Fungal microbiota dynamics and its geographic, age and gender variability in patients with cystic fibrosis.

OBJECTIVES: In cystic fibrosis (CF), there is a predisposition to bronchial colonization by potentially pathogenic microorganisms, such as fungi. Our aims were to describe the dynamics of respiratory mycobiota in patients with CF and to evaluate the geographic, age and gender variability in its distribution. METHODS: Cohort study in which 45 patients with CF from four hospitals in three Spanish cities were followed up during a 1-year period, obtaining spontaneous sputum samples every 3 to 6 months. Fungal microbiota were characterized by Internal Transcribed Spacer sequencing and Pneumocystis jirovecii was identified by nested PCR in a total of 180 samples. RESULTS: The presence of fungi were detected in 119 (66.11%) of the 180 samples and in 44 (97.8%) of the 45 patients: 19 were positive and 1 negative throughout all follow-ups and the remaining 25 presented alternation between positive and negative results. A total of 16 different genera were identified, with Candida spp. (50/180, 27.78%) and Pneumocystis spp. (44/180, 24.44%) being the most prevalent ones. The distribution of fungal genera was different among the evaluated centres (p < 0.05), by age (non-adults aged 6-17 years vs. adults aged ≥18 years) (p < 0.05) and by gender (p < 0.05). DISCUSSION: A high prevalence of fungal respiratory microbiota in patients with CF was observed, whose dynamics are characterized by the existence of multiple cycles of clearance and colonization, reporting the existence of geographic, age and gender variability in the distribution of fungal genera in this disease.

Respiratory outcomes of "new" bronchopulmonary dysplasia in adolescents: A multicenter study.

OBJECTIVE: Long-term respiratory consequences of bronchopulmonary dysplasia (BPD) in preterm infants born in the post-surfactant era ("new" BPD) remain partially unknown. The present study aimed to evaluate the respiratory outcomes of "new" BPD in adolescents who were born preterm. METHODS: This multicenter, cross-sectional study included 286 adolescents born between 2003 and 2005 (mean age: 14.2 years); among them, 184 and 102 were born extremely preterm (EP; <28 weeks' gestation) and moderate-late preterm (32 to <37 weeks' gestation), respectively. Among EP adolescents, 92 had BPD, and 92 did not. All participants underwent lung function tests, skin prick testing, and questionnaires on asthma symptoms and quality of life. RESULTS: EP adolescents with BPD had significantly lower forced expiratory volume in 1 s (FEV1 ), forced vital capacity (FVC), FEV1 /FVC ratio, and forced expiratory flow between 25% and 75% of FVC than other included adolescents. FEV1 /FVC ratios were below the lower limit of normal (z-score <-1.645) in 30.4% of EP adolescents with BPD, 13.0% of EP adolescents without BPD, and 11.8% of adolescents who were born moderate-late preterm. Bronchodilator response and air-trapping were significantly higher in BPD adolescents than in other adolescents. Diffusion capacity was significantly lower in EP adolescents than in moderate-late preterm adolescents. Asthma symptoms and quality-of-life scores were similar among groups. CONCLUSION: EP adolescents with "new" BPD had poorer pulmonary function than EP adolescents without BPD or moderate-late preterm adolescents. Further studies are needed to determine whether "new" BPD is associated with early-onset chronic obstructive pulmonary disease in adulthood.

Comorbilidades y evolución de la función pulmonar de pacientes con atresia esofágica congénita / Comorbidities and course of lung function in patients with congenital esophageal atresia

Objetivo. Describir las características epidemiológicas, el seguimiento hospitalario y la evolución de los pacientes intervenidos por atresia esofágica y su repercusión en la función pulmonar. Población y métodos. Estudio retrospectivo, longitudinal y analítico. Se revisaron las historias clínicas de pacientes con atresia esofágica nacidos entre 1996 y 2017. Se registraron datos perinatales, tipo de atresia, malformaciones asociadas, complicaciones respiratorias y digestivas, y los datos espirométricos durante tres años. Resultados. Se incluyeron 97 pacientes. El tipo de atresia más frecuente fue el III y el síndrome más frecuente, la trisomía 21. El 13,4 % fallecieron en el período neonatal. El 23,8 % de los pacientes estuvo en seguimiento por Neumología y presentó como complicaciones respiratorias exacerbaciones (el 46,4 %), sibilancias o asma (el 36 %), neumonías (el 26,8 %). El reflujo gastroesofágico fue factor de riesgo de sibilancias (OR 5,31; p = 0,002), exacerbaciones (OR 4,00; p = 0,009) y neumonías (OR 3,24; p = 0,02). En la primera espirometría (n = 20), un 65 % presentaba patrón normal; un 30 %, restrictivo, y un 5 %, mixto. En la segunda espirometría (n = 19), un 42,1 % presentaba patrón normal; un 31,6 %, restrictivo; un 15,8 %, obstructivo, y un 10,5 %, mixto. En la tercera espirometría (n = 14), el 50 % presentaba un patrón espirométrico normal; el 21,4 %, restrictivo; el 14,3 %, obstructivo, y un 14,3 %, mixto. Conclusiones. En nuestra muestra de pacientes, una importante proporción presentó comorbilidades respiratorias y digestivas. La función pulmonar empeoró progresivamente.

