Effectiveness and safety of pirfenidone for idiopathic pulmonary fibrosis.

OBJECTIVES: To assess the long-term effectiveness of pirfenidone in idiopathic pulmonary fibrosis (IPF) treatment and to establish its adverse effects profile. METHODS: Retrospective observational study in patients with IPF who initiated treatment with pirfenidone between 2011 and 2016. We collected demographic variables (age, sex); date of first and last treatment; reason for discontinuation; pulmonary function measures (forced vital capacity (FVC), carbon monoxide diffusion capacity (DLCO), and 6 min walk test (6MWT)) at treatment initiation (baseline) and at 1, 2 and 3 year follow-up; adherence to pirfenidone treatment; recorded adverse effects; and mortality. RESULTS: Thirty-one patients treated with pirfenidone were included; mean±SD age was 69±8 years, 74% were men, and 59% had a smoking history. Mean baseline values were: FVC 2.43±0.66 L (61.8±12.1%); DLCO 46.1±19.4%; and 6MWT 334±125 m. Median duration of treatment was 14±13 months, and treatment was discontinued in 58% of patients. The most frequently observed adverse effects were gastrointestinal disturbances and photosensitivity. Twenty (65%) patients were evaluated at 1 year, when mean FVC was 2.41±0.86 L (64.7±20.3%); DLCO 50.8±26.8%; and 6MWT 341±139 m. At 2 years' follow-up, 11 patients (36%) who were still taking pirfenidone were evaluated. Mean FVC was 2.34±0.79 L (66.2±14.7%); DLCO 50.0±28.3%; and 6MWT 265±121 m. At 3 years, five patients were still taking the treatment. Mean FVC was 2.71±0.84 L (71.0±24.7%); DLCO 52.6±26.7%; and 6MWT 286±139 m. Nineteen per cent of patients were non-adherent to treatment. CONCLUSIONS: Pirfenidone seems to be effective for long-term control of IPF despite substantial variability in response among individual patients. The most frequent adverse effects were digestive and cutaneous, prompting in some cases a reduction in dose or even discontinuation of the treatment.

Urinotórax e insuficiencia renal crónica: una rara asociación / Urinothorax and Chronic Renal Failure: A Rare Combination

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Insuficiencia respiratoria aguda secundaria a gota tofácea crónica con afectación laríngea y bronquial: una complicación excepcional / Acute Respiratory Failure Due to Chronic Tophaceous Gout With Laryngeal and Bronchial Involvement: An Unusual Complication

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Neumonía por S. aureus y artritis séptica esternoclavicular, una complicación insólita / S. aureus pneumonia and sternoclavicular septic arthritis: An unusual complication

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Empiema necessitatis por Corynebacterium jeikeium: preguntas del s. xix, respuestas del s. xxi / Empyema necessitatis caused by Corynebacterium jeikeium: 19th century questions, 21st century answers

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Adaptación y validación al español del cuestionario de calidad de vida OSA-18 para la evaluación del síndrome de apnea-hipopnea de sueño infantil / Adaptation and Validation of the Spanish Version of OSA-18, a Quality of Life Questionnaire for Evaluation of Children with Sleep Apnea-Hypopnea Syndrome

Objetivos: Adaptar al español y analizar la fiabilidad y la validez de la versión española del cuestionario OSA-18 de calidad de vida (CVRS) en el síndrome de apnea-hipopnea del sueño (SAHS) infantil. Método: Se incluyeron niños con sospecha de SAHS a los que se practicó polisomnografía (PSG) pre y post adenoamigdalectomía (AA). Se analizó: edad, género, clínica, PSG, datos antropométricos, grados de Brodsky y Mallampati. Se administró OSA-18 basal y entre 3-6meses post AA. Tras la traducción-retrotraducción por personas bilingües se evaluaron la consistencia interna, la fiabilidad, la validez de constructo, la validez concurrente, la validez predictiva y la sensibilidad a los cambios. Resultados: Se evaluaron 45 niños y 15 niñas: IMC18 ± 4, cuello 28 ± 5, Brodsky (0: 7%; < 25%: 12%; 25-50%: 27%; > 50 a < 75%: 45%; > 75%: 6%), IAH: 12 ± 7 pre AA. El alfa de Cronbach del global fue 0,91. Las correlaciones entre dominios fueron significativas salvo para aspectos emocionales, aunque el global se correlacionó con todos los dominios (0,50-0,90). El análisis factorial mostró una estructura prácticamente idéntica al original. En la validez concurrente, el global mostró buena correlación (0,2-0,45). En su validez predictiva, diferenció adecuadamente los niveles de gravedad según Mallampati (ANOVA p = 0,002) e índice apnea-hipopnea (ANOVA p = 0,006). La sensibilidad al cambio fue excelente, tanto en el global (p < 0,001) como en cada dominio (p < 0,001), así como la fiabilidad test-retest. Conclusiones: La adaptación española del OSA-18 es comprensible y sus características psicométricas sugieren que la versión española es equivalente a la original y puede ser empleada en países de habla hispana

