[Guidelines for Non-Invasive and Invasive Home Mechanical Ventilation for Treatment of Chronic Respiratory Failure - Update 2017]. / S2k-Leitlinie: Nichtinvasive und invasive Beatmung als Therapie der chronischen respiratorischen Insuffizienz ­ Revision 2017.

Today, invasive and non-invasive home mechanical ventilation have become a well-established treatment option. Consequently, in 2010 the German Society of Pneumology and Mechanical Ventilation (DGP) has leadingly published the guidelines on "Non-Invasive and Invasive Mechanical Ventilation for Treatment of Chronic Respiratory Failure". However, continuing technical evolutions, new scientific insights, and health care developments require an extensive revision of the guidelines.For this reason, the updated guidelines are now published. Thereby, the existing chapters, namely technical issues, organizational structures in Germany, qualification criteria, disease specific recommendations including special features in pediatrics as well as ethical aspects and palliative care, have been updated according to the current literature and the health care developments in Germany. New chapters added to the guidelines include the topics of home mechanical ventilation in paraplegic patients and in those with failure of prolonged weaning.In the current guidelines different societies as well as professional and expert associations have been involved when compared to the 2010 guidelines. Importantly, disease-specific aspects are now covered by the German Interdisciplinary Society of Home Mechanical Ventilation (DIGAB). In addition, societies and associations directly involved in the care of patients receiving home mechanical ventilation have been included in the current process. Importantly, associations responsible for decisions on costs in the health care system and patient organizations have now been involved.The currently updated guidelines are valid for the next three years, following their first online publication on the home page of the Association of the Scientific Medical Societies in German (AWMF) in the beginning of July 2017. A subsequent revision of the guidelines remains the aim for the future.

[Recommendations for respiratory muscle testing]. / Empfehlungen der Deutschen Atemwegsliga zur Messung der Atemmuskelfunktion.

Based on the tremendous impact of impaired respiratory muscle function, tests on their function play a significant role in respiratory and intensive care medicine. Besides differential diagnosing e.g. during prolonged weaning and quantification of impaired respiratory muscle function, e.g. in COPD, neuro-muscular diseases or ventilator-induced diaphragmatic dysfunction, those tests qualify for follow-up assessment, e.g. phrenic nerve lesions or specific respiratory muscle training. In general, (simple) volitional and (complex) non-volitional tests are available. Volitional tests aim at screening for potential respiratory muscle impairment, while non-volitional tests - including ultrasound application - are used to further specify low values assessed by volitional tests and to assess complex clinical conditions (e.g. intubated, sedated patients). Several tests are complementary or additive to each other. Complete assessment for respiratory muscle function, therefore, frequently requires the combination of different test regimes. The current recommendations include in-depth description and practical guidelines for the different tests and approaches to assess respiratory muscle function.

Development of a quantitative immunofluorescence assay for detection of Stenotrophomonas maltophilia antibodies in patients with cystic fibrosis.

BACKGROUND: To describe a simple quantitative immunofluorescence assay (IFA) for the detection of specific Stenotrophomonas maltophilia antibodies in serum of CF patients. METHODS: A total of 100 sera (64 CF patients and 36 healthy subjects) were collected over a period of 2 years at the University Hospital Essen, Germany. Sputum culture status classified CF patients into groups. Serologic response was determined after Pseudomonas aeruginosa absorption by indirect IFA to Sm whole cell. RESULTS: CF patients with "chronic S. maltophilia" showed significantly higher S. maltophilia antibody levels compared with healthy individuals (P<0.0001) and CF patients with "intermittent" (P=0.0315) or "never S. maltophilia/P. aeruginosa" (P=0.0002). A discriminant cut-off value of >1:120 titre was established to differentiate "CF chronic S. maltophilia" from the other groups. For "CF chronic S. maltophilia", the IFA showed sensitivity and specificity values of 70.7% and 84.7%, respectively. CONCLUSION: Our data demonstrated that quantitative IFA is a simple serological assay for the detection of specific S. maltophilia antibodies, which could be useful as a diagnostic tool for monitoring immune response of CF patients to S. maltophilia.

