Quality of life in children with diseases causing central airway obstruction: Reliability and validity of the TAPQOL questionnaire.

INTRODUCTION: The aim of this study was to analyse the psychometric performance (reliability and validity) of the TAPQOL questionnaire in measuring the global quality of life in children with central airway obstruction (CAO), which encompasses pathologies with a low prevalence that have a common anatomical or functional affectation. METHOD: The study population consisted of 109 patients with CAO attended in a national reference airway unitafter signing informed consent. The TAPQOL questionnaire was used to measure the global quality of life and its psychometric characteristics were analysed. The reliability was determined using Cronbach's α statistic and the validity was determined by means of a principal component analysis. RESULTS: The mean age was 4.8 years and there was no predominant sex. The most frequent diagnosis was laryngomalacia (24.5%), followed bysubglotticstenosis (20.2%). The lowest score in the TAPQOL questionnaire was obtained for the scales of anxiety (66.2 ± 23.7), respiratory (67.9 ± 29.6), and conduct 68.2 ± 21.4). The Cronbach's α was above 0.7 in the majority of the scales and the validity study by principal component analysis with a total variance of 76.7% fits the original 12-component model. CONCLUSIONS: TAPQOL is a valid tool to measure the health-related quality of life in patients with CAO. Measuring it in these patients indicates which fields are most affected, highlighting therapeutic needs that have not been resolved but which can be approached in normal clinical practice.

Update of the European paediatric respiratory medicine syllabus.

The 10-year-old European syllabus for paediatric respiratory medicine (PRM; also known as paediatric pulmonology) was updated by a consensus-based method using an expert task force for redrafting, and a subsequent Delphi process to achieve consensus. There was a high degree of consensus for the final syllabus, which has been streamlined and made more relevant to current practice. All modules are now mandatory apart from the undertaking of research projects, which is optional. Although there are still a number of countries in Europe which do not recognise PRM as a separate subspecialty, there are paediatric respiratory physicians practising in every country in Europe, and a current and harmonised European syllabus in the subspecialty remains important for defining the training and areas of practice of PRM practitioners.

Central-peripheral Temperature Monitoring as a Marker for Diagnosing Late-onset Neonatal Sepsis.

BACKGROUND: The prognosis for late-onset sepsis depends largely on a timely diagnosis. We assess central-peripheral temperature difference monitoring as a marker for late-onset neonatal sepsis diagnosis. METHODS: We performed a prospective, observational study focusing on a cohort of 129 very low-birth-weight infants. Thermal gradient alteration was defined as a difference of > 2°C maintained during 4 hours. We then determined its association with the late-onset sepsis variable through logistic regression. RESULTS: We enrolled 129 preterm babies in 52 months. Thermal gradient alterations showed an adjusted odds ratio for late-onset sepsis of 23.60 (95% confidence interval [CI], 6.80-81.88), with a sensitivity of 83% and negative predictive value of 94%. In 71% of cases, thermal gradient alteration was the first clinical sign of sepsis, while C-reactive protein was < 1.5 mg/dL in 64% of cases and procalcitonin < 2 ng/mL in 36%. These figures indicate potential for early diagnosis. CONCLUSIONS: Sustained increases of central-peripheral temperature differences are an early sign of evolving late-onset sepsis.

Validación de un cuestionario en castellano sobre conocimientos de asma en profesores / Validation of a Questionnaire in Spanish on Asthma Knowledge in Teachers

