Usefulness of Muscle Ultrasonography in the Nutritional Assessment of Adult Patients with Cystic Fibrosis

Muscle ultrasonography of the quadriceps rectus femoris (QRF) is a technique on the rise in the assessment of muscle mass in application of nutritional assessment. The aim of the present study is to assess the usefulness of muscle ultrasonography in patients with cystic fibrosis, comparing the results with other body composition techniques such as anthropometry, bioelectrical impedance analysis (BIA), dual-energy X-ray absorptiometry (DXA), and handgrip strength (HGS). At the same time, we intend to assess the possible association with the nutritional and respiratory status. Methods: This was a prospective observational study in adult patients with cystic fibrosis in a clinically stable situation. Muscle ultrasonography of the QRF was performed, and the results were compared with other measures of body composition: anthropometry, BIA, and DXA. HGS was used to assess muscle function. Respiratory parameters were collected, and nutritional status was assessed using Global Leadership Initiative on Malnutrition (GLIM) criteria. Results: A total of 48 patients were included, with a mean age of 34.1 ± 8.8 years. In total, 24 patients were men, and 24 patients were women. Mean BMI was 22.5 ± 3.8 kg/m2. Mean muscular area rectus anterior (MARA) was 4.09 ± 1.5 cm2, and mean muscular circumference rectus was 8.86 ± 1.61 cm. A positive correlation was observed between the MARA and fat-free mass index (FFMI) determined by anthropometry (r = 0.747; p < 0.001), BIA (r = 0.780; p < 0.001), and DXA (r = 0.678; p < 0.001), as well as muscle function (HGS: r = 0.790; p < 0.001) and respiratory parameters (FEV1; r = 0.445, p = 0.005; FVC: r = 0.376, p = 0.02; FEV1/FVC: r = 0.344, p = 0.037). A total of 25 patients (52.1%) were diagnosed with malnutrition according to GLIM criteria. Differences were observed when comparing the MARA based on the diagnosis of malnutrition (4.75 ± 1.65 cm2 in normo-nourished vs. 3.37 ± 1.04 in malnourished; p = 0.014). (...)This study was partially funded by an unrestricted grant from Vegenat Laboratories (Spain) and the Fundación SEEN-Nutricia 2021 Grant for the assessment of body composition by ultrasound. Partial funding for open access charge: Universidad de Málag

Validation of a Spanish version of the Leicester Cough Questionnaire in cystic fibrosis

Bronchiectasis; Quality of life; Respiratory diseaseBronquiectasia; Calidad de vida; Enfermedad respiratoriaBronquiectàsia; Qualitat de vida; Malaltia respiratòriaCough is a main symptom in cystic fibrosis (CF). We aim to validate a Spanish version of the Leicester Cough Questionnaire (LCQ-Sp) to measure the impact of cough in CF bronchiectasis. A prospective longitudinal multicentre study was performed. Internal consistency and score changes over a 15-day period in stable state were assessed to analyse reliability. Concurrent validity was analysed by correlation with Saint George’s Respiratory Questionnaire (SGRQ) and convergent validity by assessing the association with clinical variables. Changes in scores between stable state and the first exacerbation were assessed to analyse responsiveness. 132 patients (29.73 ± 10.52 years) were enrolled in four hospitals. Internal consistency was high for the total score and good for the three domains (Cronbach’s α 0.81–0.93). The test–retest reliability showed an intraclass correlation coefficient of 0.86 for the total score. The correlation between LCQ-Sp and SGRQ scores was −0.74. The LCQ-Sp score negatively correlated with sputum volume, and the mean score decreased at the beginning of exacerbations (16.04±3.81 vs 13.91±4.29) with a large effect size. The LCQ-Sp is a reliable, repeatable and responsive instrument to assess the impact of cough in CF bronchiectasis and is responsive to change in the event of exacerbations.The author(s) received no financial support for the research, authorship, and/or publication of this article

