[Respiratory therapy with Ez-PAP for treatment of dynamic hyperinflation in patients with severe COPD and emphysema]. / Atemtherapie mit Ez-PAP zur Behandlung der dynamischen Überblähung bei Patienten mit schwerer COPD und Lungenemphysem.

BACKGROUND: Non-pharmacological respiratory physiotherapy in treatment of COPD with severe emphysema is achieving increasing importance. Ez-PAP, a compact CPAP- or flow-PEP system, supports inspiration by using the Coanda effect in addition to a PE(E)P-effect during expiration. METHODS AND PATIENTS: 30 patients with severe COPD and emphysema and hypercapnic respiratory failure under non-(NIV) (n = 28) and invasive ventilation (n = 2) were treated in ventilator-free intervals with Ez-PAP and analyzed retrospectively. Clinical courses such as ventilator-dependency, dyspnea by visual CR-10 Borg scale, results of six-minute walk tests (6MWT), lung function tests and recordings of transcutaneous CO2 measurements were evaluated where possible. RESULTS: Analyzed patients showed less ventilator dependancy (n = 9), reported a median decrease of shortness of breath by 3,3 points (n = 10) and improved by 50,4 m in the 6MWT (n = 5). A median increase of vital capacity by 544 ml (n = 5) was shown by spirometry. Combined with manual techniques for reducing air-trapping, treatment success was documented by a median reduction of pCO2 by 7,3 mmHg (n = 6) using transcutaneous pCO2 measurement. CONCLUSION: Ez-PAP is a simple mechanical method to reduce dynamic hyperinflation in severe COPD, thus achieving relief of respiratory muscles and more effective breathing. Prospective studies of this promising method are urgently required.

Cryptococcal meningitis presenting as sinusitis in a renal transplant recipient.

Cryptococcal meningitis is a relatively common invasive fungal infection in immunocompromised patients, especially in solid organ transplant recipients. Clinical presentation typically includes fever, headache, photophobia, neck stiffness, and/or altered mental status. Unusual presentations may delay diagnosis. Therapy is challenging in renal transplant patients because of the nephrotoxicity associated with amphotericin B, the recommended treatment. We present a case of cryptococcal meningitis in a renal transplant recipient presenting as acute sinusitis with successful treatment using fluconazole as primary therapy.

Exercise reduces airway sodium ion reabsorption in cystic fibrosis but not in exercise asthma.

When ventilating large volumes of air during exercise, airway fluid secretion is essential for airway function. Since these are impaired in cystic fibrosis and exercise-induced asthma, it was the aim of this study to determine how exercise affects airway Na(+) and Cl(-) transport and whether changes depend on exercise intensity. Nasal potential was measured in Ringer's solution, with amiloride to block Na(+) transport, and in low chloride-containing isoproterenol to assess Cl(-) channels. Nasal potential was measured at rest and during submaximal and maximal bicycle ergometer exercise in individuals with cystic fibrosis, exercise-induced asthma and controls. At rest, nasal potential was significantly higher in cystic fibroses than in the others. Maximal exercise decreased nasal potentials in cystic fibrosis and controls but not in exercise asthma. Submaximal exercise decreased nasal potentials only in cystic fibrosis. Cl(-) transport was not affected. Our results indicate that nasal potentials and Na(+) transport were decreased by maximal exercise in healthy and cystic fibrosis, whereas submaximal exercise decreased potentials in cystic fibrosis only. Exercise did not affect nasal potentials in asthmatics. Decreased reabsorption during exercise might favour airway fluid secretion during hyperpnoea. This protective effect appears blunted in patients with exercise-induced asthma.

[Non-invasive ventilation: possibilities and limitations in patients with reduced ability to cough]. / Nichtinvasive Beatmung: Möglichkeiten und Grenzen bei eingeschränkter Hustenfunktion.

BACKGROUND: Neuromuscular diseases cover a wide range of pathologies, which are slowly or rapidly progressive. Besides the reduced capacity of the inspiratory muscles there is a limitation of expiration which compromises coughing. Ventilatory respiratory insufficiency (VRI) develops in stages, beginning during sleep with hypercapnia manifesting later on. Several retrospective studies have shown an improved alveolar ventilation, survival and quality of life for most NMD, even in non-bulbar ALS. The initiation of NIV is seen as apropriate when VRI in sleep develops. But patient acceptance to use NIV for more than 4 hours must be taken into account. NIV is limited by the interface which may cause skin abrasions or leakage due to pressure. NIV may be used day and night in stable conditions and with assistance experienced in mechanically-assisted coughing (MAC). The discussion of whether to perform tracheostomy remains a preeminent topic especially in ALS. CONCLUSION: NIV is effective in NMD and improves and prolongs life in slowly progressive NMD. Mask problems, leakage and bulbar dysfunction can limit the effectiveness. MAC is a tool that is very useful for elimination of bronchial secretions.

