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RESPIRATORY DISEASES (103 journals)                     

Showing 1 - 104 of 104 Journals sorted alphabetically
Advances in Respiratory Medicine     Open Access   (Followers: 7)
Advances in Thoracic Diseases     Open Access  
American Journal of Respiratory and Critical Care Medicine     Full-text available via subscription   (Followers: 257)
American Journal of Respiratory Cell and Molecular Biology     Full-text available via subscription   (Followers: 20)
American Review of Respiratory Disease     Full-text available via subscription   (Followers: 4)
Angiologia e Cirurgia Vascular     Open Access   (Followers: 1)
Annals of the American Thoracic Society     Full-text available via subscription   (Followers: 17)
Annals of Thoracic Medicine     Open Access   (Followers: 6)
Archives of Pulmonology and Respiratory Care     Open Access   (Followers: 1)
Archivos de Bronconeumología     Full-text available via subscription  
Archivos de Bronconeumología (English Edition)     Full-text available via subscription   (Followers: 1)
Asthma Research and Practice     Open Access   (Followers: 1)
BMC Pulmonary Medicine     Open Access   (Followers: 5)
BMJ Open Respiratory Research     Open Access   (Followers: 6)
Breathe     Open Access   (Followers: 4)
Canadian Journal of Respiratory, Critical Care, and Sleep Medicine     Hybrid Journal   (Followers: 1)
Canadian Respiratory Journal     Open Access   (Followers: 3)
Case Reports in Pulmonology     Open Access   (Followers: 3)
Chest     Full-text available via subscription   (Followers: 102)
Chest Disease Reports     Open Access   (Followers: 2)
Chronic Respiratory Disease     Hybrid Journal   (Followers: 9)
Clinical Lung Cancer     Hybrid Journal   (Followers: 6)
Clinical Medicine Insights : Circulatory, Respiratory and Pulmonary Medicine     Open Access   (Followers: 3)
Clinical Pulmonary Medicine     Hybrid Journal   (Followers: 2)
COPD Research and Practice     Open Access   (Followers: 1)
COPD: Journal of Chronic Obstructive Pulmonary Disease     Hybrid Journal   (Followers: 15)
Current Opinion in Pulmonary Medicine     Hybrid Journal   (Followers: 10)
Current Pulmonology Reports     Hybrid Journal  
Current Research in Tuberculosis     Open Access   (Followers: 3)
Current Respiratory Care Reports     Hybrid Journal   (Followers: 1)
Current Respiratory Medicine Reviews     Hybrid Journal   (Followers: 5)
Der Pneumologe     Hybrid Journal   (Followers: 1)
Egyptian Journal of Chest Diseases and Tuberculosis     Open Access   (Followers: 3)
ERJ Open Research     Open Access   (Followers: 3)
Eurasian Journal of Pulmonology     Open Access  
European Clinical Respiratory Journal     Open Access   (Followers: 3)
European Respiratory Journal     Full-text available via subscription   (Followers: 39)
European Respiratory Review     Open Access   (Followers: 7)
Experimental Lung Research     Hybrid Journal  
Expert Review of Respiratory Medicine     Hybrid Journal   (Followers: 5)
Heart & Lung: The Journal of Acute and Critical Care     Hybrid Journal   (Followers: 13)
Heart, Lung and Circulation     Full-text available via subscription   (Followers: 9)
Indian Journal of Respiratory Care     Open Access   (Followers: 3)
Indian Journal of Tuberculosis     Full-text available via subscription  
Influenza and Other Respiratory Viruses     Open Access   (Followers: 3)
International Journal of Chronic Obstructive Pulmonary Disease     Open Access   (Followers: 3)
Journal of Association of Chest Physicians     Open Access   (Followers: 2)
