Obstructive Fibrinous Tracheal Pasudomembrane: A very rare and life-thretening complication of the endotracheal intubation

[eng] Obstructive fibrinous tracheal pseudomembrane is a rare complication associated with endotracheal intubation. We report the case of a 10-year-old boy hospitalized for a severe abdominal trauma. The boy remained intubated for 4 days. After extubation he started to have stridor and acute respiratory distress so a reintubation was necessary. After 24 hours, an elective extubation was performed and the boy presented stridor and dyspnoea with no improvement with medical treatment. A fibrinous mobile membrane was seen during a flexible bronchoscopy. The pseudomembrane was removed and the patient remained asymptomatic. The knowledge and an early diagnosis of this pathology is very important due to be a life-threatening complication

Utility of the Pediatric Sleep Questionnaire and Pulse Oximetry as Screening Tools in Pediatric Patients with Suspected Obstructive Sleep Apnea Syndrome

Objective. To assess the screening tools in snoring patients. Material and Methods. A retrospective review of data was conducted from children between 2 and 15 years old who were referred on suspicion of obstructive sleep apnea-hypopnea (OSAH) between June 2008 and June 2011. We excluded patients with significant comorbidities. Pediatric Sleep Questionnaire (PSQ), physical exam (PE), and pulse-oximetry data were collected and correlated with the results of the nightly polygraph at home. Results. We selected 98 patients. The 22-item version of the PSQ had sensitivity of 96% and specificity of 36.8%. The overall value of the clinic predictor of OSAH (PSQ and PE together) exhibited an increased specificity 57.6% with 94.6% of sensitivity. The nocturnal home oximetry method used alone was very specific, 92.1%, but had a lower sensitivity, 77.1%. The set of clinical assessment tools used together with pulse-oximetry screening provided excellent specificity 98.1% and a positive predictive value 94.1% globally. The performance of this screening tool is related with the severity of OSAH and accuracy is better in moderate and severe cases. Conclusion. The combination of clinical assessment and pulse-oximetry screening can provide a sufficient diagnostic approach for pediatric patients with suspected OSAH at least in moderate and severe cases

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

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

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. 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. 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). 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