Concepción Prados

Breathing pattern and central ventilatory drive in mild and moderate generalised myasthenia gravis.

BACKGROUND--Myasthenia gravis is a specific autoimmune disease characterised by weakness and fatigue. Respiratory muscle weakness has been studied using the determination of maximal respiratory pressures, but the response of respiratory centres is not well characterised. This study was undertaken to determine the breathing pattern and the central ventilatory drive in patients with mild and moderate generalised myasthenia gravis. METHODS--Twenty four patients with myasthenia gravis were studied, divided into two groups. Group 1 included 13 subjects (eight women and five men aged 23-64) with mild generalised myasthenia gravis, and group 2 was composed of 15 patients (11 women and four men aged 23-69) with moderate generalised myasthenia gravis. A control group comprised 15 healthy persons with a similar age and sex distribution. Spirometric measurements and maximal respiratory pressures were performed under basal conditions in all subjects, and the rate and depth of breathing and the inspiratory occlusion pressure in the mouth in the first 0.1 second (P0.1) were measured. RESULTS--No difference was detected for parameters of breathing pattern between patients in group 1 and control subjects, although P0.1 was higher in those in group 1. Subjects in group 2 had a lower tidal ventilation, shorter inspiratory time, and a higher frequency with a higher P0.1 than control subjects. CONCLUSIONS--Mild myasthenia gravis causes increased neuromuscular drive with a normal breathing pattern. Moderate myasthenia gravis is characterised by a more rapid shallow breathing pattern.

Estudio multicéntrico de prevalencia de micobacterias ambientales en pacientes con fibrosis quística

Objetivo Evaluar la prevalencia de micobacterias ambientales (MA) en pacientes con fibrosis quistica (FQ). Pacientes y metodos Se ha realizado un estudio prospectivo de 2 anos de duracion en pacientes con FQ mayores de 6 anos, procedentes de 6 unidades monograficas. En las muestras respiratorias, recogidas cada 6 meses, se efectuo una tincion de auramina-rodamina, ademas de cultivos en medios solido y liquido. Si se detectaba una tincion o cultivo positivo para MA, se recogian 1 o 2 esputos mas y se hacia un seguimiento estricto para valorar la necesidad de tratamiento especifico. Se consideraron las siguientes variables clinicas: edad, sexo, insuficiencia pancreatica, diabetes, uso de aerosolterapia antibiotica y de azitromicina de forma continua, tratamientos con corticoides inhalados o por via oral de forma prolongada. Resultados Participaron en el estudio 220 pacientes (119 mujeres), con una edad media de 22,62 anos (rango: 6-74). El 23,6% recibia azitromicina. Se cultivaron para la deteccion de micobacterias 1.303 muestras de esputo (rango por paciente: 4-68), de las que la tincion de auramina fue positiva en 17 casos y el cultivo en 48, correspondientes a 17 pacientes (7,72%). En 88 muestras el cultivo estaba contaminado. En ningun caso se aislo Mycobacterium tuberculosis . Las MA aisladas fueron: M. avium complex (n = 10), M. abscessus (n = 6) y M. fortuitum (n = 1). Cinco de los 9 pacientes que presentaron mas de un aislamiento tuvieron deterioro clinico y se les indico tratamiento especifico. No hubo diferencias entre las variables clinicas de los pacientes con y sin aislamientos de MA. Conclusiones La prevalencia de MA en pacientes con FQ no fue muy alta (7,72%), quiza debido a la interferencia de la azitromicina sobre el crecimiento de MA. Hay que vigilar estrechamente a los pacientes con aislamientos repetidos.

Annual direct medical costs of bronchiectasis treatment Impact of severity, exacerbations, chronic bronchial colonization and chronic obstructive pulmonary disease coexistence

Patients with bronchiectasis (BE) present exacerbations that increase with severity of the disease. We aimed to determine the annual cost of BE treatment according to its severity, determined by FACED score, as well as the parameters associated with higher costs. Multicentre historical cohorts study with patients from six hospitals in Spain. The costs arising during the course of a year from maintenance treatment, exacerbations, emergency visits and hospital admissions were analysed. In total, 456 patients were included (56.4% mild BE, 26.8% moderate BE and 16.9% severe BE). The mean cost was €4671.9 per patient, which increased significantly with severity. In mild BE, most of the costs were due to bronchodilators and inhaled steroids; in severe BE, most were due to exacerbations and inhaled antibiotics. Forced expiratory volume in 1 second (FEV1%), age, colonization by Pseudomonas aeruginosa and the number of admissions were independently related to higher costs. The highest costs were found in patients with BE associated with chronic obstructive pulmonary disease, with the most exacerbations and with chronic bronchial colonization by Pseudomonas aeruginosa (PA). In conclusion, BE patients gave rise to high annual costs, and these were doubled on each advance in severity on the FACED score. FEV1%, age, colonization by PA and the number of admissions were independently related to higher costs.