Objective. To describe the epidemiological characteristics, hospital follow-up, and course of patients who underwent surgery for esophageal atresia and its consequences on lung function. Population and methods. Retrospective, longitudinal, and analytical study. The medical records of patients with esophageal atresia born between 1996 and 2017 were reviewed. Perinatal data, type of atresia, associated malformations, respiratory and gastrointestinal complications, and spirometry data were recorded over 3 years. Results. A total of 97 patients were included. The most common type of atresia was III, and the most frequent syndrome, trisomy 21; 13.4 % of patients died in the neonatal period; 23.8 % were followed up by the Department of Pulmonology, and their respiratory complications included exacerbations (46.4 %), wheezing or asthma (36 %), and pneumonia (26.8 %). Gastroesophageal reflux was a risk factor for wheezing (OR: 5.31; p = 0.002), exacerbations (OR: 4.00; p = 0.009), and pneumonia (OR: 3.24; p = 0.02). In the first spirometry (n = 20), the pattern was normal in 65 %; restrictive in 30 %; and mixed in 5 %. In the second spirometry (n = 19), the pattern was normal in 42.1 %; restrictive in 31.6 %; obstructive in 15.8 %, and mixed in 10.5 %. In the third spirometry (n = 14), the pattern was normal in 50 %; restrictive in 21.4 %; obstructive in 14.3 %, and mixed in 14.3 %.Conclusions. In our sample of patients, a large proportion had respiratory and gastrointestinal comorbidities. Lung function worsened progressively.

Comorbidities and course of lung function in patients with congenital esophageal atresia. / Comorbilidades y evolución de la función pulmonar de pacientes con atresia esofágica congénita.

OBJECTIVE: To describe the epidemiological characteristics, hospital follow-up, and course of patients who underwent surgery for esophageal atresia and its consequences on lung function. POPULATION AND METHODS: Retrospective, longitudinal, and analytical study. The medical records of patients with esophageal atresia born between 1996 and 2017 were reviewed. Perinatal data, type of atresia, associated malformations, respiratory and gastrointestinal complications, and spirometry data were recorded over 3 years. RESULTS: A total of 97 patients were included. The most common type of atresia was III, and the most frequent syndrome, trisomy 21; 13.4 % of patients died in the neonatal period; 23.8 % were followed up by the Department of Pulmonology, and their respiratory complications included exacerbations (46.4 %), wheezing or asthma (36 %), and pneumonia (26.8 %). Gastroesophageal reflux was a risk factor for wheezing (OR: 5.31; p = 0.002), exacerbations (OR: 4.00; p=0.009), and pneumonia (OR: 3.24; p = 0.02). In the first spirometry (n=20), the pattern was normal in 65 %; restrictive in 30 %; and mixed in 5 %. In the second spirometry (n = 19), the pattern was normal in 42.1 %; restrictive in 31.6 %; obstructive in 15.8 %, and mixed in 10.5 %. In the third spirometry (n = 14), the pattern was normal in 50 %; restrictive in 21.4 %; obstructive in 14.3 %, and mixed in 14.3 %. CONCLUSIONS: In our sample of patients, a large proportion had respiratory and gastrointestinal comorbidities. Lung function worsened progressively.

Objetivo: Describir las características epidemiológicas, el seguimiento hospitalario y la evolución de los pacientes intervenidos por atresia esofágica y su repercusión en la función pulmonar. Población y métodos: Estudio retrospectivo, longitudinal y analítico. Se revisaron las historias clínicas de pacientes con atresia esofágica nacidos entre 1996 y 2017. Se registraron datos perinatales, tipo de atresia, malformaciones asociadas, complicaciones respiratorias y digestivas, y los datos espirométricos durante tres años. Resultados: Se incluyeron 97 pacientes. El tipo de atresia más frecuente fue el III y el síndrome más frecuente, la trisomía 21. El 13,4 % fallecieron en el período neonatal. El 23,8 % de los pacientes estuvo en seguimiento por Neumología y presentó como complicaciones respiratorias exacerbaciones (el 46,4 %), sibilancias o asma (el 36 %), neumonías (el 26,8 %). El reflujo gastroesofágico fue factor de riesgo de sibilancias (OR 5,31; p = 0,002), exacerbaciones (OR 4,00; p = 0,009) y neumonías (OR 3,24; p = 0,02). En la primera espirometría (n = 20), un 65 % presentaba patrón normal; un 30 %, restrictivo, y un 5 %, mixto. En la segunda espirometría (n = 19), un 42,1 % presentaba patrón normal; un 31,6 %, restrictivo; un 15,8 %, obstructivo, y un 10,5 %, mixto. En la tercera espirometría (n = 14), el 50 % presentaba un patrón espirométrico normal; el 21,4 %, restrictivo; el 14,3 %, obstructivo, y un 14,3 %, mixto. Conclusiones: En nuestra muestra de pacientes, una importante proporción presentó comorbilidades respiratorias y digestivas. La función pulmonar empeoró progresivamente.