[Diaphragmatic paralysis and respiratory insufficiency]. / Zwerchfellparese und respiratorische Insuffizienz.

BACKGROUND: Phrenic nerve palsy leads to disfunction of the main respiratory muscle. With bilateral palsy dyspnoea in the supine body position will typically occur. With one-sided lesion symptoms will only appear when a second pulmonary disease is present. PATIENTS: We refer of 6 patients with bilateral and 5 with unilateral diaphragmatic paresis. In 3 patients neuralgic shoulder-arm-amyotrophy was diagnosed, in further 4 there was suspicion of it. Amyotrophic lateral sclerosis developed in 2 after 4 respectively 8 months. In 1 case a cervical operation led to palsy, mediastinitis in 1 case. Lung function tests showed a restrictive pattern, especially in bilateral palsy. RESULTS: Vital capacity was reduced by almost 50% respectively 25% in the supine body position. Mouth occlusion pressure reduction was apparent. Near normal to marked hyperkapnia developed in bilateral paresis. In 5 cases non-invasive intermittent ventilation is instituted (2 volume controlled, 3 pressure supported). Two patients died (1 patient with amyotrophic lateral sclerosis after 13 months, 1 with malignant pleurisy after 4 months). Three patients continued non-invasive intermittent ventilation since 14 to 64 months.

[Pharmacological stress echocardiography--a new non-invasive follow-up examination of intermittent ventilation]. / Medikamentöse Stressechokardiographie--eine neue nichtinvasive Untersuchung in der Verlaufsbeobachtung bei intermittierender Selbstbeatmung.

BACKGROUND: Patients with chronic respiratory insufficiency frequently develop pulmonary hypertension. Non-invasive intermittent ventilation is usually very successful in these patients to improve blood gas exchange and clinical symptoms. Alterations of pulmonary hemodynamics during non-invasive intermittent ventilation are rarely described. Pharmacological stress echocardiography of the right heart is a new method to examine pulmonary hemodynamics. Aim of this study was to answer the question whether non-invasive intermittent ventilation improves pulmonary hemodynamics. PATIENTS AND METHOD: Five patients are examined prior to and during non-invasive intermittent ventilation by right ventricular stress echocardiography at rest and during exercise. Pulmonary arterial pressure was registered and compared. The effectiveness of intermittent ventilation was evaluated with respect to blood gas analytic values. RESULTS: During non-invasive intermittent ventilation all 5 patients improved their pulmonary arterial mean (PAP mean) and systolic pressure, but no statistical significant changes could be observed during the 4.5-months follow-up. CONCLUSION: Non-invasive intermittent ventilation improves the pulmonary hemodynamics at rest and during exercise the results not being significant.

[Quality of life and long-term survival with intermittent self-ventilation in respiratory insufficiency caused by thoracic restrictive diseases]. / Lebensqualität und Langzeitüberleben unter intermittierender Selbstbeatmung bei Ateminsuffizienz infolge thorakal-restriktiver Erkrankungen.

BACKGROUND: Mortality and quality of life are to be assessed in patients with respiratory failure due to restrictive chest wall disease. Neither specific questionnaires, nor perspective studies are available. PATIENTS AND METHOD: In a retrospective analysis records of mortality and morbidity, physical and social activity, and duration of IPPV are reviewed. RESULTS: The group consists of 35 patients (mean age 56 +/- 12 years; 22 female, 13 male; 11 post tuberculosis, 24 kyphoskoliosis; IPPV duration 28.5 +/- 20.7 months). Five patients died, 3 of which during the first 4 months: In 3 death is related to respiratory symptoms, 1 is due to pulmonary embolism, 1 to disruption of aortic aneurysm. Hospitalisation is required by 12 patients in 20 instances with a mean duration of 14.2 days. Respiratory symptoms are the cause in 13 instances, special difficulties are met with occlusion of tracheostoma in 3 patients. Professional activity is performed by 14 of the 21 patients < 60 years old. Physical activity is severely reduced in 4 patients, while it is mildly to moderately compromised in 26. Seven of all patients need supplemental oxygen. CONCLUSION: In respiratory failure due to restrictive chest wall disease treatment with IPPV seems to improve long-term survival and to enhance quality of life.

