Telehealth mitigates COPD disease progression compared to standard of care: a randomized controlled crossover trial.

BACKGROUND: We showed excellent adherence and satisfaction with our telehealth care (TC) approach for COPD. Here, the results of a consecutive randomized controlled trial are presented. METHODS: Patients were randomly assigned to TC or standard care (SC). During TC, patients answered six daily questions online, and focused on the early recognition of exacerbations, in addition to SC. RESULTS: The mean increase in COPD assessment test (CAT) was 1.8 vs. 3.6 points/year in the TC and SC groups, respectively (P = 0.0015). Satisfaction with care (VAS) at baseline was 8.2; at the end of SC, 8.5 (P = 0.062); and after TC, 8.8 (P < 0.001). We detected significantly more moderate exacerbations during TC. CONCLUSION: Whilst receiving TC, the slope of the CAT increase - an indicator of the naturally progressive course of COPD - was reduced by 50%. Satisfaction with care increased with TC. The higher number of detected moderate exacerbations probably indicates a higher diagnostic sensitivity than without TC.

Physical activity and the frequency of acute exacerbations in patients with chronic obstructive pulmonary disease.

PURPOSE: Acute exacerbations (AE) in patients with COPD are associated with a decline in lung function, increased risk of hospitalization, and mortality. In this cross-sectional study we tested whether the level of objectively measured daily physical activity and exercise capacity are associated with the number of COPD exacerbations. METHODS: In 210 patients with COPD (67 % men; mean (SD) age: 63 (8) years) enrolled in The Obstructive Pulmonary Disease Outcomes Cohort of Switzerland (TOPDOCS) physical activity (PA) (steps per day, physical activity level, (PAL)), exercise capacity (6-min walking distance, (6MWD)), comorbidities, lung function, and medication were assessed. Differences between COPD patients with frequent (≥2 year) and infrequent (0-1 year) exacerbations were assessed. Univariate and multivariate analyses were performed to investigate whether the level of objectively measured daily physical activity and exercise capacity are associated with the number of COPD exacerbations. RESULTS: Patients with frequent AE had a significantly lower FEV1 and 6MWD compared to patients with infrequent AE. In univariate analysis, the number of exacerbations was inversely associated with FEV1, 6MWD, BMI, and smoking status while there was a positive association with RV/TLC and combined inhaled medication. However, there was no significant association with PAL and steps per day. In multivariate analysis, FEV1 and the use of combined inhaled medication were independently associated with the number of AE, after correction for covariates. CONCLUSIONS: The findings of this study imply that FEV1, independent of inhaled medication, is significantly associated with COPD exacerbations. Neither physical activity nor exercise capacity was independently associated with COPD exacerbations.

[Cough as a symptom--clarify or treat empirically?]. / Husten--wann abklären, wann behandeln?

Cough is one of the most prevalent symptoms for which patients seek the attention of their physicians. Cough may serve as a protective reflex but can also impair social well-being and can profoundly and adversely affect patient's quality of life. Short and self-limited cough often does not require therapy, whereas prolonged cough is bothersome and should prompt further workup. If possible, the underlying cause should be identified and treated accordingly. Often, the patient history helps to establish a working hypothesis, such as possible post-nasal drip syndrome or gastroesophageal reflux as a cause. Asthma, another frequent cause of prolonged cough, is readily diagnosed in most cases. The response to empirical therapy often "confirms" a suspected etiology, if not, extensive workup involving function testing such as bronchoprovocation, radiology, endoscopy, and extended search for exceptional causes is warranted. Productive cough is often related to a bronchopulmonary disease, whereas an irritant cough is often of an extrapulmonary origin.

Adherence to the Swiss guidelines for management of COPD: experience of a Swiss teaching hospital.

