Reliability of blood eosinophil count in steady-state bronchiectasis.

RATIONALE: The baseline value of eosinophils in peripheral blood (BEC) has been associated with different degrees of severity, prognosis and response to treatment in patients with bronchiectasis. It is not known, however, if this basal value remains constant over time. OBJECTIVES: The aim of this study was to assess whether the BEC remains stable in the long term in patients with bronchiectasis. METHODS AND MEASUREMENTS: Patients from the RIBRON registry of bronchiectasis diagnosed by computed tomography with at least 2 BEC measurements one year apart were included in the study. Patients with asthma and those taking anti-eosinophilic drugs were excluded. Reliability was assessed using the intra-class correlation coefficient (ICC). A patient with a BEC of at least 300 cells/uL or less than 100 cells/uL was considered eosinophilic or eosinopenic, respectively. Group changes over time were also calculated. MAIN RESULTS: Seven hundred and thirteen patients were finally included, with a mean age of 66.5 (13.2) years (65.8 % women). A total of 2701 BEC measurements were performed, with a median number of measurements per patient of 4 (IQR: 2-5) separated by a median of 12.1 (IQR: 10.5-14.3) months between two consecutive measurements. The ICC was good (>0.75) when calculated between two consecutive measurements (approximately one year apart) but had dropped significantly by the time of the next annual measurements. Similarly, the change from an eosinophilic or eosinopenic patient to a non-eosinophilic or non-eosinopenic patient, respectively, was less than 30 % during the first year with respect to the baseline value but was close to 50 % in later measurements. CONCLUSIONS: Given the significant changes observed in the baseline value of the BEC over time, its monitoring is necessary in patients with bronchiectasis in order to more reliably assess its usefulness.

Pseudomonas aeruginosa and lung function decline in patients with bronchiectasis.

OBJECTIVES: The objective of this study was to analyse lung function decline over time in bronchiectasis, along with the factors associated with it. METHODS: Spirometry was measured every year in this observational, prospective study in 849 patients from the Spanish Bronchiectasis Registry (RIBRON). The main outcome was the decline in the rate of forced expiratory volume during the first second (FEV1). To be included in this study, patients needed a baseline assessment and at least one subsequent assessment. FEV1 decline was analysed using a mixed-effects linear regression model adjusted for clinically significant variables. RESULTS: We recruited 849 bronchiectasis patients with at least two annual lung function measurements (follow-up range 1-4 years). A total of 2262 lung function tests were performed (mean 2.66 per patient, range 2-5). Mean baseline FEV1 was 1.78 L (standard deviation (SD) 0.76; 71.3% predicted). Mean age was 69.1 (SD 15.4) years; 543 (64% women. The adjusted rates of FEV1 decline were -0.98% predicted/year (95% confidence interval (CI) -2.41 to -0.69) and -31.6 (95% CI -44.4 to -18.8) mL. The annual FEV1 decline was faster in those patients with chronic bronchial infection by Pseudomonas aeruginosa (-1.37% (52.1 mL) vs -0.37% (-24.6 mL); p < 0.001), greater age, increased number of severe exacerbations in the previous year and higher baseline FEV1 value. DISCUSSION: In patients with bronchiectasis, the annual rate of FEV1 decline was -31.6 mL/year and it was faster in older patients and those with chronic bronchial infection by P. aeruginosa, increased number of previous severe exacerbations and higher baseline FEV1 value.

How are the ancient cystic fibrosis patients? Cystic fibrosis diagnosed over 60 years-old.

BACKGROUND AND AIMS: To specify the prevalence of patients diagnosed with CF at age of ≥60 year-old and to analyze their characteristics. PATIENTS AND METHODS: Observational study of CF patients which were diagnosed at age ≥60 year-old. The analyzed variables were: age, sex, nationality, lung function parameters, conditions present at diagnosis, microbiological characteristics and genetic findings. RESULTS: eight patients were included. 7 patients were female (87.5%) with a mean age of 70.6 years (median 71.5 years, range 60-78 years). The most important findings were: sweat test >60 mEq/l; heterozygotes F508del; bronchiectasis in CT; methicillin-sensitive Staphylococcus aureus (50%) in sputum. The most patients presented a normal or mild obstructive lung function. CONCLUSIONS: CF must also be considered a disease diagnosed in adulthood, incorporating the sweat test within the usual techniques of differential diagnosis in patients with different diseases associated with CF, because genetic counselling is esencial.

