SARS-CoV-2 infection in children with cystic fibrosis: A cross-sectional multicenter study in Spain. New waves, new knowledge.

INTRODUCTION: The association between viral infections and pulmonary exacerbations in children with cystic fibrosis (cwCF) is well established. However, the question of whether cwCF are at a higher risk of COVID-19 or its adverse consequences remains controversial. METHODS: We conducted an observational, multicenter, cross-sectional study of cwCF infected by severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) between March 2020 and June 2022, (first to sixth COVID-19 pandemic waves) in Spain. The study aimed to describe patients' basal characteristics, SARS-CoV-2 clinical manifestations and outcomes, and whether there were differences across the pandemic waves. RESULTS: During study time, 351 SARS-CoV2 infections were reported among 341 cwCF. Median age was 8.5 years (range 0-17) and 51% were female. Cases were unevenly distributed across the pandemic, with most cases (82%) clustered between November 2021 and June 2022 (sixth wave, also known as Omicron Wave due to the higher prevalence of this strain in that period in Spain). Most cwCF were asymptomatic (24.8%) or presented with mild Covid-19 symptoms (72.9%). Among symptomatic, most prevalent symptoms were fever (62%) and increased cough (53%). Infection occurring along the sixth wave was the only independent risk factor for being symptomatic. Just eight cwCF needed hospital admission. No multisystem inflammatory syndrome, persisting symptoms, long-term sequelae, or deaths were reported. CONCLUSIONS: Spanish current data indicate that cwCF do not experience higher risks of SARS-CoV-2 infection nor worse health outcomes or sequelae. Changes in patients' basal characteristics, clinical courses, and outcomes were detected across waves. While the pandemic continues, a worldwide monitoring of COVID-19 in pediatric CF patients is needed.

Incidence and Prevalence of Children's Diffuse Lung Disease in Spain.

BACKGROUND: Children's diffuse lung disease, also known as children's Interstitial Lung Diseases (chILD), are a heterogeneous group of rare diseases with relevant morbidity and mortality, which diagnosis and classification are very complex. Epidemiological data are scarce. The aim of this study was to analyse incidence and prevalence of chILD in Spain. METHODS: Multicentre observational prospective study in patients from 0 to 18 years of age with chILD to analyse its incidence and prevalence in Spain, based on data reported in 2018 and 2019. RESULTS: A total of 381 cases with chILD were notified from 51 paediatric pulmonology units all over Spain, covering the 91.7% of the paediatric population. The average incidence of chILD was 8.18 (CI 95% 6.28-10.48) new cases/million of children per year. The average prevalence of chILD was 46.53 (CI 95% 41.81-51.62) cases/million of children. The age group with the highest prevalence were children under 1 year of age. Different types of disorders were seen in children 2-18 years of age compared with children 0-2 years of age. Most frequent cases were: primary pulmonary interstitial glycogenosis in neonates (17/65), neuroendocrine cell hyperplasia of infancy in infants from 1 to 12 months (44/144), idiopathic pulmonary haemosiderosis in children from 1 to 5 years old (13/74), hypersensitivity pneumonitis in children from 5 to 10 years old (9/51), and scleroderma in older than 10 years old (8/47). CONCLUSIONS: We found a higher incidence and prevalence of chILD than previously described probably due to greater understanding and increased clinician awareness of these rare diseases.

Incidence and Prevalence of Children's Diffuse Lung Disease in Spain / Incidencia y prevalencia de las neumopatías intersticiales pediátricas en España

