Successful simultaneous stenting of a pulmonary artery and vein in pulmonary vascular stenosis due to silicosis. Case report and literature review.

A 58-year-old patient was admitted to the emergency department due to severe respiratory insufficiency. Anamnesis revealed that the patient had experienced increasing stress dyspnea for a few months. Upon imaging, an acute pulmonary embolism was excluded, but peribronchial and hilar soft tissue proliferation with compression of central parts of the pulmonary circulation was found. The patient had a history of silicosis. The histology report showed tumor-free lymph node particles with prominent anthracotic pigment and dust depositions without evidence of IgG4-associated disease. The patient was administered steroid therapy and underwent simultaneous stenting of the left interlobular pulmonary artery and the upper right pulmonary vein. As a result, a significant improvement in symptoms and physical performance was achieved. The diagnosis of inflammatory or, in particular, fibrosing mediastinal processes can be challenging and important clinical symptoms must be taken into account, especially if the pulmonary vasculature is involved. In such cases, the possibility of interventional procedures should be examined in addition to drug therapy options.

[Hypercapnic respiratory failure. Pathophysiology, indications for mechanical ventilation and management]. / Hyperkapnisches Atemversagen. Pathophysiologie, Beatmungsindikationen und -durchführung.

BACKGROUND: Acute hypercapnic respiratory failure is mostly seen in patients with chronic obstructive pulmonary disease (COPD) and obesity hypoventilation syndrome (OHS). Depending on the underlying cause it may be associated with hypoxemic respiratory failure and places high demands on mechanical ventilation. OBJECTIVE: Presentation of the current knowledge on indications and management of mechanical ventilation in patients with hypercapnic respiratory failure. MATERIAL AND METHODS: Review of the literature. RESULTS: Important by the selection of mechanical ventilation procedures is recognition of the predominant pathophysiological component. In hypercapnic respiratory failure with a pH < 7.35 non-invasive ventilation (NIV) is primarily indicated unless there are contraindications. In patients with severe respiratory acidosis NIV requires a skilled and experienced team and close monitoring in order to perceive a failure of NIV. In acute exacerbation of COPD ventilator settings need a long expiration and short inspiration time to avoid further hyperinflation and an increase in intrinsic positive end-expiratory pressure (PEEP). Ventilation must be adapted to the pathophysiological situation in patients with OHS or overlap syndrome. If severe respiratory acidosis and hypercapnia cannot be managed by mechanical ventilation therapy alone extracorporeal venous CO2 removal may be necessary. Reports on this approach in awake patients are available. CONCLUSION: The use of NIV is the predominant treatment in patients with hypercapnic respiratory failure but close monitoring is necessary in order not to miss the indications for intubation and invasive ventilation. Methods of extracorporeal CO2 removal especially in awake patients need further evaluation.

[Pulmonary hyalinizing granuloma]. / Pulmonale hyaline Granulomatose.

Benign lesions as pulmonary hyalinizing granuloma may mimic a malign disease. A 63-year old patient complained dyspnea and a weight loss of 30 kg. CT-thorax scans showed a destructive and infiltrative pulmonary process with pleural thickening. Histologic examination of transbronchial and transthoracic biopsies as well as of biopsies taken by minithoracotomy was not conclusive. Due to further progression the patient underwent a left-sided pleuropneumonectomy despite a VO2 peak of 9 ml/kg/min. Histology revealed DIP-like infiltrations, a histiocytic reaction and hyaline granulomas. Among less than 100 published cases of pulmonary hyaline granuloma a comparable rapid progression with a total functional loss of the affected lung is not reported. Mostly hyalinizing granuloma presents with infiltrations, which may mimic lung cancer, or nodular lesions, partly with cavitations or calcifications. The etiology is unknown, a persistent immunologic response to an antigenic stimulus is discussed. Associations with infections, lymphomas, amyloidosis or IgG4-related disease are reported. Some cases have features of multifocal fibrosis. In the case reported none of these associations could be found. The prognosis of pulmonary hyaline granuloma is regarded as benign. There is no effective treatment yet. Once the diagnosis has been established a conservative approach as well as a resection of nodules and a therapeutic attempt with steroids are an option. Extensive resections as pleuropneumonectomy are an exception.