QUESTIONS UNDER STUDY: Swiss guidelines for the management of chronic obstructive pulmonary disease (COPD) were published in 2002. We aimed at assessing adherence to the proposed guidelines by the physicians in charge for all patients referred to our hospital for acute exacerbations of COPD over a one year period. METHODS: In a prospective observational study, data from a questionnaire and from records of all patients referred to our hospital with acute exacerbation of COPD were collected. Diagnostic steps as well as therapeutic and prophylactic interventions were reviewed. Where applicable, interventions were stratified according to proposed levels of evidence A-D. RESULTS: 45 patients in whom the diagnosis of COPD had been made before were included. Diagnosis was established by spirometry in 71%, in the remaining diagnosis was based on clinical grounds only. Non-smoking advice was given to 69%, and 16% were offered a nicotine-replacement trial (level A). Information about a disease management plan was given in 40% of the patients (level B), 22% had done a six minute walking distance test. 27% of the patients had participated in a pulmonary rehabilitation program (level A). 93% were on regular bronchodilator therapy (level B), and 56% had regular inhaled corticosteroids (level B). CONCLUSION: Confirmation of the diagnosis of COPD by spirometry is lacking in a significant number of patients. Most patients were treated with regular bronchodilators, however, relevant over-treatment with beta-adrenergic substances and overuse of inhaled corticosteroids in mild disease stages are common. Efforts for disease prevention and education as well as awareness of the potential benefits of pulmonary rehabilitation programs are still insufficient. Efforts to improve the adherence to the Swiss guidelines for the management of COPD should be intensified.

Salvage therapy with mycophenolate mofetil for lung transplant bronchiolitis obliterans: importance of dosage.

BACKGROUND: Bronchiolitis obliterans (BO) is the most important long-term complication of lung transplantation. Treatment of this condition is often unsuccessful. METHODS: A patient presented with early BO. Despite OKT3 and the addition of methotrexate, the patient needed persistently high doses of prednisone to maintain lung function at a moderate level. Only the substitution of azathioprine by mycophenolate mofetil (MMF, 3 g/day) made it possible to reduce the dose of prednisone. RESULTS: Reduction of the dose of MMF to 2 g/day resulted in a deterioration of lung function, which improved impressively after MMF was increased again to 3 g/day. CONCLUSIONS: MMF may be a valuable therapy for lung transplant BO. However, the use of a high dose, i.e., 3 g/day, may be crucial.

Efficacy and cost effectiveness of oral ganciclovir in the prevention of cytomegalovirus disease after lung transplantation.

BACKGROUND: Cytomegalovirus is the single most frequent pulmonary pathogen in lung transplant recipients who survive at least 2 weeks. Patients at increased risk are either seropositive or have received an allograft from a donor with latent infection. Morbidity and mortality caused by cytomegalovirus disease is still considerably high. METHODS: In an open, comparative study, we evaluated the efficacy, tolerance, and cost effectiveness of postoperative ganciclovir prophylaxis: intravenous dose of 2x5 mg/kg/day for 14 days, followed by either intravenous doses of 5 mg/kg]day (five patients), or oral doses of 3x 1000 mg (nine patients) up to 90 days. Oral ganciclovir was continued until prednisone was tapered below 15 mg/day. Prophylaxed groups were compared with a historical control (eight patients) in respect to cytomegalovirus disease, in-hospital stay, overall costs, and survival. Follow-up times and the net state of immunosuppressive therapy between groups were comparable. RESULTS: Six (75%) of the non-prophylaxed patients developed cytomegalovirus disease compared to none in the intravenous and one in the oral ganciclovir group (P=0.013). The non-prophylaxed patients had a longer cytomegalovirus-related in-hospital stay (P=0.018) and nonsignificantly higher cytomegalovirus-related costs. Bronchiolitis obliterans syndrome was less frequent with prophylaxis (P=0.039), and survival tended to be better (P=0.072). The only adverse effect was a subclavian vein thrombosis in the intravenous ganciclovir group. CONCLUSIONS: In lung transplant recipients, ganciclovir prophylaxis, either intravenous or oral, is safe, well tolerated, and effective in preventing cytomegalovirus disease. Moreover, ganciclovir prophylaxis seems likely to reduce the incidence of bronchiolitis obliterans syndrome. The oral formulation might be preferable because its convenience and possibly lower costs.

Coronary artery disease in patients undergoing lung volume reduction surgery for emphysema.

OBJECTIVES: Most patients with severe pulmonary emphysema referred for lung volume reduction surgery (LVRS) have a long-standing history of cigarette smoking. Coronary artery disease (CAD) predisposes to perioperative cardiac complications. Since symptoms and signs of myocardial ischemia are often absent in patients with severe ventilatory impairment even during exercise, we investigated the prevalence of CAD in candidates for LVRS by angiography. DESIGN: We prospectively studied the prevalence of CAD by angiography and assessed the CAD risk factor profile in 41 candidates for LVRS (26 men, 15 women; mean age, 66+/-6.8 years; range, 52 to 76 years), who had no current symptoms or a history of myocardial ischemia. RESULTS: In six patients (15%), asymptomatic but significant coronary lesions (> 70% stenosis) were detected. In five patients, these findings altered the clinical management. Patients with CAD had significant higher cholesterol levels, tended to have smoked more, and had more often additional vascular risk factors. CONCLUSIONS: We found a high prevalence of angiographically significant but clinically silent CAD in this particular population of heavy smokers with advanced emphysema.