Depression and anxiety symptoms in Spanish adult patients with cystic fibrosis: associations with health-related quality of life.

BACKGROUND: Cystic fibrosis (CF) is a chronic disease with an impact on the quality of life. Self-reported symptoms of depression and anxiety were assessed in the Spanish cohort of the International Epidemiological Study on Depression and Anxiety in patients with CF (International Depression-Anxiety Epidemiological Study) and their relationship with health status and health-related quality of life (HRQoL) was evaluated. METHODS: This cross-sectional study recruited adult patients with CF at 10 Spanish centers. Patients completed the Hospital Anxiety and Depression Scale (HADS) and the Revised Cystic Fibrosis Questionnaire. Demographic and health data were recorded from medical charts. Logistic regression was used to determine the predictors of elevated symptoms of depression and anxiety (HADS≥8). RESULTS: Of the 336 participants recruited (mean age, 28.1years; 48.2% women), 41 (12.2%) had elevated depression-related scores, and 100 (29.7%) had elevated anxiety-related scores (HADS≥8). After adjusting for confounders, only less education, intravenous antibiotics, psychiatric medications and psychotherapy were significantly associated with elevated psychological symptoms. Specifically, regardless of lung function, patients who were depressed or anxious reported worse HRQoL. CONCLUSIONS: The prevalence of elevated symptoms of depression and anxiety was high in Spanish adult patients with CF, and these symptoms were associated with a decreased HRQoL.

Fibrosis quística / No disponible

No disponible

Eradication of Burkholderia cepacia Using Inhaled Aztreonam Lysine in Two Patients with Bronchiectasis.

There are not many articles about the chronic bronchial infection/colonization in patients with underlying lung disease other than cystic fibrosis (CF), especially with non-CF bronchiectasis (NCFBQ). The prevalence of B. cepacia complex is not well known in NCFBQ. The vast majority of published clinical data on Burkholderia infection in individuals with CF is comprised of uncontrolled, anecdotal, and/or single center experiences, and no consensus has emerged regarding treatment. We present two cases diagnosed with bronchiectasis (BQ) of different etiology, with early pulmonary infection by B. cepacia complex, which was eradicated with inhaled aztreonam lysine.

Fibrosis quística diagnosticada en edad adulta / Cystic fibrosis in adult age

Objetivo. Precisar la prevalencia de enfermos con fibrosis quística (FQ) diagnosticados en edades superiores a los 18 años y analizar sus características clínicas, genéticas y microbiológicas. Pacientes y métodos. Estudio observacional, transversal y descriptivo de todos los pacientes diagnosticados de FQ a edad igual o superior a los 18 años. Las variables analizadas fueron: edad actual, edad al diagnóstico, sexo, nacionalidad, parámetros de función pulmonar, patologías presentes al momento del diagnóstico, características microbiológicas y hallazgos genéticos. Resultados. Se incluyeron 89 pacientes (14,8%, del total de 600 pacientes en seguimiento en las unidades participantes), 45 mujeres (50,6%) y 44 varones (49,4%), con una edad media al diagnóstico de 36,4 años. Ochenta y un pacientes (91%) eran de nacionalidad española. La prueba del sudor fue diagnóstica en 77 de los 89 estudiados (86,5%). Las mutaciones detectadas con mayor frecuencia fueron la F508del/otra y la G542X/otra y los hallazgos clínicos más frecuentes en el momento del diagnóstico fueron las bronquiectasias en 33 pacientes (37,1%) y la esterilidad en 12 (13,5%). Los microorganismos colonizadores más frecuentes fueron Staphylococcus aureus (S.aureus) sensible a meticilina (23,6%) y Pseudomonas aeruginosa (P. aeruginosa) (13,5%). La mayoría de los pacientes presentaban una alteración ventilatoria obstructiva leve y no tenía afectación pancreática. La prueba del sudor con frecuencia ofreció resultados no concluyentes. Conclusiones. La FQ es también una enfermedad de diagnóstico en la edad adulta. Los pacientes diagnosticados en edad adulta presentan una función pulmonar levemente alterada y una baja incidencia de afectación pancreática, por lo que su pronóstico tiende a ser favorable (AU)