Objectives: To analyze the reliability and validity of the Spanish version of the OSA-18 quality of life questionnaire in children with apnea-hypopnea syndrome (SAHS). Method: Children with suspected SAHS were studied with polysomnography (PSG) before and after adenotonsillectomy (AA). Age, gender, clinical data, PSG, anthropometric data, and Mallampati and Brodsky scales were analyzed. OSA-18 was administered at baseline and 3-6months post AA. After translation and backtranslation by bilingual professionals, the internal consistency, reliability, construct validity, concurrent validity, predictive validity and sensitivity to change of the questionnaire was assessed. Results: In total, 45 boys and 15 girls were evaluated, showing BMI 18 ± 4, neck 28 ± 5, Brodsky (0: 7%; < 25%: 12%; 25-50%: 27%; > 50 to < 75%: 45%; > 75%: 6%), AHI 12 ± 7 pre AA. Global Cronbach alpha was 0.91. Correlations between domains were significant except for emotional aspects, although the total scores correlated with all domains (0.50 to 0.90). The factorial analysis was virtually identical to the original structure. The total scores showed good correlation for concurrent validity (0.2-0.45). With regard to predictive validity, the questionnaire adequately differentiated levels of severity according to Mallampati (ANOVA P = .002) and apnea-hypopnea index (ANOVA P = .006). Test-retest reliability was excellent, as was sensitivity to change, both in the total scores (P < .001) and in each domain (P < .001). Conclusions: The Spanish adaptation of the OSA-18 and its psychometric characteristics suggest that the Spanish version is equivalent to the original and can be used in Spanish-speaking countries

Adaptation and Validation of the Spanish Version of OSA-18, a Quality of Life Questionnaire for Evaluation of Children with Sleep Apnea-Hypopnea Syndrome. / Adaptación y validación al español del cuestionario de calidad de vida OSA-18 para la evaluación del síndrome de apnea-hipopnea de sueño infantil.

OBJECTIVES: To analyze the reliability and validity of the Spanish version of the OSA-18 quality of life questionnaire in children with apnea-hypopnea syndrome (SAHS). METHOD: Children with suspected SAHS were studied with polysomnography (PSG) before and after adenotonsillectomy (AA). Age, gender, clinical data, PSG, anthropometric data, and Mallampati and Brodsky scales were analyzed. OSA-18 was administered at baseline and 3-6months post AA. After translation and backtranslation by bilingual professionals, the internal consistency, reliability, construct validity, concurrent validity, predictive validity and sensitivity to change of the questionnaire was assessed. RESULTS: In total, 45 boys and 15 girls were evaluated, showing BMI 18±4, neck 28±5, Brodsky (0: 7%; <25%: 12%; 25-50%: 27%; >50 to <75%: 45%; >75%: 6%), AHI 12±7 pre AA. Global Cronbach alpha was 0.91. Correlations between domains were significant except for emotional aspects, although the total scores correlated with all domains (0.50 to 0.90). The factorial analysis was virtually identical to the original structure. The total scores showed good correlation for concurrent validity (0.2-0.45). With regard to predictive validity, the questionnaire adequately differentiated levels of severity according to Mallampati (ANOVA P=.002) and apnea-hypopnea index (ANOVA P=.006). Test-retest reliability was excellent, as was sensitivity to change, both in the total scores (P<.001) and in each domain (P<.001). CONCLUSIONS: The Spanish adaptation of the OSA-18 and its psychometric characteristics suggest that the Spanish version is equivalent to the original and can be used in Spanish-speaking countries.

Leiomiomatosis pulmonar benigna metastatizante, una causa excepcional de nódulos pulmonares / Pulmonary benign metastasizing leiomyoma, a rare cause of pulmonary nodules

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