[Obstructive sleep apnoea and oxidative stress]. / Obstruktive Schlafapnoe und oxidativer Stress.

BACKGROUND: Untreated obstructive sleep apnoea (OSA) is characterised by elevated systemic and local oxidative stress and inflammation. Non-invasive measurement of metabolites and markers of inflammation in exhaled breath condensate (EBC) is of interest in the interpretation of systemic effects of severe obstructive sleep apnoea. In patients with severe sleep apnoea (AHI ≥ 30 /h) we investigated if CPAP therapy with normalisation of AHI induces changes of H2O2 concentrations in EBC. MATERIAL AND METHODS: Patients with obstructive sleep apnea (AHI ≥ 30 /h) had H2O2 analysis of EBC after native PSG, after the first night and after 6 weeks of CPAP therapy. Only patients with normalisation of AHI (AHI ≤ 10  /h) were included in the further analysis. RESULTS: 23 patients (21 men and 2 women) fulfilled the inclusion criteria. The first night of CPAP therapy led to a significant reduction of AHI, but did not change the H2O2 concentration in EBC. After 6 weeks of CPAP therapy with normalisation of AHI (3 ± 3 /h) H2O2 in EBC showed a significant reduction from 450 ± 163 nmol/L to 294 ± 110 nmol/L. Subgroup analysis showed that in non-smokers and especially in smokers the reduction was less marked than in ex-smokers. CONCLUSION: Normalisation of AHI after 6 weeks of CPAP therapy in OSA induces a significant reduction of H2O2 in EBC. This primarily represents a reduction of local inflammation and oxidative stress in the airways, but also indicates a positive effect on elevated oxidative stress in OSA. The influence of smoking status needs further investigations, including subgroup analysis with a sufficient number of patients.

Successful noninvasive ventilation and enzyme replacement therapy in an adult patient with morbus hunter.

M. Hunter is characterized by an accumulation of mucopolysaccharides in cells, blood, and connective tissue as a consequence of a deficiency of the enzyme iduronate-2-sulfatase. Unlike enzyme replacement therapy with idursulfase in children, there is limited long-term experience in adult patients with Morbus Hunter.The case presented here describes the development of a man born in 1971 who was admitted to Hemer Lung Clinic in 2005 with severe obstructive sleep apnea, pulmonary functional impairment, and ventilatory failure (FEV 1: 0.8 L, VC: 1.0 L; pO(2): 52 mmHg; pCO(2): 81 mmHg, 6 MWT: 100 m). Initially, the patient received symptomatic treatment with noninvasive ventilation, which achieved a considerable improvement in pulmonary function and a normalization of blood gasses. Since February 2008, the patient received additional enzyme replacement therapy with idursulfase, which resulted in a further significant functional improvement (FEV1: 1.6; VC: 2.3 L; VO(2)max: 1,350 mL or 28.1 mL/kg body weight), in a normalization of prior elevated pulmonary artery pressures and also in impressive changes in the physiognomy and joint mobility. In November 2010, the polysomnography and nocturnal blood gas analysis without NIV showed only a mild obstructive sleep-related breathing disorder with no sign of hypoventilation. Therapy was changed to nocturnal CPAP therapy with a constant pressure of 6 cm H(2)O. Additional administration of oxygen was not required. With this therapy, the patient has been asymptomatic up to September 2011.Adult Hunter patients also benefit from enzyme replacement therapy and, in restrictive ventilatory defects with hypoventilation, from symptomatic therapy with noninvasive ventilation.

[Broncholithiasis, lithoptysis and broncho-oesophageal fistula]. / Broncholithiasis, Lithoptysis und broncho-ösophageale Fistel.