Objetivo: Evaluar la fiabilidad y la validez de la versión en castellano del cuestionario NewCastle Asthma Knowledge Questionnaire (NAKQ) para la determinación de conocimientos sobre asma en profesores. Métodos: Diseño y muestra: estudio observacional, transversal, en centros educativos de A Coruña con segundo ciclo de educación infantil, primaria y/o secundaria obligatoria. Los centros se seleccionaron mediante muestreo aleatorizado, estratificado según titularidad y nivel educativo (24 centros, 864 profesores). Contestaron 537 (62,1%) docentes (precisión, ± 4%, seguridad, 95%). Mediciones: Edad, sexo, formación académica, experiencia docente, antecedentes personales/familiares de asma, cuestionario NAKQ. Análisis: Evaluación de consistencia interna (alfa de Cronbach). La validez concurrente se determinó comparando la puntuación en docentes asmáticos o con familiares asmáticos y docentes sin contacto con el asma. Se evaluó la fiabilidad test-retest en 2 centros seleccionados aleatoriamente, mediante índice Kappa, metodología de Bland-Altman y el coeficiente de correlación intraclase. Resultados: La puntuación media del cuestionario NAKQ fue 15,7 ± 5,3 (mediana 17), contestando correctamente el 50,6% de ítems. El coeficiente alfa de Cronbach fue 0,824 (IC 95%: 0,802-0,845). La puntuación NAKQ fue mayor en asmáticos o con familiares próximos asmáticos (17,7 ± 3,3) que en docentes con familiares lejanos asmáticos (16,1 ± 5,4) y docentes sin contacto próximo con el asma (15,1 ± 5,6; p < 0,001). En el análisis test-retest (Kappa 0,33 a 1) no hubo diferencias entre la puntuación NAKQ en la primera y la segunda cumplimentación (diferencia media, 0,3 ± 2,3; coeficiente de correlación intraclase, 0,863). Conclusiones: Las puntuaciones obtenidas con la versión española del NAKQ en profesores de centros escolares españoles son fiables y válidas para medir su grado de conocimiento de asma

Objective: To evaluate the reliability and validity of the Spanish version of the NewCastle Asthma Knowledge Questionnaire (NAKQ) for determining asthma knowledge in teachers. Methods: Design and sample: A cross-sectional observational study in educational centers of A Coruna˜ providing preschool, primary school and/or compulsory secondary education. Centers were selected by random sampling, stratified by ownership and educational level (24 centers, 864 teachers). A total of 537 (62.1%) teachers responded (precision, ± 4%, confidence, 95%). Measurements: Age, sex, academic training, teaching experience, personal/family history of asthma, NAKQ. Analysis: Evaluation of internal consistency (Cronbach’s alpha). Concurrent validity was determined by comparing scores of asthmatic teachers or with asthmatic relatives with teachers with no contact with asthma. Test-retest reliability was evaluated in two randomly selected centers by the kappa index, BlandAltman method and intraclass correlation coefficient. Results: Mean score on the NAKQ was 15.7 ± 5.3 (median 17), correctly answering 50.6% of items. Cronbach’s alpha coefficient was 0.824 (95% CI: 0.802-0.845). NAKQ score washigher inasthmatic teachers or with close asthmatic relatives (17.7 ± 3.3) than in teachers with distant asthmatic relatives (16.1 ± 5.4) and teachers without close contact with asthma (15.1 ± 5.6; P < 0.001). In the test-retest analysis (kappa 0.33-1), there were no differences in NAKQ score between the first and second completion (mean difference, 0.3 ± 2.3; intraclass correlation coefficient, 0.863). Conclusions: Scores obtained with the Spanish version of the NAKQ in teachers of Spanish school centers are reliable and valid to measure their degree of asthma knowledge

Validation of a questionnaire in Spanish on asthma knowledge in teachers.

OBJECTIVE: To evaluate the reliability and validity of the Spanish version of the NewCastle Asthma Knowledge Questionnaire (NAKQ) for determining asthma knowledge in teachers. DESIGN AND SAMPLE: A cross-sectional observational study in educational centers of A Coruña providing preschool, primary school and/or compulsory secondary education. Centers were selected by random sampling, stratified by ownership and educational level (24centers, 864teachers). A total of 537 (62.1%) teachers responded (precision, ±4%, confidence, 95%). MEASUREMENTS: Age, sex, academic training, teaching experience, personal/family history of asthma, NAKQ. ANALYSIS: Evaluation of internal consistency (Cronbach's alpha). Concurrent validity was determined by comparing scores of asthmatic teachers or with asthmatic relatives with teachers with no contact with asthma. Test-retest reliability was evaluated in two randomly selected centers by the kappa index, Bland-Altman method and intraclass correlation coefficient. RESULTS: Mean score on the NAKQ was 15.7±5.3 (median 17), correctly answering 50.6% of items. Cronbach's alpha coefficient was 0.824 (95%CI: 0.802-0.845). NAKQ score was higher in asthmatic teachers or with close asthmatic relatives (17.7±3.3) than in teachers with distant asthmatic relatives (16.1±5.4) and teachers without close contact with asthma (15.1±5.6; P<0.001). In the test-retest analysis (kappa 0.33-1), there were no differences in NAKQ score between the first and second completion (mean difference, 0.3±2.3; intraclass correlation coefficient, 0.863). CONCLUSIONS: Scores obtained with the Spanish version of the NAKQ in teachers of Spanish school centers are reliable and valid to measure their degree of asthma knowledge.

