Einleitung Die Pathophysiologie des Post-COVID-Syndrom ist ungeklärt, Assoziationen mit Schlafapnoe sowie Zwerchfelldysfunktion wurden beschrieben.

INTRODUCTION/AIMS:The leading clinical feature of 5q-associated spinal muscular atrophy (SMA) is symmetric, proximal muscle weakness. Muscles involved in ventilation exhibit a specific pattern of denervation: intercostal muscles are severely atrophic, whereas the diaphragm muscle is less affected. The aim of this study was to investigate the involvement of diaphragmatic function by ultrasound imaging in adult patients with SMA and to quantify dynamics of diaphragmatic function during nusinersen treatment. METHODS:Diaphragmatic thickness, thickening, and excursion during quiet breathing were assessed in 24 adult patients with SMA type 2 and 3 by diaphragm ultrasound imaging before and during nusinersen treatment and were correlated with spirometric parameters. RESULTS:Diaphragm thickness was not reduced, but increased in a remarkable proportion of patients, whereas diaphragm thickening and excursion were reduced in about 20% to 30% of nusinersen-naive, adult patients with SMA types 2 and 3. During 26 months of nusinersen treatment, diaphragm thickening fraction and excursion improved. DISCUSSION:Diaphragm ultrasound imaging can provide disease- and treatment-relevant information that is not identified during routine clinical assessments and may therefore be a valuable complementary outcome measure.

Respiratory dysfunction is a common cause of morbidity and mortality in motor neuron disease (MND). However, classical volitional measures of respiratory function in these patients are impeded by, e.g., bulbar paralysis or progressive disability. Diaphragm ultrasound imaging might be a valuable tool for assessing respiratory impairment, albeit different ultrasound measures have not been systematically investigated in adult MND patients and, in particular, have not yet been comparatively applied in adult patients with amyotrophic lateral sclerosis (ALS) and spinal muscular atrophy (SMA). We hypothesized that in contrast to ALS patients, adult SMA patients show a relative sparing of diaphragm function. We retrospectively analyzed diaphragm ultrasound imaging data of 40 patients with ALS and 23 patients with SMA in comparison to a multitude of established parameters of respiratory function. Indeed, ALS patients showed more severe diaphragm dysfunction than adult SMA patients, however, diaphragm dysfunction was also common in adult SMA patients. Notably, dynamic measures of diaphragm function rather than thickness measures were impaired in ALS compared to SMA. Thus, diaphragm ultrasound imaging might be a useful tool to evaluate respiratory dysfunction in adult MND patients. Future larger and prospective studies are needed to validate our initial findings.

Einleitung suPAR als lösliche Form des zellmembrangebundenen uPAR entsteht durch Spaltung während einer Entzündung oder Immunaktivierung.

ZusammenfassungEine CPAP-Therapie ist die häufigste Behandlungsform der obstruktiven Schlafapnoe.Schwerwiegende Komplikation dieser Behandlung sind sehr selten. Pneumothorax als Lungen-Barotrauma unter der CPAP-Therapie wird in Einzelfallberichten und ausschließlich bei vorbestehenden Lungen- und Thoraxerkrankungen beschrieben.Ein 68-jähriger, pulmonal nicht vorerkrankter Schlafapnoe-Patient mit einer langjährig etablierten CPAP-Therapie wird nach einem heftigen thorakalen Schmerzereignis mit anhaltender Luftnotsymptomatik stationär aufgenommen. Radiologisch und computertomografisch bestand ein ausgedehnter rechtsseitiger Pneumothorax mit rechts basal betontem bullösem Emphysem. Nach operativer Versorgung des sekundären Spontanpneumothorax konnte am 3. postoperativen Tag die nächtliche Überdruckatmung unter reduziertem CPAP-Druck mit zufriedenstellendem Schlafapnoe-Befund und ohne Pneumothorax-Rezidiv im Verlauf wiederaufgenommen werden.Als mögliche Pneumothorax-Ursache bei dem Patienten können alveoläre entzündliche Veränderungen infolge von Überdehnung und Drucksteigerung in Alveolen, die unter jahrelanger CPAP-Behandlung mit schrittweiser Drucksteigerung entstanden sind und folglich zu ausgedehnten Lungenveränderung mit Ruptur geführt haben, angenommen werden.Zusammenfassend und schlussfolgernd soll bei Patienten, die aufgrund einer Schlafapnoe über Jahre mittels Überdruckatmung behandelt werden, bei plötzlich einsetzenden thorakalen Schmerzen mit einer Luftnotsymptomatik an einen möglichen Spontanpneumothorax gedacht werden.

