Diffuse alveolar haemorrhage in children: an international multicentre study.

Background: Paediatric diffuse alveolar haemorrhage (DAH) is a rare heterogeneous condition with limited knowledge on clinical presentation, treatment and outcome. Methods: A retrospective, descriptive multicentre follow-up study initiated from the European network for translational research in children's and adult interstitial lung disease (Cost Action CA16125) and chILD-EU CRC (the European Research Collaboration for Children's Interstitial Lung Disease). Inclusion criteria were DAH of any cause diagnosed before the age of 18 years. Results: Data of 124 patients from 26 centres (15 counties) were submitted, of whom 117 patients fulfilled the inclusion criteria. Diagnoses were idiopathic pulmonary haemosiderosis (n=35), DAH associated with autoimmune features (n=20), systemic and collagen disorders (n=18), immuno-allergic conditions (n=10), other childhood interstitial lung diseases (chILD) (n=5), autoinflammatory diseases (n=3), DAH secondary to other conditions (n=21) and nonspecified DAH (n=5). Median (IQR) age at onset was 5 (2.0-12.9) years. Most frequent clinical presentations were anaemia (87%), haemoptysis (42%), dyspnoea (35%) and cough (32%). Respiratory symptoms were absent in 23%. The most frequent medical treatment was systemic corticosteroids (93%), hydroxychloroquine (35%) and azathioprine (27%). Overall mortality was 13%. Long-term data demonstrated persistent abnormal radiology and a limited improvement in lung function. Conclusions: Paediatric DAH is highly heterogeneous regarding underlying causes and clinical presentation. The high mortality rate and number of patients with ongoing treatment years after onset of disease underline that DAH is a severe and often chronic condition. This large international study paves the way for further prospective clinical trials that will in the long term allow evidence-based treatment and follow-up recommendations to be determined.

Paediatric patients with a tracheostomy: a multicentre epidemiological study.

Changes in the indications for tracheostomy in children have led to the progressively greater involvement of the paediatric pulmonologist in the care of these patients. The aim of this study was to review the current profile of tracheostomised children in Spain. We undertook a longitudinal, multicentre study over 2 yrs (2008 and 2009) of all patients aged between 1 day and 18 yrs who had a tracheostomy. The study, involving 18 Spanish hospitals, included 249 patients, of whom 150 (60.2%) were <1 yr of age. The main indications for the procedure were prolonged ventilation (n=156, 62.6%), acquired subglottic stenosis (n=34, 13.6%), congenital or acquired craniofacial anomalies (n=25, 10%) and congenital airway anomalies (n=24, 9.6%). The most frequent underlying disorders were neurological diseases (n=126, 50.6%) and respiratory diseases (n=98, 39.3%). Over the 2-yr study period, 92 (36.9%) children required ventilatory support, and decannulation was achieved in 59 (23.7%). Complications arose in 117 patients (46.9%). Mortality attributed to the underlying condition was 12.5% and that related directly to the tracheostomy was 3.2%. Respiratory complexity of tracheostomised children necessitates prolonged, multidisciplinary follow-up, which can often extend to adulthood.

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.

Utility of fiberoptic bronchoscopy for difficult airway in neonates.

BACKGROUND: Newborns diagnosed with craniofacial malformations or laryngeal and tracheal alterations may often need advanced airway-management for airway stabilization. Although fiberoptic bronchoscopy (FB) is currently the gold standard for difficult airway management, there is a scarcity of published data on the application of FB in newborns for intubation and controlled extubation (CE). OBJECTIVES: This study describes a case series where FB is used for intubation and/or extubation to manage newborns with difficult airway in either urgent procedures or scheduled ones. METHODS: All FB were carried out on newborns with difficult airway in the neonatal unit over the period January 2005 to December 2018. Patient characteristics were collected from clinical reports, description of the technique from the procedure report. RESULTS: 66 FBs were performed from a total of 40 newborns, a median age of 25 days and a weight of 3217 g. Eighteen were ex-premature babies (45%). Six (15%) had craniocervical malformations. 17 (25.7%) FBs were performed for tracheal intubation (TI), 6 in emergency situations, 34 (51.5%) for CE, and 15 (22.7%) for precise tube placement. Clearing of the airway was achieved in all cases and thus there were no failed TIs. In 32 cases (94.1%), CE was successfully performed. In 6 cases, withdrawal of the ET midprocedure was decided to postpone as 4 of these required extra treatment prior to renewed extubation attempt and 2 needed a tracheostomy. 2 patients required subsequent FB reintubation due to airway pathology. Complications during the procedure were mild desaturations (3%) and deep desaturations (7.5%). CONCLUSIONS: FB is very safe and highly useful when performing intubation in neonates with difficult airway but is essential during extubation to avoid any risk of failure with a potentially fatal outcome. Neonatal units of tertiary hospitals should provide equipment and training to manage these neonates. WHAT IS ALREADY KNOWN?: A failed intubation or extubation can lead to cardiac arrest and anoxic brain damage and/or to death in a neonate. Fiberoptic bronchoscopy is recommended as the safest tool for neonates with difficult airway. However, there is little concrete data published in the current literature to support the recommendations. WHAT IS NEW: Fiberoptic bronchoscopy is an essential tool to avoid the risk of a failed extubation in neonates with difficult airway.