Sex bias in diagnostic delay in bronchiectasis : An analysis of the Spanish Historical Registry of Bronchiectasis

Diagnostic delay is common in most respiratory diseases, particularly in bronchiectasis. However, sex bias in diagnostic delay has not been studied to date. Objective: Assessment of diagnostic delay in bronchiectasis by sex. Methods: The Spanish Historical Registry of Bronchiectasis recruited adults diagnosed with bronchiectasis from 2002 to 2011 in 36 centres in Spain. From a total of 2113 patients registered we studied 2099, of whom 1125 (53.6%) were women. Results: No differences were found for sex or age (61.0 ± 20.6, p = 0.88) or for localization of bronchiectasis (p = 0.31). Bronchiectasis of unknown aetiology and secondary to asthma, childhood infections and tuberculosis was more common in women (all p s 2 years). Independent factors associated with this sex bias were age at onset of symptoms, smoking history, daily expectoration and reduced lung function

Sex bias in diagnostic delay in bronchiectasis: An analysis of the Spanish Historical Registry of Bronchiectasis

Diagnostic delay is common in most respiratory diseases, particularly in bronchiectasis. However, sex bias in diagnostic delay has not been studied to date. Objective: Assessment of diagnostic delay in bronchiectasis by sex. Methods: The Spanish Historical Registry of Bronchiectasis recruited adults diagnosed with bronchiectasis from 2002 to 2011 in 36 centres in Spain. From a total of 2113 patients registered we studied 2099, of whom 1125 (53.6%) were women. Results: No differences were found for sex or age (61.0 ± 20.6, p = 0.88) or for localization of bronchiectasis (p = 0.31). Bronchiectasis of unknown aetiology and secondary to asthma, childhood infections and tuberculosis was more common in women (all ps 2 years). Independent factors associated with this sex bias were age at onset of symptoms, smoking history, daily expectoration and reduced lung function