The impact of atmospheric pollutants on the prevalence of atopic eczema in 6-7-year-old schoolchildren in Spain; ISAAC Phase III.

Atopic Eczema (AE) is a chronic inflammatory skin disease that affects children and adults, and alters quality of life with a high morbidity rate and severe economic burden. The objective of the present work was to analyse specific atmospheric pollutants (O3, NO, PM10 and SO2) affecting the prevalence of diagnosed AE and its symptoms among 6-7-year-old schoolchildren.The participants included 21311 schoolchildren aged 6-7 years from 8 Spanish regions, whose parents completed the ISAAC Phase III questionnaire to ascertain AE diagnosis and symptoms. The mean levels (µg/m3) of O3, NO, PM10 (particles 10 micrometers or less in diameter) and SO2 were determined in each geographical area. Participating in this study.According to these mean levels, three levels of exposure to each pollutant were considered: level 1 (percentiles 0-25); level 2 (percentiles 26-74); level 3 (percentiles 75-100). Exposure to O3 was associated with increased prevalence of rashes (exposure level 2, Odds Ratio (OR): 1.22, 95% Confidence Interval (95%CI): 1.02-1.45; level 3 OR: 1.33, 95%CI: 1.10-1.61) and diagnosed AE (level 2, OR: 1.27, 95%CI: 1.17-1.39; level 3 OR: 1.27, 95%CI: 1.15-1.41). An association was found between the level of NO and a drop in the prevalence of diagnosed AE (exposure level 2, OR: 0.88, 95%CI: 0.81-0.95; level 3 OR: 0.85, 95%CI: 0.74-0.97). There was also an association between the highest exposure level to PM10 and a reduced prevalence of rashes (level 3 OR: 0.42, 95%CI: 0.22-0.81) and diagnosed AE (level 3 OR: 0.53, 95%CI: 0.38-0.75). Future studies into exposure to O3 and its relationship with allergic diseases may be conducted in order to prevent this association.

The association between acetaminophen and asthma: should its pediatric use be banned?

During the last few decades, a huge epidemiological effort has been made all over the world in order to cast some light on the origin of asthma (or 'wheezing disorders' as a general term) and its recent increase in prevalence. The focus on genetic factors has failed to show any genetic signal strong enough to be seriously considered, and the tiny genetic signals found have never been appropriately replicated. The focus on environmental factors has provided some variable signals on the role of infections, allergens and bacterial substances, the direction of which have curiously varied from protecting to inducing asthma. The only environmental factor that has launched a large and consistent epidemiological signal, found in almost every epidemiological study addressing the issue, is previous acetaminophen exposure, which consistently increases the prevalence and clinical manifestations of every wheezing disorder under study. Is acetaminophen a real asthma promoter or an innocent bystander?

Atopic dermatitis and indoor use of energy sources in cooking and heating appliances.