Background Children's diffuse lung disease, also known as children's Interstitial Lung Diseases (chILD), are a heterogeneous group of rare diseases with relevant morbidity and mortality, which diagnosis and classification are very complex. Epidemiological data are scarce. The aim of this study was to analyse incidence and prevalence of chILD in Spain. Methods Multicentre observational prospective study in patients from 0 to 18 years of age with chILD to analyse its incidence and prevalence in Spain, based on data reported in 2018 and 2019. Results A total of 381 cases with chILD were notified from 51 paediatric pulmonology units all over Spain, covering the 91.7% of the paediatric population. The average incidence of chILD was 8.18 (CI 95% 6.28–10.48) new cases/million of children per year. The average prevalence of chILD was 46.53 (CI 95% 41.81–51.62) cases/million of children. The age group with the highest prevalence were children under 1 year of age. Different types of disorders were seen in children 2–18 years of age compared with children 0–2 years of age. Most frequent cases were: primary pulmonary interstitial glycogenosis in neonates (17/65), neuroendocrine cell hyperplasia of infancy in infants from 1 to 12 months (44/144), idiopathic pulmonary haemosiderosis in children from 1 to 5 years old (13/74), hypersensitivity pneumonitis in children from 5 to 10 years old (9/51), and scleroderma in older than 10 years old (8/47). Conclusions We found a higher incidence and prevalence of chILD than previously described probably due to greater understanding and increased clinician awareness of these rare diseases.

Antecedentes Las neumopatías intersticiales pediátricas, también conocidas con el acrónimo chILD (del inglés children's Interstitial Lung Diseases), es un grupo heterogéneo de enfermedades raras con morbimortalidad relevante, cuyo diagnóstico y clasificación son complejos. Los estudios epidemiológicos son escasos. El objetivo de este trabajo fue analizar la incidencia y la prevalencia de chILD en España. Métodos Estudio prospectivo observacional multicéntrico en pacientes de 0 a 18 años afectos de chILD para analizar la incidencia y la prevalencia en España, a partir de datos recogidos en 2018 y 2019. Resultados Se recogieron 381 casos de chILD entre 51 unidades de neumología pediátrica de toda España, que cubrían el 91,7% de la población pediátrica. La incidencia promedio fue 8,18 (IC 95%: 6,28-10,48) casos nuevos/millón de niños por año. La prevalencia promedio fue de 46,53 (IC 95%: 41,81-51,62) casos/millón de niños. El grupo de edad con mayor prevalencia fue el de niños menores de un año. Se observaron diferentes entidades en niños de 2 a 18 años en comparación con niños de 0 a 2 años. Los diagnósticos más frecuentes fueron: glucogenosis intersticial pulmonar primaria en neonatos (17/65), hiperplasia de células neuroendocrinas en lactantes de uno a 12 meses (44/144), hemosiderosis pulmonar idiopática en niños de uno a 5 años (13/74), neumonía por hipersensibilidad en niños de 5 a 10 años (9/51) y esclerodermia en mayores de 10 años (8/47). Conclusiones Encontramos una mayor incidencia y prevalencia de chILD que las descritas previamente, probablemente debido a un mayor conocimiento y detección de estas enfermedades raras.

[Patient with respiratory compromise controlled by a pediatric home hospitalization unit.] / Paciente con compromiso respiratorio controlado por una unidad pediátrica de hospitalización a domicilio.

OBJECTIVE: The use of paediatric home oxygen therapy is initiated in neonatal chronic lung disease; later it spreads to other patients with chronic hypoxemia. The frequency and need for oxygen therapy is growing; this, together with the family benefits, justify the implementation of a home treatment plan. Our objective with this work was to know and describe the characteristics of the patients admitted to the Pediatric HaD Unit of a tertiary hospital, the average cost per patient of oxygen therapy during 13 years of review, and evaluate the quality of the service. METHODS: Retrospective descriptive study by reviewing records of patients admitted to home care hospital at home of a paediatric tertiary care hospital (n=124) for 13 years (2000-2013), to learn and to describe the characteristics of the unit and these patients, and costs. After that, it was done statistical analysis through t-Student. RESULTS: 124 patients with chronic hypoxemia patients with mean age of 2.2 years. The most frequent cause was lung diseases, including ECPN as the most important. They accounted 14.2% of hospitalizations in home care hospital at home department, and 60% were achieved clinical stability. The mean cost was 1,991.1€ per patient, which supposes 93% of economical saving in comparison with conventional hospitalization (3,988.91€). CONCLUSIONS: The use of oxygen in HaD improves the quality of life and survival of our patients, reducing the number of readmissions and hospital costs.