Normas para control y seguimiento de niños con displasia broncopulmonar (enfermedad pulmonar crónica de la infancia) / Control and follow-up standards in children with bronchopulmonary dysplasia (infant chronic lung disease)

La displasia broncopulmonar (enfermedad pulmonar crónica de la infancia) constituye un grupo heterogéneo de enfermedades de etiopatogenia multifactorial y fisiopatología multisistémica. Su frecuencia ha aumentado en los últimos años debido principalmente a la mayor supervivencia de los recién nacidos prematuros de muy bajo peso al nacer que presentan interrupción del desarrollo vascular y pulmonar unido a alteraciones funcionales generadas por el déficit de surfactante y relacionadas con la inmadurez. Sin embargo, se ha controlado la gravedad de estos cuadros gracias a los cambios realizados en la práctica clínica. Para un adecuado control y seguimiento multidisciplinario, abordamos esta afección con el objetivo de elaborar un plan de actuación cuando estos neonatos se encuentran en su hogar tras el alta hospitalaria...

Right ventricular dysfunction in adolescents with mild cystic fibrosis.

BACKGROUND: In cystic fibrosis (CF) patients the right ventricle (RV) suffers a progressive deterioration, but it is not clear when these changes begin. The aim of this study was to analyze the RV function in CF patients with mild respiratory disease. METHODS: Color-Doppler-Echocardiographic studies were prospectively performed in CF adolescent patients and an age-matched control group. Findings were correlated with pulmonary function tests (PFT), genotype, chronic bacterial colonization, pancreatic status and clinical scores. Only patients with mild CF were selected. RESULTS: Thirty seven CF patients and 40 healthy controls were recruited. In CF patients all echocardiographic parameters were abnormal compared to controls. Doppler analysis showed slightly elevated pulmonary artery pressure values, and abnormal relaxation and systolic function for all indexes. No correlation was found with any of the features studied. CONCLUSIONS: In CF patients, abnormalities in the structure and function of the RV may be present at early stages of the disease. These abnormalities are subclinical and do not correlate with clinical scores, PFT, genotype, chronic bacterial colonization or pancreatic insufficiency.

[Inhaled antibiotic therapy in cystic fibrosis]. / Antibioterapia inhalada en la fibrosis quística.

Cystic fibrosis is the most frequent fatal genetically-transmitted disease among Caucasians. Chronic bronchial infection, especially by Pseudomonas aeruginosa, is the main cause of morbidity and mortality in this disease. Aerosolized antibiotic therapy achieves high drug concentrations in the airway with low toxicity, allowing chronic use. Currently, two antibiotics have been approved for inhalation therapy, tobramycin inhalation solution and colistimethate sodium aerosol. There is less evidence from clinical trials for the latter. The main indication for these drugs is chronic bronchial colonization by P. aeruginosa, although there is increasing evidence of the importance of the primary infection by this bacterium, whether treated by oral or intravenous antibiotics or not. More controversial is the use of aerosolized antibiotic therapy in bacterial prophylaxis or respiratory exacerbations. For many years, intravenous formulations of distinct antibiotics for aerosolized use have been employed, which are in distinct phases of research for use in nebulizer therapy. In addition to being used to treat P. aeruginosa infection, aerosolized antibiotics have been used to treat other pathogens such as methicillin-resistant Staphylococus aureus, Mycobacterium abscessus and Aspergillus fumigatus.