Long-term outcomes of necrotizing pneumonia.

INTRODUCTION: Necrotizing pneumonia (NP) is a serious complication of community-acquired pneumonia characterised by the destruction of normal lung parenchyma. No study has evaluated the repercussions of the lung damage in the years following the episode. The aim of this study was to assess the long-term impact on lung function and respiratory symptoms in children hospitalised due to NP. METHODS: We analysed outcomes in children given a diagnosis of NP between January 2003 and April 2016. We selected patients aged more than 4 years capable of undergoing a lung function test, that had been followed up for at least 2 years. The patients completed a respiratory questionnaire and underwent a lung function test. RESULTS: We included a total of 24 patients (12 male). The median age at the time of diagnosis was 28 months, the median length of stay was 15 days, and 18 patients required pleural drainage. The mean duration of follow-up after NP was 8.75 years. During the evaluation, none of the patients exhibited asthma, cough, or exercise-induced symptoms. Three children had a second episode of pneumonia that did not require hospital admission. The spirometry results were the following (given as mean±standard deviation): FEV1 Z-score, -0.47±0.65; FVC Z-score, -0.56±0.73; and FEV1/FVC Z-score, 0.19±0.98. We found no evidence of obstructive pulmonary disease or restrictive patterns. CONCLUSIONS: The long-term outcomes of paediatric NP are good. However, patients exhibited mildly impaired lung function several years after the episode. We recommend follow-up of these patients due to potential impairments in lung function in adulthood.

Diagnosis and management of aspiration using fiberoptic endoscopic evaluation of swallowing in a Pediatric Pulmonology Unit.

BACKGROUND AND OBJECTIVES: Swallowing disorders lead to chronic lung aspiration. Early detection and treatment of aspiration in children with dysphagia are important to prevent lung damage. Diagnosis of aspiration, which may be silent, requires an instrumental study such as fiberoptic endoscopic evaluation of swallowing (FEES). Despite its usefulness, it is rarely practiced by pediatric pulmonologists. This study aimed to evaluate the feasibility and utility of FEES performed in the pediatric respiratory unit of a tertiary hospital, analyze the clinical characteristics, endoscopic findings and proposed treatments, and identify the factors associated with penetration or aspiration. METHODS: Medical records of 373 children with suspected aspiration who were referred to the pediatric respiratory unit for FEES were reviewed retrospectively. Clinical characteristics, FEES findings, and the proposed treatments were analyzed. RESULTS: Laryngeal penetration/aspiration was seen in 47.9% of the patients. The most common associated conditions were neurological disease and prematurity. The most frequently observed endoscopic finding was altered laryngeal sensitivity (36.5%). Intervention was recommended in 54.2% of the patients. Complications were not seen during any of the procedures. The multivariate logistic regression model revealed an independent association between aspiration and alterations in laryngeal sensitivity (odds ratio [OR], 5.68), pharyngeal pooling (OR, 11.47), and post-swallowing food residues (OR, 8.08). CONCLUSIONS: The FEES procedure performed by pediatric pulmonologists is a reliable method for diagnosing aspiration in children. It can be safely executed by trained pulmonologists, and significant endoscopic signs other than aspiration can guide in the diagnosis and management recommendations.

The Impact of the SARS-CoV-2 Pandemic on the Emergency Department and Management of the Pediatric Asthmatic Patient.

INTRODUCTION: Asthma exacerbation is among the commonest causes for pediatric emergency room visits, and respiratory viruses are frequent triggers of such exacerbations. Few studies have evaluated the consequences of the novel human coronavirus that causes the illness currently known as COVID-19, in the pediatric population. PURPOSE: The objective of this study was to analyze the impact of the COVID-19 pandemic and lockdown measures on the emergency department in the pediatric asthmatic patient. PATIENTS AND METHODS: This retrospective observational study evaluated pediatric patients treated at the Pediatric Emergency Service for wheezing episodes. Changes in the number and characteristics of these patients over the same period of 2019 as compared to 2020 during the month following the alarm declaration (March 14 to April 15) were evaluated. RESULTS: In total, data of 30 asthma patients managed in the period after the declaration of the coronavirus pandemic and of 158 asthma patients managed in the pre-COVID-19 period were included. In 2020, patient visits decreased by 82% in 2019. No statistically significant differences among age, sex, oxygen saturation, fever status, or number of severe bronchospasm episodes were found. Nebulized medication usage was reduced significantly since the alarm declaration. No significant increase in requests for complementary testing in the COVID-19 period was found. No patient requiring hospital admission was found to be PCR SARS-CoV-2 positive. Median time spent in the emergency department decreased from 180 minutes in 2019 to 85 minutes in the COVID-19 era. CONCLUSION: The COVID-19 pandemic and ensuing lockdown measures have led to an extraordinary reduction in emergency visits to the pediatric service. The ongoing pandemic has also led to improvements in the approach to asthma exacerbations and wheezing, to reduce the risk of exposure to the virus, such as increased use of pressurized metered dose inhaler and decreased time in the Emergency Department.

