Bronchiectasis: A Complex, Heterogeneous Disease. / Las bronquiectasias: una enfermedad compleja y heterogénea.

Most areas of respiratory medicine continue to use an Oslerian approach, based on signs and symptoms, in which the disease is the center of all activity. However, this paradigm is changing. Now that lung diseases have been recognized as heterogeneous and complex, we are moving towards more personalized, precise, patient-oriented medicine. The aim of this review was to define the current state of the knowledge on bronchiectasis, or, more accurately, the bronchiectasis syndrome, as a multidimensional, systemic, heterogeneous, complex disease. We explore the advances that have already been made, and above all the many steps that are still to be taken. We also propose some tools which might facilitate the application of these concepts in clinical practice, and help us continue our journey towards a more holistic view of this disease.

[Diagnosis and treatment of bronchiectasis. Spanish Society of Pneumology and Thoracic Surgery]. / Diagnóstico y tratamiento de las bronquiectasias. SEPAR.

Bronchiectasis is the end result of several different diseases that share principles of management. The clinical course usually involves chronic bronchial infection and inflammation, which are associated with progression. The cause of bronchiectasis should always be investigated, particularly when it can be treated. We recommend evaluating etiology, symptoms, bronchial colonization and infection, respiratory function, inflammation, structural damage, nutritional status, and quality of life in order to assess severity and to monitor clinical course. Care should be supervised by specialized units, at least in cases of chronic bronchial infection, recurrent exacerbations, or when there is a cause that is likely to respond to treatment. Improving symptoms and halting progression are the goals of management, which is based on treatment of the underlying cause and of acute or chronic infections and on the drainage of secretions. Complications that arise must also be treated. Antibiotic prescription is guided by how well infection is being controlled, and this is indicated by the color of sputum and a reduction in the number of exacerbations. We recommend inhaled antibiotics in cases of chronic bronchial infection that does not respond to oral antibiotics, when these cause side effects, or when the cause is Pseudomonas species or other bacteria resistant to oral antibiotics. Inhaled administration is also advisable to treat initial colonization by Pseudomonas species.

[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.

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

Fundamento y objetivo: La disminución de la densidad mineral ósea (DMO) es una complicación de la fibrosis quística (FQ) que se observa al aumentar la esperanza de vida de estos pacientes. El objetivo del presente estudio ha sido valorar la evolución de la DMO tras la instauración de un protocolo de prevención y tratamiento de la osteoporosis. Pacientes y método: Realizamos un estudio prospectivo en 21 pacientes adultos (10 mujeres y 11 varones) con FQ de una unidad multidisciplinaria, con una edad media de 24,3 años (extremos, 19-44). Se evaluaron los resultados de la DMO según los diferentes tratamientos administrados y se relacionó el cambio relativo anual en el porcentaje de la DMO con los observados en la función pulmonar, índice de masa corporal y puntuaciones radiológica de Brasfield y clínica de Shwachman, durante un período de seguimiento medio de 3 años. Las 3 pautas de tratamiento instauradas fueron: medidas preventivas generales, suplementos de calcio y vitamina D, y alendronato a dosis de 10 mg diarios con suplementos de calcio y vitamina D. Resultados: Inicialmente se observó que el 14,2% de los pacientes con FQ presentaban una disminución acusada de la masa ósea respecto a la población control de su misma edad y sexo, con una puntuación Z inferior a ­2 desviaciones estándar en columna lumbar y/o fémur total. Otro 38% presentaban descensos de la puntuación Z entre ­1 y ­2 desviaciones estándar. La disminución anual progresiva de la DMO durante los 3 años fue generalizada en todas las áreas anatómicas analizadas: ­0,52% (1,87) en columna lumbar, ­1,17% (1,91) en fémur total y ­2,16% (2,65) en cuello de fémur. En fémur el porcentaje de cambio relativo anual de DMO se relacionó con el observado en el volumen espiratorio forzado en el primer segundo (p < 0,05). Sólo los pacientes tratados con alendronato no sufrieron pérdida progresiva de masa ósea. El tratamiento con calcio, vitamina D y alendronato fue más eficaz para prevenir la disminución de la DMO en fémur total y cuello femoral que las medidas preventivas generales (p < 0,05) y en fémur total fue más eficaz que los suplementos de calcio con vitamina D (p < 0,05). Conclusiones: Nuestro trabajo confirma que los pacientes adultos jóvenes españoles con FQ tienen una disminución de la DMO respecto a la población control y que, a lo largo de la enfermedad, esta disminución es progresiva. Esta pérdida de masa ósea no se previene con medidas generales ni con suplementos de calcio y vitamina D, y únicamente en los pacientes tratados con alendronato se produce un aumento de la DMO

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