ABSTRACT
La fibrosis pulmonar idiopática (FPI) es una enfermedad crónica que afecta principalmente a adultos a partir de la quinta década de la vida. Se caracteriza por la presencia de disnea progresiva, declinación funcional sostenida y una supervivencia media al diagnóstico menor de tres años. Hasta ahora, el foco del tratamiento ha sido la mejoría de la supervivencia, pero para muchos pacientes, una mejoría en la calidad de vida (QoL) es un objetivo más realista. A pesar del importante impacto que la enfermedad tiene sobre los aspectos físicos, emocionales y sociales, la investigación enfocada en la QoL es muy limitada. Actualmente, no existe ningún instrumento que esté totalmentevalidado como apropiado en la evaluación de la QoL específicamente en pacientes con FPI. Los instrumentos de medición genéricos que se han utilizado en los pacientes con FPI incluyen el instrumento de 100 ítems de la OMS (WHOQoL-100), la escala de calidad debienestar (QWB) y el instrumento de 36 ítems (SF-36), ninguno de los cuales fue diseñado específicamente para pacientes con FPI. En los estudios revisados, los pacientes con FPIpresentaron afectación de la HRQoL en casi todos los dominios de la vida, pero los más afectados fueron los relacionados con el funcionamiento físico, los síntomas y niveles de independencia. Se requiere de más investigaciones para aprender sobre cómo esta enfermedad afecta a nuestros pacientes, si se adaptan a su enfermedad a lo largo del tiempo y, si lo hacen, de qué manera. Además, se necesita demostrar si alguno de losinstrumentos de medición de QoL existentes es confiable y válido para evaluar la HRQoL a lo largo del tiempo en la población de pacientes con FPI.
Idiopathic pulmonary fibrosis (IPF) is a chronic disease that primarily affects adults from the fifth decade of life. It is characterized by progressive dyspnea, sustained functional decline and survival of less than three years after diagnosis. So far, the treatment has focused on improving survival, but for many patients, an improvement in quality of life (QoL) is a more realistic goal. Despite the significant impact the disease has on physical, emotional and social features of the patients life, research focused on QoL is very limited.Currently, there are no fully validated instruments appropriate for the specific evaluation of QoL in patients with IPF. Generic measurement tools used in patients with IPF include the WHO 100 items instrument (WHOQOL-100), the quality scale of well-being (QWB)and the 36 items instrument (SF-36), but none of them was designed specifically for patients with IPF. In the reviewed studies, patients with IPF showed impairment of the health related QoL in almost all aspects of life, but those related to physical functioning,symptoms and levels of independence were the most affected ones. More research is needed to learn about how this disease affects IPF patients, whether they adapt themselves to their disease over time, and if so, how. Furthermore, research needs to showwhether the QoL measurement tools available are reliable and valid measures for health related QoL over time among patients with IPF.
Subject(s)
Humans , Idiopathic Pulmonary Fibrosis , Quality of Life , Disease Progression , Prognosis , Reproducibility of ResultsABSTRACT
The epidemiology and prognosis of bronchioalveolar carcinoma (BAC) is different from adenocarcinoma. We studied 28 patients who underwent surgical resection and in whom a final histologic diagnosis of bronchioalveolar carcinoma was made. Sixty one percent of patients were asymptomatic at the time of diagnosis. The most usual radiological presentations were pulmonary nodule (11), pulmonary mass (6) and diffuse infiltrate (6). Nine patients (9/25) were never smokers. Bronchoscopy provided the diagnosis only in 4/26 patients. Patients were classified as stage IA 11/24, IB 5/24 IIIB in 2/24 and IV in 6/24. Five-year survival for the entire group was 65%. Patients with focal lesions had a longer survival. The survival in patients who underwent potentially curative resections was 62.8 +/- 44.0 months. When surgery was considered non-curative, survival was 21.2 +/- 19.5 months (p = 0.0064). Five-year survival in stage IA patients was 80%. Survival of patients with non-mucinous histology was longer than those with mucinous type carcinomas (54.9 +/- 25.0 months vs. 5.33 +/- 4.04, p < 0.0001) but the 5-year-survival was not statistically different. In conclusion, BAC has a favourable prognosis. The best survival rate was found in focal forms, represented by early tumor, nodes, metastasis (TNM) stages and non-mucinous BAC among the histological groups.
Subject(s)
Female , Humans , Male , Middle Aged , Adenocarcinoma, Bronchiolo-Alveolar/mortality , Lung Neoplasms/mortality , Adenocarcinoma, Bronchiolo-Alveolar/pathology , Adenocarcinoma, Mucinous/mortality , Adenocarcinoma, Mucinous/pathology , Bronchoscopy , Kaplan-Meier Estimate , Neoplasm Staging , Lung Neoplasms/pathology , Prognosis , Survival RateABSTRACT
A group of pulmonologists from different sites of Argentina convened to establish consensus guidelines for treatment of acute and chronic bronchial asthma. General acceptance that in fatal asthma diagnosis and hospital admission are usually too late and treatment insufficient prompted the need for this meeting. The purpose of treatment was devised to keep the patient symptomless, decrease frequency of exacerbations and the risk of severe attacks. Peak expiratory flow rate (PEFR) measurement in all patients was decided. inhalation of anti-inflammatory drugs (corticosteroids, CE, and/or disodium cromoglycate, DSG, in those younger than 20 years) was established as first line of treatment. Inhaled CE (even in high doses such as 2 mg/day) do not provoke significant adverse systemic effects (immune depression, Cushing syndrome, hyperglycemia in diabetics or osteopenia). Secondary local adverse effects are however frequent: oral and pharyngeal candidiasis and dysphonia. It is advisable considering present evidence, that bronchodilators (Bd) be used preferentially on demand. On account of small bronchodilator effect and frequent secondary adverse effects, use of theophylline should be limited to patients not adequately responsive to anti-inflammatory drugs in high dosage. Immunotherapy is not useful in asthma. Four clinical levels were defined in chronic asthma considering severity of dyspnea, frequency of nocturnal bronchial obstruction, levels of PEFR and amount of required Bd. Guidelines of treatment were established for each clinical level considering increasing dosage of CGS, inhaled CE (up to 2 mg/day) and regular administration of Bd. Indications for systemic CE administration were also established. Three levels of acute asthma (sudden worsening of symptoms) were accepted based on clinical evidence and PEFR values. Treatment was quantitatively adjusted to severity. Criteria for hospital admission either in emergency or intensive care areas and treatment procedures were established.