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.

Galactomannan in bronchoalveolar lavage fluid for diagnosis of invasive aspergillosis in non-hematological patients.

BACKGROUND: The role of galactomannan (GM) in serum or bronchoalveolar lavage fluid (BALF) for the diagnosis of invasive pulmonary aspergillosis (IPA) has been extensively evaluated in hematological patients, however its performance in non-hematological patients is not well established. METHODS: We performed a multicenter retrospective study in 3 university hospitals in Madrid, Spain between 2010 and 2014. The study population comprised patients with chronic obstructive pulmonary disease (COPD) and patients with immunosuppressive conditions in whom IPA was suspected and for whom BALF GM was available. Patients with hematological disorders were excluded. RESULTS: A total of 188 patients (35 with COPD and 153 with immunosuppressive conditions) were analyzed, and 31 cases of IPA (proven or probable) were identified. The global sensitivity of BALF GM (optical density index [ODI] ≥ 1.0) was 77.4%; sensitivity was higher in patients with immunosuppressive conditions than in patients with COPD (81.8% vs 66.7%; p: 0.38). In COPD patients, the best performance was obtained for BALF GM (ODI ≥ 0.5), although sensitivity (88.9%) was similar to that of BALF fungal culture (88.9%). The sensitivity of GM in serum was very poor in both populations (36.4% and 11.6%, respectively). CONCLUSIONS: In the present series, the diagnostic performance of BALF GM was good for IPA in non-hematological patients, especially in patients with immunosuppressive conditions.

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)

Elevación diafragmática como hallazgo radiológico de un tumor fibroso aislado de pleura / No disponible

No disponible

Tratamiento con TOBI en pacientes con fibrosis quística mayores de 18 años / Treatment with TOBI (Tobramycin Inhalation) in patients over 18 years old with cystic

La fibrosis quística (FQ) es una enfermedad autosómica recesiva causada por mutaciones, siendo la más conocida la delección F508del (70%). Se han descrito más de 1.000 mutaciones asociadas a esta enfermedad. La enfermedad pulmonar es una de las causas más importantes de morbilidad y mortalidad relacionadas con la FQ. Es muy frecuente que se colonicen por determinados gérmenes, siendo uno de los más importantes la P. aeruginosa. Una vez que existe una infección crónica, los enfermos se tratan con antibioterapia en aerosol, empleando con más frecuencia la colimicina y la tobramicina libre de conservantes (TOBI®). Son pocos los estudios que han analizado este último antibiótico en nuestro país, por lo que nos hemos propuesto observar la evolución funcional y la clínica de los pacientes mayores de 18 años que lo están empleando. Hemos estudiado a 13 enfermos, 7 hombres y 6 mujeres, con una edad media de 28,0 ± 11,0 años, con un IMC medio de 20,84 ± 1,99 y 11,0 ± 4,0 años de colonización por P. aeruginosa. Se comprobó la función pulmonar desde 6 meses antes (FEV1: 37,7 ± 9,98%, FVC: 57,10 ± 8,97%, FEV1/FVC: 56,20 ± 12,90) hasta los 6 primeros meses tras iniciar el TOBI® (FEV1: 36,50 ± 10,70%, FVC: 55,57 ± 13,69%, FEV1/FVC: 56,0 ± 11,70%), así como el número de exacerbaciones e ingresoshospitalarios, en este espacio de tiempo. El número de exacerbaciones fue significativamente mayor en el período analizado antes de empezar con TOBI® (p < 0,05). No hemos encontrado otros hallazgos significativos en el resto de las variables analizados. Los efectos secundarios han sido muy escasos. Creemos que se podrían realizar más trabajos entre nuestros enfermos para conocer la eficacia y la seguridad de este antibiótico administrado por vía inhalada y ajustar los períodos on-off (AU)

Cystic fibrosis (CF) is a genetic disease, authosomic recesive, caused by more than 1.000 mutations, being the most known F508del (70%). The pulmonary disease is one of the cause of morbility and mortality related to CF. It is very common they are colonized by several microorganims, being one of the most important P. aeruginosa. Once, patients present chronic infection, they are treated with aerosolized antibiotics, using more colistin and free-preservative tobramicin (TOBI®). There are few studies that they are analized the last antibiotic in our country, so we have proponed to observe the functional and clinic evolution of the patients older than 18 years, who have used it. We have studied 13 patients, 7 males and 6 females, with and age of 28.0 ± 11.0 years, with a BMI of 20.84 ± 1.99 and 11.0 ± 4.0 years of colonization due to P. aeruginosa. The pulmonary function was studied since 6 months before (FEV1: 37.7 ± 9.98%, FVC: 57.10 ± 8.97%, FEV1/FVC: 56.20 ± 12.90) until six months later the begining of using TOBI® (FEV1: 36.50 ± 10.70%, FVC: 55.57 ± 13.69%, FEV1/FVC: 56.0 ± 11.70%), as well as the number of exacerbations and hospitalizations in this period of time. The number of exacerbations was significatively higher during the period analized before starting with TOBI® (p < 0.05). We have not seen other significative findings in the other studied variables. The secundary effects have been very little. We think that more works could carry out between aur patients to know the efficacy and security of this antibiotic administered by inhaled way adjusting the periods on-off (AU)

Effect on single-breath washout and lung function of elastase-induced emphysema in rats.

Lung volumes, diffusing capacity (DLCO), quasi-static pressure-volume curves (P-V), forced expiration (FE) and He-SF6 single-breath washout (SBW) were performed in Wistar rats with emphysema induced by different doses of pancreatic elastase in saline, instilled intratracheally 6 wk prior to the tests. Emphysema was quantitatively assessed by mean linear intercept (Lm) measurements on 5-microns lung sections. Lung volume, P-V curve, and FE dependence on Lm, as well as the nonsignificant dependence of DLCO on Lm, are generally similar to results reported by others. The most interesting observation concerns the SBW: N2 slopes of the alveolar plateau, compared for identical lung volumes, did not change with the degree of emphysema. By contrast, the He-SF6 slope difference did depend significantly on the degree of emphysema. Based on the diffusion front theory, the present work suggests that in rats with elastase-induced emphysema, the phase III slope modifications relate mainly to elastic and not to structural alterations.

Pleuropulmonary infections due to nontyphoid strains of Salmonella.

Medical records of 11 patients with nontyphoid Salmonella pleuropulmonary disease studied from 1960 to 1986 in a general hospital were reviewed. Eight patients (73%) were 60 years old or older, and the median age was in the seventh decade. There was no seasonal variation in the prevalence. The infection was hospital acquired in 4 patients (36%). All patients had one or more (median, 1.5) major underlying diseases. Seven of them had previous abnormalities of the lung or pleura. Severe immunosuppression was present in 7 cases. Pneumonia occurred in 8 patients, lung abscesses in 2, and empyema in 1. All patients with pneumonia had positive blood cultures. A gastrointestinal source of pulmonary infection was not probable because only 2 patients had positive stool cultures. We suggest that the reticulo-endothelial system could be the source of hematogenous spread of nontyphoid Salmonella. The overall mortality was 63%. Pulmonary infection due to Salmonella should be considered among the pathogens associated with gram-negative bacillary pneumonia in elderly patients who are immunosuppressed and have underlying pulmonary disease. Pathogenesis of this infection remains to be clarified.