High flow nasal cannula versus standard low flow nasal oxygen during flexible bronchoscopy in children: A randomized controlled trial.

BACKGROUND: Hypoxemia is the most frequent complication of fiberoptic bronchoscopy (FB) in children. Guidelines recommend oxygen supplementation and conventional nasal prongs (NC) are used for this purpose. The aim of this study was to evaluate if the use of high-flow nasal cannula therapy (HFNC) in children undergoing FB result in a lower incidence of hypoxemia than standard oxygen administration. METHODS: Patients aged 1 month-16 years undergoing elective FB were included in a prospective randomized controlled, nonblinded, single-center clinical trial and randomly assigned to receive oxygen via NC or HFNC. Patients' baseline characteristics were recorded pre-bronchoscopy. The primary outcome was oxygen desaturation during the procedure defined as saturation less than 94%. RESULTS: An intention to treat analysis for 53 patients receiving NC and 51 receiving HFNC, showed HFNC patients were less likely to have hypoxemia than were NC patients (p = .011), with an absolute risk reduction of 0.27 (95% confidence interval [CI]: 0.08-0.45) and a number needed to treat of 3.75 (95% CI: 2.22-12.04). Moderate hypoxemia (SpO2 ≥ 90% and <94%, and <60 s) was observed significantly less often with HFNC than with NC (p = .012). Severe hypoxemia (SpO2 < 90% and >30 s) was not different between groups. Patients undergoing bronchoalveolar lavage (BAL) presented fewer desaturations with HFNC (p = .0003). CONCLUSIONS: HFNC offers optimized oxygenation during elective FB with a significant reduction in desaturations and can be considered for oxygen administration, especially when BAL is performed.

[Long-term outcomes of necrotizing pneumonia]. / Resultados a largo plazo de la neumonía necrotizante.

INTRODUCTION: Necrotizing pneumonia (NP) is a serious complication of community-acquired pneumonia characterised by the destruction of normal lung parenchyma. No study has evaluated the repercussions of the lung damage in the years following the episode. The aim of this study was to assess the long-term impact on lung function and respiratory symptoms in children hospitalised due to NP. METHODS: We analysed outcomes in children given a diagnosis of NP between January 2003 and April 2016. We selected patients aged more than 4 years capable of undergoing a lung function test, that had been followed up for at least 2 years. The patients completed a respiratory questionnaire and underwent a lung function test. RESULTS: We included a total of 24 patients (12 male). The median age at the time of diagnosis was 28 months, the median length of stay was 15 days, and 18 patients required pleural drainage. The mean duration of follow-up after NP was 8.75 years. During the evaluation, none of the patients exhibited asthma, cough, or exercise-induced symptoms. Three children had a second episode of pneumonia that did not require hospital admission. The spirometry results were the following (given as mean ± standard deviation): FEV1 z-score, -0.47±0.65; FVC z-score, -0.56±0.73; and FEV1/FVC z-score, 0.19±0.98. We found no evidence of obstructive pulmonary disease or restrictive patterns. CONCLUSIONS: The long-term outcomes of paediatric NP are good. However, patients exhibited mildly impaired lung function several years after the episode. We recommend follow-up of these patients due to potential impairments in lung function in adulthood.

Spanish multicentre study on morbidity and pathogenicity of tracheal bronchus in children.