Broncho-oesophageal fistulas in broncholithiasis are extremely rare. Preceding lithoptysis is only seen infrequently. We report on a 68-year-old patient who complained of cough for more than 3 years. 5 months prior to admission he had hemoptysis and expectorated several greyish stones of up to 5 mm diameter. Endoscopy revealed a small excavation at the medial wall of the left main bronchus. Compared to a former CT, an actual CT scan of the thorax showed a small fistula between the oesophagus and the left main bronchus and revealed a missing calcification at this site. The patient underwent a left-sided thoracotomy with excision of the fistula, suture of the oesophagus and interponation of a flap of the M. latissimus dorsi. The demonstration of broncholith migration with CT scans before and after lithoptysis, the development of a left-sided fistula and its demonstration in the CT scan as well as endoscopically have not been reported in this combination before.

[Influence of adaptive servoventilation on B-type natriuretic petide in patients with Cheyne-Stokes respiration and mild to moderate systolic and diastolic heart failure]. / Einfluss der Adaptiven Servoventilation auf das BNP bei Patienten mit Cheyne-Stokes-Atmung und leicht- bis mittelgradiger systolischer und diastolischer Herzinsuffizienz.

BACKGROUND: CPAP therapy has a variable effect on central sleep apnea with Cheyne-Stokes respiration (CSA-CSR). Adaptive servoventilation (ASV) is more effective in normalising breathing in patients with heart failure. We hypothesised that, by normalising AHI, ASV reduces elevated BNP levels in patients with mild systolic and diastolic heart failure. METHODS: From April 2004 to October 2006, patients with CSA-CSR with and without concomitant obstructive sleep apnea (OSA), clinical evidence of heart failure, regardless EF, and elevated BNP levels (> 100 pg/mL) were selected for treatment with ASV, unless CPAP therapy had reduced AHI to < 15 per hour of sleep. Follow-up polysomnographies and BNP analyses were performed after 6 weeks. RESULTS: 15-male patients (AHI 48.3 +/- 14.6/h) fulfilled all inclusion criteria; 7 patients had CSA-CSR + OSA, 8 had CSA-CSR. After 6 weeks of ASV, BNP decreased from 415 +/- 196 pg/mL to 264 +/- 146 pg/mL (p = 0.0009). There was only a significant BNP reduction in the CSA-CSR+OSA subgroup (p = 0.0002). CONCLUSION: ASV can normalise AHI in patients with mild systolic and diastolic heart failure and CSA-CSR +/- OSA, thus leading to a significant reduction of BNP levels. These findings suggest that effective suppression of sleep apnoea in such heart failure patients improves cardiac function.

Ion mobility spectrometry for the detection of volatile organic compounds in exhaled breath of patients with lung cancer: results of a pilot study.

BACKGROUND: Analysis of exhaled breath, especially of volatile organic compounds (VOCs), is of increasing interest in the diagnosis of lung cancer. Compared with other methods of breath analysis, ion mobility spectrometry (IMS) offers a tenfold higher detection rate of VOCs. By coupling the ion mobility spectrometer with a multicapillary column as a pre-separation unit, IMS offers the advantage of an immediate twofold separation of VOCs with visualisation in a three-dimensional chromatogram. The total analysis time is about 500 s compared with gas chromatography/mass spectrometry (GC/MS) of about 1 h. It therefore seemed reasonable to test IMS in breath analysis. METHODS: In a pilot study, 32 patients with lung cancer were subjected to a breath analysis by IMS. Their IMS chromatograms were compared with those of 54 healthy controls. An IMS that was built for special clinical application was used to identify characteristic peaks of VOCs which might be relevant for the diagnosis of lung cancer in exhaled air of 10 ml volume. RESULTS: By a combination of 23 peak regions within the IMS chromatogram, patients with lung cancer, including a patient with carcinoma in situ, were classified and differentiated from healthy persons with an error rate of zero. CONCLUSION: Breath analysis by IMS can detect a discriminating combination of VOCs in patients with lung cancer. By pattern recognition without the need for chemical analysis of the underlying VOCs, IMS has the potential to facilitate lung cancer diagnosis.

