Multidimensional approach to non-cystic fibrosis bronchiectasis: the FACED score.

Bronchiectasis is a multidimensional disease and, therefore, its severity or prognosis cannot be adequately quantified by analysing one single variable. The objective of the present study was to develop a multidimensional score that classifies the severity of bronchiectasis according to its prognosis. This is an observational multicentre study including 819 patients diagnosed with non-cystic fibrosis bronchiectasis using high-resolution computed tomography. 397 subjects were selected at random to construct the score while the remaining 422 were used for its validation. The outcome was 5-year all-cause mortality after radiological diagnosis. A logistic regression analysis was used to select the variables included in the final score. The final seven-point score incorporated five dichotomised variables: forced expiratory volume in 1 s % predicted (F, cut-off 50%, maximum value 2 points); age (A, cut-off 70 years, maximum value 2 points); presence of chronic colonisation by Pseudomonas aeruginosa (C, dichotomic, maximum value 1 point); radiological extension (E, number of lobes affected, cut-off two lobes, maximum value 1 point); and dyspnoea (D, cut-off grade II on the Medical Research Council scale, maximum value 1 point) to construct the FACED score. The validation cohort confirmed the score's validity. We conclude that this easy-to-use multidimensional grading system proved capable of accurately classifying the severity of bronchiectasis according to its prognosis.

Validation of a Quality of Life Questionnaire for Bronchiectasis: psychometric analyses of the Spanish QOL-B-V3.0.

PURPOSE: Bronchiectasis is a chronic disease, leading to worsening of health-related quality of life. This study evaluated the psychometric properties of a new patient-reported outcome for non-cystic fibrosis bronchiectasis, the Quality of Life Questionnaire Bronchiectasis, translated into Spanish (QOL-B-Sp-V3.0). METHODS: This prospective study recruited clinically stable patients with non-cystic fibrosis bronchiectasis at 4 Spanish centers. Health status was assessed with multiple indicators (dyspnea, exacerbations, bronchorrhea, etc.), microbiological, radiological, spirometric, and anthropometric parameters plus St-George Respiratory Questionnaire (SGRQ). Psychometric analyses included internal consistency, test-retest reliability, convergent validity, predictive validity, and responsivity to change. RESULTS: The 207 stable patients (mean age 57.2 years) had a Bhalla score of 11.53 ± 7.39 and FEV1% of 68.3 ± 22.2 %. One hundred and sixty-one stable patients repeated the test 2 weeks later, and 80 patients who had an exacerbation within 6 months of the assessment also repeated it. Internal consistency was high across all scales (Cronbach's alpha >0.70). Thirty-six of 37 items correlated more strongly with their assigned scale than a competing scale. Test-retest coefficients were strong (intraclass correlations r = 0.68-0.88). All scales, except Treatment Burden, discriminated significantly between patients with mild, moderate, and severe disease according to FEV1% and other respiratory parameters. Strong convergence was found between the QOL-B-Sp-V3.0 and SGRQ. Significant correlations were found between QOL-B-Sp-V3.0 and various clinical, spirometric, radiological, and anthropometric variables. Significant differences were found on all QOL-B-Sp-V3.0 scales, except emotional functioning, between the baseline responses and onset of an exacerbation; robust sensitivity to change was observed on the Respiratory Symptoms scale. CONCLUSIONS: The QOL-B-Sp-V3.0 questionnaire demonstrated strong reliability and validity. Scores were reproducible after 2 weeks, and it discriminated between patients who varied in severity and was responsive to changes related to exacerbation.

Impact of CFTR modulator therapy on body composition as assessed by thoracic computed tomography: A follow-up study.