[Intermittent self-ventilation in neuromuscular diseases. Comparison of lung function parameters in ventilated and non-ventilated patients]. / Intermittierende Selbstbeatmung bei neuromuskulären Erkrankungen. Vergleich der Lungenfunktionsparameter bei beatmeten und nichtbeatmeten Patienten.

BACKGROUND: Based on neuromuscular-disease patients in our case, we investigate the possibility of elaborating criteria by which to judge when home intermittent mechanical ventilation should be commenced and to access its consequences over an extended period. PATIENTS AND METHODS: Out of 24 patients with neuromuscular diseases (6 female, 18 male, mean age 32 years), 14 were treated with IPPV. They were suffering from hypercapnic respiratory failure with heavy sleep disruption and corresponding daily symptoms. RESULTS: The aim was to calculate significant examination parameters to facilitate indication for IPPV. The clinical situation of patients dependent on respiratory support improved under IPPV. We witnessed a "ventilation-saving effect" and--despite progression of the basic disease within the monitoring period--no deterioration of the blood-gas situation. CONCLUSIONS: It is possible to elaborate criteria for the indication of IPPV based on our group of neuromuscular-disease patients.

[Follow-up of intermittent self-ventilation (ISB). Mortality and causes]. / Verlauf unter intermittierender Selbstbeatmung (ISB). Mortalität und ihre Ursachen.

BACKGROUND: Intermittent positive pressure ventilation (IPPV) has an impact on morbidity and survival in patients with chronic respiratory failure. We analyse the causes of death in relation to the disease, the age, the effectiveness of and the compliance with IPPV. PATIENTS: The course of 108 patients, who were on IPPV for a mean of 24 (SD +/- 21) months, is analysed. RESULTS: Twenty-seven patients die (7/7 AIS, 2/17 muscular dystrophy, 8/45 kyphoskoliosis and TB sequelae, 2/7 other neurological diseases, 6/16 COPD bronchiectasis, 1/1 fibrosis of the lang). The cause of death is in 21 hypoventilation or respiratory infection due to progressive disease (9 patients), air leakage after occlusion of tracheostoma (2 patients), bronchitis (6 patients), discontinuation of IPPV (3 patients). Two patients develop pulmonary failure (atypical mycobacteriosis and asbestosis, pulmonary embolism). Non-pulmonary causes of death are present in 2 (rupture of aortic aneurysm, pleural carcinosis). CONCLUSIONS: A safe interface for IPPV is needed for longer survival. An efficient system of home-care has to be developed.

[Noninvasive self-ventilation--successful transition aid in the waiting period before lung transplantation?]. / Nichtinvasive Selbstbeatmung--erfolgreiche Uberbrückungshilfe in der Wartezeit vor Lungentransplantation?

BACKGROUND: Non-invasive mechanical ventilation is used with success for ventilatory failure due to derangements of the respiratory pump. Efficacy in pulmonary diseases is controversial. Still non-invasive intermittent ventilation is reported as a mean of bridging the period till lung transplantation can be performed. PATIENTS AND METHODS: We report of 20 patients from 5 centers who were treated with non-invasive intermittent ventilation while lung transplantation was planned. RESULTS AND CONCLUSION: Non-invasive intermittent ventilation was seen as successful in 14 patients. Of these 4 are transplanted after up to 2 years, 4 are still waiting, 2 have not yet entered a lung transplantation program, 1 continued non-invasive intermittent ventilation after refusal to be enlisted, 3 meanwhile died. In the group of non-successful non-invasive intermittent ventilation 2 patients were transplanted after a short waiting period. All remaining 4 patients died. It seems that non-invasive intermittent ventilation may be helpful in prolonging the period of survival while waiting for lung transplantation.

[The role of non-invasive positive pressure ventilation in lung volume reduction surgery of pulmonary emphysema--a survey of German hospitals]. / Die Rolle der intermittierenden Selbstbeatmung bei Volumenreduktionschirurgie des Lungenemphysems.