Journal of Asthma     Hybrid Journal   (Followers: 4)
Journal of Asthma Allergy Educators     Hybrid Journal   (Followers: 4)
Journal of Bronchology & Interventional Pulmonology     Hybrid Journal   (Followers: 4)
Journal of Clinical Tuberculosis and Other Mycobacterial Diseases     Open Access  
Journal of Heart and Lung Transplantation     Hybrid Journal   (Followers: 12)
Journal of Respiratory Medicine     Open Access   (Followers: 4)
Journal of Respiratory Research     Open Access   (Followers: 1)
Journal of Tuberculosis Research     Open Access   (Followers: 1)
Jurnal Respirasi     Open Access  
Karger Kompass Pneumologie     Full-text available via subscription   (Followers: 1)
Kindheit und Entwicklung     Hybrid Journal  
Lung     Hybrid Journal   (Followers: 2)
Lung Cancer     Hybrid Journal   (Followers: 16)
Lung Cancer International     Open Access   (Followers: 2)
Lung Cancer: Targets and Therapy     Open Access   (Followers: 3)
Lung India     Open Access   (Followers: 1)
Multidisciplinary Respiratory Medicine     Open Access   (Followers: 4)
npj Primary Care Respiratory Medicine     Open Access   (Followers: 2)
Open Journal of Respiratory Diseases     Open Access   (Followers: 1)
Open Respiratory Medicine Journal     Open Access   (Followers: 1)
Paediatric Respiratory Reviews     Hybrid Journal   (Followers: 11)
Pediatric Quality & Safety     Open Access  
Pediatric Respirology and Critical Care Medicine     Open Access   (Followers: 1)
Pulmonary Circulation     Open Access   (Followers: 4)
Pulmonary Medicine     Open Access   (Followers: 2)
Pulmonary Pharmacology & Therapeutics     Hybrid Journal   (Followers: 2)
Pulmonary Therapy     Open Access   (Followers: 1)
Pulmonology and Respiratory Research     Open Access   (Followers: 1)
Respiratory Care     Full-text available via subscription   (Followers: 10)
Respiratory Investigation     Full-text available via subscription  
Respiratory Medicine     Hybrid Journal   (Followers: 18)
Respiratory Medicine : X     Open Access  
Respiratory Medicine Case Reports     Open Access  
Respiratory Medicine CME     Hybrid Journal  
Respiratory Medicine Extra     Full-text available via subscription   (Followers: 1)
Respiratory Physiology & Neurobiology     Hybrid Journal   (Followers: 4)
Respiratory Research     Open Access   (Followers: 1)
Respirology     Hybrid Journal   (Followers: 5)
Respirology Case Reports     Open Access  
Revista Americana de Medicina Respiratoria     Open Access  
Revista Chilena de Enfermedades Respiratorias     Open Access  
Revista Inspirar     Open Access  
Revista ORL     Open Access  
Revista Portuguesa de Pneumologia     Open Access  
Sarcoidosis Vasculitis and Diffuse Lung Disese     Full-text available via subscription   (Followers: 3)
Seminars in Respiratory and Critical Care Medicine     Hybrid Journal   (Followers: 14)
Sleep Medicine Reviews     Hybrid Journal   (Followers: 17)
The Clinical Respiratory Journal     Hybrid Journal   (Followers: 3)
The International Journal of Tuberculosis and Lung Disease     Full-text available via subscription   (Followers: 8)
The Lancet Respiratory Medicine     Full-text available via subscription   (Followers: 35)
Therapeutic Advances in Chronic Disease     Open Access   (Followers: 7)
Therapeutic Advances in Respiratory Disease     Open Access   (Followers: 1)
Thorax     Hybrid Journal   (Followers: 38)
Translational Respiratory Medicine     Open Access   (Followers: 1)
Tuberculosis     Hybrid Journal   (Followers: 12)
Tuberculosis Research and Treatment     Open Access   (Followers: 3)
Пульмонология     Full-text available via subscription  