OBJECTIVE: Treatment with cystic fibrosis transmembrane conductance regulator (CFTR) modulators in individuals with cystic fibrosis (CF) has brought a significant change in forced expiratory volume in 1 second (FEV1) and clinical parameters. However, it also results in weight gain. The aim of our study is to evaluate the effect of CFTR modulator treatment on body composition, measured by computed tomography (CT). METHODS: Adult subjects with CF under follow-up at La Princesa University Hospital were recruited. All of them were on elexacaftor-tezacaftor-ivacaftor (ELX/TEZ/IVA) treatment. Body composition analysis was conducted using CT scans and an open-source software. The results were then compared with bioimpedance estimations, as well as other clinical and spirometry data. RESULTS: Our sample consisted of 26 adult subjects. The fat mass compartments on CT scans correlated with similar compartments on bioimpedance, and normal-density muscle mass exhibited a strong correlation with phase angle. Higher levels of very low-density muscle prior to treatment were associated with lower final FEV1 and less improvement in FEV1 after therapy. We observed an increase in total body area (P < 0.001), driven by increases in total fat mass (P < 0.001), subcutaneous fat (P < 0.001), visceral fat (P = 0.002), and intermuscular fat (P = 0.022). The only muscle compartment that showed an increase after treatment was very low-density muscle (P = 0.032). CONCLUSIONS: CT scans represent an opportunity to assess body composition on CF. Combination treatment with CFTR modulators, leads to an improvement in FEV1 and to an increase in body mass in all compartments primarily at the expense of fat mass.

Efficacy and Safety of Dry Powder Antibiotics: A Narrative Review.

The use of inhaled antibiotics was initially almost exclusively confined to patients with cystic fibrosis (CF). However, it has been extended in recent decades to patients with non-CF bronchiectasis or chronic obstructive pulmonary disease who present with chronic bronchial infection by potentially pathogenic microorganisms. Inhaled antibiotics reach high concentrations in the area of infection, which enhances their effect and enables their long-term administration to defeat the most resistant infections, while minimizing possible adverse effects. New formulations of inhaled dry powder antibiotics have been developed, providing, among other advantages, faster preparation and administration of the drug, as well as avoiding the requirement to clean nebulization equipment. In this review, we analyze the advantages and disadvantages of the different types of devices that allow the inhalation of antibiotics, especially dry powder inhalers. We describe their general characteristics, the different inhalers on the market and the proper way to use them. We analyze the factors that influence the way in which the dry powder drug reaches the lower airways, as well as aspects of microbiological effectiveness and risks of resistance development. We review the scientific evidence on the use of colistin and tobramycin with this type of device, both in patients with CF and with non-CF bronchiectasis. Finally, we discuss the literature on the development of new dry powder antibiotics.

Continuous Glucose Monitoring as an Additional Tool in Early Cystic Fibrosis-Related Diabetes Monitoring and in Evaluation of Short-Term Sitagliptin Response.

Cystic fibrosis-related diabetes (CFRD) is a complication associated with a negative prognosis in patients with cystic fibrosis (CF). Although the oral glucose tolerance test (OGTT) is the widely recommended screening test for CFRD diagnosis, continuous glucose monitoring (CGM) is increasingly considered a useful and easy-to-perform test for diagnosis and follow-up in clinical practice. Regarding CFRD treatment, although insulin is the classic approved pharmacological option, incretins could also be a helpful alternative in early stages. CGM could be also a useful tool to measure the early response to this therapy. METHODS: We studied 25 CF patients with abnormal OGTT results and compared glucose and insulin levels during the OGTTs with CGM results as a tool for early CFRD diagnosis. In addition, we evaluated glycaemic control with CGM before and after treatment with sitagliptin. RESULTS: A correlation was found between lower plasma insulin levels during the OGTTs and higher average sensor glucose (p = 0.009) and hyperglycaemic excursions (p = 0.017). The CGM data on sitagliptin treatment (n = 25) showed an average glycaemic improvement from 124.2 to 117.2 mg/dL (p = 0.002) with a 5.6-point standard deviation of glucose decrease (p < 0.001). Hyperglycaemic excursions ≥200 mg/dL diminished 57.1% (p = 0.021). Both time in range and time above 180 mg/dL improved during treatment (p = 0.036 and p = 0.006, respectively). CONCLUSION: CGM is a useful tool that offers valuable information for both the diagnosis and the management of CFRD. Lower plasma insulin levels during OGTTs are associated with a poor ambulatory glucose profile in CGM. Sitagliptin could play an important role in the treatment of the early stages of CFRD.