Zusammenfassung Einleitung Die Implantation eines pleuralen getunnelten Dauerkatheters (indwelling pleural catheter = IPC) stellt bei symptomatischen rezidivierenden benignen und malignen Pleuraergüssen (BPE und MPE) neben einer Pleurodese eine weitere etablierte Therapiemethode dar.Zur Sicherheit des IPC, insbesondere zu Pneumothorax und Katheterinfektionen, existierten wenige Studien.Ziel unserer Untersuchung war, die Komplikationshäufigkeit nach IPC-Anlage und deren prädiktive Faktoren bei Patienten mit BPE vs. MPE zu ermitteln. Methoden Retrospektive Analyse aller IPC-Implantationen im Bereich Pneumologie am Universitätsklinikum Dresden im Zeitraum von 2015 – 2018. Ergebnisse Bei 86 Patienten (je 43 m/f; Alter 66,9 ± 13,3 Jahre) wurde bei symptomatischem BPE und MPE ein IPC implantiert. Ein BPE bzw. MPE bestand bei 12,8 % (11/86) bzw. 87,2 % (75/86) der Erkrankten.Als Sofortkomplikation nach IPC-Anlage war bei 43/86 (50 %) Patienten ein meist kleiner, asymptomatischer Pneumothorax nachweisbar. 34/43 (79 %) Patienten bedurften diesbezüglich keiner spezifischen Therapie. Bei 9/43 war ein IPC-Sog im Median über 3 Tage erforderlich. 8/43 Patienten wiesen einen großen Pneumothorax mit partieller/kompletter Regredienz im Median nach 2 Tagen auf.Bei 15,1 % (13/86) der Gesamtgruppe und 36,4 % (4/11) der BPE vs. 12 % (9/75) der MPE kam es im Median nach 87 (BPE/MPE 116/87) Tagen zu einer Katheterinfektion. Diese war bei BPE (p = 0,035), großem Pneumothorax (4/8 Patienten; p = 0,015) und längerer Katheterverweildauer (124 ± 112 vs. 71 ± 112 Tage; p = 0,07) häufiger. Schlussfolgerung Kleine Pneumothoraces sind häufig nach IPC-Implantation, bedürfen aber meist keiner spezifischen Therapie. Bei 15,1 % aller Patienten war im Median nach 87 Tagen eine Katheterinfektion nachweisbar. Diese trat häufiger bei BPE, längerer Katheterverweildauer und großen Pneumothoraces auf.

Background Implant of indwelling pleural catheters (IPC) represents an established therapy method in addition to pleurodesis for symptomatic recurrent benign and malignant pleural effusions (BPE and MPE). There are only few studies on IPC safety during follow-up, especially with regard to infection and pneumothorax rates. The aim of our investigation was to determine the complication frequency after IPC implant and its predictive factors in patients with BPE vs. MPE. Methods Retrospective analysis of all IPC implantations in the pneumology department at the University Hospital Dresden during 2015-2018. Results An IPC was implanted in 86 patients (43m/f each; age 66.9 +/- 13.3 years) with symptomatic BPE and MPE. BPE and MPE was present in 12.8% (11/86) and 87.2% (75/86) of the patients, respectively. A predominantly small and asymptomatic pneumothorax was detectable as an immediate complication in 43/86 (50%) of patients; 34/43 (79%) of patients did not require any specific therapy. For 9/43 patients, IPC suction was required for a median period of three days; 8/43 patients had a large pneumothorax with partial or complete regression after a median period of two days. Catheter infection developed in 15.1% (13/86) of the total group and 36.4% (4/11) of the BPE vs. 12% (9/75) of the MPE after a median period of 87 (BPE/MPE 116/87) days. This was more common in BPE (p=0.035), large pneumothorax (4/8 patients; p=0.015) and longer catheter dwell times (124 +/- 112 vs. 71 +/- 112 days; p=0.07). Conclusion Small pneumothoraxes are frequent after IPC implantation, but usually do not require specific therapy. IPC infection was detected in 15.1% of all patients after a median period of 87 days. This was more common in patients with BPE, longer catheter dwell times and large pneumothorax.