Stenotrophomonas maltophilia in cystic fibrosis: improved detection by the use of selective agar and evaluation of antimicrobial resistance.

BACKGROUND: We implemented a selective medium for improved detection of Stenotrophomonas maltophilia in sputum samples from CF patients. We also performed antimicrobial susceptibility testing with eight antibiotics. METHODS: A total of 623 consecutive sputum samples from 165 CF patients in a German CF center were cultured onto conventional media and onto Steno medium agar (SMA). All isolates confirmed as S. maltophilia by biochemical and molecular methods were subjected to antimicrobial susceptibility testing. The following agents were tested by Etest: ceftazidime, levofloxacin, moxifloxacin, tigecycline, trimethoprim-sulfamethoxazole, fosfomycin, colistin, and ticarcillin-clavulanate acid. RESULTS: Conventional media supported the growth of S. maltophilia in 7.1% of samples, whereas SMA supported its growth in 11.6%, increasing the detection rate to 64%. Trimethoprim-sulfamethoxazole and tigecycline exhibited the highest in vitro activity, whereas ceftazidime, colistin, and ticarcillin-clavulanate acid exhibited higher resistance rates. CONCLUSIONS: SMA is a promising medium allowing improved isolation of S. maltophilia from sputum samples from CF patients. Trimethoprim-sulfamethoxazole and tigecycline demonstrated excellent inhibitory effects against S. maltophilia, which may suggest a potential clinical effect.

[Non-invasive and invasive mechanical ventilation for treatment of chronic respiratory failure. S2-Guidelines published by the German Medical Association of Pneumology and Ventilatory Support]. / Nichtinvasive und invasive Beatmung als Therapie der chronischen respiratorischen Insuffizienz. S2-Leitlinie herausgegeben von der Deutschen Gesellschaft für Pneumologie und Beatmungsmedizin e. V.

The field of mechanical ventilation is highly important in pulmonary medicine. The German Medical Association of Pneumology and Ventilatory Support ["Deutsche Gesellschaft für Pneumologie und Beatmungsmedizin e. V. (DGP)"] therefore has formulated these guidelines for home mechanical non-invasive and invasive ventilation. Non-invasive home mechanical ventilation can be administered using various facial masks; invasive home mechanical ventilation is performed via a tracheostomy. Home mechanical ventilation is widely and increasingly accepted as a treatment option for chronic ventilatory failure which most often occurs in COPD, restrictive lung diseases, obesity-hypoventilation syndrome and neuromuscular disorders. Essential for the initiation of home mechanical ventilation are the presence of symptoms of ventilatory failure and the detection of hypoventilation, most importantly hypercapnia. These guidelines comprise general indication criteria along with disease-specific criteria summarised by treatment algorithms. In addition, the management of bronchial secretions and care of paediatric patients are addressed. Home mechanical ventilation must be organised around a specialised respiratory care centre with expertise in patient selection, the initiation and the control of home mechanical ventilation. In this regard, the guidelines provide detailed information about technical requirements (equipment), control and settings of mechanical ventilation as well as organisation of patient care. A key requirement for home mechanical ventilation is the qualification of specialised home-care services, which is addressed in detail. Independent living and the quality of respiratory care are of highest priority in patients receiving home mechanical ventilation, since home mechanical ventilation can interfere with the integrity of a patient and often marks a life-sustaining therapy. Home mechanical ventilation has been shown to improve health-related quality of life of patients with chronic ventilatory failure. Long-term survival is improved in most patient groups, even though the long-term prognosis is often severely limited. For this reason, ethical issues regarding patient education, communication with ventilated patients at the end of life, living will, testament and medical care during the dying process are discussed.

Non-invasive ventilation reduces respiratory tract infections in children with neuromuscular disorders.