Influence of age and gender on upper airway resistance in NREM and REM sleep.

The prevalence of irregular breathing during sleep is age and gender dependent, but the reason for this is unknown. This study tested the hypothesis that older men have a greater sleep-related increase in respiratory resistance. In 48 healthy subjects, 12 in each of four groups of younger and older men and women, airway resistance was measured during wakefulness and sleep using a mask, pneumotachograph, and catheter-mounted pressure sensors. Total respiratory resistance and total "low-flow," and "high-flow" oropharyngeal resistance were analyzed from 170,000 breaths, high flow being at rates above 50% maximal inspiratory flow. High-flow oropharyngeal and total respiratory resistance increased during non-rapid eye movement (NREM) sleep in all groups but not low-flow resistance. Total respiratory resistance increased from 12 +/- 1.2 cmH(2)O. l(-1). s(-1) awake to 16.2 +/- 2.4 in NREM sleep in young men, from 22.8 +/- 3.6 to 33.6 +/- 5.4 in young women, from 18 +/- 3 to 34.8 +/- 4.8 in older men, and from 26.6. +/- 4.2 to 34.2 +/- 6 in older women. The percentage of change in total respiratory resistance from awake to NREM sleep was not different between age groups or genders. We conclude that there are no major age or gender differences in the changes in airway resistance with sleep in normal subjects.

Redo lung volume reduction surgery in a patient with alpha1-antitrypsin deficiency.

Lung volume reduction surgery is a palliative procedure that improves dyspnea and pulmonary function in selected patients with advanced emphysema. Postoperative benefit is sustained for an individual period and depends on the emphysema morphology, the surgical technique, and other not yet well-defined factors. The question whether lung volume reduction surgery can be performed a second time on the same thoracic cavity is often raised but experience in this regard is lacking. We describe a patient who has undergone a successful redo operation 2 years after the initial lung volume reduction surgery.

Radiologic emphysema morphology is associated with outcome after surgical lung volume reduction.

BACKGROUND: Lung volume reduction surgery is known to alleviate dyspnea and to improve pulmonary function, performance in daily activity, and quality of life in selected patients with severe pulmonary emphysema. We investigated the role of radiologically assessed emphysema morphology on functional outcome after a lung volume reduction operation. METHODS: The preoperative chest computed tomograms in 50 consecutive patients who had undergone surgical lung volume reduction were retrospectively reviewed by 6 physicians blinded to the clinical outcome. Emphysema morphology was determined according to a simplified classification (ie, homogeneous, moderately heterogeneous, and markedly heterogeneous; lobe predominance). We studied the impact of these morphologic aspects on functional outcome at 3 months. RESULTS: We found a fair interobserver agreement applying our classification system. Functional improvement after surgical lung volume reduction was best in markedly heterogeneous emphysema with an increase in forced expiratory volume in 1 second of 81% +/- 17% (mean +/- standard error, n = 17) compared with 44% +/- 10% (n = 16) for intermediately heterogeneous emphysema. But also in patients with homogeneous emphysema clinical relevant improvement of function could be observed (increase in forced expiratory volume in 1 second = 34% +/- 6%; n = 17). CONCLUSIONS: The morphologic type of emphysema, assessed by a simplified surgically oriented classification, is an important predictor of surgical outcome. Lung volume reduction surgery may also improve dyspnea and lung function in homogeneous emphysema.

Thoracoscopic bilateral lung volume reduction for diffuse pulmonary emphysema.