CPAP is the most common treatment for obstructive sleep apnea. Serious complications from this treatment are very rare. Pneumothorax following lung barotrauma under CPAP therapy has been described in case reports only in patients with pre-existing lung and thoracic diseases. A 68-year-old sleep apnea patient without pre-existing lung or thoracic diseases and with established CPAP therapy since many years was admitted to the hospital after a severe thoracic pain event with persistent shortness of breath. Chest radiograph and computed tomography showed an extensive right-sided pneumothorax with basal bullous emphysema. After surgical treatment of the secondary spontaneous pneumothorax, on the third postoperative day CPAP with reduced pressure was re-introduced with satisfactory sleep apnea findings and without pneumothorax recurrence. As possible cause of pneumothorax in the patient, alveolar inflammatory changes due to over-distention and increased pressure in the alveoli was assumed, which can occur after years of CPAP treatment with gradual pressure increase. In summary, in sleep apnea patients treated with CPAP for years, after sudden onset of thoracic pain and shortness of breath possible spontaneous pneumothorax should be considered.

Einleitung Der IPC stellt bei symptomatischen rezidivierenden BPE/MPE neben einer Pleurodese eine weitere etablierte Therapiemethode dar.

Chronic granulomatous disease (CGD) should be considered as a differential diagnosis in children and adolescents with frequent infections, especially when caused by certain specific pathogens.This case report describes a 64-year-old female with multiple recurrent and complicated bronchopulmonary infections, caused by common, but also rare pathogens, autoimmune phenomena, malignancies and recurrent organizing pneumonia (OP) with granulomas. Finally, the patient was diagnosed with p47phox-deficient chronic granulomatous disease (CGD).Individuals with a primary immunodeficiency may survive multiple complications and may be diagnosed at an advanced age especially if the affected structure shows residual activity. When confronted with patients with recurrent bronchopulmonary infections, especially with certain specific rare pathogens, in combination with organizing pulmonary granulomas as well as autoimmune phenomena, CGD should be considered even in elderly patients. Delayed diagnosis significantly increases mortality and morbidity in such cases.

BACKGROUND Periodic limb movements in sleep (PLMS) are repetitive movements usually of the legs strongly associated with Restless-legs syndrome (RLS), which appear more frequently in males, older age and other sleep disturbances, such as sleep-disordered breathing (SDB). Patients with Parkinson's disease (PD) suffer from various sleep disturbances including REM sleep behavior disorder, RLS and PLMS. Although a dopaminergic pathophysiology of PLMS is discussed, no systematic data on PLMS side-to-side distribution in PD and its correlation with asymmetry of motor symptoms are available. OBJECTIVE This study aimed at elucidating PLMS asymmetry in correlation to that of motor symptoms in PD compared to SDB and RLS. METHODS Cross-sectional, retrospective analysis of two polysomnography (PSG) recordings per patient scoring PLMS separately for both legs. RESULTS Of 105 patients (44 PD, 44 age- and sex-matched SDB and 17 RLS patients) PLMS measures (number of PLM, PLM-Index, PLM-arousal index) showed significant side-to-side differences in all disease entities in both PSGs (P < 0.001; Wilcoxon rank test). PLM-Index asymmetry (PLM-I difference of >5/h between both sides) was observed less frequently in PD (34% of patients) compared to RLS (77%, P < 0.05) and SDB (59%, P < 0.05; χ2 test). In asymmetric PD patients, predominant side of PLMS was more stable than in SDB and RLS comparing the two PSGs, but we did not detect an agreement between PLMS predominant side with that of motor symptoms in PD patients. CONCLUSIONS Only the minority of PD patients shows asymmetric PLMS distribution with relatively high night-to-night stability but no correlation with motor symptom asymmetry.

Pulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary hypertension. So far some 200 cases have been published worldwide. Since the latest classification, agreed at a meeting in Venice in 2003, PVOD is included in the group of pulmonary arterial hypertension (PAH). It has been hypothesized that it represents endothelial dysfunction of unknown cause. Although it has many features in common with idiopathic PAH, it partially differs from it phenotypically. After exclusion of known causes of pulmonary hypertension, such as diseases of pulmonary interstitial tissue, acral clubbing and rales suggest PVOD. The findings typical in the computed tomogram of the chest include prominent septal lines, centro-acinar opacities and mediastinal lymphadenopathy. In addition to arterial lesions PVOD is particularly characterized by lesions in the venous vascular bed. PVOD has a poor prognosis. Treatment with medication approved for PAH may carry the risk of pulmonary edema. Currently the immediate listing for lung- or combined lung-heart-transplantation is recommended, once the diagnosis of PVOD has been established.