OBJETIVO: El empleo de oxigenoterapia domiciliaria pediátrica se inicia en la enfermedad pulmonar crónica neonatal (EPCN), extendiéndose posteriormente a otros pacientes con hipoxemia crónica. La frecuencia y necesidad de oxigenoterapia está en crecimiento, esto, junto a los beneficios familiares, justifican la implantación de un plan de tratamiento domiciliario. Nuestro objetivo con este trabajo fue conocer y describir las características de los pacientes ingresados en la Unidad de HaD Pediátrica de un hospital de tercer nivel, el coste medio por paciente de la oxigenoterapia durante 13 años de revisión, y evaluar la calidad del servicio. METODOS: Estudio descriptivo retrospectivo mediante revisión de las historias de los pacientes ingresados en hospitalización a domicilio pediátrica (HaD) de un hospital de tercer nivel (n=124) durante 13 años (2000-2013), con descripción de las distintas características de la unidad, los pacientes incluidos y el coste. Posteriormente, se realizó análisis estadístico con t-Student. RESULTADOS: Se analizaron 124 pacientes con hipoxemia crónica, con una edad media de 2,2 años, siendo la causa más frecuente las enfermedades pulmonares y entre ellas la EPCN. Estos pacientes representaron el 14,2% de los ingresos en HaD, y en el 60% fue posible la retirada de la oxigenoterapia por buena evolución clínica. El Coste total medio por paciente fue de 1.991,1€, lo que supone un ahorro respecto a la modalidad de hospitalización convencional (3.988,91€) del 93% en nuestro centro. CONCLUSIONES: El uso de oxígeno en HaD mejora la calidad de vida y supervivencia de nuestros pacientes, disminuyendo el número de reingresos y costes hospitalarios.

Effects of music therapy as an adjunct to chest physiotherapy in children with cystic fibrosis: A randomized controlled trial.

Airway clearance therapy (ACT) is considered an important approach to improve airway clearance in children with cystic fibrosis (CF). Daily ACT administration requires substantial commitments of time and energy that complicate ACT and reduce its benefits. It is crucial to establish ACT as a positive routine. Music therapy (MT) is an aspect of integrative strategies to ameliorate the psycho-emotional consequences of chronic diseases, and a MT intervention could help children with CF between the ages of 2 and 17 develop a positive response. The aim of this randomized controlled trial was to evaluate the effects of specifically composed and recorded instrumental music as an adjunct to ACT. We compared the use of specifically composed music (Treated Group, TG), music that the patient liked (Placebo Group, PG), and no music (Control Group, CG) during the usual ACT routine in children with CF aged from 2 to 17. The primary outcomes, i.e., enjoyment and perception of time, were evaluated via validated questionnaires. The secondary outcome, i.e., efficiency, was evaluated in terms of avoided healthcare resources. Enjoyment increased after the use of the specifically composed music (children +0.9 units/parents +1.7 units; p<0.05) compared to enjoyment with no music (0 units) and familiar music (+0.5 units). Perception of time was 11.1 min (±3.9) less than the actual time in the TG (p<0.05), 3.9 min (±4.2) more than the actual time in the PG and unchanged in the CG. The potential cost saving related to respiratory exacerbations was €6,704.87, while the cost increased to €33,524.35 in the CG and to €13,409.74 in the PG. In conclusion, the specifically composed, played and compiled instrumental recorded music is an effective adjunct to ACT to establish a positive response and is an efficient option in terms of avoided costs. Trial registered as ISRCTN11161411. ISRCTN registry (www.isrctn.com).