Non-invasive ventilation (NIV) has been shown to improve gas exchange and survival in patients with chronic neuromuscular disorders. Little is known about its influence on respiratory tract infections (RTIs). Twenty-four patients with regular use of NIV and 11 patients without NIV with neuromuscular disorders answered a questionnaire concerning the use of NIV and assisted coughing techniques, the status of influenza and pneumococcus vaccination, and the frequency and severity of RTIs. Additionally, we performed a retrospective chart review of twelve patients who were ventilated over a period of at least 5 years. In the first year of NIV consultations of a general practitioner due to RTI decreased from 9.2+/-20.8 to 3.2+/-5.3 per year (P<0.005), the number of antibiotic treatment due to RTI decreased from 4.1+/-3.4 to 1.9+/-2.2 per year (P<0.005) and the number of hospital admissions due to RTI decreased from 1.6+/-1.7 to 0.7+/-1.3 per year (P<0.005). Vaccinations against influenza and/or pneumococcus did not have a significant influence on the rate of infections. In 12 patients using NIV for more than 5 years the incidence of RTI requiring hospital admission decreased from 0.54+/-0.41/year in the pre-ventilation period to 0.12+/-0.09/year in the NIV period (P<0.005). NIV had a favorable impact on respiratory infectious complications in children with neuromuscular disorders.

Predictors of severe chest infections in pediatric neuromuscular disorders.

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.

Respiratory failure in Pompe disease: treatment with noninvasive ventilation.

In this study, noninvasive ventilation (NIV) was prospectively applied to eight patients (35.8 +/- 11.4 years) with late-onset Pompe disease and respiratory failure apparent from severe restrictive lung disease, nocturnal hypoxemia (83 +/- 8%), and daytime hypercapnia (66.7 +/- 17.9 mm Hg). The impact of NIV on respiratory function was followed for 34 +/- 17 months. Despite further decrease of vital capacity and inspiratory muscle strength, NIV normalized oxygen saturation during sleep (96 +/- 1%), daytime carbon dioxide tensions (44.1 +/- 3.6 mm Hg), and symptoms.

[Sleep-disordered breathing in neuromuscular diseases]. / Schlafbezogene Atmungsstörungen bei neuromuskulären Erkrankungen.

UNLABELLED: Sleep-disordered breathing is common in neuromuscular diseases but remains poorly defined in its relationship to daytime respiratory function. METHODS: We prospectively compared supine lung and respiratory muscle function with results of polysomnography/capnometry in 35 patients with progressive myopathies (age 32.5 +/- 15 years). RESULTS: 32/35 patients had restrictive ventilatory defect, 9/32 had hypercapnic respiratory failure by day (PaCO(2) 66.0 +/- 15.7 mm Hg). Supine inspiratory vital capacity (IVC(S)) correlated with maximal inspiratory muscle pressure (R = 0.75), respiratory muscle strain (P0.1/PImax, R = - 0.68), and daytime blood gases (p < 0.005 for all). SDB in the way of REM-sleep hypopneas, circumscribed hypoventilation episodes and sleep-stage independent continuous hypoventilation (PtcCO(2) > 50 mmHg > 50% of sleep time) was common at IVC(S)< 60% pred, and preceded daytime hypercapnia. IVC(S) correlated with nocturnal SaO(2) (R = 0.64), PtcCO(2) (R = - 0.87), percent light sleep (R = 0.67) and deep sleep (R = - 0.76). IVC(S) correlated only marginally with respiratory disturbance index (total sleep, R = - 0.45; REM-sleep, R = - 0.44). IVC(S) < 60% was 96% sensitive, 78% specific for presence of SDB. IVC(S) < 20 % was 89% sensitive, 96% specific for daytime hypercapnia. CONCLUSIONS: IVC(S) correlates with respiratory muscle function, daytime and nocturnal blood gases, and is highly predictive of SDB and daytime hypercapnia.

Long-term noninvasive ventilation in children and adolescents with neuromuscular disorders.