           

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Revista Chilena de Enfermedades Respiratorias
Number of Followers: 0  

  This is an Open Access Journal Open Access journal
ISSN (Print) 0717-5698 - ISSN (Online) 0717-7348
Published by SciELO Homepage  [912 journals]
  • Chilean clinical practice guidelines for idiopathic pulmonary fibrosis,
           2019

    •  
  • History and methodology of the first Chilean idiopathic pulmonary fibrosis
           guidelines, 2019

    • Abstract: Resumen Desde 2017 los miembros de la Comisión de Enfermedades Pulmonares Intersticiales Difusas de la Sociedad Chilena de Enfermedades Respiratorias hemos trabajado en la elaboración de las primeras guías de fibrosis pulmonar idiopática (FPI) del país, necesidad evidente para fomentar el diagnóstico precoz y adecuado de la enfermedad y establecer una base para posible incorporación de su cuidado en cobertura de seguros de salud especiales. Se elaboraron 5 preguntas de revisión de evidencia y el resto se trabajó en formato de preguntas de contexto. Un grupo de metodólogos graduaron la evidencia siguiendo la metodología GRADE.Since 2017, the members of the Commission of Diffuse Interstitial Lung Diseases of the Chilean Society of Respiratory Diseases have worked in the development of the first guidelines of idiopathic pulmonary fibrosis (IPF) in the country, an obvious need to encourage early and adequate diagnosis of the disease and establish a basis for possible incorporation of IPF patients care into special health insurance coverage. Five evidence review questions were prepared and the remainder were worked out in context question format. A group of methodologists graduated the evidence following the GRADE methodology.
       
  • Definition, pathogenesis and risk factors of idiopathic pulmonary fibrosis

    • Abstract: Resumen La fibrosis pulmonar idiopática (FPI) es una forma específica de neumonía intersticial idiopática, de tipo fibrosante crónica y progresiva, con patrón radiológico y/o histológico de neumonía intersticial usual (NIU). Su patogenia es compleja, el modelo más aceptado actualmente es basado en las células epiteliales alveolares, aberrantemente activadas que conducen a la proliferación de fibroblastos y su diferenciación a miofibroblastos que depositan matriz extracelular y destruyen irreversiblemente la arquitectura pulmonar. No existe un claro factor inicial que explique la activación y posterior mantención del mecanismo de la fibrosis. El factor de crecimiento transformante beta (TGF-β) liberado por las células epiteliales alveolares se ha implicado como unos de los principales conductores de la inducción y proliferación de fibroblastos alterados que persiste mucho tiempo después de la estimulación inicial, lo que explicaría en gran parte el comportamiento clínico progresivo y crónico.Idiopathic pulmonary fibrosis (IPF) is a specific form of idiopathic interstitial pneumonia, of chronic and progressive fibrosing type, with radiological and / or histological pattern of usual interstitial pneumonia (UIP). Its pathogenesis is complex, the most accepted model currently is based on the fact that the alveolar epithelial cells, aberrantly activated, lead to the proliferation of fibroblasts and their differentiation to myofibroblasts that deposit extracellular matrix and irreversibly destroy the pulmonary architecture. There is no clear initial trigger that explains the activation and subsequent maintenance of the fibrosis mechanism. The transforming growth factor beta (TGF-β), released by the alveolar epithelial cells, has been implicated as one of the main drivers of the induction and proliferation of altered fibroblasts that persists long after the initial stimulation, which would largely explain progressive and chronic clinical behavior.
       
  • How is idiopathic pulmonary fibrosis suspected in primary health care'

    • Abstract: Resumen La fibrosis pulmonar idiopática (FPI) es una enfermedad pulmonar de sintomatología inespecífica. La tos crónica y disnea progresivas en adultos mayores fumadores o exfumadores son los síntomas y características clínicas más frecuentes. Por lo tanto, es usual que el diagnóstico sea tardío. La atención primaria constituye el primer contacto del paciente con el sistema de salud. Por esto es necesaria la entrega de toda la información posible a los médicos, enfermeras y kinesiólogos que atienden adultos con problemas respiratorios, ya que sólo se diagnosticará FPI si se piensa en FPI.Idiopathic pulmonary fibrosis (IPF) is a lung disease of nonspecific symptomatology. Progressive chronic cough and dyspnea in older smokers or ex-smokers are the most frequent symptoms and clinical features. Therefore, it is usual for the diagnosis to be late. Primary care constitutes the patient’s first contact with the health system. Therefore, it is necessary to deliver all possible information to physicians, nurses and physical therapists who care for adults with respiratory problems, since IPF will only be diagnosed if FPI is considered.
       
