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Fundamento Estudiar la presentacion clinica y actitud terapeutica ante la hemoptisis amenazanteen los pacientes con fibrosis quistica (FQ). Pacientes y metodo Pacientes con FQ mayores de 18 anos atendidos en la Comunidad Autonomade Madrid que presentaron hemoptisis amenazante entre enero de 1990 y diciembre de 1999. Resultados Doce pacientes (4 mujeres y 8 varones) con FQ presentaron 36 episodios de hemoptisisamenazante. En 30 casos fue masiva y en 6 recurrente. La afeccion pulmonar era moderada-grave. Diez pacientes tenian colonizacion pulmonar por Pseudomonas aeruginosa. Delos 36 episodios de hemoptisis, 13 (36%) se solucionaron con tratamiento antibiotico y tres(8%) con tratamiento antibiotico y fibrobroncoscopia. En los 20 episodios restantes se realizoembolizacion de las arterias bronquiales. La eficacia de la embolizacion para el control de lahemoptisis fue de un 80% (16 de 20 episodios) con una sesion de embolizacion, de un 85%(17/20) con dos sesiones y de un 95% (19/20) con tres sesiones. No hubo ninguna complicacionmayor por la embolizacion. La tasa de recurrencia por episodio fue de un 69% (24/35 episodios,en 6 pacientes), con un tiempo medio de recurrencia de 13 meses. Durante el seguimientono se produjeron muertes secundarias a la hemoptisis. Conclusiones La hemoptisis amenazante es frecuente en los pacientes con FQ y afeccion pulmonarmoderada-grave. Si la hemorragia no cesa con tratamiento conservador (incluyendo antibioticos),debe realizarse embolizacion de las arterias bronquiales. Esta tecnica es efectiva ysegura cuando la lleva a cabo personal experto.

Lymphangioleiomyomatosis (LAM) is a rare lung disease that predominantly affects young females and generally progresses to respiratory failure. There is not sufficient evidence to support the routine use of any treatment in LAM. The only treatment for severe LAM is currently lung transplantation. Activation of mammalian target of rapamycin (mTOR) signalling pathway has been observed in LAM. LAM is often associated with angiomyolipoma in the kidneys. mTOR inhibitor sirolimus reduces angymiolipoma volumes. Some reports have shown improvement in lung function with sirolimus in LAM. We report 3 women with LAM, with a rapid decline in lung function and symptoms and who were treated with sirolimus.

Background A very weak relationship has been reported between the health-related quality of life (HRQL) of patients with asthma and their degree of airway hyper-responsiveness (AHR), evaluated in terms of sensitivity. However, this relationship still has not been sufficiently explored for bronchial reactivity indices. Objectives To analyse the relationship between bronchial reactivity and sensitivity with the HRQL of patients with stable asthma, identifying the functional parameters that determine HRQL. Methods In 103 consecutive patients with stable asthma, HRQL was evaluated using the Asthma Quality of Life Questionnaire (AQLQ). Patients underwent spirometry and non-specific bronchial provocation with methacoline. Sensitivity (PD20) and reactivity (dose–response slope (DRS), continuous index of responsiveness (CIR) and bronchial reactivity index (BRI)) of the dose–response curve were analysed. Results BRI presented significant differences with different degrees of asthma severity. Although patients with AHR showed poorer quality of life than patients without AHR, the AQLQ total score was not related to PD20 but rather to DRS (r=−0.784), CIR (r=−0.712) and BRI (r=−0.776). The indices of bronchial reactivity reached a negative correlation with all the domains of the AQLQ. In a multiple linear regression model, BRI, DRS, FIV1 (forced inspiratory volume in 1 s) and VCIN (inspiratory vital capacity) were identified as independent predictors of the AQLQ total score (r2=0.742, p

