Prevalencia de aspergilosis broncopulmonar alérgica en niños con fibrosis quística / Prevalence of bronchopulmonary aspergillosis in children with cystic fibrosis

La aspergilosis broncopulmonar alérgica (ABPA) es una reacción de hipersensibilidad secundaria al Aspergillus fumigatus (Af) que complica la evolución en fibrosis quística (FQ). Existen pocos estudios pediátricos de su prevalencia publicados en el mundo y en Chile se desconoce. El objetivo de este trabajo fue estimar la prevalencia de ABPA en niños con FQ en un hospital de referencia, explorar factores de riesgo y describir los criterios diagnósticos, tratamiento y evolución. Se incluyeron retrospectivamente los niños con FQ atendidos en un hospital terciario en Santiago de Chile (Hospital Roberto del Río) entre los años 2011 a 2019, se identificaron aquellos con diagnóstico de ABPA. Se registraron criterios diagnósticos según la Cystic Fibrosis Foundation, presencia de factores de riesgo, tratamientos recibidos y efectos adversos. De 65 pacientes con FQ atendidos en este período, la prevalencia de ABPA fue del 12%. El promedio de edad al diagnóstico fue ± 11 años (5-17 años), predominando la edad adolescente y el género masculino. El 50% cumplieron con los criterios clásicos, el 87,5% usaron antibióticos y el 62,5% corticoides inhalados. La respuesta favorable al tratamiento inicial con corticoides y antifúngico vía oral fue 62,5%, con una exacerbación al momento del estudio. El 25% se comportaron como refractario y el 12,5% respondieron a tratamiento con pulsos de metilprednisolona. El 37,5% presentaron eventos adversos relacionados a corticoides. La prevalencia de ABPA observada es comparable a las series publicadas. Se necesitan trabajos prospectivos para conocer la prevalencia nacional y su tendencia a lo largo de los años, identificando factores de riesgo.

Allergic bronchopulmonary aspergillosis (ABPA) is a hypersensitivity response to Aspergillus fumigatus (Af) and worsens outcome in children with cystic fibrosis (CF). Its prevalence varies in the literature, but we do not know it in Chile. The aim of the study was to know the prevalence of ABPA in children with CF and to describe risk factors, diagnostic criteria, treatment and outcome. We included all patients with CF seen in a tertiary hospital in Santiago, Chile (Hospital Roberto del Río), between 2011 and 2019; ABPA cases (CF Foundation diagnostic criteria) were identified for the estimation of the prevalence. Risk factors, diagnostic criteria and treatment were recorded, as proposed by the Cystic Fibrosis Foundation. A total of 65 patients with CF were identified in the study period, with a prevalence of 12% (8 cases). Mean age at diagnosis ± 11 years (5-17), more frequent in adolescence and male. CF Foundation criteria diagnostic were identified in 50% of cases, with high frequency of antibiotic use (87,5%) and inhaled steroids (62,5%). Positive oral steroids and antifungal treatment response was 62,5%. Refractary response was 25% and 12,5% needed intravenous metilprednisolone pulses. A 37,5% of cases presented adverse effects to steroids. Prevalence of ABPA is comparable to literature. A prospective study is needed to identified national prevalence and trends, identifying risks factors.

[Characterization of the physical capacity in children of the Chilean National Program of Cystic Fibrosis]. / Caracterización de la capacidad física en niños del Programa Nacional de Fibrosis Quística de Chile.