Nutrition, cystic fibrosis and the digestive tract

Comparative Study; English Abstract; Review;The prevalence of hyponutrition in cystic fibrosis is high although it may vary according to the different studies. Detection of hyponutrition should be done by combining different methods, depending on their availability. However, the simplest and most validated criterion is to measure at each visit the weight (and height in children) in order to calculate the body mass index and categorizing hyponutrition according to absolute criteria: in adults < 18.5 kg/m(2), and in children as percentiles of the body mass index. Worsening of the nutritional status is directly related with the decrease in lung function parameters and it has been proposed as a morbidity (and even mortality) predictive factor in people with cystic fibrosis, independently of the level of pulmonary dysfunction. Exocrine pancreatic insufficiency is present is approximately 70-90% of the patients with cystic fibrosis and the genotype-phenotype correlation is high. Most of the patients with exocrine pancreatic insufficiency tolerate a high-fat diet provided that they are treated with pancreatic enzymes at appropriate doses. The prevalence of diabetes increases with age, reaching up 40% of the cases in patients older than 30 years. Clinical liver involvement is less prevalent (it approximately affects 1/3 of the patients). Other intestinal complications such as meconial ileus, gastroesophageal reflux, obstruction of the distal intestine, or fibrosing colon disease may also condition malnourishment. In patients with cystic fibrosis, a usual high-fat diet providing 120%-150% of the recommended calories is advised. If the nutritional goals are not achieved or maintained with diet modifications, artificial supplements may be added, although the recommendation for their use has not been endorsed by solid scientific evidences. The most frequently used preparations usually are polymeric or hypercaloric. The indications for enteral (through a tube, especially gastrostomy) or parenteral nutritional support are similar to those used in other pathologies. Dietary and nutritional control should be included in a multidisciplinary program allowing the improvement of the functional capacity and the quality of life and reducing, at least from a theoretical viewpoint, the morbimortality associated to malnourishment in these patients.YesLa prevalencia de desnutrición en fibrosis quística es elevada aunque variable según los estudios. La detección de la misma debe realizarse mediante la combinación de diferentes métodos, en función de la disponibilidad de los mismos. No obstante, el criterio más sencillo y validado es medir en cada visita el peso (y la talla en niños)para calcular el índice de masa corporal y clasificar la desnutrición en criterios absolutos; en adultos, con valores menores a 18,5 kg/m2 y en niños, con percentiles del índice de masa corporal menores a 10. El empeoramiento del estado de nutrición guarda una relación directa con el descenso de los parámetros de función pulmonar y se ha propuesto como un factor predictor de morbilidad e incluso de mortalidad en personas con fibrosis quística, independientemente del grado de disfunción pulmonar. La insuficiencia pancreática exocrina está presente en aproximadamente el 70 al 90% de los pacientes con fibrosis quística y la correlación entre genotipo y fenotipo es alta. La mayoría de los pacientes con insuficiencia pancreática exocrina toleran una dieta alta en grasa si son tratados con enzimas pancreáticas a las dosis adecuadas. La prevalencia de diabetes aumenta con la edad alcanzando hasta el 40% de los casos en pacientes mayores de 30 años. La afectación hepática clínica es menos prevalente (aproximadamente afecta a 1/3 de los pacientes). Otras complicaciones intestinales como el íleo meconial, el reflujo gastroesofágico, la obstrucción del intestino distal o la colopatía fibrosante pueden condicionar también malnutrición. En los pacientes con fibrosis quística se recomienda que la ingesta habitual aporte entre el 120 y 150% de las calorías recomendadas y que contenga alto contenido en grasas. Si no consiguen alcanzar o mantener los objetivos nutricionales previstos con las modificaciones de la dieta, se pueden adicionar suplementos artificiales, si bien la recomendación de su empleo no está avalado en evidencias científicas sólidas. Los preparados más empleados suelen ser poliméricos e hipercalóricos. Las indicaciones de soporte nutricional enteral por sonda (especialmente gastrostomía) o parenteral son similares a las empleadas en otras patologías. El control dietético y nutricional debe incluirse en un programa multidisciplinar que permita mejorar la capacidad funcional, la calidad de vida y reducir, al menos teóricamente,la morbi-mortalidad asociada a la malnutrición en estos pacientes

Nutrición, fibrosis quística y aparato digestivo Nutrition, cystic fibrosis and the digestive tract