Filamentous fungi in the airway of patients with cystic fibrosis: Just spectators?

BACKGROUND: There are important advances in the management of bacterial infection in patients with cystic fibrosis (CF), but there are many gaps in the field of fungal infections. AIMS: The aim of this study was to analyse whether chronic respiratory filamentous fungal colonization had clinical impact and whether antifungal treatment can change the disease. METHODS: The prospective, bicentric and descriptive study was carried out within a 3-year follow-up period, with four-month periodicity medical controls. Adult patients from two CF units of tertiary hospitals were included. Clinical, microbiological, analytical and spirometric variables were collected. Quality of life was evaluated in a subgroup, using the Spanish version of the Revised Cystic Fibrosis Quality of Life Questionnaire (CFQ-R). To statistically analyze the evolution of forced expiratory along time (volume of air blown out in 1 second -FEV1-) and the forced vital capacity (FVC), mixed linear models were carried out. RESULTS: From the ninety-eight patients under study, 40 suffered chronic filamentous fungal colonization. The presence of filamentous fungi in airway was associated to an annual fall of FEV1 and FVC of 0.029 and 0.017 litres, respectively (p<0.001). In addition, worse quality of life based on CFQ-R, significant when concerning physical condition and emotional state, was also linked with the fungal colonization. Protocolized antifungal therapy, nebulized or oral, improved FEV1 in 0.023 and 0.024 litres per year, respectively (p<0.001). CONCLUSIONS: Chronic filamentous fungal colonization in patients with CF is associated with a significant annual decline of lung function that persists over time. Chronic antifungal therapy slows down this progression, mainly in the patient with more advanced disease.

[Predictive factors for survival in patients with idiopathic pulmonary fibrosis]. / Factores predictivos de supervivencia en pacientes con fibrosis pulmonar idiopática.

BACKGROUND AND OBJECTIVE: To determine the median survival and determinants of survival in patients with idiopathic pulmonary fibrosis (IPF). PATIENTS AND METHOD: We retrospectively evaluated 29 IPF patients who died in the pneumology department between 2001 and 2006. Data from the time of diagnosis until death were analysed. Determinants of survival including age at diagnosis, gender, history of cigarette smoking, fibrosis-emphysema association, and lung function tests were analysed. RESULTS: The mean age at diagnosis was 69.7, 16 patients were men (55.2%). The median survival time, from the time of diagnosis, was 28.47 months (20.44-36.5 IC95%). There was a significant difference in survival between age groups. The median survival time in patients older than 65 years was 22.40 months and in patients younger than 65 years it was 56.37 months (p<0.001). There was no significant difference in survival when other determinants of survival. were evaluated. CONCLUSION: The median survival of IPF patients is low. It is necessary to continue investigating to find more effective and selective therapeutic strategies.

Spanish Guidelines on the Evaluation and Diagnosis of Bronchiectasis in Adults. / Normativa sobre la valoración y el diagnóstico de las bronquiectasias en el adulto.

In 2008, the Spanish Society of Pulmonology (SEPAR) published the first guidelines in the world on the diagnosis and treatment of bronchiectasis. Almost 10 years later, considerable scientific advances have been made in both the treatment and the evaluation and diagnosis of this disease, and the original guidelines have been updated to include the latest scientific knowledge on bronchiectasis. These new recommendations have been drafted following a strict methodological process designed to ensure the quality of content, and are linked to a large amount of online information that includes a wealth of references. These guidelines cover aspects ranging from a consensual definition of bronchiectasis to an evaluation of the natural course and prognosis of the disease. The topics of greatest interest and some new areas are addressed, including epidemiology and economic costs of bronchiectasis, pathophysiological aspects, the causes (placing particular emphasis on the relationship with other airway diseases such as chronic obstructive pulmonary disease and asthma), clinical and functional aspects, measurement of quality of life, radiological diagnosis and assessment, diagnostic algorithms, microbiological aspects (including the definition of key concepts, such as bacterial eradication or chronic bronchial infection), and the evaluation of severity and disease prognosis using recently published multidimensional tools.

