Results of the Diaphragmatic Plication Database: 10 Years' Experience.

BACKGROUND: Unilateral diaphragmatic paralysis or paresis (UDP) in adults is an often overlooked disease which relevantly impairs the patient's lung function and quality of life. Particularly in idiopathic UDP, there is no evidence for conservative therapy and only little evidence for surgical therapy. METHODS: The method involves retrospective single-center analysis of patients with UDP persistent for at least 1 year who were operated by diaphragmatic resection, plication, and augmentation with a polypropylene mesh. The patients were tested for lung and diaphragmatic function, six-minute walk test (6MWT), and blood gas analysis before, 3 and 12 months after surgery. RESULTS: In total, 85 patients received surgery for UDP. The most frequent reasons for UDP were idiopathic (67%), iatrogenic (mainly cardiac and cervical spine surgery; 24%), and trauma (9%). The mean operation time was 84 ± 24 minutes, the length of hospital stay 8.4 ± 3.9 days, chest tubes were removed after 11.7 ± 4.1 days. Overall morbidity was 42%, mortality 0%. Forced expiratory volume in one second (FEV1) in supine position improved by 12.4% absolute, vital capacity by 11.8% absolute, and sniff nasal inspiratory pressure by 1.4 kPa 12 months after surgery (p <0.001 each). Total lung capacity increased by 6.8% absolute at 12 months (p = 0.001) The 6MWT distance improved by 45.9 m at 3 months and 50.9 m at 12 months (p = 0.001, each). CONCLUSION: Surgical therapy for UDP is highly effective in the long term. The superiority over conservative treatments needs to be evaluated prospectively with standardized physiotherapeutic protocols. FEV1 in supine position and 6MWT are easy to perform tests and represent statistically and patient-relevant outcomes.

[Neuralgic amyotrophy: a common cause of unilateral and bilateral diaphragmatic pareses]. / Die neuralgische Amyotrophie: eine häufige Ursache uni- oder bilateraler Zwerchfellparesen.

There are several causes for unilateral or bilateral diaphragmatic paresis. The most common cause is an (intraoperative) injury to the phrenic nerve.However, in up to 20% of cases, no explanation can be found despite extensive workup. Neuralgic amyotrophy (NA, also known as Parsonage-Turner syndrome) is a common underdiagnosed multifocal autoimmune-inflammatory disease that predominantly affects proximal nerve segments of the upper extremities. Classic symptoms include acute onset of severe pain in the shoulder girdle with delayed onset of paresis of the shoulder and arm muscles. In at least 7% of cases, the phrenic nerve is also affected. Based on the annual incidence of NA of 1:1000, the entity as a cause of diaphragmatic dysfunction is probably not as uncommon as previously thought. However, clinical experience shows that this diagnosis is often not considered, and diaphragmatic paresis gets wrongly classified as idiopathic.This is particularly disastrous because in the early stage of NA, medical therapy with corticosteroids is mostly not considered and the possibility that surgical repair of the diaphragm may be performed prematurely, given that the condition may resolve spontaneously many months after symptom onset.The aim of the present article is to raise awareness of the entity of NA as a cause of diaphragmatic paresis and to establish a standardized approach to diagnosis and treatment.

Home Non-Invasive Positive Pressure Ventilation in Chronic Obstructive Pulmonary Disease: Why, Who, and How?

Advanced chronic obstructive pulmonary disease (COPD) might result in chronic hypercapnic ventilatory failure. Similar to neuromuscular and restrictive chest wall diseases, long-term non-invasive positive pressure ventilation (NPPV) is increasingly used in chronic hypercapnic COPD. This review describes the methods, patient selection, ventilatory strategies, and therapeutic effects of long-term Home-NPPV based on randomized controlled clinical trials published since 1985 in English language retrieved from the databases PubMed and Scopus. Long-term NPPV is feasible and effective in stable, non-exacerbated COPD patients with daytime hypercapnia with arterial pressure of carbon dioxide (PaCO2) levels ≥50 mm Hg (6.6 kPa), if the applied ventilatory pressures and application times improve baseline hypercapnia by at least 20%. Patients who survived an acute hypercapnic exacerbation might benefit from long-term NPPV if hypercapnia persists 2-4 weeks after resolution of the exacerbation. Pressure-controlled ventilation or pressure-support ventilation with adequate minimum backup breathing frequencies, in combination with nasal masks or oronasal masks have been successfully used in all larger clinical trials. Ventilatory strategies with mean inspiratory pressures of up to 28 cm H2O are well-tolerated by patients, but limitations exist in patients with impaired cardiac performance. Home-NPPV with a PaCO2-reductive approach might be considered as an additional treatment option in patients with stable chronic hypercapnic COPD.