OBJECTIVE: In a prospective study, we investigated the functional results, complications and survival of bilateral video-assisted thoracoscopic (VAT) lung volume reduction (LVR) in a selected group of patients with severe, nonbullous pulmonary emphysema. From January 1994 to September 1996, 42 of 143 candidates (13 female, 29 male, 42-78 years) were operated. They were short of breath on minimal exertion due to severe airflow obstruction and hyperinflation (FEV1 < 30%) pred., TLC > 130% pred., RV > 200% pred.). METHODS: LVR was performed bilaterally by VAT using endoscopic staplers without buttressing the staple lines. Pulmonary function test (PFT), MRC dyspnea score and 12 min walking distance were assessed preoperatively, at 3, 6 and 12 months. In addition lung function was measured at hospital discharge. RESULTS: The patients reported a marked relief of dyspnea, which persisted at all follow-up visits (P<0.001). FEV1 increased from 0.80 +/- 0.24 (L) to 1.14 +/- 0.41 (L) postoperatively, a 43% gain (P < 0.001). A relevant increase of FEV1 persisted for at least 1 year. The residual volume to total lung capacity ratio decreased from 0.64 to 0.56 at hospital discharge. The mean 12 min walking distance increased from 500 +/- 195 (m) to 770 +/- 222 (m) after 1 year (P < 0.001). The mean hospital stay was 13 +/- 5.5 days (median 12.0), drainage time was 9 +/- 4.3 (median 8.0) days. There was no 30 day mortality. Three patients died between 2 and 15 months postoperatively by non surgery related reasons. One patient underwent lung transplantation 5 months after surgical lung volume reduction. CONCLUSIONS: In a selected group of patients with severe, nonbullous pulmonary emphysema, bilateral LVR by VAT results in instantaneous postoperative improvement in pulmonary function and dyspnea. These favorable effects, including an amelioriation in exercise performance, lasted for at least 1 year.

Effect of lung volume reduction surgery on pulmonary hemodynamics in severe pulmonary emphysema.

OBJECTIVE: The presence of pulmonary hypertension in severe pulmonary emphysema has been considered a relative contraindication to lung volume reduction surgery (LVRS). There was concern that resection of lung tissue might further increase pulmonary artery pressure. To address this point, the prevalence of pulmonary hypertension in candidates for LVRS was investigated. The changes in pulmonary artery pressures after bilateral videoassisted thoracoscopic resection was studied in patients with homo- and heterogeneously destroyed emphysematous lungs. DESIGN: The pulmonary arterial pressures by right heart catheterization were prospectively assessed, before and 6 months after LVRS in 21 consecutive patients (15 males, six females, mean (+/- S.E.) age: 62 +/- 1.9, range 42-74 years). All were former smokers and three had ZZ-AT1 deficiency. The inclusion criteria were: (a) severe bronchial obstruction (FEV1 < 35% predicted); (b) pulmonary hyperinflation (RV/TLC > 0.60); and (c) absence of hypercapnia (PaCO2 < 50 mmHg). RESULTS: The FEV1 had increased from 28 +/- 2% to 35 +/- 3% of the predicted value (P < 0.05) 6 months after surgery. The RV/TLC had declined from 0.65 +/- 0.02 to 0.55 +/- 0.02; PaO2 increased (66 +/- 1 versus 71 +/- 2 mmHg, P = 0.04), PaCO2 (38 +/- 2 versus 36 +/- 1 mmHg, P = 0.26) did not change. The pulmonary artery mean pressure (PAPmean) remained unchanged (18 +/- 1 versus 19 +/- 1 mmHg, P = 0.26). In six patients PAPmean was > or = 20 mmHg (up to 24 mmHg) preoperatively. After 6 months, six patients had a PAPmean > or = 20 mmHg (up to 31 mmHg). CONCLUSIONS: In patients with severe emphysema who are candidates for LVRS (but have only mild to moderate hypoxemia and a PaCO2 < 50 mmHg) we found no relevant pulmonary hypertension and pulmonary artery pressure did not change significantly after surgery. Therefore, routine right heart catheterization is not mandatory for preoperative evaluation.

[Diagnosis of sleep apnea syndrome--role of family practitioner]. / Diagnose des Schlafapnoe Syndroms--Rolle des Hausarztes.

The obstructive sleep apnea syndrome is a highly prevalent and underdiagnosed disease. Repetitive arousals due to upper airway obstruction lead to hypersomnia. Due to the insidious onset, patients often underestimate the severity of their symptoms. Relatives can give helpful additional informations and should be involved in history taking and motivation for treatment. In general, GP's are confronted with the problem of fatigue and hypersomnia in the first line. They play the most important role in selecting patients for further investigations. The patient history helps to separate hypersomnia from fatigue. Hypersomnia indicates falling asleep at daytime in unappropriate situations, especially when the subject is passive. Often, a multidisciplinary approach including respiratory physicians, ENT specialists, orthopedic dentists and neurologists is warranted. Anatomic narrowing of the upper airway must be ruled out by clinical investigation. Overnight sleep studies detect apneas/hypopneas and repetitive arousals. Continuous positive airway pressure, applied by a nose mask and a flow generator remains the mainstay of therapy. Motivation, counseling and troubleshooting especially in the beginning of this therapy are of outmost importance. Ongoing research aims to improve comfort of nose masks and optimize function of flow generators.