Broncoscopias en unidades de cuidados intensivos neonatales / Bronchoscopies in Neonatal Intensive Care Units

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Auscultación asimétrica: lo que la verdad esconde / Asymmetric auscultation: What the truth hides

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ABCA3 Deficiency in a Newborn with Respiratory Failure / Deficiencia de ABCA3 en un recién nacido con insuficiencia respiratoria

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Resultados del programa de screening neonatal de fibrosis quística en Andalucía tras 5 años de su implantación / Results of the Andalusian Cystic Fibrosis Neonatal Screening Program, 5 Years After Implementation

Introducción: Andalucía dispone de screening neonatal de fibrosis quística (SNFQ) desde mayo 2011, basado en doble determinación de tripsinógeno inmunorreactivo ([TIR] [TIR1/TIR2]). Si el screening es positivo realizamos un test del sudor y si es positivo o dudoso solicitamos genética. Objetivo: Analizar el SNFQ, basado en los resultados de los primeros 4,5 años. Material y método: Estudio descriptivo prospectivo de los neonatos sometidos a SNFQ. Se recogen los niveles de TIR, cloruro en sudor, mutaciones. Mediante SPSS12.0 se realizó análisis estadístico. Resultados: Desde mayo 2011 a diciembre 2016, 474.953 neonatos fueron sometidos a SNFQ. Mil ochenta y siete (0,23%) presentaron TIR2 elevado. Desde la implantación del SNFQ se diagnosticaron 73 casos con fibrosis quística; 60 de ellos fueron diagnosticados mediante un SNFQ positivo, mientras que 13 no. Concretamente un paciente comenzó con clínica clásica de fibrosis quística y se comprobó que no se había realizado el SNFQ por decisión paterna; los 12 restantes tuvieron un SNFQ negativo (falsos negativos). De estos, un paciente fue diagnosticado presintomáticamente al tener su hermano gemelo con SNFQ positivo; otro con cloruro en el límite alto de la normalidad se diagnosticó presintomáticamente mediante genética; 10 pacientes comenzaron clínicamente. Excluyendo los pacientes con íleo meconial, la sensibilidad y especificidad del programa de SNFQ asciende al 85,71 y 99,78% respectivamente. La incidencia de la enfermedad en Andalucía es de 1/6.506 recién nacidos vivos. Conclusión: Los presentes resultados nos permiten reflexionar sobre posibles áreas de mejoras adicionales del algoritmo del SNFQ, que debe pasar por la introducción de estudios genéticos para así aumentar la sensibilidad y disminuir los falsos positivos

Introduction: Cystic fibrosis neonatal screening (CFNS), based on double determination of immunoreactive trypsinogen ([IRT] [IRT1/IRT2]), has been available in Andalusia since May 2011. If screening is positive, a sweat test is performed, and if that is positive or inconclusive, genetic testing is requested. Objective: To analyze CFNS, based on results from the first 4.5 years of the program. Materials and methods: Prospective descriptive study of neonates undergoing CFNS. IRT levels, sweat chloride, and mutations were recorded. Statistical analysis was performed using SPSS 12.0. Results: Between May 2011 and December 2016, 474,953 neonates underwent CFNS. Of these, 1,087 (0.23%) had elevated IRT2. Since CFNS was introduced, 73 cases of cystic fibrosis were diagnosed; 60 were diagnosed by positive CFNS, and 13 were diagnosed by other means. In one case, the patient developed a typical clinical picture of cystic fibrosis, but had not undergone CFNS at the decision of the parents; the remaining 12 had a negative CFNS (false negatives). Of these, one patient was diagnosed before symptoms developed, as his twin brother had a positive CFNS result; another had chloride at the upper limit of normal, and was subsequently diagnosed with genetic testing before symptoms appeared; and 10 patients developed clinical signs and symptoms. Excluding patients with meconium ileus, sensitivity and specificity of the CFNS program were 85.71% and 99.78%, respectively. The incidence of the disease in Andalusia is 1/6,506 live births. Conclusion: These results are a basis for reflection on possible areas for improvement of the CFNS algorithm, and thought may be given to the introduction of genetic studies to increase sensitivity and reduce false positives

Results of the Andalusian Cystic Fibrosis Neonatal Screening Program, 5 Years After Implementation. / Resultados del programa de screening neonatal de fibrosis quística en Andalucía tras 5 años de su implantación.