Fibrosis quística: consenso sobre el tratamiento del neumotórax y de la hemoptisis masiva y sobre las indicaciones del trasplante pulmonar

La fibrosis quística (FQ), con una incidencia de un caso cada 2.500 recién nacidos vivos, es la enfermedad hereditaria más frecuente en la raza caucásica. Se produce como consecuencia de la ausencia o la alteración de la función de una glucoproteína, llamada cystic fibrosis transmembrane conductance regulator (CFTR)1. La patogenia de la enfermedad no está aclarada, aunque parece ser que el círculo vicioso obstrucción-infeccióninflamación es el productor del daño pulmonar causante, a su vez, de las bronquiectasias quísticas y la fibrosis pulmonar propias de la enfermedad2. El diagnóstico de FQ se establece en el primer año de vida en el 70% de los casos, fundamentalmente por infecciones respiratorias agudas o persistentes o por malnutrición3. El tratamiento de la alteración pulmonar es uno de los pilares fundamentales para mejorar la supervivencia de estos enfermos. La expectativa de vida de los pacientes con FQ ha aumentado durante las últimas décadas debido, fundamentalmente, a la aparición de nuevos tratamientos y a la creación de unidades multidisciplinarias encargadas del cuidado de estos pacientes. A medida que aumenta la edad de los pacientes pueden ocurrir, con mayor frecuencia, una serie de complicaciones pulmonares graves, como el neumotórax y la hemoptisis masiva. La hemoptisis masiva tiene casi siempre su origen en las arterias bronquiales. La inflamación crónica que sufren las paredes bronquiales de los pulmones de los pacientes con FQ produce una hipertrofia de las mismas, junto con la formación de vasos nuevos. Esta hipervascularización conlleva una propensión al sangrado, especialmente durante las exacerbaciones infecciosas respiratorias. La forma de presentación de los neumotórax en la FQ no difiere de la que acontece en otras enfermedades respiratorias diferentes de este proceso. Es una complicación grave, con importante riesgo vital inmediato, pues ocurre fundamentalmente en los pacientes con afectación respiratoria importante3. El trasplante pulmonar debe ser la última opción terapéutica para los pacientes con una enfermedad pulmonar terminal. Con el objetivo de conseguir unas pautas consensuadas sobre el tratamiento del neumotórax y de la hemoptisis masiva en los pacientes con FQ, así como sobre las indicaciones y contraindicaciones del trasplante pulmonar en esta enfermedad, la Fundación Sira Carrasco, para ayuda contra la FQ organizó, el día 23 de abril de 1999, en Madrid, unos encuentros de consenso con profesionales especializados en el tratamiento de la FQ. Los resultados de esta discusión y los consensos alcanzados se detallan en el presente documento.

Relationship Between Exhaled Carbon Monoxide and Airway Hyperresponsiveness in Asthmatic Patients

The study objectives were to analyze the changes in exhaled carbon monoxide (COex) induced by histamine provocation challenge in asthmatic patients and to evaluate the relationship between COex and airway sensitivity and reactivity. Levels of COex were measured in 105 nonsmoking mildly asthmatic subjects before and after histamine provocation challenge. Dose‐response curves were characterized by their sensitivity (PD20) and reactivity. Dose‐response slope (DRS), continuous index of responsiveness (CIR), and bronchial reactivity index (BRI) were determined as reactivity indices. Bronchial challenge was positive for 47 subjects and negative for 58. The COex levels rose significantly after bronchial challenge in the positive response group (4.49 ± 0.4 vs. 5.74 ± 0.57 ppm, p = 0.025) and in the negative response group (2.84 ± 0.25 vs 4.00 ± 0.41 ppm, p = 0.000). An inverse relation between basal COex and PD20 was found (r = − 0.318, p = 0.030). In all subjects, a proportional direct relationship between COex and DRS (r = 0.214, p = 0.015), CIR (r = 0.401, p = 0.000), and BRI (r = 0.208, p = 0.012) was observed. On stepwise multiple linear regression analysis, COex only significantly correlated with CIR (multiple r2 = 0.174, p = 0.000). In conclusion, exhaled CO determination is a noninvasive inflammatory marker of the respiratory tract, which shows an acceptable association with airway hyperresponsiveness.

Isolation of Nocardia Species in Patients With Cystic Fibrosis

The isolation of Nocardia species from the respiratory secretions of patients with cystic fibrosis presents problems with important clinical implications. From the sputum culture of a total of 387 patients with cystic fibrosis, Nocardia species was isolated in 9 cases (2%; 8 females and 1 male) with a mean (SD) age of 17 (7) years. Sixty-seven percent of the patients were asymptomatic and no relevant radiographic or analytical changes were detected. In only 3 patients was of Nocardia species isolated again in successive samples. Two patients were not treated, 7 were treated with cotrimoxazole and 3 with minocycline; in 2 cases therapy was intravenous. After a mean follow-up of 48 (33) months, all patients had improved. Isolation of Nocardia species from the secretions of patients with cystic fibrosis does not necessarily imply infection and the need for treatment should be assessed on an individual basis.

Amyloid goitre and hypothyroidism secondary to cystic fibrosis.

Although cystic fibrosis (CF) is still the most frequently fatal childhood disease, many adults now survive into their third and fourth decades. Uncommon complications of chronic diseases, such as amyloidosis, while infrequent, may now appear during the course of CF in adulthood. We present a case of a patient with CF who was diagnosed with hypothyroidism due to amyloid deposits in the thyroid.

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.

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.

Tres formas no idiopáticas de bronquiolitis obliterante con neumonía en organización

La bronquiolitis obliterante con neumonia en organizacion es la enfermedad mas importante de los bronquiolos. Se ha descrito una forma idiopatica, de gran trascendencia en el diagnostico diferencial de los casos incipientes de fibrosis pulmonar, y varias formas asociadas con el consumo de farmacos, infecciones, enfermedades del colageno y focal. Se presentan los casos de 3 pacientes con bronquiolitis obliterante con neumonia en organizacion, en relacion con una artritis reumatoide y consumo de sales de oro, con infeccion por el virus de inmunodeficiencia humana y una forma focal asociada a hidatidosis pulmonar.