Tracheal bronchus (TRB) has been generally considered an anatomical variant of the tracheobronchial tree without a precise pathological effect. Its prevalence is estimated to be between 0.2% to 3% of all children undergoing bronchoscopy and scientific information has been limited to case reports or small case series. Our working hypothesis was that TRB could trigger by itself recurrent or persistent respiratory symptoms. The objective of this retrospective and multicentre study of children with a diagnosis of TRB, coming from the main paediatric pulmonology units of Spain, was to determine the anatomical and clinical characteristics, including comorbidities, of TRB in childhood and their impact in the patients' clinical outcomes. One hundred thirty-three patients from 13 institutions were included in the study. Mean diagnostic age was 3.4 years and flexible bronchoscopy was the initial diagnostic method in 85% of cases. All TRB were located on the right wall of the trachea: 76% in the lower third and 24% in the carina. The most common clinical manifestations were obstructive bronchitis (53.3%) and recurrent pneumonia (46.6%), usually affecting the right upper lobe. Regarding associated anomalies, 33% had tracheomalacia, 32% congenital cardiovascular malformations, 28% gastroesophageal reflux, 22.5% congenital tracheal stenosis, and 8.3% Down syndrome. This series appears to be the most extensive published to date addressing this topic and, according to our data, TRB does not appear to be a mere incidental finding but is more likely linked to a wide range of congenital anomalies and contributes by itself to the recurrent respiratory symptomatology that these children exhibit.

Risk of varicella complications in children treated with inhaled steroids.

We have studied the complications of varicella in children treated with inhaled steroids. From 1995 to 2005, 3/213 (1.4%) children hospitalized on the island of Mallorca, Spain, for varicella had received inhaled steroids. The rate of hospitalization for varicella complications in children receiving inhaled steroids was 17/100,000 children taking inhaled steroids, a rate similar to the rest of the children's population (18.2/100,000). Inhaled steroids did not increase the risk of varicella complications (relative risk 0.94; 95% confidence interval: 0.3-2.89).

Neonatal swallowing assessment using fiberoptic endoscopic evaluation of swallowing (FEES).

OBJECTIVES: Swallowing disorders which lead to aspiration are common in premature infants with a postmenstrual age (PMA) of >36 weeks. Aspiration is often silent and the unique symptom is desaturation during feeding. The aim of this study was 1) to determine the number of prematures with desaturations during feeding due to aspiration, using Fiberoptic Endoscopic Evaluation of Swallowing (FEES); 2) to relate clinical factors and FEES findings to aspiration; and 3) to describe type and efficacy of suggested treatments. METHODS: A retrospective review of 62 ex-premature babies with a median PMA of 40 weeks which underwent FEES due to persistent feeding desaturations. Aspiration was related to other FEES findings and to clinical and demographic data. The efficacy of the treatment was evaluated during the FEES and by comparing recorded desaturations during feeding 7 days before and after FEES. RESULTS: A total of 44 (71%) infants were diagnosed with aspiration and/or penetration. No relation was found to demographic or clinical data. The accumulation of saliva and residues post-swallowing were related to aspiration (P < 0.01). In 77.3% of the infants, use of a thickener seemed to reduce aspiration during FEES and was suggested as a treatment. 13.6% of infants received anti-reflux treatment after FEES and 9.1% required gastrostomy. CONCLUSIONS: Aspiration is very frequent in premature infants who present desaturations during feeding and FEES is a useful method for diagnosing and suggesting treatments.

[Spanish patients with central hypoventilation syndrome included in the European Registry. The 2015 data]. / Pacientes españoles con síndrome de hipoventilación central incluidos en el Registro europeo. Datos del 2015.

INTRODUCTION: Congenital Central Hypoventilation Syndrome (CCHS) is a very rare genetic disease. In 2012 the European Central Hypoventilation Syndrome (EuCHS) Consortium created an online patient registry in order to improve care. AIM: To determine the characteristics and outcomes of Spanish patients with CCHS, and detect clinical areas for improvement. MATERIALS AND METHOD: An assessment was made on the data from Spanish patients in the European Registry, updated on December 2015. RESULTS: The Registry contained 38 patients, born between 1987 and 2013, in 18 hospitals. Thirteen (34.2%) were older than 18 years. Three patients had died. Genetic analysis identified PHOX2B mutations in 32 (86.5%) out of 37 patients assessed. The 20/25, 20/26 and 20/27 polyalanine repeat mutations (PARMs) represented 84.3% of all mutations. Longer PARMs had more, as well as more severe, autonomic dysfunctions. Eye diseases were present in 47%, with 16% having Hirschsprung disease, 13% with hypoglycaemia, and 5% with tumours. Thirty patients (79%) required ventilation from the neonatal period onwards, and 8 (21%) later on in life (late onset/presentation). Eight children (21%) were using mask ventilation at the first home discharge. Five of them were infants with neonatal onset, two of them, both having a severe mutation, were switched to tracheostomy after cardiorespiratory arrest at home. Approximately one-third (34.3%) of patients were de-cannulated and switched to mask ventilation at a mean age of 13.7 years. Educational reinforcement was required in 29.4% of children attending school. CONCLUSION: The implementation of the EuCHS Registry in Spain has identified some relevant issues for optimising healthcare, such as the importance of genetic study for diagnosis and assessment of severity, the high frequency of eye disease and educational reinforcement, as well as some limitations in ventilatory techniques.

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.