  • Differential diagnosis of idiopathic pulmonary fibrosis

    • Abstract: Resumen Para el diagnóstico certero de fibrosis pulmonar idiopática (FPI) es de vital importancia la presencia de un patrón tomográfico definitivo de neumonía intersticial usual (NIU), en un contexto clínico adecuado. El interrogatorio dirigido, el uso de cuestionarios validados, una evaluación reumatológica acuciosa y exámenes complementarios son importantes para descartar causas secundarias de fibrosis pulmonar como neumonitis por hipersensibilidad (NHS), enfermedades del tejido conectivo (ETC), toxicidad por drogas y algunas neumoconiosis que pueden imitar el patrón radiológico y muchas veces dificultar un diagnóstico adecuado de FPI.For the accurate diagnosis of idiopathic pulmonary fibrosis (IPF), the presence of a definitive tomographic pattern of usual interstitial pneumonia (UIP) is of vital importance, in an appropriate clinical context. Targeted interrogation, the use of valid questionnaires, an acute rheumatologic evaluation and complementary examinations are important to rule out secondary causes such as hypersensitivity pneumonitis (HP), connective tissue diseases (CTD), drug toxicity and some pneumoconiosis that can mimic the radiological pattern and often hinder a clear diagnosis of IPF.
       
  • High resolution computed tomography in the diagnosis of idiopathic
           pulmonary fibrosis

    • Abstract: Resumen El patrón de neumonía intersticial usual (NIU) en la tomografía computada de alta resolución (TCAR) tiene un alto grado de correlación con el patrón histológico lo que permite obviar en dichos casos la necesidad de realizar biopsia pulmonar. La exactitud del diagnóstico de NIU en TCAR se basa en la detección de signos específicos de fibrosis como el panal y las densidades reticulares con bronquiolectasias por tracción. La actualización de las guías 2018 de práctica clínica de la ATS/ERS/JRS/ALAT de Fibrosis Pulmonar Idiopática (FPI) y la declaración de consenso de la Sociedad Fleischner proponen una nueva forma de clasificar los patrones tomográficos tomando en cuenta los aspectos antes considerados. Si bien la presencia de panal sigue siendo el hallazgo principal para caracterizar el patrón NIU, la distribución del reticulado intersticial y la presencia de bronquiolectasias por tracción en el contexto clínico especifico puede ser suficiente para realizar el diagnóstico de fibrosis pulmonar idiopática.Usual interstitial pneumonia pattern (UIP) in high-resolution computed tomography (HRCT) has a high degree of correlation with the histological pattern which makes it possible to obviate in these cases the need for lung biopsy. The accuracy of a UIP diagnosis in HRCT is based on the detection of specific signs of fibrosis such as honeycomb and reticular densities with traction bronchiolectasis. The update of the 2018 Clinical Practice Guidelines of the ATS / ERS / JRS / ALAT of Idiopathic Pulmonary Fibrosis (IPF) and the Fleischner Society White Paper proposed a new way of classifying the tomographic patterns taking into account the aspects previously considered. Although the presence of honeycomb remains the main finding to characterize the UIP pattern, the distribution of interstitial reticulate and the presence of traction bronchiolectasis, in the specific clinical context, may be sufficient to make the diagnosis of idiopathic pulmonary fibrosis.
       