Aim. To know the prevalence of the patients diagnosed of cystic fibrosis (CF) older than 18 years old of five specific Spanish Units and to analyze their clinical, genetic and microbiological characteristics. Patients and methods. Observational, cross-sectional, descriptive study of patients diagnosed with CF at age or older than 18 years. The variables analyzed were: current age, age at diagnosis, sex, nationality, lung function parameters, pathologies presented at diagnosis, microbiological features and genetic findings. Results. Eigthy nine patients (14.8% of the total of 600 CF patients followed at the participating units), of which 45 patients were female (50.6%) and 44 were males (49.4%), were included with a mean age at diagnosis of 36.4 years. Eigthy one patients (91%) were Spaniards. The sweat test was diagnostic in 77 (86.5%) of the patients studied. The sweat test was diagnostic in 77 of the 89 patients studied (86.5%). The most frequently detected mutations were F508del/other and G542X/other, and the most frequent clinical findings at diagnosis were the presence of bronchiectasis in 33 patients (37.1%) followed by sterility in 12 patients (13.5%). The most common colonizing organisms were meticillin-sensitive Staphylococcus aureus (S.aureus) (23.6%) and Pseudomonas aeruginosa (P. aeruginosa) (13.5%). Most patients presented a mild obstructive ventilatory defect and had no pancreatic involvement. The sweat test used to be indeterminate. Conclusions. CF is also a disease which diagnosis can be in adulthood. CF patients diagnosed in adulthood have a mild lung function and lower incidence of pancreatic involvement, so their prognosis tends to be favorable (AU)

Cystic fibrosis in adult age. / Fibrosis quística diagnosticada en edad adulta.

AIM: To know the prevalence of the patients diagnosed of cystic fibrosis (CF) older than 18 years old of five specific Spanish Units and to analyze their clinical, genetic and microbiological characteristics. PATIENTS AND METHODS: Observational, cross-sectional, descriptive study of patients diagnosed with CF at age or older than 18 years. The variables analyzed were: current age, age at diagnosis, sex, nationality, lung function parameters, pathologies presented at diagnosis, microbiological features and genetic findings. RESULTS: Eigthy nine patients (14.8% of the total of 600 CF patients followed at the participating units), of which 45 patients were female (50.6%) and 44 were males (49.4%), were included with a mean age at diagnosis of 36.4 years. Eigthy one patients (91%) were Spaniards. The sweat test was diagnostic in 77 (86.5%) of the patients studied. The sweat test was diagnostic in 77 of the 89 patients studied (86.5%). The most frequently detected mutations were F508del/other and G542X/other, and the most frequent clinical findings at diagnosis were the presence of bronchiectasis in 33 patients (37.1%) followed by sterility in 12 patients (13.5%). The most common colonizing organisms were meticillin-sensitive Staphylococcus aureus (S.aureus) (23.6%) and Pseudomonas aeruginosa (P. aeruginosa) (13.5%). Most patients presented a mild obstructive ventilatory defect and had no pancreatic involvement. The sweat test used to be indeterminate. CONCLUSIONS: CF is also a disease which diagnosis can be in adulthood. CF patients diagnosed in adulthood have a mild lung function and lower incidence of pancreatic involvement, so their prognosis tends to be favorable.

Infección por micobacterias no tuberculosas en pacientes con bronquiectasias no causadas por fibrosis quística / Non-tuberculous mycobacterial infection in patients with non-cystic fibrosis bronchiectasias

Objetivo. Conocer la prevalencia y características de la infección por micobacterias no tuberculosas en enfermos con bronquiectasias no relacionadas con la fibrosis quística. Pacientes y métodos. Estudio descriptivo retrospectivo de pacientes adultos con bronquiectasias no relacionadas con la fibrosis quística con un seguimiento de al menos dos años. Resultados. Se incluyeron 68 pacientes, 50 mujeres (73,5%), con una edad media de 63,31±16,2 años. La etiología más frecuente fue la enfermedad pulmonar obstructiva crónica (28 pacientes, 41,2%) con una afectación leve-moderada y colonización por Pseudomonas aeruginosa (P. aeruginosa) (70,6%). Siete pacientes (10,3%) presentaron infección por micobacterias no tuberculosas, tratándose de Mycobacterium avium complex en 6 ocasiones (7,35%). Cuatro (57,14%) pacientes recibieron tratamiento antimicrobiano específico. En los enfermos infectados fue menor la frecuencia de P. aeruginosa y el uso de corticoides inhalados. No hubo diferencias espirométricas significativas entre los pacientes infectados por micobacterias no tuberculosas y los no infectados. Conclusiones. Las bronquiectasias no relacionadas con la fibrosis quística podrían considerarse un factor de riesgo para la infección por micobacterias no tuberculosas(AU)