BACKGROUND: Atopic dermatitis (AD) prevalence has considerably increased worldwide in recent years. Studying indoor environments is particularly relevant, especially in industrialised countries where many people spend 80% of their time at home, particularly children. This study is aimed to identify the potential association between AD and the energy source (biomass, gas and electricity) used for cooking and domestic heating in a Spanish schoolchildren population. METHODS: As part of the ISAAC (International Study of Asthma and Allergies in Childhood) phase III study, a cross-sectional population-based survey was conducted with 21,355 6-to-7-year-old children from 8 Spanish ISAAC centres. AD prevalence, environmental risk factors and the use of domestic heating/cooking devices were assessed using the validated ISAAC questionnaire. Crude and adjusted odds ratios (cOR, aOR) and 95% confidence intervals (CIs) were obtained. A logistic regression analysis was performed (Chi-square test, p-value < 0.05). RESULTS: It was found that the use of biomass systems gave the highest cORs, but only electric cookers showed a significant cOR of 1.14 (95% CI: 1.01-1.27). When the geographical area and the mother's educational level were included in the logistic model, the obtained aOR values differed moderately from the initial cORs. Electric heating was the only type which obtained a significant aOR (1.13; 95% CI: 1.00-1.27). Finally, the model with all selected confounding variables (sex, BMI, number of siblings, mother's educational level, smoking habits of parents, truck traffic and geographical area), showed aOR values which were very similar to those obtained in the previous adjusted logistic analysis. None of the results was statistically significant, but the use of electric heating showed an aOR close to significance (1.14; 95% CI: 0.99-1.31). CONCLUSION: In our study population, no statistically significant associations were found between the type of indoor energy sources used and the presence of AD.

Central-peripheral temperature gradient: an early diagnostic sign of late-onset neonatal sepsis in very low birth weight infants.

AIMS: We assessed central-peripheral temperature gradient alteration for the diagnosis of late-onset neonatal sepsis and compared earliness detection of this sign with altered blood cell count and C-reactive protein. METHOD: Thirty-one preterm babies (<1500 g or <32 weeks) participated in an observational prospective study. Axillary (central) and sole (peripheral) temperatures were continuously monitored with a thermal probe (ThermoTracer; Dräger Medical AGF & Co. KgaA, Lübeck, Germany) adjusting incubator air temperature for a thermal gradient <1.5°C. Central-peripheral temperature alteration was defined as a thermal gradient >2°C that could not be corrected with protocolized air temperature modifications. Proven (positive blood culture) sepsis and probable late-onset sepsis were recorded. RESULTS: Late-onset sepsis was diagnosed in 11 neonates (proven, 9; probable, 2). Thermal gradient alteration was present in 12 cases, in association with the onset of sepsis in 10 and concomitantly with a ductus arteriosus and stage 1 necrotizing enterocolitis in 2. Thermal gradient alteration had a sensitivity of 90.9% [95% confidence interval (CI), 62.3-98.4] and specificity of 90% (95% CI, 69.9-97.2%), and in 80% of cases, it occurred before abnormal laboratory findings. CONCLUSIONS: Central-peripheral temperature gradient monitoring is a feasible, non-invasive, and simple tool easily applicable in daily practice. An increase of >2°C showed a high-sensitivity and specificity for the diagnosis of late-onset sepsis.

Screening for symptoms of obstructive sleep apnea in children with severe craniofacial anomalies: assessment in a multidisciplinary unit.

OBJECTIVE: To assess the incidence of airway obstruction symptoms and the presence of obstructive sleep apnea in children with severe craniofacial anomalies by a proactive screening program using a standard questionnaire and cardiorespiratory polygraphy. PATIENTS AND METHODS: Children with severe craniofacial anomalies referred to our paediatric airway unit from February 2001 to June 2011 were eligible to be included in this retrospective, single centre study. Symptoms of airway obstruction were proactively investigated using the shorter version of the Pediatric Sleep Questionnaire (PSQ). Obstructive sleep apnea was assessed by means of cardiorespiratory polygraphy. Demographic data and reason for referral were also recorded. Primary outcomes were the prevalence of symptoms of airway obstruction and OSA. RESULTS: 44 children (24 girls) with severe craniofacial anomalies (15 Crouzon, 13 Apert, 9 Goldenhar, 5 Treacher-Collins, 2 Pfeiffer) were included, at a mean age of 5 years (range 8 months to 14 years). Reason for referral was routine follow up in 30 patients and overt OSA symptoms and signs in the remaining 14. PSQ results showed symptoms of airway obstruction in 82% of patients, being snoring the most frequent symptom (64.1%) followed by apneas (33.3%). Polygraphic studies showed inconclusive results in 8 children (18.2%), normal apnea-hypopnea index (AHI) in 16 (36.4%), mild obstructive sleep apnea in 9 (20.4%), moderate in 4 (9.1%) and severe obstructive sleep apnea in 7 (15.9%). CONCLUSIONS: Children with craniofacial anomalies have a high prevalence of symptoms of airway obstruction and obstructive sleep apnea that support a proactive screening strategy in this highly selected population.