[Giant cell arteritis and aortic dissection in 2 siblings]. / Riesenzellarteriitis und Aortendissektion bei einem Geschwisterpaar.

We present the case of two female siblings with temporal arteritis, polymyalgia and type B aortic dissection. Aortic aneurysms in families are well known in Marfan's and Ehlers-Danlos syndrome; they can, however, occur without signs of a collagen disorder. In patients with arteritis temporalis the incidence of aortic aneurysms is higher than normal. In a retrospective survey of 20,591 autopsies there were 443 aortic aneurysms (2%), 30 (7%) being a consequence of arteritis. Arteritic processes caused 15% of the thoracic and 5% of the abdominal aneurysms. This is the first description of temporal arteritis, polymyalgia and aortic dissection in siblings. The observation illustrates the danger of aneurysmatic aortic dilatation and/or dissection at a later or chronic stage of arteritis.

Sleep-related laryngospasm.

The term "sleep-related laryngospasm" refers to episodic, abrupt interruption of sleep accompanied by feelings of acute suffocation followed by stridor. The condition is included in the diagnostic and coding manual of the American Sleep Disorders Association (ASDA), but there are few references in the peer-reviewed literature. Our description of the distinct clinical picture associated with this condition is based on an analysis of the histories of a series of 10 patients. The patients and their families gave precise, uniform accounts of the dramatic attacks. Diagnostic work-up included pulmonary and gastroenterological assessment. All patients reported sudden awakening from sleep due to feelings of acute suffocation, accompanied by intense fear. Apnoea lasting 5-45 s was followed by stridor. Breathing returned to normal within minutes. Patients were left exhausted by the attacks. Nine of our 10 patients had evidence of gastro-oesophageal reflux and six responded to antireflux therapy. We conclude that the nocturnal choking attacks (and the occasional daytime attacks experienced by some of the patients) are caused by laryngospasm. The pathogenesis of the apparent underlying laryngeal irritability is unknown. The condition may be related to a gastro-oesophageal reflux.

Chronic necrotizing pulmonary aspergillosis complicated by pneumothorax.

A 61-year-old man presented with left-sided pneumothorax. On the chest computed tomograghy (CT), severe bilateral emphysema and left-sided pleural thickening were seen. His pneumothorax was drained with a chest tube. Because of a persistent air leakage, video-thoracoscopic wedge-resection of the suspected fistula and muscle-sparing minithoracotomy with extensive wedge resections of the left upper lobe were performed. Biopsy specimens showed micronodular mycetomas with septate hyphae highly suggestive of Aspergillus. The fungus destructed the lung tissue without vessel invasion. The patient had not been taking immunosuppressant drugs and had no prior opportunistic infections. Itraconazole was begun, the lung was expanded and the patient recovered. We propose that extensive resection of affected lung tissue in combination with long-term antifungal therapy with itraconazole is a valuable therapeutic option in patients with a complicated course of chronic necrotizing pulmonary aspergillosis (CNPA).

Recurrent brain abscesses in an HIV-positive patient with hereditary hemorrhagic telangiectasia and arteriovenous malformations of the lung.

Patients with hereditary hemorrhagic telangiectasia are at risk for the development of brain abscesses. The history of a 47-year-old man infected with the human immunodeficiency virus and with hereditary hemorrhagic telangiectasia is reported. Within eight months, the patient presented twice with life-threatening cerebral abscesses at different sites. On both occasions, Streptococcus anginosus was cultured from the abscess material. Treatment consisted of parenteral antibiotics and neurosurgical drainage. After treatment of the second occurrence, the patient was placed on a prophylactic regimen of clindamycin. He remains relapse-free and is clinically stable 24 months after the second episode.

Bronchial manifestation of acute febrile neutrophilic dermatosis (Sweet's syndrome).

We describe a 62 yr old female with acute febrile neutrophilic dermatosis (AFND) or Sweet's syndrome, involving the bronchial mucosa. The patient presented with classical skin manifestations of AFND accompanied by bilateral pneumonia, pleural effusions and lesions of the oral mucosa. The diagnosis was confirmed by skin and mucosal biopsies. Bronchoscopy revealed raised pustules, similar to the lesions of the skin, suggesting bronchial involvement of AFND. The patient responded to systemic corticosteroids only for a short period and died as a result of multiorgan failure. Postmortem findings confirmed dense, focal neutrophilic infiltrates of the bronchi and bronchioles. No underlying disease was found.