INTRODUCTION: Cystic fibrosis (CF) is an inherited, progressive, multisystem disease. Better physical capacity may slow disease progression, thus improving prognosis and survival. The objective of this research was to evaluate the physical capacity of children admitted to the National CF Pro gram of the Metropolitan Region, Chile. PATIENTS AND METHOD: A multicenter, cross-sectional stu dy design was used. The inclusion criteria were children aged 6 to 12 years enrolled in the National CF Program; Tanner sexual maturity stage I, no respiratory exacerbations in the last 30 days, and no musculoskeletal pathologies. The maximum aerobic capacity was assessed through the peak oxygen uptake (VO2peak) and determined with an incremental protocol in a magnetic cycle ergometer connected to an ergo-spirometer with which, at the same time, respiratory gases, oxygen consumption and carbon dioxide production values every 30 seconds, anaerobic threshold, and maximum workload were analyzed. The values of forced vital capacity (FVC), forced expiratory volume in 1 second (FEVJ, FEVj/FVC ratio, and forced expiratory flows between 25% and 75% of vital capacity were assessed through ergo-spirometry. At the beginning of the ergo-spirometry, arterial oxygen saturation, respiratory rate, heart rate, blood pressure, tidal volume and the per ception of lower extremity fatigue and dyspnea were recorded using the modified Borg scale. The test lasted approximately 10 minutes. RESULTS: The clinical records of 43 children collected from six health centers were reviewed. Out of these, 29 children met inclusion criteria, and 23 were re cruited. Two children were unable to participate, reducing the final subject group to 21 (13 males, 8 females). The mean age was 8.8 ± 2 years; weight 30.5 ± 10.9 kg; height 1.32 ± 0.11 m; and body mass index 17.1 ± 3.5 (z-score 0.01 ± 1.34). More than half of the children (61%) had normal weight. The obtained VO2peak was 43.7 ± 6.5 ml/min/kg (106.7 ± 19.8% of the predictive values). Only 10% of the children had values lower than those predicted by sex and age. No correlations were found between VO2peak and anthropometric and pulmonary function variables. Conclu sion: Most of the evaluated children (90%) had physical capacity similar to healthy subjects by sex and age.

Caracterización de la capacidad física en niños del Programa Nacional de Fibrosis Quística de Chile / Characterization of the physical capacity in children of the Chilean National Program of Cystic Fibrosis

Resumen: Introducción: La fibrosis quística (FQ) es una enfermedad multisistémica hereditaria y progresiva. Una mejor capacidad física puede retardar la progresión de la enfermedad, mejorando así el pronós tico y la supervivencia. El objetivo de esta investigación fue evaluar la capacidad física de los niños admitidos en el programa nacional de FQ de la Región Metropolitana, Chile. Pacientes y Método: Se utilizó un diseño de estudio transversal multicéntrico. Los criterios de inclusión fueron: niños de 6 a 12 años de edad, incluidos en el Programa Nacional de FQ, madurez sexual Tanner I, ausencia de exacerbaciones respiratorias en los últimos 30 días y ausencia de enfermedades musculoesqueléticas. La capacidad aeróbica máxima fue evaluada a través del consumo pico de oxígeno (VO2pico) y se determinó con un protocolo incremental en un cicloergómetro magnético conectado a un ergoespirómetro en el que paralelamente se analizaron los gases respiratorios: valores de consumo de oxí geno y producción de dióxido de carbono cada 30 segundos, umbral anaeróbico y carga máxima de trabajo. Además, se evaluaron los valores de capacidad vital forzada (CVF), volumen espiratorio al primer segundo (VEFj), relación VEFj/CVF y los flujos espiratorios forzados entre el 25 y 75% de la capacidad vital. Durante la prueba se registró: saturación arterial de oxígeno, frecuencia respiratoria, frecuencia cardíaca, presión arterial, volumen corriente y se consultó la percepción de fatiga de extre midades inferiores y disnea a través de la escala de Borg modificada. La duración aproximada del test fue alrededor de 10 minutos. Resultados: Se revisaron los registros clínicos de 43 niños, recogidos en seis centros de salud. Veintinueve niños cumplieron los criterios de inclusión siendo 23 reclutados. Dos niños no pudieron participar, reduciendo el grupo final de sujetos a 21 (13:8 varones:mujeres). La edad media fue de 8,8 ± 2 años; el peso fue de 30,5 ± 10,9 kg; la talla fue de 1,32 ± 0,11 m y el índice de masa corporal fue de 17,1 ± 3,5 (z-score 0,01 ± 1,34). Más de la mitad (61%) de los niños estaba eutrófico. El VO2pico obtenido fue de 43,7 ± 6,5 ml/min/kg (106,7 ± 19,8% de los valores teóricos de referencia). Sólo el 10% de los niños tenían valores inferiores a los valores teóricos esperados para población normal, ajustados por sexo y edad. No se encontraron correlaciones entre el VO2pico y las variables antropométricas y de función pulmonar. Conclusión: La mayoría de los niños evaluados (90%) tenían capacidad física similar a los valores teóricos de referencia para niños sanos ajustados por sexo y edad.

