 35/49 patients (71%). Twenty-four (49%) had sleep disordered breathing with nocturnal hypercapnic hypoventilation. Inspiratory vital capacity and peak inspiratory pressure, but not symptom score, correlated with sleep disordered breathing and severity of nocturnal hypercapnic hypoventilation. Sleep disordered breathing-onset was predicted by inspiratory vital capacity<60% (sens. 97%, spec. 87%). Sleep disordered breathing with nocturnal hypercapnic hypoventilation was predicted by inspiratory vital capacity<40% (sens. 96%, spec. 88%) and PaCO(2)>40 mmHg (sens. 92%, spec. 72%,). Sleep disordered breathing can reliably be predicted from simple daytime respiratory function tests, which, if applied systematically, will improve recognition of nocturnal respiratory failure.

Diagnostic performance of single airflow channel recording (ApneaLink) in home diagnosis of sleep apnea

BackgroundApneaLink is a novel single-channel screening device for sleep apnea detection which is based on pressure-transduced measurement of oronasal airflow, summarised as respiratory disturbance index per hour of recording time (RDIApneaLink). We tested ApneaLinks diagnostic performance in a patient population with high prevalence of sleep apnea.MethodsApneaLink was applied simultaneously with in-laboratory polysomnography (PSG) (nu2009=u2009102, 24 female, age 54.7xa0years) and sequentially with PSG in the unattended home setting (nu2009=u2009131, 37 female, age 59.1xa0years). Predictive values were computed for apnea-defining thresholds of apnea–hypopnea index (AHI)u2009≥u20095/h, ≥10/h, ≥15/h. Night-to-night variability (NNV) was assessed over three consecutive nights (nu2009=u200955, 10 female, age 48.9xa0years).ResultsRDIApneaLink correlated well with apnea–hypopnea index on PSG (PSGAHI) on simultaneous (ru2009=u20090.98, bias −0.7) and unattended home application (ru2009=u20090.95, bias −0.6). Predictive values were highest at AHIu2009≥u200910/h (in-laboratory: sensitivity 91.1%, specificity 87.5%, LR+ (positive likelihood ratio) 7.4, LR− 0.1; home: sensitivity 80%, specificity 85.5%, LR+5.5, LR− 0.2). NNV was low

Nächtliche nichtinvasive Beatmung bei Kindern und Jugendlichen mit neuromuskulären Erkrankungen: Einfluss auf Schlaf und Symptome

left( {V = 0.58 pm 0.44,{text{range}},0 - 1.69} right)

Hyperinsufflationsassistiertes Hustenmanöver bei neuromuskulären Erkrankungen

.ConclusionApneaLink is an accurate screening tool for sleep apnea in a population with high prevalence of the disorder.

Classification of sleep and wake using a novel minimal-contact single-channel device

Chest infections are serious complications in neuromuscular disorders. The predictive values of lung and respiratory muscle function including peak cough flow still remain unclear. We performed retrospective analysis of 46 children and adolescents (12.7+/-3.7 years) in whom lung function, respiratory muscle function and peak cough flows had been obtained. Data were related to: (1). number of chest infections and days of antibiotic treatment the year prior to the study and (2). history of severe chest infection requiring hospital admission. The number of chest infections and the number of days treated with antibiotics correlated with Inspiratory Vital Capacity IVC, peak cough flow PCF and Peak Expiratory Pressure PEP. Twenty-two patients were hospitalized at least once due to severe chest infection. IVC (0.65 vs. 1.44 l; P<0.0001) and PCF (116 vs. 211 l/min; P<0.0005) in these patients were significantly lower than in the non-hospitalized group. IVC<1.1l and PCF<160 l/min were specific and sensitive thresholds to discriminate between patients who had already suffered severe chest infections and those who had not. Therefore, spirometry and peak cough flow are reliable tests to identify patients at high risk for severe chest infections. Patients with IVC below 1.1l and/or PCF below 160 l/min should be well monitored and introduced to assisted coughing techniques.