Einleitung: Die Bronchiolitis obliterans mit organisierender Pneumonie (BOOP) ist eine Lungenerkrankung mit typischen klinischen, radiologischen und histologischen Merkmalen. In ca. 50% der Fälle kann kein Auslöser fest gestellt werden (idiopathische Verlaufsform). Die BOOP spricht gewöhnlich gut auf Kortikosteroid-Behandlung an, rezidiviert aber in einem 1/3 der Fälle.

Einleitung: Nächtliche Sauerstoff-Entsättigungen (nSE) werden häufig bei Patienten mit einer idiopathischen pulmonal arteriellen Hypertonie nachgewiesen. Bei Patienten mit einer portopulmonalen Hypertonie (POPH) fehlen Angaben über Häufigkeit, Schweregrad und eventuelle Ursachen von nSE.

INTRODUCTION:Nocturnal oxygen desaturations (NOS) are a common feature in patients with idiopathic pulmonary arterial hypertension. In patients with portopulmonary hypertension (PPHT), the frequency, degree and potential causes of NOS are unknown.PATIENTS AND METHODS:We retrospectively analyzed patients with PPHT, in whom right heart catheterization, pulmonary function testing, measurements of arterial oxygen tension (paO(2)) during daytime both at rest and at maximum physical exercise on cycle ergometer, and nocturnal polygraphy including pulse oximetry were performed. The data of these examinations were analyzed.RESULTS:Six patients with moderate to severe PPHT were included. Mean pulmonary artery pressure was 50+/-8 mmHg. Lung function did not reveal relevant pathological findings. Pulse oximetry demonstrated moderate to severe NOS as classified by duration of oxygen desaturation <90 % in 5 patients. Mean paO(2) at rest and during maximum exercise was 10.6+/-1.2 kPa and 9.2+/-1.6 kPa, respectively. One of 5 patients with moderate to severe NOS demonstrated exertional hypoxemia with a paO(2) value of <8.0 kPa. None of the patients showed sleep apnea.CONCLUSION:Moderate to severe nocturnal hypoxemia seems to be common and is not related to sleep apnoea and lung function in patients with PPHT. The absence of exertional hypoxemia does not exclude NOS. We recommend to perform overnight oximetry in the routine examination of PPHT patients because nocturnal oxygen supplementation should be considered in patients with NOS.

Einleitung: Cheyne-Stokes Atmung (CSA) ist häufig bei Patienten mit einer chronischen Herzinsuffizienz und einer linksventrikulären Ejektionsfraktion <40% nachweisbar1.

Obstructive sleep apnea during pregnancy, associated with arterial hypertension, pre-eclampsia and adverse outcome of the newborn, has been described. Usually it can successfully be treated with non-invasive ventilation. A 36-year-old, twin-pregnant woman at 28 + 6 weeks of gestation presented with the complaints of snoring and nocturnal oxygen desaturations. Polysomnography confirmed the diagnosis of severe obstructive sleep apnea (respiratory disturbance index [RDI] 104/h, minimal oxygen saturation in pulse oximetry [SpO2/min] 75%). First therapeutic approaches including oxygen supplementation, non-invasive ventilation with continuous positive airway pressure (CPAP) or bilevel positive airway pressure--spontaneous (BiPAP-S) remained without success. Only after ventilation with bilevel positive airway pressure in spontaneous/timed modus (BiPAP-ST) combined with additional oxygen supplementation a satisfactory treatment result could be achieved (RDI 32/h, SpO2/min 85%). Because of progressive pre-eclampsia despite therapy at 31 + 0 weeks of gestation cesarean section was performed and 2 healthy children were delivered. After surgery non-invasive ventilation had to be continued for another six weeks before the condition improved. In contrast to the documented benefits of non-invasive positive pressure ventilation in pregnant women the literature, in our case even with sufficient therapy of sleep apnea progressive pre-eclampsia and premature delivery could not be prevented. Possible explanations include the short treatment duration and existence of twin pregnancy.