Abstract: Introduction: Cystic fibrosis (CF) is an inherited, progressive, multisystem disease. Better physical capacity may slow disease progression, thus improving prognosis and survival. The objective of this research was to evaluate the physical capacity of children admitted to the National CF Pro gram of the Metropolitan Region, Chile. Patients and Method: A multicenter, cross-sectional stu dy design was used. The inclusion criteria were children aged 6 to 12 years enrolled in the National CF Program; Tanner sexual maturity stage I, no respiratory exacerbations in the last 30 days, and no musculoskeletal pathologies. The maximum aerobic capacity was assessed through the peak oxygen uptake (VO2peak) and determined with an incremental protocol in a magnetic cycle ergometer connected to an ergo-spirometer with which, at the same time, respiratory gases, oxygen consumption and carbon dioxide production values every 30 seconds, anaerobic threshold, and maximum workload were analyzed. The values of forced vital capacity (FVC), forced expiratory volume in 1 second (FEVj), FEVj/FVC ratio, and forced expiratory flows between 25% and 75% of vital capacity were assessed through ergo-spirometry. At the beginning of the ergo-spirometry, arterial oxygen saturation, respiratory rate, heart rate, blood pressure, tidal volume and the per ception of lower extremity fatigue and dyspnea were recorded using the modified Borg scale. The test lasted approximately 10 minutes. Results: The clinical records of 43 children collected from six health centers were reviewed. Out of these, 29 children met inclusion criteria, and 23 were re cruited. Two children were unable to participate, reducing the final subject group to 21 (13 males, 8 females). The mean age was 8.8 ± 2 years; weight 30.5 ± 10.9 kg; height 1.32 ± 0.11 m; and body mass index 17.1 ± 3.5 (z-score 0.01 ± 1.34). More than half of the children (61%) had normal weight. The obtained VO2peak was 43.7 ± 6.5 ml/min/kg (106.7 ± 19.8% of the predictive values). Only 10% of the children had values lower than those predicted by sex and age. No correlations were found between VO2peak and anthropometric and pulmonary function variables. Conclu sion: Most of the evaluated children (90%) had physical capacity similar to healthy subjects by sex and age.

Bone mineral density, lung function, vitamin D and body composition in children and adolescents with cystic fibrosis: a multicenter study.

BACKGROUND: cystic fibrosis (CF) is the most common inherited disease in Caucasian population. Nowadays, long survival has led to the emergence of new complications, such as CF bone disease (CFBD), which is characterized by increased fracture risk. OBJECTIVES: evaluate the association of bone mineral density (BMD) with lung function and BMD with 25-hydroxivitamin D (25OHD) plasmatic levels in children/adolescents with CF. METHODS: we conducted a multicenter, cross-sectional study with clinically stable CF patients between five and 18 years. Weight, height, pubertal development, BMD and body composition (DXA), pulmonary function (FEV1 and FEF25-75) and 25OHD plasmatic levels were measured. Patients answered food intake and physical activity surveys. p values under 0.05 were considered as statistically significant. RESULTS: thirty-seven patients were enrolled, 51% with normal respiratory function. Mean BMD Z-score in lumbar spine and in total body less head were -0.4 and -0.5 respectively. Twenty seven percent had a fat free mass index below the third percentile, 89% had 25OHD levels lower than 30 ng/ml and 78.4% had a low calcium intake. We did not find any correlations between BMD Z-scores, lung function or 25OHD levels. Patients with fat free mass (FFM) below the third percentile had BMD Z-score lower than -1 more frequently, in both locations (p < 0.006 and p < 0.001, respectively). CONCLUSIONS: although most assessed patients had normal BMD and normal lung function, a high proportion had low: FFM, calcium intake and 25OHD levels. The association between low FFM and low BMD highlights the importance of improving body composition in CF patients, in order to prevent future CFBD.