La prevalencia de desnutrición en fibrosis quística es elevada aunque variable según los estudios. La detección de la misma debe realizarse mediante la combinación de diferentes métodos, en función de la disponibilidad de los mismos. No obstante, el criterio más sencillo y validado es medir en cada visita el peso (y la talla en niños) para calcular el índice de masa corporal y clasificar la desnutrición en criterios absolutos; en adultos, con valores menores a 18,5 kg/m² y en niños, con percentiles del índice de masa corporal menores a 10. El empeoramiento del estado de nutrición guarda una relación directa con el descenso de los parámetros de función pulmonar y se ha propuesto como un factor predictor de morbilidad e incluso de mortalidad en personas con fibrosis quística, independientemente del grado de disfunción pulmonar. La insuficiencia pancreática exocrina está presente en aproximadamente el 70 al 90% de los pacientes con fibrosis quística y la correlación entre genotipo y fenotipo es alta. La mayoría de los pacientes con insuficiencia pancreática exocrina toleran una dieta alta en grasa si son tratados con enzimas pancreáticas a las dosis adecuadas. La prevalencia de diabetes aumenta con la edad alcanzando hasta el 40% de los casos en pacientes mayores de 30 años. La afectación hepática clínica es menos prevalente (aproximadamente afecta a 1/3 de los pacientes). Otras complicaciones intestinales como el íleo meconial, el reflujo gastroesofágico, la obstrucción del intestino distal o la colopatía fibrosante pueden condicionar también malnutrición. En los pacientes con fibrosis quística se recomienda que la ingesta habitual aporte entre el 120 y 150% de las calorías recomendadas y que contenga alto contenido en grasas. Si no consiguen alcanzar o mantener los objetivos nutricionales previstos con las modificaciones de la dieta, se pueden adicionar suplementos artificiales, si bien la recomendación de su empleo no está avalado en evidencias científicas sólidas. Los preparados más empleados suelen ser poliméricos e hipercalóricos. Las indicaciones de soporte nutricional enteral por sonda (especialmente gastrostomía) o parenteral son similares a las empleadas en otras patologías. El control dietético y nutricional debe incluirse en un programa multidisciplinar que permita mejorar la capacidad funcional, la calidad de vida y reducir, al menos teóricamente, la morbi-mortalidad asociada a la malnutrición en estos pacientes.The prevalence of hyponutrition in cystic fibrosis is high although it may vary according to the different studies. Detection of hyponutrition should be done by combining different methods, depending on their availability. However, the simplest and most validated criterion is to measure at each visit the weight (and height in children) in order to calculate the body mass index and categorizing hyponutrition according to absolute criteria: in adults < 18.5 kg/m², and in children as percentiles of the body mass index. Worsening of the nutritional status is directly related with the decrease in lung function parameters and it has been proposed as a morbidity (and even mortality) predictive factor in people with cystic fibrosis, independently of the level of pulmonary dysfunction. Exocrine pancreatic insufficiency is present is approximately 70-90% of the patients with cystic fibrosis and the genotype-phenotype correlation is high. Most of the patients with exocrine pancreatic insufficiency tolerate a high-fat diet provided that they are treated with pancreatic enzymes at appropriate doses. The prevalence of diabetes increases with age, reaching up 40% of the cases in patients older than 30 years. Clinical liver involvement is less prevalent (it approximately affects 1/3 of the patients). Other intestinal complications such as meconial ileus, gastroesophageal reflux, obstruction of the distal intestine, or fibrosing colon disease may also condition malnourishment. In patients with cystic fibrosis, a usual high-fat diet providing 120%-150% of the recommended calories is advised. If the nutritional goals are not achieved or maintained with diet modifications, artificial supplements may be added, although the recommendation for their use has not been endorsed by solid scientific evidences. The most frequently used preparations usually are polymeric or hypercaloric. The indications for enteral (through a tube, especially gastrostomy) or parenteral nutritional support are similar to those used in other pathologies. Dietary and nutritional control should be included in a multidisciplinary program allowing the improvement of the functional capacity and the quality of life and reducing, at least from a theoretical viewpoint, the morbimortality associated to malnourishment in these patients