The role of bronchoscopy in the management of patients with severe craniofacial syndromes.

PURPOSE: The purpose of this study is to assess the incidence of airway anomalies in children with severe craniofacial syndromes and to establish the role of bronchoscopy in the care of these patients. METHODS: Consecutive children with craniofacial syndromes, including both bony deformities of the skull and face, in which a bronchoscopy was performed between 1995 and 2010 were retrospectively reviewed. RESULTS: Thirty-six patients (22 boys, 14 girls; mean age, 39 months) were studied. Craniofacial synostosis was present in 21 patients (Crouzen syndrome, 11; Apert syndrome, 7, Pfeiffer syndrome, 3) and craniofacial dysostosis in 15 (Goldenhart syndrome, 8; Treacher Collins syndrome, 7). In 30 patients (83.3%), bronchoscopy was performed because of respiratory symptoms (apneic episodes, 22; respiratory distress, 13; stridor, 6; cyanosis, 1) and, in the remaining 6 (asymptomatic children), during guided tracheal intubation before a surgical procedure. Airway anomalies were found in 69.4% of patients (70% in symptomatic patients). Management consisted of tracheotomy in 13 patients, adenoidectomy/tonsillectomy in 13, glossopexy in 5, antireflux surgery in 3, and supraglottoplasty in 2. CONCLUSIONS: Airway anomalies occurred in 70% of children with severe craniofacial syndromes and respiratory symptoms. Bronchoscopy should be performed routinely in this selected group of patients, and the entire airway must be examined. Treatment should be tailored to each individual patient.

[Prevalence of atopic eczema and nutritional factors in 6-7 year old children]. / Prevalencia de dermatitis atópica y factores nutricionales en niños de 6-7 años.

OBJECTIVES: To describe the prevalence of atopic eczema (AE) and to find the characteristics of the diet of children with AE. DESIGN: Descriptive, transversal study. SETTING: Primary schools. MAIN MEASUREMENTS: The study used the standardized and validated questionnaire of the ISAAC phase III study, which finds the prevalence of AE in children and enables inter-centre comparison to be conducted. A questionnaire about food consumption was included. The prevalence ratio (PR) of AE and food consumption (95% confidence intervals) was calculated. RESULTS: A total of 28 448 children aged 6-7 were included in the study. The prevalence of clinically diagnosed AE was 15.35% in boys and 15.24% in girls. The foods most often consumed were: dairy and cereals, followed by rice, pasta, and eggs. The least consumed were: butter, margarine, and fast food. The PR for children who consumed pasta was (PR=1.35), seafood (PR=1.28), cereals (PR=1.26), eggs (PR=1.13), and meat (PR=1.09). CONCLUSIONS: The prevalence of AE in Spain was 15.3%, and was associated with food consumption. The consumption of dairy products, cereals, and olive oil was appropriate. The intake of fruit, vegetables, seafood, and legumes was below the recommended amounts. Future studies on the question are needed.

Prevalencia de dermatitis atópica y factores nutricionales en niños de 6-7 años / Prevalence of atopic eczema and nutritional factors in 6-7 year old children