Evolution of Web-Based Training Videos Provided by the German Respiratory League for the Correct Inhalation Technique.

BACKGROUND/OBJECTIVE: To assess whether and how the use of scientifically established Web-based training videos for teaching correct inhalation technique in patients with chronic airway diseases has become accepted among the wider population. METHODS: The viewing trends of 141 freely available YouTube videos (full playing time, 01:31-04:37 min:s) provided by the German Respiratory League, covering a broad range of internationally prescribed devices, were analyzed over a 10-year period. Specific emphasis was placed both on German and international videos. RESULTS: The total number of views was 3,350,678. Non-German videos (English, Russian, Turkish, Greek, Arabic, Farsi, and Slovakian) accounted for 23.2% of the views. The number of views steadily increased between 2011 and 2020 with a mean annual increase of 54.0% (range 24.5/119.9%) compared to the respective previous year. By 2020, the incidence of views per 100,000 German inhabitants was 725 for German videos only and 1,030 for all videos. In terms of the annual trend, there were two peak viewing periods, namely in spring and late fall, while the lowest amount of views occurred in summer. CONCLUSION: This study highlights the rising impact of Web-based training videos used for teaching the correct use of inhalation devices, with a steady increase in the number of annual views and a clear seasonal peaking of views in spring and late fall.

The Italian Version of the Severe Respiratory Insufficiency Questionnaire.

BACKGROUND: The Severe Respiratory Insufficiency Questionnaire (SRI) is a multidimensional instrument for health-related quality of life (HRQL) assessment in patients with chronic respiratory failure (CRF). The SRI has originally been developed in German in 2003, but 15 translated versions have been created during the last 18 years with the exclusion of the Italian translation. AIMS OF THE STUDY: The present project was aimed at creating an Italian version of the SRI. METHODS: Professional forward-translation and back-translation procedures have been provided based on the original German version by independent translators, and this was followed by final reconciliation. RESULTS: The Italian SRI contains 49 items covering 7 different subscales which can summarize to one Summary Score. CONCLUSIONS: The Italian SRI is a multidimensional instrument, which can be used for HRQL assessment in Italian-speaking patients with CRF. Validation of the Italian version of the questionnaire is formally required in the future.

[The digitalization of pneumology and the role of medical societies]. / Die Digitalisierung der Pneumologie und die Rolle der Fachgesellschaften.

BACKGROUND: Medicine is becoming increasingly digitalized. Digital applications are finding their way into health care. The aim of the study was to record the attitudes of members of the German Respiratory Society (DGP) towards digitalization. METHODS: The study was conducted in cooperation with the DGP, the German Respiratory League and the Health Innovation Hub (HIH) of the German Federal Ministry of Health. Data were gathered with the help of an online questionnaire (July/August 2021), analyzed descriptively and supplemented with a subgroup comparison regarding proactivity of the members. RESULTS: 284 questionnaires were complete and included in the analysis (31% female); 76.4% believed that digitalization would change their daily professional life within five years. 47.2% had prescribed or planned to prescribe Digital Health Applications (DiGA). Lack of technology skills of patients and the time required for health professionals were seen as critical (49.3 and 47.5%). Regarding DiGA, scientific proof of efficacy (48.9%) and ease of use for patients (47.9%) were rated as most important. The subgroup comparison showed that 42.7% actively informed themselves about digital medicine and that this group saw more opportunities for meaningful use of DiGA. CONCLUSION: The members of the DGP have mostly a positive view about the profound changes expected from digital medicine. The more active the role of DGP members and other professional societies, the more digital elements can add value to practice.

The Severe Respiratory Insufficiency Application (SRI App): a pilot randomised controlled trial.

An application (app) for the Severe Respiratory Insufficiency Questionnaire (SRI) has been designed and developed for mobile devices. In a randomised controlled trial comprising 60 patients with chronic respiratory failure, the app was compared with the classic paper SRI. Thereby, it was shown that the SRI app is a practical tool that is well accepted. Missing values can be completely avoided by using the SRI app. Finally, reliability, convergent and discriminant validities were established. Accordingly, for the individual SRI subscales, Cronbach's alpha ranged between 0.56 and 0.81 (app) and between 0.54 and 0.83 (paper), respectively. The multilingual SRI app is accessible free of charge for non-profit research purposes.