Nutrition, cystic fibrosis and the digestive tract

Comparative Study; English Abstract; Review;The prevalence of hyponutrition in cystic fibrosis is high although it may vary according to the different studies. Detection of hyponutrition should be done by combining different methods, depending on their availability. However, the simplest and most validated criterion is to measure at each visit the weight (and height in children) in order to calculate the body mass index and categorizing hyponutrition according to absolute criteria: in adults < 18.5 kg/m(2), and in children as percentiles of the body mass index. Worsening of the nutritional status is directly related with the decrease in lung function parameters and it has been proposed as a morbidity (and even mortality) predictive factor in people with cystic fibrosis, independently of the level of pulmonary dysfunction. Exocrine pancreatic insufficiency is present is approximately 70-90% of the patients with cystic fibrosis and the genotype-phenotype correlation is high. Most of the patients with exocrine pancreatic insufficiency tolerate a high-fat diet provided that they are treated with pancreatic enzymes at appropriate doses. The prevalence of diabetes increases with age, reaching up 40% of the cases in patients older than 30 years. Clinical liver involvement is less prevalent (it approximately affects 1/3 of the patients). Other intestinal complications such as meconial ileus, gastroesophageal reflux, obstruction of the distal intestine, or fibrosing colon disease may also condition malnourishment. In patients with cystic fibrosis, a usual high-fat diet providing 120%-150% of the recommended calories is advised. If the nutritional goals are not achieved or maintained with diet modifications, artificial supplements may be added, although the recommendation for their use has not been endorsed by solid scientific evidences. The most frequently used preparations usually are polymeric or hypercaloric. The indications for enteral (through a tube, especially gastrostomy) or parenteral nutritional support are similar to those used in other pathologies. Dietary and nutritional control should be included in a multidisciplinary program allowing the improvement of the functional capacity and the quality of life and reducing, at least from a theoretical viewpoint, the morbimortality associated to malnourishment in these patients.YesLa prevalencia de desnutrición en fibrosis quística es elevada aunque variable según los estudios. La detección de la misma debe realizarse mediante la combinación de diferentes métodos, en función de la disponibilidad de los mismos. No obstante, el criterio más sencillo y validado es medir en cada visita el peso (y la talla en niños)para calcular el índice de masa corporal y clasificar la desnutrición en criterios absolutos; en adultos, con valores menores a 18,5 kg/m2 y en niños, con percentiles del índice de masa corporal menores a 10. El empeoramiento del estado de nutrición guarda una relación directa con el descenso de los parámetros de función pulmonar y se ha propuesto como un factor predictor de morbilidad e incluso de mortalidad en personas con fibrosis quística, independientemente del grado de disfunción pulmonar. La insuficiencia pancreática exocrina está presente en aproximadamente el 70 al 90% de los pacientes con fibrosis quística y la correlación entre genotipo y fenotipo es alta. La mayoría de los pacientes con insuficiencia pancreática exocrina toleran una dieta alta en grasa si son tratados con enzimas pancreáticas a las dosis adecuadas. La prevalencia de diabetes aumenta con la edad alcanzando hasta el 40% de los casos en pacientes mayores de 30 años. La afectación hepática clínica es menos prevalente (aproximadamente afecta a 1/3 de los pacientes). Otras complicaciones intestinales como el íleo meconial, el reflujo gastroesofágico, la obstrucción del intestino distal o la colopatía fibrosante pueden condicionar también malnutrición. En los pacientes con fibrosis quística se recomienda que la ingesta habitual aporte entre el 120 y 150% de las calorías recomendadas y que contenga alto contenido en grasas. Si no consiguen alcanzar o mantener los objetivos nutricionales previstos con las modificaciones de la dieta, se pueden adicionar suplementos artificiales, si bien la recomendación de su empleo no está avalado en evidencias científicas sólidas. Los preparados más empleados suelen ser poliméricos e hipercalóricos. Las indicaciones de soporte nutricional enteral por sonda (especialmente gastrostomía) o parenteral son similares a las empleadas en otras patologías. El control dietético y nutricional debe incluirse en un programa multidisciplinar que permita mejorar la capacidad funcional, la calidad de vida y reducir, al menos teóricamente,la morbi-mortalidad asociada a la malnutrición en estos pacientes

Depression and anxiety symptoms in bronchiectasis: associations with health-related quality of life

Bronchiectasis causes pulmonary infections and loss of lung function, resulting in chronic respiratory symptoms and worsening health-related quality of life. The aims of this study were to measure symptoms of depression and anxiety in a sample of patients with bronchiectasis and evaluate their relationship to health outcomes and health-related quality of life.This cross-sectional study included adolescents and adults with bronchiectasis. Patients completed the hospital anxiety and depression scale and the St. George respiratory questionnaire. Health outcome data, including clinical, radiological and spirometric values, were recorded from medical charts.Ninety-three participants with bronchiectasis of any aetiology were recruited: 20 % had elevated depression-related scores and 38 % had elevated anxiety-related scores. Increased symptoms of depression and anxiety were significantly associated with age; anxiety was associated with more frequent exacerbations. Regression analyses indicated that after controlling for demographic (gender and age) and clinical variables (exacerbations frequency, daily sputum, aetiology and spirometry), both depression and anxiety symptoms predicted significantly worse health-related quality of life. In comparison with other predictors, psychological symptoms explained the largest amount of variance in health-related quality of life.Symptoms of depression and anxiety were significant predictors of health-related quality of life in patients with bronchiectasis, independently of respiratory involvement, gender, age or other variables