Aims. To know the characteristics and prevalence of non-tuberculous mycobacterial infection infection in patients with non-cystic fibrosis bronchiectasis. Patients and methods. A retrospective descriptive study of NCFB adult patients whose disease had been followed-up for at least two years was performed. Results. A total of 68 subjects were included, 50 females (73.5%), with mean age of 63.31± 16.2 years. The most frequent etiology of the non-cystic fibrosis bronchiectasis was COPD in 28 cases (41.2%) with a light-moderate pulmonary involvement and Pseudomonas aeruginosa (P. aeruginosa) colonization (70.6%). Seven patients (10.3%) had MNT infection, six of whom had Mycobacterium avium complex (7.35%). Four patients (57.14%) were treated. In the infected patients, P. aeruginosa and the use of inhaled steroids were observed with less frequency. There were no significant differences between the infected and non-infected patients in relation to spirometric values. Conclusions. The non-cystic fibrosis bronchiectasis could be considered a risk factor for non-tuberculous mycobacterial infection(AU)

[Non-tuberculous mycobacterial infection in patients with non-cystic fibrosis bronchiectasias]. / Infección por micobacterias no tuberculosas en pacientes con bronquiectasias no causadas por fibrosis quística.

AIMS: To know the characteristics and prevalence of non-tuberculous mycobacterial infection infection in patients with non-cystic fibrosis bronchiectasis. PATIENTS AND METHODS: A retrospective descriptive study of NCFB adult patients whose disease had been followed-up for at least two years was performed. RESULTS: A total of 68 subjects were included, 50 females (73.5%), with mean age of 63.31± 16.2 years. The most frequent etiology of the non-cystic fibrosis bronchiectasis was COPD in 28 cases (41.2%) with a light-moderate pulmonary involvement and Pseudomonas aeruginosa (P. aeruginosa) colonization (70.6%). Seven patients (10.3%) had MNT infection, six of whom had Mycobacterium avium complex (7.35%). Four patients (57.14%) were treated. In the infected patients, P. aeruginosa and the use of inhaled steroids were observed with less frequency. There were no significant differences between the infected and non-infected patients in relation to spirometric values. CONCLUSIONS: The non-cystic fibrosis bronchiectasis could be considered a risk factor for non-tuberculous mycobacterial infection.

¿Qué saben los pacientes adultos con fibrosis quística de su enfermedad? Adherencia al tratamiento / What do patients suffering cystic fibrosis know about their disease?

Introducción: La fibrosis quística (FQ) ha aumentado la supervivencia debido al avance del tratamiento multidisciplinar. Nos hemos propuesto averiguar si los pacientes de una unidad de adultos con FQ cumplen su tratamiento habitual, así como observar qué nivel de conocimiento tienen sobre su enfermedad, sus terapias y sus implicaciones. Material y métodos: Hemos realizado un estudio descriptivo transversal, con variable cualitativo ordinal y ciego simple, en una Unidad de adultos con FQ que contestaron un cuestionario donde se preguntó sobre el tratamiento de la enfermedad y su administración, hábitos tóxicos, conocimiento de su enfermedad y preocupación por su presente o futuro. Resultados: El cuestionario fue contestado por 35 pacientes (40%) durante los años 2000-2001, lo que suponía el 40% del total de enfermos, con una edad media de 31,43 ± 8,49 años. Un 85,7% (30 pacientes) contestaron saber qué tratamiento llevaban y lo anotaron correctamente. Un total de 24 pacientes (68,57%) precisaban enzimas pancreáticas. Al preguntar sobre el conocimiento de la enfermedad, el 94,3% (33 pacientes) se consideraba informado sobre la misma y el 91,4% (32 pacientes) dijo que la información dada por sus médicos era adecuada. Lo que más les preocupaba era el empeoramiento de su calidad de vida, 14,3% (5 pacientes), la preocupación por el futuro, 11,4% (4 pacientes), las limitaciones en la vida diaria, 8,6% (3 pacientes) y su mortalidad o tener recaídas, 2,9% (1 paciente). No respondieron a esta pregunta un 11,4% (4 pacientes). Conclusiones: la mayoría de los enfermos que contestaron la encuesta están enterados de su enfermedad y de su tratamiento y tiene claras sus preocupaciones por el futuro. Sin embargo, contestaron menos de la mitad del total de los pacientes, lo que indica que se deberían indagar las causas de este problema (AU)