Objetivo. Describir la prevalencia de dermatitis atópica (DA) y conocer las características de la dieta en niños con DA. Diseño. Estudio descriptivo, transversal. Emplazamiento. Centros escolares de educación primaria. Mediciones principales. Se realizó un estudio mediante la utilización del cuestionario validado y estandarizado del estudio ISAAC de fase III, que permite conocer la prevalencia de DA en niños y compararla entre centros. Se acompañó de una encuesta sobre consumo de alimentos. Se calcularon la razón de prevalencia (RP) de DA en relación con el consumo de alimentos y los intervalos de confianza del 95%. Resultados. Se incluyó en el estudio a un total de 28.448 niños de 6-7 años. La prevalencia de DA clínicamente diagnosticada fue del 15,35% en niños y del 15,24% en niñas. Los alimentos más consumidos fueron la leche y los cereales, seguidos del arroz, la pasta y los huevos. Los alimentos menos consumidos fueron la mantequilla, la margarina y la comida rápida. Se observó una razón de prevalencia de DA en los niños que consumían pasta (RP = 1,35), pescado (RP = 1,28), cereales (RP = 1,26), huevos (RP = 1,13) y carne (RP = 1,09). Conclusiones. La prevalencia de DA en España fue del 15,3% asociada con el consumo de alimentos. El consumo de lácteos, cereales y aceite de oliva es adecuado. La ingesta de fruta, verdura, pescado y legumbres es inferior a la aconsejada. Sería necesario realizar futuros estudios sobre el tema

Objectives. To describe the prevalence of atopic eczema (AE) and to find the characteristics of the diet of children with AE. Design. Descriptive, transversal study. Setting. Primary schools. Main measurements. The study used the standardized and validated questionnaire of the ISAAC phase III study, which finds the prevalence of AE in children and enables inter-centre comparison to be conducted. A questionnaire about food consumption was included. The prevalence ratio (PR) of AE and food consumption (95% confidence intervals) was calculated. Results. A total of 28 448 children aged 6-7 were included in the study. The prevalence of clinically diagnosed AE was 15.35% in boys and 15.24% in girls. The foods most often consumed were: dairy and cereals, followed by rice, pasta, and eggs. The least consumed were: butter, margarine, and fast food. The PR for children who consumed pasta was (PR=1.35), seafood (PR=1.28), cereals (PR=1.26), eggs (PR=1.13), and meat (PR=1.09). Conclusions. The prevalence of AE in Spain was 15.3%, and was associated with food consumption. The consumption of dairy products, cereals, and olive oil was appropriate. The intake of fruit, vegetables, seafood, and legumes was below the recommended amounts. Future studies on the question are needed

[Time trends in prevalence of symptoms of allergic rhinitis in 13-14 year-old schoolchildren in 8 areas of Spain between 1993-1994 and 2001-2002 according to the International Study of Asthma and Allergies in Childhood (ISAAC)]. / Evolución temporal de la prevalencia de síntomas de rinitis alérgica en escolares de 13-14 años de 8 áreas españolas entre 1993-1994 y 2001-2002 según el Estudio Internacional sobre Asma y Alergias en la Infancia (ISAAC).

BACKGROUND AND OBJECTIVE: Our goal was to estimate the time trends in the prevalence of symptoms of allergic rhinitis (AR) and its geographic variations. POPULATION AND METHOD: During 2001-2002, the phase 3 of ISAAC study was carried out, and a comparison with the phase 1 (1993-1994) was done. This cross sectional study included 30,046 schoolchildren 13-14 years-old from 9 centers (Asturias, Barcelona, Bilbao, Cartagena, Castellon, Madrid, Pamplona, Valencia, and Valladolid). A questionnaire about rhinitis symptoms self-administered to schoolchildren was used at schools and institutes with a participation rate of 100% to 75.8%. RESULTS: A divergent time trend of AR prevalence symptoms was observed with a decrease in Barcelona and Bilbao, a stabilization in Cartagena, Pamplona, and Valencia, and an increase in Castellon; Madrid and Valladolid; however, there was an overall increase in nasal allergy. The prevalence of rhinoconjunctivitis in the past year (Prevalence Ratio [PR] = 1,13; 95% Confidence Interval [CI], 1.07-1.19) and nasal allergy ever (PR = 1.22; 95% CI, 1.15-1.30) increased after adjusting for gender, center and season change. The prevalence of rhinoconjunctivitis and nasal allergy varied according to centres, Madrid, Asturias, Cartagena and Bilbao exhibiting the higher prevalence. Taken as a whole, these data indicate a middle prevalence of AR when compared with other countries following an ISAAC methodology. CONCLUSIONS: Two patterns of time trend of prevalence of symptoms of AR were observed. The investigation of risk factors at local level could be important in order to undertake preventive measures.

[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.