  • Histopathological criteria for the diagnosis of idiopathic pulmonary
           fibrosis

    • Abstract: Resumen En la última actualización de las Guías de Práctica Clínica de la ATS/ERS/JRS/ALAT de fibrosis pulmonar idiopática (FPI) se propone una nueva forma de clasificar los patrones histopatológicos en 4 tipos: definitivo de neumonía intersticial usual (NIU), probable NIU, indeterminado y alternativo a NIU. Una remodelación fibrótica heterogénea de la arquitectura normal del parénquima pulmonar, con cicatrización destructiva en forma de “panal de abejas”, presencia de focos fibroblásticos y distribución predominantemente subpleural y paraseptal, con escaso infiltrado inflamatorio intersticial de tipo crónico, asociado a la ausencia de elementos sugerentes de causas secundarias como distribución bronquiolocéntrica, predominio de infiltrados intersticiales inflamatorios o granulomas mal formados, permite un diagnóstico certero de FPI en un escenario clínico-radiológico adecuado.In the latest update of the ATS/ERS/JRS/ALAT Clinical Practice Guidelines for idiopathic pulmonary fibrosis (IPF), a new way of classifying histopathological patterns in 4 types is proposed: definitive usual interstitial pneumonia (UIP), probable UIP, indeterminate and alternative to UIP. A heterogeneous fibrotic remodeling of the normal architecture of the pulmonary parenchyma, with destructive scarring in the form of “honeycomb”, presence of fibroblastic foci and predominantly subpleural and paraseptal distribution, with scarce chronic interstitial inflammatory infiltrate, associated with the absence of elements suggestive of secondary causes such as bronchiolocentric distribution, predominance of inflammatory interstitial infiltrates or poorly formed granulomas, allows an accurate diagnosis of IPF in an appropriate clinical-radiological scenario.
       
  • Rheumatological evaluation of the patient with interstitial lung disease

    • Abstract: Resumen Las Enfermedades del Tejido Conectivo (ETC) comprenden un grupo heterogéneo de patologías multisistémicas de origen autoinmune. La Enfermedad pulmonar intersticial (EPI) asociada a ETC (EPI-ETC) es frecuente y empeora el pronóstico de la ETC. Las EPI-ETC representan aproximadamente 15-30% del total las EPI y se presentan con las mismas formas histopatológicas y radiológicas descritas para las EPI idiopáticas. Esto pone en evidencia la importancia de incorporar en forma rutinaria a reumatología en el comité multidisciplinario para el diagnóstico y manejo de las EPI.Connective Tissue Diseases (CTD) comprise a heterogeneous group of multisystemic pathologies of autoimmune origin. Interstitial lung disease (ILD) associated with CTD (CTD-ILD) is common and and it worsens the prognosis of CTD. CTD-ILD represent approximately 15-30% of the universe of ILD and have the same histopathological and radiological forms described for idiopathic ILD. This highlights the importance of routinely incorporate a rheumatologist into the multidisciplinary committee for the diagnosis and management of ILD.
       
  • Multidisciplinary discussion for the diagnosis of idiopathic pulmonary
           fibrosis

    • Abstract: Resumen Antes de la publicación de la clasificación ATS/ERS 2002 de las neumonías intersticiales idiopáticas (NII), la evaluación histopatológica se consideraba la referencia de oro para el diagnóstico de las enfermedades pulmonares intersticiales (EPI). Sin embargo, varios estudios posteriores mostraron que las concordancias interobservador entre anatomopatólogos expertos torácicos eran sorprendentemente pobres ya que las apariencias histopatológicas pueden superponerse entre entidades distintas. Por lo anterior, se hace necesario un nuevo sistema diagnóstico que sirva de patrón de oro en pacientes con EPI. Es así como nace el concepto de discusión multidisciplinaria, para referirse a una reunión que permita la integración de todos los datos clínicos, radiológicos y patológicos disponibles para un paciente individual y así poder determinar un diagnóstico de trabajo.Prior to the publication of the 2002 ATS / ERS classification of idiopathic interstitial pneumonias (IIP), the histopathological evaluation was considered the gold standard for the diagnosis of interstitial lung diseases (ILD). However, several subsequent studies showed that interobserver concordances between expert lung pathologists were surprisingly poor, since histopathological appearances may overlap between different entities. Therefore, a new diagnostic system that serves as a gold standard in patients with ILD became necessary. This is how the concept of multidisciplinary discussion was born, to refer to a meeting that allows the integration of all the clinical, radiological and pathological data available for an individual patient and thus be able to determine a working diagnosis.
       