MCC/IMS signals in human breath related to sarcoidosis-results of a feasibility study using an automated peak finding procedure.

A feasibility study using an ion mobility spectrometer coupled with a multi-capillary column (MCC) was started to identify characteristic peaks of volatile compounds in exhaled human breath samples of 10 ml volume. The breath of 20 patients with sarcoidosis and suspicion of sarcoidosis because of mediastinal lymph node enlargement was investigated. Using a set of procedures for data processing and scoring a sector of interest was determined within the IMS-chromatogram. It could be shown that a procedure related to a single peak in the IMS-chromatogram delivers differentiation into the two groups of patients with confirmed sarcoidosis and those suffering no sarcoidosis. The potential biomarker is characterized by the following parameters: inverse mobility (1/K(0)) 0.53 ± 0.01 V s cm(-2)-retention time 22 ± 5 s. These results are a first step in breath analysis by MCC/IMS in patients with sarcoidosis by an automated procedure applied to IMS-chromatograms directly.

Ion mobility spectrometry in the diagnosis of sarcoidosis: results of a feasibility study.

A feasibility study with ion mobility spectrometry (IMS) was started to find characteristic peaks of volatile organic compounds in exhaled air of 10 mL sampling volume, which might be relevant for the diagnosis of sarcoidosis. Therefore, breath samples of 9 patients with sarcoidosis and suspicion of sarcoidosis because of mediastinal lymph node enlargement were investigated. The 5 patients with confirmed sarcoidosis showed a highly congruent distribution of metabolites in exhaled air which was different in main component analyses from patients with unspecific mediastinal lymph node enlargement. These results are a first step in breath analysis by IMS in patients with sarcoidosis. The IMS as a new method in breath analysis and the first results of the investigations are presented and discussed in detail.

Detection of human metabolites using multi-capillary columns coupled to ion mobility spectrometers.

The human breath contains indicators of human health and delivers information about different metabolism processes of the body. The detection and attribution of these markers provide the possibility for new, non-invasive diagnostic methods. In the recent study, ion mobility spectrometers are used to detect different volatile organic metabolites in human breath directly. By coupling multi-capillary columns using ion mobility spectrometers detection limits down to the ng/L and pg/L range are achieved. The sampling procedure of human breath as well as the detection of different volatiles in human breath are described in detail. Reduced mobilities and detection limits for different analytes occurring in human breath are reported. In addition, spectra of exhaled air using ion mobility spectrometers obtained without any pre-concentration are presented and discussed in detail. Finally, the potential use of IMS with respect to lung infection diseases will be considered.

[Telomerase in lung cancer. Testing the activity of the "immortaligy enzyme" bronchial biopsies increases the diagnostic yield in cases of suspected peripheral bronchogenic carcinomas]. / Telomeraseaktivität beim Bronchialkarzinom. Der Nachweis des "Unsterblichkeitsenzyms" in Bürstenbiopsien erhöht die diagnostische Ausbeute bei Verdacht auf ein peripheres Karzinom.

The proliferative capability is time-limited in normal somatic cells by the shortening of their chromosomal ends, the telomeres (Hayflick limit). An important feature of malignant cells is their immortality. The probably most common mechanism of tumour cells to achieve unlimited replicability is the activation of the enzyme telomerase. The reverse transcriptase can compensate the loss of telomeres. Using a PCR-based TRAP assay we found telomerase activity in tumour biopsies, exsudates and bronchial washings in various thoracic malignancies. In 38 of 47 patients with suspected peripheral lung cancer eventually surgery or invasive procedures proved a malignancy. In fluoroscopically guided bronchial brushings from 25 of these 38 patients (66%) the TRAP assay revealed telomerase activity. There was a single false positive case (tuberculosis) and with a single exception, the simultaneously taken brushes of the contralateral lobes were all telomerase negative. In 23 patients (61%) tumour cells were found in the cytological examination. In 33 patients at least one marker was positive. Thus the combination of cytology and telomerase test in bronchial brush biopsies attained a diagnostic yield of 87%.