The aim of the current study was to investigate the long-term impact of nocturnal noninvasive (positive-pressure) ventilation (NIV) on sleep, sleep-disordered breathing (SDB) and respiratory function in children and adolescents with progressive neuromuscular disorders (NMD). Thirty patients (12.3 +/- 4.1 yrs) 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 +/- 0.6 kPa (53.7 +/- 9.9 to 41.6 +/- 4.8 mmHg), diurnal carbon dioxide arterial tension from 6.3 +/- 1.6 to 5.4 +/- 0.5 kPa (47.5 +/- 11.9 to 40.6 +/- 3.6 mmHg). 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 +/- 111 mL x yr(-1) but remained stable in 25 children with other conditions (8 +/- 78 mL x 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 (positive-pressure) ventilation has favourable long-term impact on nocturnal and diurnal gas exchange and sleep and in patients with non-Duchenne neuromuscular disorders on vital capacity as well. It is indicated in children and adolescents with symptomatic sleep-disordered breathing or ventilatory insufficiency due to neuromuscular disorders.

Patterns and predictors of sleep disordered breathing in primary myopathies.

BACKGROUND: Sleep disordered breathing (SDB) is common in neuromuscular diseases but its relationship to respiratory function is poorly defined. A study was undertaken to identify distinct patterns of SDB, to clarify the relationships between SDB and lung and respiratory muscle function, and to identify daytime predictors for SDB at its onset, for SDB with continuous hypercapnic hypoventilation, and for diurnal respiratory failure. METHODS: Upright and supine inspiratory vital capacity (IVC, % predicted), maximal inspiratory muscle pressure (PImax), respiratory drive (P(0.1)), respiratory muscle effort (P(0.1)/PImax), and arterial blood gas tensions were prospectively compared with polysomnography and capnometry (PtcCO(2)) in 42 patients with primary myopathies. RESULTS: IVC correlated with respiratory muscle function and gas exchange by day and night. SDB evolved in three distinct patterns from REM hypopnoeas, to REM hypopnoeas with REM hypoventilation, to REM/non-REM (continuous) hypoventilation, and preceded diurnal respiratory failure. SDB correlated with IVC and PImax which yielded highly predictive thresholds for SDB onset (IVC <60%, PImax <4.5 kPa), SDB with continuous hypoventilation (IVC <40%, PImax <4.0 kPa), and SDB with diurnal respiratory failure (IVC <25%, PImax <3.5 kPa). CONCLUSION: Progressive ventilatory restriction in neuromuscular diseases correlates with respiratory muscle weakness and results in progressive SDB which, by pattern and severity, can be predicted from daytime lung and respiratory muscle function.

Sleep-disordered breathing and respiratory failure in acid maltase deficiency.

BACKGROUND: Sleep-disordered breathing (SDB) and respiratory failure (RF) are complications of acid maltase deficiency (AMD), a rare hereditary myopathy. OBJECTIVE: To define the relationship between lung and respiratory muscle function, to establish incidence and patterns of SDB, and to determine daytime predictors of SDB. METHODS: Sitting and supine lung and respiratory muscle function tests were obtained in 27 subjects with juvenile and adult AMD (aged 39 +/- 19 years) and compared with outcomes of polysomnography. RESULTS: Ventilatory restriction was present in 17/27 subjects. Inspiratory vital capacity (IVC) correlated (p < 0.005) with peak inspiratory muscle pressure (PIP, R = 0.61), respiratory muscle strain (P(0.1)/P(0.1max), R = -0.68), and gas exchange by day (PaO(2): R = 0.71; PaCO(2): R = -0.64) and night (SaO(2): R = 0.73; P(tc)CO(2): R = -0.75). Diaphragm weakness (DW) was present in 13 subjects, 10 of whom had hypercapnic RF (PaCO(2) 65 +/- 7 mm Hg), and was associated with longer disease course. SDB was found in 13 subjects, 12 with DW. It was characterized by REM-sleep hypopneas that, as ventilatory restriction worsened, were complemented by hypoventilation (P(tc)CO(2) > 50 mm Hg) first in REM sleep, then in non-REM sleep (p < 0.005). SDB was predicted by DW (sensitivity 80%, specificity 86%) and nocturnal hypoventilation by IVC < 40% (sensitivity 80%, specificity 93%). Noninvasive ventilation, instituted for daytime respiratory failure or nocturnal hypoventilation, normalized daytime and nocturnal gas exchange (p < 0.005). CONCLUSION: Vital capacity correlates with respiratory muscle function in AMD. Diaphragm weakness is the major cause of SDB and RF. SDB and nocturnal hypoventilation are predictable from daytime function tests.