Nueva formulación de tobramicina inhalada en polvo seco para la colonización crónica por Pseudomonas aeruginosa en fibrosis quística / A new inhaled dry-powder formulation of tobramycin for chronic colonization by pseudomonas aeruginosa in cystic fibrosis

La sobreinfección por Pseudomonas aeruginosa (PA) es frecuente en los pacientes con fibrosis quística (FQ). El tratamiento antibiótico por via inhalatoria es el más recomendado (..) (AU)

Superinfection with Pseudomonas aeruginosa (PA) is common in patients with cystic (..) (AU)

Chronic hepatitis C treatment in a cystic fibrosis patient in the pulmonary pre-transplant stage.

The standard treatment of chronic hepatitis C, pegylated interferon and ribavirin (pegI/R), has many limitations in both effectiveness and secondary effects, which makes it unsuitable or even contraindicated for some patients. In hepatitis C virus-infected cystic fibrosis patients this treatment could increase respiratory infections with subsequent pulmonary function deterioration. On the contrary, hepatitis C virus (HCV) infection may make lung transplant (LT) unfeasible. We present the case of a cystic fibrosis-young man diagnosed with HCV infection during LT assessment who was treated with pegI/R. In spite of the lung function worsening and respiratory infections, he managed to complete treatment and even sustained virological response (SVR). At present he is on LT waiting list.

Tratamiento de hepatitis crónica C en un paciente con fibrosis quística en situación de pretrasplante pulmonar / Chronic hepatitis C treatment in a cystic fibrosis patient in the pulmonary pre-transplant stage

El tratamiento estándar de la hepatitis crónica C, interferón pegilado (INF-peg) y ribavirina (RBV), puede ser inadecuado o incluso estar contraindicado en algunos pacientes debido a sus limitaciones en cuanto a eficacia y efectos adversos. En pacientes con fibrosis quística infectados por el virus de la hepatitis C (VHC) el tratamiento antiviral podría aumentar las infecciones respiratorias con el consiguiente empeoramiento de la función pulmonar. Por contra, la infección por VHC podría desestimar a estos pacientes para un necesario trasplante pulmonar. Presentamos el caso de un varón con fibrosis quística diagnosticado de infección VHC durante su evaluación previa al trasplante pulmonar. El paciente fue tratado con INF-peg y RBV. A pesar del empeoramiento en la función pulmonar y numerosas infecciones respiratorias intercurrentes, logró completar el tratamiento y obtener respuesta viral sostenida, encontrándose actualmente en lista de espera(AU)

The standard treatment of chronic hepatitis C, pegylated interferon and ribavirin (pegI/R), has many limitations in both effectiveness and secondary effects, which makes it unsuitable or even contraindicated for some patients. In hepatitis C virus-infected cystic fibrosis patients this treatment could increase respiratory infections with subsequent pulmonary function deterioration. On the contrary, hepatitis C virus (HCV) infection may make lung transplant (LT) unfeasible. We present the case of a cystic fibrosisyoung man diagnosed with HCV infection during LT assessment who was treated with pegI/R. In spite of the lung function worsening and respiratory infections, he managed to complete treatment and even sustained virological response (SVR). At present he is on LT waiting list(AU)

Cuidados paliativos en pacientes con enfermedades respiratorias crónicas en fase avanzada / No disponible