Spanish Guidelines on Treatment of Bronchiectasis in Adults. / Normativa sobre el tratamiento de las bronquiectasias en el adulto.

In 2008, the Spanish Society of Pulmonology (SEPAR) published the first guidelines in the world on the diagnosis and treatment of bronchiectasis. Almost 10 years later, considerable scientific advances have been made in both the treatment and the evaluation and diagnosis of this disease, and the original guidelines have been updated to include the latest therapies available for bronchiectasis. These new recommendations have been drafted following a strict methodological process designed to ensure quality of content, and are linked to a large amount of online information that includes a wealth of references. The guidelines are focused on the treatment of bronchiectasis from both a multidisciplinary perspective, including specialty areas and the different healthcare levels involved, and a multidimensional perspective, including a comprehensive overview of the specific aspects of the disease. A series of recommendations have been drawn up, based on an in-depth review of the evidence for treatment of the underlying etiology, the bronchial infection in its different forms of presentation using existing therapies, bronchial inflammation, and airflow obstruction. Nutritional aspects, management of secretions, muscle training, management of complications and comorbidities, infection prophylaxis, patient education, home care, surgery, exacerbations, and patient follow-up are addressed.

Pneumonic and non-pneumonic exacerbations in bronchiectasis: Clinical and microbiological differences.

OBJECTIVES: Despite the clinical relevance of exacerbations in bronchiectasis (BE), little is known about the microbiology and outcomes of pneumonic (CAP) vs. non-pneumonic (NOCAP) exacerbations. METHODS: This study compares clinical and microbiological characteristics of CAP vs. NOCAP in adults with BE. We performed a multicenter prospective observational study of consecutive cases of NOCAP and CAP from four Spanish hospitals (2011-2015). RESULTS: We recruited 144 patients, 47 of them CAP (33%) cases. CAP patients were older, with a larger representation of males, more comorbidities, higher arterial hypertension and COPD but less chronic bronchial infection and previous history of exacerbations. Clinical presentation was similar, excepting creatinine, C-reactive protein (C-RP), glucose and leukocytes which were higher in CAP. C-RP of 8.38 mg/dL showed a significant predictive discrimination for CAP. Streptococcus pneumoniae and Pseudomonas aeruginosa were the first causes of CAP and NOCAP, respectively. The rate of microbiological concordance with previous chronic bronchial infection was variable. Main clinical outcomes (mortality, length of stay, etc.) were similar in the two groups. Chronic bronchial infection and history of frequent exacerbations (≥ 2/year) were associated with a reduced risk of CAP. CONCLUSIONS: CAP and NOCAP in BE had similar clinical presentation with the exception of fever, leukocytosis, and C-RP. Microbiology also differed. A cut-off value of C-RP ≥ 8.38 mg/dL can predict CAP in bronchiectasis.

[Polymyositis and interstitial lung disease with a favorable response to corticosteroids and methotrexate]. / Polimiositis y afectación pulmonar intersticial con buena respuesta a glucocorticoides y metotrexato.

Polymyositis is a rare collagen disease that can involve the lungs. Between 5% and 30% of patients with polymyositis present interstitial lung disease at diagnosis or during the course of disease. Onset is usually insidious and involves dyspnea and nonproductive cough. Several histopathological findings are associated with polymyositis and the most common is nonspecific interstitial pneumonia. The prognosis of interstitial lung disease associated with polymyositis is better than that of idiopathic pulmonary fibrosis, since most patients respond to treatment with corticosteroids and immunosuppressants. We report the case of a 60-year-old woman with dyspnea and muscle weakness who was diagnosed with polymyositis and interstitial lung disease (radiography indicated possible nonspecific interstitial pneumonia). The patient responded well to prednisone and methotrexate.

Clinical impact of chronic obstructive pulmonary disease on non-cystic fibrosis bronchiectasis. A study on 1,790 patients from the Spanish Bronchiectasis Historical Registry.