  • Pharmacological treatment in the FPI. Disease modifying drugs

    • Abstract: Resumen La historia natural de la fibrosis pulmonar idiopática (FPI) es heterogénea e impredecible. Aunque el curso de la enfermedad, sin tratamiento, es inevitablemente progresiva y de mal pronóstico. Los tratamientos históricos han variado desde corticosteroides e inmunosupresores (azatioprina, ciclofosfamida), hasta colchicina y N-acetilcisteína. En las últimas décadas se han realizado múltiples ensayos terapéuticos fallidos. Sin embargo, desde el año 2014 en los Estados Unidos, Europa y otros países, dos drogas, denominadas terapia antifibrótica o modificadoras de la enfermedad, están aprobadas para el tratamiento de la FPI: nintedanib y pirfenidona. La terapia antifibrótica, tiene como objetivo enlentecer en hasta 50% la declinación de la función pulmonar en pacientes con FPI.The natural history of idiopathic pulmonary fibrosis (IPF) is heterogeneous and unpredictable. The course of the disease without treatment, is inevitably progressive, with a poor prognosis. Historical treatments have varied from corticosteroids and immunosuppressants (azathioprine, cyclophosphamide), to colchicine and N-acetylcysteine. In the last decades, multiple failed therapeutic trials have been carried out. However, since 2014 in the United States, Europe and other countries, two drugs, called antifibrotic therapy or disease modifiers, are approved for the treatment of IPF: nintedanib and pirfenidone. The purpose of antifibrotic therapy is to slow down the decline in lung function in patients with IPF up to 50%.
       
  • Adjuvant therapies to antifibrotic therapy in IPF. Importance of
           multidisciplinary management

    • Abstract: Resumen La fibrosis pulmonar idiopática (FPI) se caracteriza por presentar una capacidad funcional reducida, disnea e hipoxia inducida por el ejercicio, lo que disminuye su tolerancia al esfuerzo y limita su capacidad de realizar actividades diarias. Las comorbilidades son frecuentes y su presencia contribuyen al empeoramiento de la calidad de vida y aumento de la mortalidad. Por lo anterior, es que además de las terapias antifibróticas, los pacientes con FPI se benefician de un enfoque integral de la atención que puede incluir: pesquisa, diagnóstico y tratamiento de comorbilidades, ingreso a protocolos de investigación, manejo sintomático, cuidados paliativos, oxígeno suplementario, rehabilitación pulmonar, educación y apoyo por un equipo multidisciplinario.Idiopathic pulmonary fibrosis (IPF) is characterized by reduced functional capacity, dyspnea and exercise-induced hypoxia, which decreases tolerance to exertion and limits the ability to perform daily activities. Comorbidities are frequent and their presence contribute to worsening quality of life and increased mortality. Therefore, in addition to antifibrotic therapies, patients with IPF benefit of a comprehensive approach to care that may include: screening, diagnosis and treatment of comorbidities, admission to research protocols, symptomatic management, palliative care, supplementary oxygen, pulmonary rehabilitation, education and support by a multidisciplinary team.
       