Antibioterapia inhalada en la fibrosis quística / Inhaled antibiotic therapy in cystic fibrosis

La fibrosis quística es la enfermedad genética letal más frecuente en la población caucasiana. La infecciónbronquial crónica, especialmente por Pseudomonas aeruginosa, es la principal causa de morbimortalidad deesta patología. El tratamiento antibiótico por aerosol alcanza altas concentraciones en la vía aérea con bajatoxicidad, por lo que permite el empleo crónico. En la actualidad hay 2 antibióticos aprobados para su usoinhalatorio, la tobramicina en solución para inhalación y el colistimetato de sodio, existiendo con este últimomenos evidencias en estudios clínicos. La indicación fundamental es la colonización bronquial crónica por P.aeruginosa, aunque cada vez se demuestra más relevancia en la primoinfección por esta bacteria, acompañadao no de antibióticos por vía oral o intravenosos. Más controvertido es el uso de la aerosolterapia antibiótica enla profilaxis bacteriana o en la exacerbación respiratoria. Durante muchos años se han estado empleandoformulaciones intravenosas de distintos antibióticos en aerosol, las cuales están en distintas fases de investigaciónpara su lanzamiento como presentación por vía nebulizada. Además de su indicación en el tratamientode la infección por P. aeruginosa se han empleado otros antibióticos en aerosol para otros patógenos comoStaphylococus aureus resistentes a meticilina, Mycobacterium abscessus o Aspergillus fumigatus(AU)

Cystic fibrosis is the most frequent fatal genetically-transmitted disease among Caucasians. Chronic bronchialinfection, especially by Pseudomonas aeruginosa, is the main cause of morbidity and mortality in this disease.Aerosolized antibiotic therapy achieves high drug concentrations in the airway with low toxicity, allowingchronic use. Currently, two antibiotics have been approved for inhalation therapy, tobramycin inhalationsolution and colistimethate sodium aerosol. There is less evidence from clinical trials for the latter. The mainindication for these drugs is chronic bronchial colonization by P. aeruginosa, although there is increasingevidence of the importance of the primary infection by this bacterium, whether treated by oral or intravenousantibiotics or not. More controversial is the use of aerosolized antibiotic therapy in bacterial prophylaxis orrespiratory exacerbations. For many years, intravenous formulations of distinct antibiotics for aerosolized usehave been employed, which are in distinct phases of research for use in nebulizer therapy. In addition to beingused to treat P. aeruginosa infection, aerosolized antibiotics have been used to treat other pathogens such asmethicillin-resistant Staphylococus aureus, Mycobacterium abscessus and Aspergillus fumigatus(AU)

Diagnóstico del síndrome aspirativo recurrente y reflujo gastroesofágico en enfermos con procesos respiratorios de repetición en la edad pediátrica / Diagnosis of chronic aspiration syndrome and gastro-esophageal reflux in patients with recurrent respiratory manifestations in childhood

The chronic aspiration syndrome and the gastroesophageal reflux, wether associated or not, are much more frequent in childhood than generally believed. These diseases cause chronic or recurrent bronchopulmonary manifestations, sometimes difficult to diagnose, due to a varying clinical presentation and to the fact that no specific diagnostic criteria exists. Diagnosis of gastroesophageal reflux is simple, however, diagnosis of recurrent aspiration syndrome is not so easy. In addition, when one or the other of the two is diagnosed, it can be difficult to establish the relationship between microaspiration and respiratory affectation or demonstrate that gastroesophageal reflux is the cause of the respiratory symptoms of our patient.For the diagnosis, it is necessary to do a complete and well focused clinical history analysing the factors wich predispose the condition. Clinical exploration is also important. The diagnosis tests should be carried out according to clinical suspicion and knowing, as far as possible, their predictive value, sensitivity and specificity, following a standardized and agreed upon diagnostic protocol.

El síndrome aspirativo recurrente y el reflujo gastroesofágico, asociados o no, son bastante más frecuentes de lo que se cree en la edad pediátrica. Estas entidades ocasionan enfermedad broncopulmonar crónica o recurrente difícil de diagnosticar en ocasiones, debido a una presentación clínica muy variable y a la no existencia de criterios diagnósticos específicos. El diagnostico de reflujo gastroesofágico es fácil, pero no ocurre lo mismo con el diagnóstico del síndrome aspirativo recurrente. Además, aun cuando diagnostiquemos una u otra entidad, puede ser difícil relacionar microaspiración y afección respiratoria o demostrar que el reflujo gastroesofágico es la causa de la sintomatología respiratoria de nuestro paciente. Para el diagnóstico es necesaria la realización de una historia clínica exhaustiva y bien dirigida que analice los factores predisponentes. La exploración clínica también es importante. Las pruebas complementarias seriadas deben ser realizadas de acuerdo con la sospecha clínica y conociendo, en lo posible, su valor predictivo, sensibilidad y especificidad, según un protocolo diagnóstico estandarizado y consensuado.

Exacerbación respiratoria y aspergilosis broncopulmonar alérgica en fibrosis quística / Respiratory exacerbation and allergic bronchopulmonary

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