BACKGROUND: Few studies have evaluated the coexistence of bronchiectasis (BE) and chronic obstructive pulmonary disease (COPD) in series of patients diagnosed primarily with BE. The aim of this study was to analyse the characteristics of patients with BE associated with COPD included in the Spanish Bronchiectasis Historical Registry and compare them to the remaining patients with non-cystic fibrosis BE. METHODS: We conducted a multicentre observational study of historical cohorts, analysing the characteristics of 1,790 patients who had been included in the registry between 2002 and 2011. Of these, 158 (8.8%) were registered as BE related to COPD and were compared to the remaining patients with BE of other aetiologies. RESULTS: Patients with COPD were mostly male, older, had a poorer respiratory function and more frequent exacerbations. There were no differences in the proportion of patients with chronic bronchial colonisation or in the isolated microorganisms. A significantly larger proportion of patients with COPD received treatment with bronchodilators, inhaled steroids and intravenous antibiotics, but there was no difference in the use of long term oral or inhaled antibiotherapy. During a follow-up period of 3.36 years, the overall proportion of deaths was 13.8%. When compared to the remaining aetiologies, patients with BE associated with COPD presented the highest mortality rate. The multivariate analysis showed that the diagnosis of COPD in a patient with BE as a primary diagnosis increased the risk of death by 1.77. CONCLUSION: Patients with BE related to COPD have the same microbiological characteristics as patients with BE due to other aetiologies. They receive treatment with long term oral and inhaled antibiotics aimed at controlling chronic bronchial colonisation, even though the current COPD treatment guidelines do not envisage this type of therapy. These patients' mortality is notably higher than that of remaining patients with non-cystic fibrosis BE.

Etiology of Bronchiectasis in a Cohort of 2047 Patients. An Analysis of the Spanish Historical Bronchiectasis Registry. / Etiología de las bronquiectasias en una cohorte de 2.047 pacientes. Análisis del registro histórico español.

INTRODUCTION: Bronchiectasis is caused by many diseases. Establishing its etiology is important for clinical and prognostic reasons. The aim of this study was to evaluate the etiology of bronchiectasis in a large patient sample and its possible relationship with demographic, clinical or severity factors, and to analyze differences between idiopathic disease, post-infectious disease, and disease caused by other factors. METHODS: Multicenter, cross-sectional study of the SEPAR Spanish Historical Registry (RHEBQ-SEPAR). Adult patients with bronchiectasis followed by pulmonologists were included prospectively. Etiological studies were based on guidelines and standardized diagnostic tests included in the register, which were later included in the SEPAR guidelines on bronchiectasis. RESULTS: A total of 2,047 patients from 36 Spanish hospitals were analyzed. Mean age was 64.9years and 54.9% were women. Etiology was identified in 75.8% of cases (post-Infection: 30%; cystic fibrosis: 12.5%; immunodeficiencies: 9.4%; COPD: 7.8%; asthma: 5.4%; ciliary dyskinesia: 2.9%, and systemic diseases: 1.4%). The different etiologies presented different demographic, clinical, and microbiological factors. Post-infectious bronchiectasis and bronchiectasis caused by COPD and asthma were associated with an increased risk of poorer lung function. Patients with post-infectious bronchiectasis were older and were diagnosed later. Idiopathic bronchiectasis was more common in female non-smokers and was associated with better lung function, a higher body mass index, and a lower rate of Pseudomonas aeruginosa than bronchiectasis of known etiology. CONCLUSIONS: The etiology of bronchiectasis was identified in a large proportion of patients included in the RHEBQ-SEPAR registry. Different phenotypes associated with different causes could be identified.

[Efficiency of the home intravenous antibiotics treatment in cystic fibrosis]. / Eficiencia del tratamiento antibiótico intravenoso domiciliario en la fibrosis quística.