Bone mineral density, lung function, vitamin d and body composition in children and adolescents with cystic fibrosis: a multicenter study / Densidad mineral ósea, función pulmonar, vitamina D y composición corporal en niños y adolescentes con fibrosis quística: estudio multicéntrico

Background: cystic fibrosis (CF) is the most common inherited disease in Caucasian population. Nowadays, long survival has led to the emergence of new complications, such as CF bone disease (CFBD), which is characterized by increased fracture risk. Objectives: evaluate the association of bone mineral density (BMD) with lung function and BMD with 25-hydroxivitamin D (25OHD) plasmatic levels in children/adolescents with CF. Methods: we conducted a multicenter, cross-sectional study with clinically stable CF patients between five and 18 years. Weight, height, pubertal development, BMD and body composition (DXA), pulmonary function (FEV1 and FEF25-75) and 25OHD plasmatic levels were measured. Patients answered food intake and physical activity surveys. p values under 0.05 were considered as statistically significant. Results: thirty-seven patients were enrolled, 51% with normal respiratory function. Mean BMD Z-score in lumbar spine and in total body less head were -0.4 and -0.5 respectively. Twenty seven percent had a fat free mass index below the third percentile, 89% had 25OHD levels lower than 30 ng/ml and 78.4% had a low calcium intake. We did not find any correlations between BMD Z-scores, lung function or 25OHD levels. Patients with fat free mass (FFM) below the third percentile had BMD Z-score lower than -1 more frequently, in both locations (p < 0.006 and p < 0.001, respectively).Conclusions: although most assessed patients had normal BMD and normal lung function, a high proportion had low: FFM, calcium intake and 25OHD levels. The association between low FFM and low BMD highlights the importance of improving body composition in CF patients, in order to prevent future CFBD

Introducción: la fibrosis quística (FQ) es la enfermedad hereditaria más frecuente en la población caucásica. La mayor sobrevida alcanzada ha favorecido la aparición de la enfermedad ósea (EO) asociada, con el consiguiente aumento del riesgo de fracturas. Objetivo: evaluar la asociación de la densidad mineral ósea (DMO) con la función pulmonar y con la concentración plasmática de 25-hidroxivitamina D (25OHD) en niños y adolescentes con FQ. Métodos: estudio transversal y multicentrico, de pacientes con FQ de entre cinco y 18 años, clínicamente estables. Se evaluó peso, talla, desarrollo puberal, DMO y composición corporal (DXA), función pulmonar (FEF25-75 y VEF1), 25OHD plasmática e ingesta alimentaria y actividad física por encuestas. Se consideró una diferencia significativa si p < 0,05. Resultados: ingresaron 37 pacientes, 51% con función pulmonar normal. Los DMO-z promedio en columna lumbar y cuerpo total sin cabeza fueron -0,4 y -0,5, respectivamente. El 27% tuvo un índice de masa libre de grasa (IMLG) < p3, el 89% tuvo niveles insuficientes o deficientes de 25OHD y el 78,4% tuvo déficit de ingesta de calcio. No encontramos correlación entre el DMO-z con la función pulmonar ni con la concentración de 25OHD. Los pacientes con MLG < p3 tuvieron con mayor frecuencia DMO-z baja o en riesgo, en ambas localizaciones (p = 0,006 y p = 0,001 respectivamente). Conclusiones: aunque la mayoría de los pacientes tuvo una DMO y una función pulmonar normal, una alta proporción de sujetos presentaron déficit de MLG, baja ingesta de calcio y deficit de 25OHD. La asociación entre déficit de MLG y menor DMO revela la importancia de mejorar este factor para prevenir la enfermedad ósea futura