Portable NIV for patients with moderate to severe COPD: two randomized crossover trials.

BACKGROUND: Long-term non-invasive ventilation (NIV) is as an established treatment option for chronic hypercapnic COPD patients. Beneficial effects have also been shown during exercise, but this is restricted to rehabilitation programs. New portable NIV (pNIV) devices may now enable NIV application during walking at home. STUDY DESIGN AND METHODS: In two randomized crossover trials, the impact of pNIV on dyspnea and endurance capacity was investigated in patients with moderate to severe COPD. Participants performed a standardized 6-min walking test, with and without pNIV, using a pre-set inspiratory/expiratory positive airway pressure of 18/8 cmH2O. The first study was performed in NIV-naïve patients (Study I), while the second study was performed in those already established on long-term NIV (Study II). RESULTS: 38 patients (66.9 ± 7.4 years, mean FEV1: 30.3 ± 8%pred) and 23 patients (67.6 ± 8.7 years, mean FEV1: 29.8 ± 10.4%pred) participated in Study I and II, respectively. In Study I, the mean difference in the Borg Dyspnea Scale (BDS, primary outcome) score following walking was 3.2 (IQR 2-4) without pNIV, compared to 2.6 (IQR 1-4) with pNIV (ΔBDS 0.65, P = 0.04), while walking distance increased from 311.8 m (95%CI 276.9-346.6 m) to 326.3 m (95%CI 291.5-361.2 m) (P = 0.044) when pNIV was used. Accordingly, in Study II, the mean difference in BDS was 4.4 (IQR 3-6) without pNIV, compared to 4.5 (IQR 3-6) with pNIV (ΔBDS 0.09, P = 0.54), while walking distance decreased from 291.5 m (95%CI 246.1-336.9 m) to 258.4 m (95%CI 213-303.8 m) (P ≤ 0.001). INTERPRETATION: The use of a pNIV device during walking can improve dyspnea and walking distance in patients with moderate to severe COPD. Patients who do not already receive long-term NIV therapy are more likely to benefit compared to those undergoing long-term NIV. Careful patient selection is mandatory. Clinical Trial Register: DRKS00013203; DRKS00012913 registered October 20th 2017 and October 16th 2017; https://www.drks.de/drks_web/.

Long-Term Auscultation in Chronic Obstructive Pulmonary Disease: Renaissance of an Ideograph of Medical Care.

BACKGROUND: Electronic auscultation technology has advanced dramatically in the last few years. Therefore, long-term pulmonary auscultation could provide additional information about respiratory system by monitoring acute chronic obstructive pulmonary disease (AECOPD) exacerbations or by identifying wheezing phenotypes amongst stable COPD patients. OBJECTIVES: Comparison of respiratory sounds in stable versus AECOPD patients recorded with a portable respiratory sound monitor over a period of 24 h. METHODS: This prospective trial evaluated cough and wheezing events using an auscultation monitor specially developed for this purpose with 4 integrated highly sensitive microphones, in stable and severely AECOPD patients for a period of 24 h in an inpatient setting. RESULTS: Twenty stable COPD patients (12 male, 60%) and 20 severely exacerbated COPD patients (14 male, 70%) were analyzed. In AECOPD patients, long-term auscultation revealed a significantly higher number of wheezing epochs than stable COPD patients (591 [IQR: 145-1,645] vs. 152 [IQR: 90-400]; p = 0.021). Conversely, cough epochs did not differ between AECOPD and stable COPD patients (213 [IQR: 140-327] vs. 162 [IQR: 123-243]; p = 0.256). The Borg-dyspnea scale, CAT score, and total CCQ score each showed no correlation with wheezing frequency, while CAT and CCQ scores did correlate with coughing frequency. CONCLUSION: Wheezing, but not coughing, occurs more frequently in AECOPD patients than in stable COPD patients, indicating that severe wheezing is an important clinical sign of exacerbation, while coughing is not. Therefore, the patterns of wheezing and coughing, as assessed by long-term auscultation, differ in stable versus exacerbated COPD patients.

Living conditions and autonomy levels in COPD patients receiving non-invasive ventilation: impact on health related quality of life.