[Importance of medical history in diagnosis of respiratory insufficiency in patients suffering from neuromuscular diseases and thoracic deformities]. / Stellenwert der Anamnese in der Diagnostik der respiratorischen Insuffizienz bei Patienten mit neuromuskulären Erkrankungen und Thoraxdeformitäten.

Patients suffering from neuromuscular diseases and thoracic deformities may develop global respiratory failure during their illness. We wanted to judge clinical parameters and information from the patients' medical history to reliably, quickly and noninvasively diagnose a ventilatory failure. Therefore we evaluated 105 situations with and without mechanical ventilation from 29 patients with indication for noninvasive nocturnal mask ventilation. 6 clinical parameters (e.g. heart rate, oxygen saturation, relative vital capacity), 2 test results (pH and partial pressure of carbon dioxide (pCO2)) and 6 parameters from the patients' medical history (e.g. nycturia, frontal headache in the morning, breathlessness) were investigated. After statistical evaluation we could show a relation between heart rate and pCO2 (Spearman's correlation: r = 0.331, p = 0.001, n = 105; one-tailed significance: r = 0.335, p = 0.038, n = 29). Significant differences between the groups of nycturia incidence indicate a tight relation between the incidence of nycturia and the height of hypercapnia levels (ANOVA--analysis of variance: p = 0.001). Using logistic regression we could show that information regarding medical history, especially nycturia, frontal headache and indrawings, gives important indications for global respiratory failure (sensitivity 97.62-100%, specificity 57.14-76.19%). Pathogenesis needs to be elaborated further.

[The incidental heart sound. Study of clinical diagnostic certainty and possible causes]. / Das akzidentelle Herzgeräusch. Untersuchung zur klinischen Diagnosesicherheit und möglichen Ursachen.

The diagnosis "innocent murmur" is made when there is no evidence for a heart disease either by history nor by examination, auscultation, electrocardiogram or echocardiography. The purpose of the present study was to determine the usefulness of echocardiography for diagnosing of an innocent heart murmur and further to compare the findings of ultrasound with those of the clinical examination. Children were accepted for the study after clinical examination and evaluation of the electrocardiogram (but before echocardiography) only when two independent pediatricians did not realize any symptoms of a heart disease. A total number of 200 children were examined; 121 were male and 79 female. 194 children (97%) had an innocent murmur, six children (3%) a heart disease [atrial septum defect (3), bicuspid aortic valve (1), aortic stenosis (1) or pulmonary stenosis]. The sensitivity and positive predictive value of clinical examination and auscultation ranged from 92% to 99%, the specificity from 50% to 60%. In six cases the initial diagnosis had to be changed after echocardiography. Three times an innocent murmur was diagnosed as a heart disease and another three times a pathological murmur was regarded as innocent. Left ventricular bands were seen in 53 (26,5%) children and they significantly correlated with the musical heart murmur (77%). If there was no false tendon, the murmur was mostly an uncharacteristic one (74% (less than 0,001). The echocardiographic findings were not different of those children without a murmur or heart disease. The average peak velocity in the ascending aorta was 143,1 +/- 23,6 cm/s (87-215 cm/s), the shortening fraction 36,2 +/- 6,3%. We could not find a correlation between velocity, shortening fraction and aortic diameter.