INTRODUCTION: Cystic fibrosis neonatal screening (CFNS), based on double determination of immunoreactive trypsinogen ([IRT] [IRT1/IRT2]), has been available in Andalusia since May 2011. If screening is positive, a sweat test is performed, and if that is positive or inconclusive, genetic testing is requested. OBJECTIVE: To analyze CFNS, based on results from the first 4.5 years of the program. MATERIALS AND METHODS: Prospective descriptive study of neonates undergoing CFNS. IRT levels, sweat chloride, and mutations were recorded. Statistical analysis was performed using SPSS 12.0. RESULTS: Between May 2011 and December 2016, 474,953 neonates underwent CFNS. Of these, 1,087 (0.23%) had elevated IRT2. Since CFNS was introduced, 73 cases of cystic fibrosis were diagnosed; 60 were diagnosed by positive CFNS, and 13 were diagnosed by other means. In one case, the patient developed a typical clinical picture of cystic fibrosis, but had not undergone CFNS at the decision of the parents; the remaining 12 had a negative CFNS (false negatives). Of these, one patient was diagnosed before symptoms developed, as his twin brother had a positive CFNS result; another had chloride at the upper limit of normal, and was subsequently diagnosed with genetic testing before symptoms appeared; and 10 patients developed clinical signs and symptoms. Excluding patients with meconium ileus, sensitivity and specificity of the CFNS program were 85.71% and 99.78%, respectively. The incidence of the disease in Andalusia is 1/6,506 live births. CONCLUSION: These results are a basis for reflection on possible areas for improvement of the CFNS algorithm, and thought may be given to the introduction of genetic studies to increase sensitivity and reduce false positives.

Tracheal bronchus diagnosed in children undergoing flexible bronchoscopy.

This paper describes the clinical features of paediatric patients with tracheal bronchus (TB) identified with flexible bronchoscopy (FB) in a tertiary care hospital. A retrospective review of every FB with diagnosis of TB carried out in our centre since 1990 was performed which considered specifically: age at diagnosis, gender, semiology, somatic anomalies, tracheal bronchus type, other bronchoscopic findings and clinical progress. Out of 1665 FB in 1337 patients, TB was found in 26 (1.9%). The median age was 15 months (age range 1 month-13 years), with no gender differences. Of 26 patients, a total of 24 had associated congenital pathologies (92.3%) (heart disease 69%, chromosomal abnormality 35% and spinal fusion defect 11%). FB was performed for a number of reasons including: recurrent or persistent wheezing, pneumonia or atelectasis, persistent stridor and refractory cough. The type of TB identified was primarily ectopic lobar of the right upper lobe (73%) and the apical supernumerary (11.5%). In addition to TB, other defects were found by FB in 90% of cases, such as tracheomalacia, bronchomalacia or tracheal stenosis. The review confirmed a higher prevalence of TB than expected in previous series and highlighted its association with other airway or somatic malformations, notably congenital cardiac defects and spinal fusion defects. All patients were managed conservatively for the TB.

Atelectasia masiva: ¿siempre cuerpo extraño? / Massive pulmonary atelectasis: Is it always a foreign body?

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Perinatal Tuberculosis: Is It a Forgotten Disease?

Perinatal tuberculosis is an uncommon condition but with a high mortality and a challenging diagnosis. We present 4 cases of perinatal tuberculosis managed between 1991 and 2014 in a Spanish Tertiary Hospital. The infection should be considered in patients with progressive respiratory symptoms and with a poor response to conventional antibiotic therapy, especially in those with positive epidemiologic risk. Bronchoscopy can be a useful tool for diagnosis.

Bronquitis plástica en pediatría: manejo y tratamiento / Management and Treatment of Pediatric Plastic Bronchitis

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