BACKGROUND: Research on health-related quality of life (HRQL) has become increasingly important in recent decades. However, the impact of both living conditions and the level of autonomy impairments on HRQL in COPD patients receiving non-invasive ventilation (NIV) is still unclear. METHODS: The Severe Respiratory Insufficiency Questionnaire (SRI) was used to measure HRQL in a prospective cohort of COPD patients in whom home NIV was already established. Data on sociodemographics, clinical characteristics and standardized levels of autonomy impairment were evaluated. A multiple linear regression analysis was performed to identify the factors associated with a reduced HRQL. RESULTS: A total of 137 patients (67.0 ± 7.8 years, 45% female) were assessed. The mean SRI Summary Score was 54.1 ± 16.9 (95%CI: 51.1-57.1; N = 127). Regular ambulatory care was provided in 76% of patients, but only 37% underwent pulmonary rehabilitation. Overall, 69% of patients lived with family members, while 31% lived alone (family situation). Autonomy impairment levels were most serious in 3%, serious in 14%, and significant in 29% of patients, while 54% had no impairments at all. Of note, higher levels of autonomy impairment were markedly associated with lower SRI scores (regression coefficient - 6.5 ± 1.1 per level; P < 0.001). In contrast, family situation (0.2 ± 3.0; P = 0.959), ambulatory care by a respiratory specialist (1.7 ± 3.6; P = 0.638), and pulmonary rehabilitation (- 0.8 ± 3.1; P = 0.802) did not appear to influence HRQL. Possible subgroup effects were evident for the factors "impaired autonomy" and "living in a nursing home" (P = 0.016). CONCLUSION: A higher level of autonomy impairment has been identified as the major determinant of reduced HRQL in COPD-patients receiving long-term NIV, particularly in those living in a nursing home. Trial Registration German Clinical Trials Register (DRKS00008759).

[Invasive and Non-Invasive Home Mechanical Ventilation in Germany - A Rapid Development with Large Regional Differences]. / Invasive und nicht-invasive außerklinische Beatmung in Deutschland.

BACKGROUND: The number of patients using home mechanical ventilation (HMV) is steadily increasing in Germany. Detailed data on inpatient initiation and control of HMV are not available. This, however, is absolutely necessary in order to optimize the medical care structures in Germany. Regional diversities must be taken into consideration in order to provide care structures that reflect the local needs. METHOD: The data sets of the German Federal Statistical Office on the OPS (Operation and Procedure Classification System) for HMV from 2008 to 2019 were analysed (N = 572,494). RESULTS: Between 2008 and 2019 there was a doubling of the number of HMV initiations and controls. The number of initiations (N = 17,958) and controls (N = 49,140) was highest in 2019. Furthermore, at the state level, the development of HMV is very heterogeneous. Finally, the increases were particularly due to an increase in non-invasively ventilated patients. CONCLUSION: The rapid increase in HMV is reaching capacity limits of the existing healthcare structure. New healthcare structures should provide an integrated approach between outpatient and inpatient care in order to ensure a high quality of care for patients receiving HMV without compromising the human and economic resources of the system.

[Role of Non-Invasive Strategies in the Treatment of Acute Hypoxemic Respiratory Failure Related to COVID-19]. / Bedeutung nicht-invasiver Verfahren in der Therapie des akuten hypoxämischen Versagens bei COVID-19.

Non-invasive strategies such as HFOT (high-flow oxygen therapy), CPAP (continuous positive airway pressure) and NIV (non-invasive ventilation) are increasingly being used during the COVID-19 pandemics in order to treat acute hypoxemic respiratory failure related to COVID-19, and this is aimed at avoiding intubation. This review article summarizes the current evidence by also emphasizing its heterogeneity. Importantly, current evidence suggests that these non-invasive strategies can be successfully used even in case of severe respiratory failure and are, thus, indeed capable of avoiding intubation, and consequently, tube-related complications. In contrast, it also remains to be emphasized that prolonged spontaneous breathing supported by non-invasive treatment strategies is also prone to complications. In particular, late NIV failure is associated with substantially deteriorated outcome, which is suggested to be meaningful in view of NIV failure rates still being high in Germany. Finally, the current article also refers to a parallel article that addresses the discussion being held in the public media in Germany concerning this topic. Here, its textual questionability, but also its negative consequences for both the research community and the general society are elaborated. In this context, the importance of national and regularly updated guidelines is emphasized.

Is Outpatient Control of Long-Term Non-Invasive Ventilation Feasible in Chronic Obstructive Pulmonary Disease Patients?