BACKGROUND: Since the first publication by Cooper et. al. in 1994 of lung volume reduction surgery (LVRS) of emphysema a marked respiratory failure with hyperkapnia (PaCO2 > 55 Torr) has been regarded as an exclusion criterion for LVRS. PATIENTS AND METHOD: In a survey in German hospitals the question was asked whether non-invasive nasal ventilation (NIPPV) has a role in the management of LVRS. Of 12 hospitals 6 had experience with NIPPV and LVRS in a total of 19 patients with a mean FEV1 of 0.64 +/- 0.101. RESULTS: LVRS improved FEV1 by 0.20 +/- 0.181. Preoperative NIPPV was short (< 6 months) in 8 patients and resulted in improvement of physical condition and getting the patient used to NIPPV for better perioperative management. In 5 cases NIPPV was used on a long-term basis in order to allow the patient to be included in the LVRS program. In fact 7 of these 13 patients needed ventilation perioperatively, and 4 had to continue long-term NIPPV after surgery. In further 3 patients NIPPV was applied only perioperatively. One patient had to resume NIPPV after 15 months. Two patients started NIPPV 1 resp. 12 months after surgery. Two patients had bronchial cancer which was resected. Four patients died: 1 perioperatively after intubation, 2 after 3 resp. 13 months due to respiratory failure, 1 for cancer relapse after 20 months. CONCLUSION: NIPPV may be helpful in the planning and management of LVRS in patients with ventilatory failure with hypercarbia.

[Postoperatively increased oxygen demand and hemoptysis after extubation]. / Postoperativ erhöhter Sauerstoffbedarf und Hämoptysen nach Extubation.

A 22-year-old man on postoperative day 1 after open reduction with internal fixation of a distal fibula fracture presented with hemoptysis and increased oxygen demand. X-ray and contrast-enhanced computed tomography revealed bilateral patchy opacities and mediastinal emphysema. After bronchoscopy and bronchoalveolar lavage, a diagnosis of alveolar hemorrhage was made. The medical history revealed regular abuse of marihuana and cocaine.

[Dyspnea in a 45-year-old man with liver cirrhosis]. / Dyspnoe bei einem 45-jährigen Patienten mit Leberzirrhose.

HISTORY AND ADMISSION FINDINGS: During evaluation for a liver transplantation in a 45-year-old man with alcoholic liver he complained of exertional dyspnea. He had grade 3 chronic obstructive pulmonary disease (COPD) after nicotine abuse of 50 pack-years. One and a half years earlier the patient had been treated for tuberculosis. INVESTIGATIONS: The diagnostic procedures showed a respiratory insufficiency with a PO(2) of 52 mm Hg. Notable was a missing improvement of oxygenation after the supply of 100 % oxygen. Lung perfusion scintigraphy showed a shunt of about 14 %, a contrast-medium echocardiography demonstrating a right-to-left shunt. These findings indicated that the patient had a hepatopulmonary syndrome. TREATMENT AND COURSE: The only causal therapy of a hepatopulmonary syndrome was a liver transplantation. However, the tuberculosis diagnosed one and a half years earlier had not been treated adequately, so that an antituberculotic therapy was essential before starting an immunosuppression. Therefore the only option of treatment was to continue the long-term oxygen therapy. As a matter of differential diagnosis and because of the intrapulmonary shunt, a pulmonary venous disconnection for a shunt caused by tuberculous cavities or by the COPD had to be taken into account. CONCLUSION: Treating patients with advanced liver cirrhosis and dyspnea a hepatopulmonary syndrome must be taken into consideration. Typically there will be found a right-left-shunt. In addition, coexistent comorbidities as a reason for dyspnea have to be excluded.

[Relapsing solitary pulmonary nodule in a patient with ulcerative colitis]. / Rezidivierender pulmonaler Rundherd bei Colitis ulcerosa.

ANAMNESIS AND CLINICAL FINDINGS: A 46-year-old patient with ulcerative colitis, known since she was 27 years old, was found to have a mesenteric granulomatous necrotizing inflammation containing acid-fast bacilli ten years ago. Treatment against tuberculosis was initiated. The ulcerative colitis was treated with mesalazin and, in times of peak activity, additionally with prednisolon. During periods of chronic activity the patient received azathioprin. Six years ago a solitary pulmonary nodule (coin lesion) was detected radiologically in the inferior lobe of the left lung. INVESTIGATIONS: Laboratory investigations revealed mild inflammation (CRP 1.5 mg/dl). Computed tomography showed a solitary nodule in the left lower pulmonary lobe (diameter 27 mm). Fungi, actinomyces and mycobacteria were not found. TREATMENT AND COURSE: The pulmonary nodule was resected. During the following years several relapses occurred. After the second relapse lobectomy was performed revealing mycobacterial DNA (not further identified). Treatment against non-tuberculous mycobacteria with clarithromycin, rifampicin and ethambutol was started for 12 months. A third relapse in the form of another solitary pulmonary node occurred one year later. But this nodule regressed spontaneously in part without any additional therapy. CONCLUSION: In this case the spontaneous regression after the third relapse may represent better control of the ulcerative colitis. An infection seems less likely retrospectively.