Objetivos: analizar la bibliografía existente sobre los diferentes aspectos relacionados con la aplicación de los cuidados paliativos en las Enfermedades Respiratorias Crónicas en Fase Avanzada (ERCA), para delimitar los pacientes susceptibles de recibir estos cuidados, las necesidades de los enfermos y las medidas más importantes que deben ser adoptadas para su tratamiento desde una perspectiva de mejora de su calidad de vida. Material y métodos: revisión narrativa, focalizando en los criterios pronósticos en las ERCA, las necesidades de estos pacientes, su integración en los programas de cuidados paliativos y el tratamiento de la disnea. Resultados: las ERCA agrupan a una serie de procesos crónicos respiratorios, de evolución progresiva e incurable, que cursan con insuficiencia respiratoria y una importante sintomatología Y déficit funcional, ocasionando alta dependencia y frecuentación de los servicios sanitarios. Hay un generalizado consenso en la necesidad de integrar los cuidados paliativos en la atención a los pacientes con ERCA que contrasta con el escaso porcentaje de estos enfermos incluidos en programas de cuidados paliativos. Las necesidades de los pacientes con ERCA son similares a las de los pacientes con cáncer avanzado. Los programas de seguimiento domiciliario han mostrado su efectividad para reducir el número de ingresos y mejorar la calidad de vida de estos enfermos. Su implantación en el marco de los programas de cuidados paliativos facilitaría la inclusión de estos enfermos. Mejorar la comunicación en los pacientes con ERCA, facilitando su participación en la toma de decisiones debe ser uno de los objetivos prioritarios en el seguimiento de estos enfermos. La disnea es el síntoma más prevalente en los pacientes con ERCA y el que más disconfort produce. Se deben utilizar escalas de dificultad respiratoria que permitan evaluar y monitorizar la efectividad de los tratamientos empleados. Los opioides son una de las piedras angulares en el tratamiento de la disnea. Un ensayo terapéutico con estos fármacos resulta imprescindible en el manejo de enfermos con disnea refractaria a otras medidas de tratamiento. Conclusiones: los programas de cuidados paliativos deberían incluir pacientes con ERCA con el objetivo de focalizar en la mejora de su calidad de vida (AU)

Objectives: to assess current bibliography on the various aspects related to palliative care implementation for advanced-stage chronic respiratory disorders (ACRDs) in order to recognize patients who will benefit from this care, the needs of patients, and the most significant measures to be adopted from an improved quality of life standpoint. Material and methods: a narrative review focused on ACRD prognostic criteria, patient needs, patient incorporation to palliative care programs, and dyspnea management. Results: ACRDs include a number of chronic respiratory conditions that are both progressive and incurable, and manifest with respiratory failure, significant symptoms, and function impairment, which leads to a high rate of dependency on and use of healthcare services. There is general consensus on the need to integrate palliative care into the management of ACRD patients, which is in contrast to the scarce proportion of such patients already included in palliative care programs. The needs of these ACRD patients are similar to those of subjects with advanced cancer. Home follow-up programs have proven effective to reduce hospitalization rates and to improve quality of life. Their implementation within a palliative care frame would facilitate patient inclusion. Communication improvement for patients with ACRD to help in their taking part in decision making must be a priority goal in the follow-up of these individuals. Dyspnea is the most prevalent and discomfort-inducing symptom among patients with ACRD. Respiratory distress scales should be used to assess and monitor treatment effectiveness. Opioids are a keystone in the management of dyspnea. A therapy cycle with these drugs is essential in the management of patients with dyspnea refractory to other therapeutic efforts. Conclusions: palliative care programs should include patients with ACRD and focus on improving their Quality of life (AU)

Síndrome de Swyer-James-McLeod; descripción de un caso y breve revisión de la literatura / Swyer-James-McLeod Syndrome: a case report and brief review of the literature

Resumen. El síndrome de Swyer-James-McLeod se caracteriza por ser una patología adquirida, generalmente de causa infecciosa, en las primeras etapas de la vida. Ello provoca una hipoplasia del pulmón afectado, con características anatomopatológicas que se describen como una bronquiolitis obliterante adquirida. La clínica que presentan los pacientes es muy variada, desde formas paucisintomáticas hasta cuadros graves con infecciones respiratorias severas de repetición (AU)

Abstract. The Swyer-James-McLeod syndrome is characterized by being an acquired condition, generally due to an infectious cause, in the early stages of life. It causes hypoplasia of the affected lung, with anatomic-pathological characteristics that are described as acquired bronchiolitis obliterans. The symptoms presented by the patients vary greatly, going from paucisymptomatic forms to severe pictures with recurrent severe respiratory infections (AU)