BACKGROUND: Long-term non-invasive ventilation (NIV) is an established and increasingly used treatment option for patients with chronic hypercapnic chronic obstructive pulmonary disease (COPD). Following inpatient NIV establishment, inpatient control visits regularly occur thereafter. However, it remains unclear whether such control visits can also be performed in an outpatient setting, which, in turn, would reduce costs, patient burden and the complications related to hospitalization. OBJECTIVES: To investigate an outpatient setting with predefined criteria for hospitalization for patients with chronic hypercapnic COPD. METHODS: An outpatient clinic located within the hospital in the vicinity of the respiratory care unit provided predefined criteria for hospitalization of COPD patients receiving long-term NIV therapy. The results of this setting were retrospectively analysed. RESULTS: A total of 130 outpatient visits (80 patients) were analysed. In 93 cases (71.5%), hospital admission was not necessary, while hospitalization was performed in 37 cases (28.5%). Out of these, 7 cases with acute conditions required prompt hospitalization. Patients without hospitalization had better PaCO2 values (45.40 ± 5.27 vs. 50.05 ± 8.04, p = 0.002) and Severe Respiratory Insufficiency Questionnaire Summary scores (55.54 ± 19.74 vs. 41.82 ± 19.59, p = 0.012). CONCLUSION: Outpatient control of long-term NIV in a hospital setting is feasible and has the capacity to identify stable COPD patients in whom NIV therapy is sufficient according to predefined criteria. These patients may not require hospitalization and may account for more than two thirds of cases.

Long-Term Oxygen Therapy: Comparison of the German and British Guidelines.

BACKGROUND: The German guideline on long-term oxygen therapy (LTOT) was published in 2008 by the German Respiratory Society (DGP), while the British Thoracic Society (BTS) published their most recent guideline in 2015. OBJECTIVES: The aim of the present article was to highlight the major areas of consensus and disagreement in the recently published BTS and DGP guidelines on LTOT. METHODS: The BTS and DGP guidelines were directly compared in terms of congruencies and differences. A critical appraisal was then performed and authors' suggestions were provided. RESULTS: The 2 guidelines are almost congruent in 2 major areas, namely, (1) the indication criteria for LTOT in chronic obstructive pulmonary disease (COPD) patients at rest and (2) the recommended duration of LTOT over a 24-h period. However, 8 major areas in which the guidelines differ considerably were identified: (1) techniques for blood gas analysis; (2) timing of LTOT in stable patients; (3) LTOT in post-exacerbation COPD patients; (4) ambulatory oxygen therapy; (5) nocturnal oxygen therapy; (6) titration of oxygen flow rates; (7) follow-up visits; and (8) LTOT for patients who still smoke. Furthermore, the BTS guideline is much more detailed, includes more references (161 vs. 71) and is more up to date than the DGP guideline. CONCLUSION: There are major differences between the 2 guidelines. Many of the aspects raised by the BTS guideline appear to be reasonable with regard to the current literature, clinical experience and prescription practices. However, an international consensus on LTOT is lacking.

[Treatment alternatives for amyopathic MDA5-positive dermatomyositis]. / Therapiealternativen bei amyopathischer MDA5-positiver Dermatomyositis.

We report a case of an atypical course of therapy in amyopathic MDA5-antibody-positive dermatomyositis with interstitial lung disease. Due to the poor prognosis, early therapy with cyclophosphamide followed by rituximab was carried out initially in addition to the administration of prednisolone. Due to therapy failure, treatment was switched to mycophenolate mofetil. This showed a surprisingly rapid positive course in terms of interstitial lung disease, skin manifestation, and general disease activity.

The use of digital web-based video training for correct inhalation technique during the COVID-19 pandemic.

Scientifically validated web-based training videos for proper inhalation technique were increasingly used by an international audience during the pandemic. Translations into additional languages would support a larger patient population. https://bit.ly/3lYQwsD.

The Impact of Non-Invasive Ventilation on Sleep Quality in COPD Patients.