[Q fever endemic in the Palatinate]. / Q-Fieberendemie in der Vorderpfalz.

In 1989 investigations revealed a small scale outbreak of Q fever in the area of Vorderpfalz several kilometers west of the city of Ludwigshafen/Rhein. In 16 cases the diagnosis of Q fever has been serologically confirmed by complement fixation test (CF), the estimated number of unknown cases, however, may be considerable. A flock of sheep was determined as the source of infection. Due to the prevalence of the tick species Dermacentor marginatus, one of the main vectors of the infection, the area is considered one of the natural foci of Q fever. Epidemiological aspects as well as case reports are shown.

Necrotizing bronchitis, angiitis, and amyloidosis associated with chronic Q fever.

The authors report the clinical, radiological and histological findings in a 63-year-old male patient who developed severe necrotizing bronchitis, necrotizing angiitis, and secondary amyloidosis of the right upper love and intermediate bronchus. The patient expired due to respiratory insufficiency. At the age of 27 years, the patient had had radiotherapy of the mediastinum because of suspected Hodgkin's disease. Acute pneumonia suggestive of Q-fever infection was diagnosed at the age of 48. Progressive restrictive lung disease developed during the last decade. Serological evaluation revealed IgM and IgA high titers against Coxiella burnetii. IgA, complement and amyloid deposits were detected in the walls of small arteries. Bronchial lavage and pleural effusions displayed numerous activated T lymphocytes. Analysis of endogenous lectins revealed alterations of the pulmonary defense system. The clinical history, histological and immunological findings suggest that chronic Q fever may induce remarkable changes in the immune system, comparable to autoimmune-reactive diseases.

Elastinolytic activity of alveolar macrophages in smoking-associated pulmonary emphysema.

Current concepts of pathomechanisms leading to acquired emphysema suggest that alveolar macrophages (AM) activated by cigarette smoking may cause an elastase/antielastase imbalance localized to the microenvironment formed by phagocytes and lung tissue. A functional cell assay was used to evaluate the cell-associated elastinolytic activity of AM. AM were obtained by bronchoalveolar lavage from patients with emphysema and from patients with non obstructive chronic pulmonary diseases (non-COPD) and cultured under serum-free conditions in direct contact with 3H-labeled elastin particles. Elastinolytic activity was calculated from the released radioactivity in culture supernatants and expressed as micrograms of 3H-elastin degraded x 10(-5) AM x 72 h-1. AM of patients with emphysema had significantly higher elastinolytic activity compared to that of non-COPD patients (median: 10.8 versus 4.1 micrograms; P < 0.01). Further differentiation of patients revealed the lowest median activity in sarcoidosis (2.3 micrograms). In respect to smoking habits there was a major difference between smokers with emphysema degraded more than twice the amount of elastin than smokers in the non-COPD group (median:11 versus 3.9 micrograms, P = 0.01). From these data we conclude that AM-derived elsatinolytic proteases may be involved in the destruction of lung elastin, which is thought to be the key event occurring in the pathogenesis of pulmonary emphysema.

[Adult Leigh syndrome. A rare differential diagnosis of central respiratory insufficiency]. / Das adulte Leigh-Syndrom. Eine seltene Differentialdiagnose einer zentralen Atemregulationsstörung.

Subacute necrotizing encephalomyelopathy (Leigh's syndrome) is a rare neurodegenerative disease in the adult. The precise metabolic defect is unknown, but abnormalities of a mitochondrial enzyme system related to cytochrome-c oxidase or pyruvate dehydrogenase are described. The clinical picture usually consists of an altered breathing pattern, oculomotor paralysis, other signs of cranial nerve dysfunction, ataxia, myoclonic jerks, nystagmus, generalized seizures, optic atrophy and demyelinating peripheral neuropathy. Hypopnea leads to CO2-retention with consecutive loss of consciousness demanding mechanical ventilation. Respiratory failure is the most frequent cause of death. Here we describe two patients with adult onset Leigh's syndrome and we discuss the longterm treatment strategies including vitamin B1 and CPAP mask.