Bronchiectasis is the end result of several different diseases that share principles of management. The clinical course usually involves chronic bronchial infection and inflammation, which are associated with progression. The cause of bronchiectasis should always be investigated, particularly when it can be treated. We recommend evaluating etiology, symptoms, bronchial colonization and infection, respiratory function, inflammation, structural damage, nutritional status, and quality of life in order to assess severity and to monitor clinical course. Care should be supervised by specialized units, at least in cases of chronic bronchial infection, recurrent exacerbations, or when there is a cause that is likely to respond to treatment. Improving symptoms and halting progression are the goals of management, which is based on treatment of the underlying cause and of acute or chronic infections and on the drainage of secretions. Complications that arise must also be treated. Antibiotic prescription is guided by how well infection is being controlled, and this is indicated by the color of sputum and a reduction in the number of exacerbations. We recommend inhaled antibiotics in cases of chronic bronchial infection that does not respond to oral antibiotics, when these cause side effects, or when the cause is Pseudomonas species or other bacteria resistant to oral antibiotics. Inhaled administration is also advisable to treat initial colonization by Pseudomonas species.

Aims: To estimate the prevalence of malnutrition in chronic obstructive pulmonary disease (COPD) patients hospitalized for exacerbation and to evaluate its clinical and prognostic influence on the exacerbation. Subjects/Methods: The subjects were 78 consecutive patients with moderate-to-severe COPD who were admitted to hospital with a diagnosis of exacerbation. Nutritional status was assessed by means of body mass index (BMI), bioelectric impedance analysis and levels of plasmatic albumin. Previous spirometry, 6-min walk test, severity of the exacerbation, days of hospitalization and readmission in the following 3 months were also evaluated. Results: Malnutrition [BMI Conclusions: The high prevalence of malnutrition among hospitalized COPD patients is related to their lung function and exercise tolerance. Moreover, nutritional parameters during exacerbation are related to length of hospitalization and readmission.

Objective Patients with cystic fibrosis are at great risk of infection by nontuberculous mycobacteria from the environment because of certain predisposing factors such as bronchiectasis, malnutrition, and diabetes. The aim of this study was to analyze the mycobacterial content of sputum smears and cultures from adult patients with cystic fibrosis attended at a specialized unit for adults from March 1997 through December 2001. Patients and methods Sputum samples were collected prospectively according to a protocol applied at each visit, and during most exacerbations staining and culture for mycobacteria were ordered in addition to the usual cultures for bacteria and fungi. A tuberculin test was performed at the end of the study. Results Twenty-eight patients (16 men) with cystic fibrosis were enrolled. The mean (SD) age was 25.3 (6.7) years. A total of 251 samples were cultured (range in number of samples per patient, 1-31). The mean period of follow up was 40.3 (22.1) months. The sputum smear was positive in 29 cases (4 patients); the culture was positive in 7 patients. More than 3 samples were positive in only 4 patients. Mycobacterium abscessus was isolated in 3 cases, Mycobacterium avium complex in 2 and Mycobacterium simiae in 1 and other an unidentified rapid growth Mycobacterium species. The Mantoux test was positive in 5 patients. Two of the 4 patients in whose samples mycobacteria were isolated repeatedly required treatment. Conclusions The prevalence of nontuberculous mycobacterial infection is high in patients with cystic fibrosis. Staining and culture for mycobacteria should be carried out regularly and whenever exacerbation of pulmonary symptoms cannot be attributed to bacteria usually found in such patients. Patients with recurrent isolations of mycobacteria should be monitored closely.

Wegener's granulomatosis is a systemic vasculitis of unknown etiology, primarily affecting the upper and lower respiratory tract and the kidneys, although there is a form restricted to the lungs. Wegener's granulomatosis occurs infrequently, with an estimated annual incidence of 8.5 cases per million. The principal symptoms at onset usually involve the upper and lower airway. The central nervous system is involved in approximately 22% of cases, but neurological signs occur infrequently among the initial clinical manifestations. Proteinase 3-specific antineutrophil cytoplasmic antibodies are useful serological markers for establishing a diagnosis, which should, however, be confirmed by a tissue biopsy of the affected organ.