Nichtinvasive Beatmung bei neuromuskulären Erkrankungen

SummaryAimSleep disordered breathing (SDB) is an inevitable complication of progressive neuromuscular disorders (NMD). Aim of the study was to prospectively investigate the impact of three months of non-invasive ventilation (NIV) on sleep and SDB-associated symptoms in children and adolescents with advanced NMD.MethodsTwenty-two patients (11.1±4.3 years) with symptomatic SDB were enrolled in the study and evaluated with polysomnography and a symptom questionnaire. Twenty patients were treated with pressure assisted ventilation during sleep. The impact of NIV on SDB, sleep and symptoms was re-evaluated after 3 months of NIV.ResultsIn 2/22 patients, after adenotomy, SDB was no longer demonstrable. In the remaining 20 patients NIV improved SDB (Respiratory Disturbance Index 1.8±1.6 vs. 9.2±4.5/h; minimal SpO2 91±3 vs. 79±12%). Three months of treatment resulted in shortened sleep latency (17±13 vs. 42±38 minutes), improved sleep efficiency (95±3 vs. 89±9%) and less EEG-arousals (11±5 vs. 22±9/h, p<0.001 for all). Sleep-stage proportion did not change. Patients reported significant improvements of sleep quality, morning headaches, mood, concentration and daytime sleepiness (p<0.001 for all).ConclusionsIn children with NMD intermittent nocturnal NIV results in objective and subjective improvements of sleep quality and symptoms associated with SDB.ZusammenfassungZielSchlafbezogene Atmungsstörungen (SBAS) sind eine obligate Komplikation von progredienten neuromuskulären Erkrankungen (NME). Ziel der Studie war die prospektive Untersuchung des Einflusses von drei Monaten nichtinvasiver Beatmung (NIB) auf den Schlaf und die mit SBAS assoziierten Symptome von Kindern und Jugendlichen mit fortgeschrittenen NME.MethodeZweiundzwanzig Patienten mit symptomatischen SBAS (11,1±4,3 Jahre) wurden in die Studie eingeschlossen und mit einer Polysomnographie und einem Symptomfragebogen untersucht. 20 Patienten wurden während des Nachtschlafes mit druckgesteuerten Beatmungsgeräten über Masken beatmet, die Evaluation wurde nach drei Monaten Behandlung wiederholt.ErgebnisseBei zwei Patienten waren nach Adenotomie keine SBAS mehr nachweisbar. Bei den übrigen 20 Patienten normalisierte die NIB die Atmung und den Gasaustausch im Schlaf (Respiratory Disturbance Index 1,8±1,6 vs. 9,2±4,5/h und minimale SpO2 91±3 vs. 79±12%). Nach drei Monaten wurde während der Beatmung eine kürzere Einschlaflatenz (17±13 vs. 42±38 Minuten), eine höhere Schlafeffizienz (95±3 vs. 89±9%) und weniger EEG-Arousals (11±5 vs. 22±9/h) gemessen (alle p<0,001). Die Schlafstadienverteilung änderte sich nicht. Die subjektive Schlafqualität, morgendlichen Kopfschmerzen, Stimmung, Konzentrationsfähigkeit und Tagesmüdigkeit wurden als signifikant gebessert beurteilt (alle p<0,001).SchlussfolgerungDie intermittierende nächtliche NIB führt mittelfristig zu einer objektiven und subjektiven Verbesserung der Schlafqualität sowie der mit SBAS assoziierten Tagessymptome.AIMnSleep disordered breathing (SDB) is an inevitable complication of progressive neuromuscular disorders (NMD). Aim of the study was to prospectively investigate the impact of three months of non-invasive ventilation (NIV) on sleep and SDB-associated symptoms in children and adolescents with advanced NMD.nnnMETHODSnTwenty-two patients (11.1 +/- 4.3 years) with symptomatic SDB were enrolled in the study and evaluated with polysomnography and a symptom questionnaire. Twenty patients were treated with pressure assisted ventilation during sleep. The impact of NIV on SDB, sleep and symptoms was re-evaluated after 3 months of NIV.nnnRESULTSnIn 2/22 patients, after adenotomy, SDB was no longer demonstrable. In the remaining 20 patients NIV improved SDB (Respiratory Disturbance Index 1.8 +/- 1.6 vs. 9.2 +/- 4.5/h; minimal SpO2 91 +/- 3 vs. 79 +/- 12%). Three months of treatment resulted in shortened sleep latency (17 +/- 13 vs. 42 +/- 38 minutes), improved sleep efficiency (95 +/- 3 vs. 89 +/- 9%) and less EEG-arousals (11 +/- 5 vs. 22 +/- 9/h, p < 0.001 for all). Sleep-stage proportion did not change. Patients reported significant improvements of sleep quality, morning headaches, mood, concentration and daytime sleepiness (p < 0.001 for all).nnnCONCLUSIONSnIn children with NMD intermittent nocturnal NIV results in objective and subjective improvements of sleep quality and symptoms associated with SDB.

Progrediente neuromuskuläre Erkrankungen

The aim of this study was to assess the validity of an artificial neural network based on flow-related spectral entropy as a diagnostic test for obstructive sleep apnoea and Cheyne-Stokes respiration. A data set of 37 subjects was used for spectral analysis of the airflow by performing a fast Fourier transform. The examined intervals were divided into epochs of 3 min. Spectral entropy S was applied as a measure for the spread of the related power spectrum. The spectrum was divided into several frequency areas with various subsets of spectral entropy. We studied 11 subjects with obstructive apnoeas (n = 267 epochs), 10 subjects with obstructive hypopnoeas (n = 80 epochs), 11 subjects with Cheyne-Stokes respiration (n = 253 epochs) and 5 subjects with normal breathing in non-REM sleep (n = 174 epochs). Based on spectral entropy an artificial neural network was built, and we obtained a sensitivity of 90.2% and a specificity of 90.9% for distinguishing between obstructive apnoeas and Cheyne-Stokes respiration, and a sensitivity of 91.3% and a specificity of 94.6% for discriminating between obstructive hypopnoeas and normal breathing in non-REM sleep. This resulted in an accuracy of 91.5% for identifying flow patterns of obstructive sleep apnoea, Cheyne-Stokes respiration and normal breathing in non-REM sleep. It is concluded that the use of an artificial neural network relying on spectral analysis of the airflow could be a useful method as a diagnostic test for obstructive sleep apnoea and Cheyne-Stokes respiration.