BACKGROUND: Non-invasive ventilation (NIV) has been shown to be the most appropriate therapy for COPD patients with chronic respiratory failure. While physiological parameters and long-term outcome frequently serve as primary outcomes, very few studies have primarily addressed the impact of NIV initiation on sleep quality in COPD. METHODS: This single-center prospective cohort study comprised NIV-naïve patients with COPD. All patients underwent polysomnographic evaluation both at baseline and at 3 months follow-up, accompanied by the assessment of health-related quality of life (HRQL) using the Severe Respiratory Insufficiency Questionnaire (SRI) and the Epworth Sleepiness Scale (ESS). A subgroup evaluation was performed to address the impact of comorbid obstructive sleep apnea syndrome (OSAS). RESULTS: Forty-six patients were enrolled and twenty-five patients completed the follow-up period (66.7 ± 7.4 years). NIV resulted in an increase in slow-wave sleep (+2% (-3.5/7.5), p = 0.465) and rapid eye movement sleep (+2.2% (-1.0/5.4), p = 0.174), although no statistical significance could be detected. ESS (-1.7(-3.6/0.1), p = 0.066) also showed a positive trend. Significant improvements in the Respiratory Disturbance Index (RDI) (-12.6(-23.7/-1.5), p = 0.027), lung function parameters, transcutaneous PCO2 and the SRI summary scale (4.5(0.9/8), p = 0.016) were observed. CONCLUSION: NIV therapy does not decrease sleep quality and is even capable of improving HRQL, transcutaneous PaCO2, daytime sleepiness and RDI, and the latter especially holds true for patients with comorbid OSAS.

Anemia Severely Reduces Health-Related Quality of Life in COPD Patients Receiving Long-Term Home Non-Invasive Ventilation.

PURPOSE: To assess the influence of anemia on health-related quality of life (HRQL) in COPD patients receiving long-term non-invasive ventilation (NIV). PATIENTS AND METHODS: In this prospective single-center cohort study, COPD patients on long-term NIV were analyzed between June 2015 and May 2020. Linear multiple regression analyses were performed using the results of the Severe Respiratory Insufficiency Questionnaire (SRI) along with the following variables: sex, age, body mass index, duration of NIV, exacerbation history (≤1 versus >1 in the previous year), the updated Charlson comorbidity index, hemoglobin levels and anemia (WHO criteria). RESULTS: Anemia was identified in 32.8% (N=128). Anemia (mean difference -8.4, 95% CI -2.0/-14.9 SRI points, P=0.011) and exacerbations (mean difference -9.9, 95% CI -4.3/-15.5 SRI points, P=0.001) each had a negative impact on SRI summary scores. Exacerbations were negatively associated with six out of seven SRI subscale scores, while anemia was negatively associated with four out of seven. SRI summary scores dropped by 1.5 points for every g/dl of hemoglobin (P=0.08). No other variables had an influence on the SRI scores. CONCLUSION: The present study has shown that within a cohort of COPD patients undergoing long-term NIV, one-third were identified as anemic. Furthermore, anemia, like exacerbation history, was found to have a considerable negative impact on HRQL that is specific to patients with chronic respiratory failure. CLINICAL TRIAL REGISTRATION: German Clinical Trials Registry (DRKS00008759).

Development of the Diaphragmatic Paralysis Questionnaire: a simple tool for patient relevant outcome.

OBJECTIVES: Measurement tools of health-related quality of life (HRQL) that are specific for the underlying disorder are inevitably needed to assess HRQL changes following specific treatment strategies. The aim of the current study was to develop a questionnaire assessing HRQL in patients with unilateral diaphragmatic paresis. METHODS: Firstly, topics of health impairments covering physical, psychological, social and functional aspects were predefined by a physician expert panel to ensure face validity. Secondly, all predefined topics were rated by a patient group with unilateral diaphragmatic paresis (untreated: n = 11; postoperative: n = 9) using a 5-point Likert scale ranging from 'not relevant at all' (-2) to 'absolutely relevant' (+2) to guarantee content validity. Thirdly, only relevant topics (0 to +2) were used for item development, while non-relevant items (<0) were not subject for item development. RESULTS: In total, 20 patients rated a total of 43 topics covering a broad spectrum of health impairment. 21 were considered as relevant for item development. Items are answered on a 5-point Likert scale ranging from 'completely untrue' (-2) to 'always true' (+2). The Diaphragmatic Paralysis Questionnaire (DPQ) Summary Score ranges from 0 (worst HRQL) to 100 (best HRQL). Finally, the German DPQ was professionally translated and transculturally adapted into English, Italian, Dutch, French, Greek and Spanish, using translation/back-translation procedures. CONCLUSIONS: The DPQ is the first diseases-specific HRQL measure developed for patients with diaphragmatic paresis. In addition, the DPQ is available in 7 languages free of charge for non-profit purposes. CLINICAL TRIAL REGISTRATION: German clinical trials register: DRKS00017056.