BACKGROUND AND OBJECTIVE: The objective of our study was to determine the costs saving with the implementing of a home intravenous antibiotic treatment (HIVAT) program for patients with cystic fibrosis and to compare it with the conventional system (inpatient). PATIENTS AND METHOD: Consecutive patients in an adults cystic fibrosis unit were selected who received some days of HIVAT, between January 2002 and December 2004. For the analysis of costs saving of the HIVAT, we used the difference between the total costs of the avoided stay days and the costs generated by the domiciliary therapy (drugs, expendable equipment) and by the ambulatory medicine unit in case the patients were not hospitalized. All patients received a therapy with an intravenous antibiotic for a minimum of 14 days. All these data were provided by the accounting service of the hospital with the aid management Clinical Financier Program (GECLIF). RESULTS: 22 patients with cystic fibrosis needed 85 intravenous antibiotics treatments during the 3 years of the study, of which: 43 cycles were completely domiciliary, 14 inpatient and 28 were combined (hospital and home). The 71 cycles of HIVAT originated 909 days at home, with an average (standard deviation) of 12.80 (4.18) days and 43 treatments in ambulatory medicine unit. The home antibiotic treatments that originated greater cost (3,964.34 Euro) was meropenem (1 g/6 h) i.v. with linezolid (600 mg/12 h) via oral combination during 14 days, and in second place the association of ceftazidime, tobramycine and linezolid, whose cost in cycle of 14 days was of 2464.84 Euro. The average saving cost in the 3 years of study was of 2,647.29 Euro by each cycle of HIVAT and global 197,689.78 Euro. CONCLUSIONS: HIVAT obtained important sanitary costs saving and this was greater every year, not due to the increase of days at home, but due to the rising cost per day of hospital stays every new year.

[Protocol for prevention and treatment of osteoporosis in patients with cystic fibrosis]. / Efectividad en la aplicación de tres protocolos de prevención y tratamiento de la osteoporosis en pacientes adultos con fibrosis quística.

BACKGROUND AND OBJECTIVE: Reduction of bone mineral density (BMD) is a complication of cystic fibrosis (CF) which is observed in parallel to the increment of life expectancy in these patients. The aim of this study was to analyze the evolution of BMD following the application of a protocol for the prevention and treatment of osteoporosis. PATIENTS AND METHOD: We performed a multidisciplinary prospective study in 21 adult patients with CF with a mean age of 24.3 (r: 19-44) years. We evaluated BMD results depending on the treatment schedule, and the annual relative change of BMD percentage was correlated with changes observed in respiratory function, corporal mass index (CMI), Brasfield radiologic score and Shwachman clinical score for a 3 years follow up period. Three regimens of treatment were applied: general measurements, supplementation of calcium and vitamin D, and 10 daily mg of alendronate plus calcium and vitamin D supplements. RESULTS: Basal assessment showed that 14.2% of CF patients had a marked diminution of bone mass with respect to an age and sex matched control population, with a Z score of < -2 DE in lumbar vertebral and/or total femur. Another 38% showed a lessening of Z score between -1 and -2.5 DE. We observed a progressive annual reduction of BMD in all the anatomic areas analyzed: -0.52% (1.87) in lumbar spine, -1.17% (1.91) in total femur and -2.16% (2.65) in neck femur. The hip BMD annual decrement was related to that observed in FEV1. Only patients treated with alendronate did not suffer progressive BMD lose. Treatment with a combination of calcium, vitamin D and alendronate was more efficient in hip BMD than calcium plus vitamin D alone (p < 0.05). Also, this combination was better than no treatment at hip and femoral neck levels (p < 0.05). CONCLUSIONS: Our work confirms that Spanish young adults patients with CF show low control matched BMD, and that it even worsens at follow-up. This decrement is not adequately halted with preventive treatment with supplements of calcium and vitamin D, and only patients treated with alendronate show increments of their BMD.

Long-term safety and efficacy of tobramycin in the management of cystic fibrosis.