Validación de la versión española del cuestionario revisado de calidad de vida para fibrosis quística en adolescentes y adultos (CFQR 14+ Spain)

Estudiar la validez y fiabilidad de la versión española del cuestionario revisado de calidad de vida para fibrosis quística (FQ) en adolescentes y adultos (CFQR 14+ Spain). Se estudiaron 43 adolescentes y adultos con FQ, clínicamente estables. Se utilizaron como medidas del estado de salud parámetros radiológicos, espirométricos, número de reagudizaciones, prueba de la marcha de 6 min, antropométricos (índice de masa corporal, desnutrición de masa magra), grasa en heces y el cuestionario respiratorio de St.George (SGRQ). El alfa de Cronbach fue ≥0,70 para todas las escalas, excepto para «síntomas digestivos» y «carga de tratamiento». Cuarenta ítems (de 50) presentaron correlaciones ítems-escala mayores a 0,70 y el 98% mayores a 0,40. La reproductibilidad test-retest (coeficiente de Spearman) osciló entre 0,49–0,95 y el coeficiente de correlación intraclase alcanzó puntuaciones mayores de 0,70 en 10 de 12 escalas. Todas las dimensiones correlacionaron significativamente con las puntuaciones del SGRQ. Se observaron correlaciones significativas entre las dimensiones del cuestionario y las variables respiratorias y nutricionales que pretendían medir y permitió diferenciar adecuadamente los distintos grados de gravedad en función de los parámetros respiratorios evaluados. La desnutrición y la malabsorción condicionaron significativamente peores puntuaciones solo en algunos dominios relacionados (como problemas con el peso). El efecto suelo fue menor al 15% en todas las dimensiones y el efecto techo fue elevado en 7 dimensiones. El cuestionario CFQR 14+ Spain es válido y fiable para su aplicación en España, aunque podría ser mejorado en algunas de las subescalas. The aim of this study was to assess the validity and reliability of the Spanish version of the revised disease-specific health related quality of life questionnaire for adolescents and adults with cystic fibrosis (CFQR 14+ Spain). A total of 43 cystic fibrosis (CF) patients completed the CFQR 14+ Spain. Forced expiratory volume in 1 second, in percentage of predicted - FEV 1 (%)-, number of respiratory exacerbations, 6-minute walk test, Bhalla score (based on computerized tomography of the chest), fat-free mass index, body mass index (BMI), faecal fat and St George's Respiratory Questionnaire were included as measurements of health status. Ten out of the twelve scales had alpha coefficients above 0.70. Test–retest correlations (Spearman) ranged from 0.49 to 0.95 and they were significant in all scales. Intraclass correlations ranged from 0.47 to 0.95 (ten out of the twelve scales were >0.70) forty out of the fifty ítems have correlations between items and scale above 0.70. All the CFQR+14 scales, except the digestive symptoms scale, discriminated significantly between patients with mild, moderate and severe disease (according to FEV 1 (%)). Other respiratory parameters also discriminated significantly between patients with mild-moderate and severe disease. Only some scales discriminated significantly between nourished and malnourished patients. All of the scales met standards for floor effects (<15% of the responders with the lowest score) but not for ceiling effects (only five out of the twelve). The Spanish CFQR14+ (Spain) is a reliable and valid instrument for measuring the health-related quality of life in Spanish adolescents and adults with CF, though with the exception of a few of its subscales