  • How to monitor patients with idiopathic pulmonary fibrosis'

    • Abstract: Resumen La fibrosis pulmonar idiopática (FPI) se ha clasificado en enfermedad leve o temprana-moderada-severa o Avanzada, sin puntos de corte en parámetros clínicos, funcionales o imagenológicos. No existe aún consenso en cual es el principal parámetro que se debe medir. Si bien las variables funcionales como la capacidad vital forzada (CVF), capacidad de difusión de monóxido de carbono (DLCO) y test de caminata de 6 minutos se han utilizado de forma rutinaria en la practica clínica y en los principales estudios clínicos de tratamiento muchas veces no son representativos de la evolución clínica. Por lo anterior se han desarrollado, índices o puntajes compuestos como la escala GAP (Gender-Age-Physiology) que podrían ser útiles en el seguimiento de los pacientes.Idiopathic pulmonary fibrosis (IPF) has been classified as mild or early - moderate - severe or advanced disease, with no cut-off points in clinical, functional or imaging parameters. There is no consensus yet on which is the main parameter to be measured although the functional variables such as forced vital capacity (FVC), carbon monoxide diffusion capacity (DLCO) and 6-minute walk test, have been routinely used in clinical practice and in the main clinical studies of treatment, are often not representative of the clinical evolution. Therefore, composite indices or scores such as the GAP (Gender-Age-Physiology) scale have been developed that could be useful in the follow-up of patients.
       
  • Lung transplant in patients with idiopathic pulmonary fibrosis

    • Abstract: Resumen El trasplante pulmonar representa una opción terapéutica en pacientes con fibrosis pulmonar avanzada que presentan un curso progresivo a pesar de tratamiento médico, mejorando su sobrevida y calidad de vida. Es de vital importancia que el neumólogo conozca cuando referir a un paciente y estar familiarizado con los requisitos necesarios y contraindicaciones para enviar un paciente a un centro de trasplante. La derivación oportuna permite realizar un completo estudio, además de identificar las contraindicaciones y condiciones clínicas susceptibles de modificar y/o corregir antes de un trasplante.Pulmonary transplantation represents a therapeutic option in patients with advanced pulmonary fibrosis who have a progressive course despite medical treatment, improving their survival and quality of life. It is vital that the pulmonologist knows when to refer a patient and be familiar with the requirements and contraindications. Timely referral grants the necessary time for a complete study, in addition to identifying contraindications and clinical conditions that could be modified and / or corrected before a transplant.
       
  • Acute exacerbations in patients with idiopathic pulmonary fibrosis

    • Abstract: Resumen Clásicamente entendemos como exacerbación de la Fibrosis pulmonar idiopática (FPI) a un deterioro respiratorio, clínicamente significativo, sin causa evidente. En la actualidad se prefiere el concepto de “exacerbación aguda gatillada” para referirnos a aquella que se genera en el contexto de infección, aspiración, toxicidad por drogas, tromboembolismo pulmonar, insuficiencia cardiaca o posterior a procedimientos invasivos. Mientras que se reserva el termino de “exacerbación aguda idiopática” a aquella en la que no encontramos un gatillante. El pronóstico es ominoso, con mortalidad elevada, con cifras que fluctúan entre 50-90% dependiendo de la necesidad de soporte ventilatorio. Por lo que muchas veces una exacerbación aguda puede ser el evento final de un paciente con FPI. El tratamiento no es del todo claro, no existe evidencia robusta del beneficio de terapias, históricamente los corticoides se han utilizados como terapia estándar, sin embargo la evidencia actual cuestiona los beneficios de dicho tratamiento.Classically we understand as an exacerbation of Idiopathic Pulmonary Fibrosis (IPF) to a clinically significant respiratory deterioration, without obvious cause. At present, the concept of “acute triggered exacerbations” is preferred to refer to those that are generated in the context of infection, aspiration, drug toxicity, pulmonary thromboembolism, heart failure or after invasive procedures. While the term “idiopathic acute exacerbations” is reserved for those in which we do not find a trigger. The prognosis is ominous and the mortality is high, with figures that fluctuate between 50 to 90% depending on the need for ventilatory support. Many times an acute exacerbation can be the final event of a patient with IPF. The treatment is not entirely clear, there is no robust evidence of the benefit of therapies, historically corticosteroids have been used as standard therapy, however current evidence questions the benefits of such a treatment.
       
 
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