Our goal was to evaluate the clinical characteristics and management of life-threatening hemoptysis in patients with cystic fibrosis (CF).We included adult CF patients followed up at the Cystic Fibrosis Units of the Autonomous Community of Madrid who had life-threatening hemoptysis from June 1990 to December 1999.Twelve CF patients (4 females) developed 36 episodes of life-threatening hemoptysis (30 massive and 6 recurrent). Lung disease was moderate to severe. Sputum cultures revealed Pseudomonas aeruginosa in 10 patients. Thirteen episodes (36%) resolved upon antibiotic treatment and 3 (8%) after antibiotic therapy and bronchoscopy. Bronchial artery embolization (BAE) was performed in 20 of 36 events. Immediate technique success was achieved in 80% episodes (16 of 20) after one session, 85% (17/20) after two sessions, and 95% (19/20) after three sessions. No major complications associated with the procedure were seen. The overall recurrence rate per episode was 69% (24 of 35 episodes in 6 patients) with a mean time of recurrence of 13 months. There were no massive hemoptysis-associated deaths during the follow-up.Life-threatening hemoptysis is a frequent complication in CF patients who have moderate or severe lung disease. When conservative therapeutic measures (including antibiotics) fail to control it, BAE should be performed. When performed by expert professionals, BAE is effective and safe to immediate control of life-threatening hemoptysis in patients with CF.

215 Adherence with TOBI® among cystic fibrosis patients with Pseudomonas aeruginosa infection V.N. Joish1, S.S. Sansgiry2, S.H. Boklage1, P.A. Chopra2, S. Sethi3. 1Bayer Healthcare Pharmaceuticals Inc., Health Economics and Outcomes Research, Wayne, United States; 2University of Houston, Clinical Sciences and Administration, Houston, United States; 3University at Buffalo, State University of New York, Buffalo, United States

Bronchiectasis is the end result of several different diseases that share principles of management. The clinical course usually involves chronic bronchial infection and inflammation, which are associated with progression. The cause of bronchiectasis should always be investigated, particularly when it can be treated. We recommend evaluating etiology, symptoms, bronchial colonization and infection, respiratory function, inflammation, structural damage, nutritional status, and quality of life in order to assess severity and to monitor clinical course. Care should be supervised by specialized units, at least when there is a history of chronic bronchial infection, recurrent exacerbations, or a cause that is likely to respond to treatment. Improving symptoms and halting progression are the goals of management, which is based on treatment of the underlying cause and of acute or chronic infections and on the drainage of secretions. Complications that arise must also be treated. Antibiotic prescription is guided by monitoring how well infection is being controlled, and this is indicated by the color of sputum and a reduction in the number of exacerbations. We recommend inhaled antibiotics when bronchial infection is chronic and does not respond to oral antibiotics or when these cause side effects, or when the cause is Pseudomonas species or other bacteria resistant to oral antibiotics. Inhaled administration is also advisable to treat initial colonization by Pseudomonas species.

Introduction: A20 is a negative regulator of TLR driven NF-uB signalling. This regulatory function is reliant on the formation of a ubiquitin editing complex comprising A20, Ring Finger protein (RNF)11, Itch (E3 ligase) and the adaptor protein TAX1BP1 [1]. In CF epithelial cells LPS reduces the expression of all complex members. Moreover, A20 does not interact with other complex members or target proteins [2,3]. Here we sought to determine the role of CFTR in the down-regulation of A20 ubiquitin editing genes. Methods: 16HBE41obronchial epithelial cells were transfected with CFTR siRNA. Primary nasal epithelial cells (NECs) from CF patients [F508del homozygous (n = 6), or R117H/F508del heterozygous (n = 5)] and age-matched controls (n = 6) were fully differentiated at air-liquid interface and treated with LPS (P. aeruginosa, Sigma) for 24h. A20, RNF11, Itch and TAX1BP1 mRNA expression was assessed by qPCR and analysed by ANOVA. Results: Following CFTR knockdown (transfection efficiency 72%±5.34%, n = 3) A20 mRNA was reduced by 43% (P < 0.01). This would indicate that A20 expression may be regulated by CFTR. In line with this, expression of A20, RNF11, Itch and TAX1BP1 was significantly reduced (P < 0.01–0.001) in NECs from both CF groups compared with controls. Moreover, the reduction in A20, RNF11 and TAX1BP1 (P < 0.05) expression was more pronounced in the F508del than R117H group. Conclusion: Expression of the A20 ubiquitin editing complex reflects CF disease severity and may prove a novel predictor of inflammation. The CF Trust UK (PJ541) supported this work