Cystic fibrosis (CF) is a fatal inherited disease caused by mutations in the CF transmembrane conductance regulator (CFTR) gene whose mortality is conditioned by a progressive decline in lung function. Bacterial infections play a key role in this decline. Chronic bacterial infection in CF patients varies over time and the presence of Pseudomonas aeruginosa in sputum is a marker of poor prognosis. P. aeruginosa is eradicated from the airways using inhaled antibiotics administered in various formulations and devices. Antipseudomonal antibiotics have extended the survival of CF patients to 40 years. Tobramycin is a bactericidal aminoglycoside antibiotic with demonstrated activity against gram-negative microorganisms. Initially, the drug was administered as an inhaled parenteral solution. Subsequently, a specific tobramycin inhalation solution was developed. PulmoSphere™ technology enables dry tobramycin powder to be formulated for inhalation (tobramycin inhalation powder) using a small and portable capsule-based breath-activated device (T-326). Chronic colonization by P. aeruginosa is the main indication for aerosol antibiotic therapy. The American Cystic Fibrosis Foundation, European guidelines, and Spanish consensus guidelines provide different recommendations for eradication.

[Home intravenous antibiotic treatments in cystic fibrosis units of Madrid]. / Tratamiento antibiótico intravenoso domiciliario en las unidades de fibrosis quística de la Comunidad de Madrid.

BACKGROUND AND OBJECTIVE: A descriptive study of the home intravenous antibiotic treatment (HIVAT) course in cystic fibrosis (CF) units of Madrid in an 18 month period. Different patient features were recorded, antibiotherapy, intravenous access, complications and their resolutions. We accessed the improvement of the pulmonary function, forced vital capacity (FVC) and forced expiratory volume in one second (FEV1) at the end of the treatment. PATIENTS AND METHOD: For an 18 months period (January 2002-June 2003) the patients with CF who received HIVAT and fulfilled the previously fixed criteria were included. The next clinic variables were collected: age, sex, bacterial colonization of respiratory tree, pancreatic function and pulmonary function in steady phase, before and after HIAT. RESULTS: 56 patients, 31 male and 25 female, were given 90 courses of HIAT (34 patients received only one course). Mean age was 20.06 (8.07) years. 57.1% of the patients were colonized by Pseudomonas aeruginosa. The most frequently used antibiotics were ceftazidime and tobramycin. Courses of treatment lasted a mean of 4.08 (5.09) days inpatient and 11.89 (4.96) at home. In 87.7% of the course were used the intravenous cannulae, Port-a-cath in 6.7% and Venocath in 7.8%. The intravenous access was replaced, in the 64.4% of the cases, by the nurse of the CF Unit, in the 21.4% in the emergency room of the nearest hospital and in 11.9% in primary care center. Three occasions skin reactions were reported. The parameters of pulmonary function improved significatively after HIVAT. CONCLUSIONS: The HIVAT is a therapeutic option that, following predetermined inclusion criteria, has a low complication rate and improves pulmonary function.

[Patients with cystic fibrosis managed at the cystic fibrosis units of Madrid: cross-sectional study of 387 subjects]. / Pacientes con fibrosis quística atendidos en las unidades de fibrosis quística de la Comunidad de Madrid: estudio transversal de 387 casos.

BACKGROUND AND OBJECTIVE: Our objective was to describe the clinical and genetics features of patients with cystic fibrosis (CF) attended in Madrid. PATIENTS AND METHOD: This was a descriptive, cross-sectional study of CF patients attended during 2001. Demographic, genetic, anthropometric, pancreatic insufficiency, diabetes mellitus, Pseudomonas aeruginosa colonization, lung function and body mass index (BMI) (z score) data were recorded and compared with the American CF Registry. RESULTS: 387 patients were included, most of them living in Madrid (n = 247 [63%]), 209 males (54%), with a mean age (SD) of 15.15 (10.42) years (younger than 18 years, 248, 64.24%). F508del was the most common mutation (52.8% of chromosomes) with 104 homozygous patients. Pancreatic insufficiency was present in 310 subjects (80.1%), diabetes mellitus in 30 (7.8%) and P. aeruginosa colonization in 126 (33.1%). Lung function was measured in 309 patients: mean of FEV1 and FVC predicted values (SD) was 82.5 (27.11) and 89.32 (21.89), respectively. The mean BMI z score was 0.0796 (1.18). CONCLUSIONS: CF patients from Madrid have a good nutritional status, less P. aeruginosa colonization, less pancreatic insufficiency and better lung function than those of the American CF Registry. The lower prevalence of homozygous F508del in our population may explain, at least partly, our findings.