Persistent tachypnea of infancy: Follow up at school age.

AbstractBackgroundPersistent tachypnea of infancy (PTI) is a rare pediatric lung disease of unknown origin. The diagnosis can be made by clinical presentation and chest high resolution computed tomography after exclusion of other causes. Clinical courses beyond infancy have rarely been assessed.MethodsPatients included in the Kids Lung Register diagnosed with PTI as infants and now older than 5 years were identified. Initial presentation, extrapulmonary comorbidities, spirometry and clinical outcome were analyzed.ResultsThirty‐five children older than 5 years with PTI diagnosed as infants were analyzed. At the age of 5 years, 74% of the patients were reported as asymptomatic and did not develope new symptoms during the observational period at school‐age (mean, 3.9 years; range, 0.3‐6.3). At the age of about 10 years, none of the symptomatic children had abnormal oxygen saturation during sleep or exercise anymore. Lung function tests and breathing frequency were within normal values throughout the entire observational period.ConclusionsPTI is a pulmonary disease that can lead to respiratory insufficiency in infancy. As at school age most of the previously chronically affected children became asymptomatic and did not develop new symptoms. We conclude that the overall clinical course is favorable

Lung disease caused by ABCA3 mutations

Background Knowledge about the clinical spectrum of lung disease caused by variations in the ATP binding cassette subfamily A member 3 (ABCA3) gene is limited. Here we describe genotype-phenotype correlations in a European cohort. Methods We retrospectively analysed baseline and outcome characteristics of 40 patients with two disease-causing ABCA3 mutations collected between 2001 and 2015. Results Of 22 homozygous (15 male) and 18 compound heterozygous patients (3 male), 37 presented with neonatal respiratory distress syndrome as term babies. At follow-up, two major phenotypes are documented: patients with (1) early lethal mutations subdivided into (1a) dying within the first 6 months or (1b) before the age of 5 years, and (2) patients with prolonged survival into childhood, adolescence or adulthood. Patients with null/null mutations predicting complete ABCA3 deficiency died within the 1st weeks to months of life, while those with null/other or other/other mutations had a more variable presentation and outcome. Treatment with exogenous surfactant, systemic steroids, hydroxychloroquine and whole lung lavages had apparent but many times transient effects in individual subjects. Conclusions Overall long-term (>5 years) survival of subjects with two disease-causing ABCA3 mutations was <20%. Response to therapies needs to be ascertained in randomised controlled trials

Is Hyperuricemia Overlooked when Treating Pediatric Tuberculosis Patients with Pyrazinamide?

The treatment of tuberculosis (TB) requires long-term multiple drug use. Hyperuricemia is frequently reported in adults, but there are few data for the pediatric population. This study aimed to review drug-related side effects in pediatric patients that received treatment for TB. Patients with active TB undergoing treatment were followed for drug-related side effects. During the 7 year period, 23 patients with a mean age of 7.9 +/- 4.66 years were treated. Drug-related side effects were observed in 14 patients. Hyperuricemia occurred in 12 of the 14 patients, vs. hepatotoxicity in 2. In all, eight of the patients with hyperuricemia had >= 2 episodes during pyrazinamide (PZA) therapy. Based on these findings, we devised an algorithm that could be used for the management of hyperuricemia in patients receiving PZA because of TB, and recommend that hyperuricemia be closely monitored during PZA therapy

Quality of life and exercise capacity in patients with primary ciliary dyskinesia

28th International Congress of the European-Respiratory-Society (ERS) -- SEP 15-19, 2018 -- Paris, FRANCEWOS: 000455567102045…European Respiratory So

Respiratory Bronchiolitis-Associated Interstitial Lung Disease in Childhood: New Sequela of Smoking.

Childhood interstitial lung diseases are rare disorders of largely unknown etiology characterized by variable types and degrees of parenchymal inflammation. Disease spectrum and prognosis considerably from those in adults. Respiratory bronchiolitis-associated interstitial lung disease (RB-ILD) is a well-described entity occurring almost exclusively in adults who are current heavy cigarette smokers. We describe an 11-year-old boy with failure to thrive, dry cough, and exertional dyspnea for 1 year who was diagnosed with RB-ILD due to heavy passive smoking exposure. Although RB-ILD is well defined in smoking adults, there are no reports in the English literature in nonactive smokers, especially in childhood

Pigeon breeder's disease as a cause of hypersensitivity pneumonia in children

Background and objectives. Hypersensitivity pneumonia is a complex condition due to exposure time, intensity, different clinical presentation, and treatment practices. We aimed to evaluate the patients that were diagnosed with hypersensitivity pneumonia (HSP) due to exposure to pigeons and a review of the literature for diagnosis and treatment of Pigeon Breeder's Disease (PBD) in children. Method. Between the years of 2009-2018, patients who were diagnosed with HSP due to PBD were included in the study in a pediatric pulmonology department. Findings of our patients, treatments, and prognoses were compared with 17 articles in the literature about PBD in children. Results. In a 9 year-period, 6 patients were diagnosed as HSP due to PBD. The mean age of the patients was 8.8 +/- 5.4 years and the average duration of pigeon exposure was 60.1 +/- 6.5 days. Precipitating antibodies were positive in 3 patients. In four cases, symptoms were resolved with only prevention of pigeon exposure. Two patients who had close contact with pigeons needed oxygen supplementation and steroid therapy. Conclusion. Hypersensitivity pneumonia should be considered for the differential diagnosis of patients that present with respiratory distress, cough, fever, and weight loss. Prolonged exposure and close contact may worsen the clinical symptoms. In most cases, only exposure prevention is enough, while steroid therapy, oxygen support, and intensive care monitoring may be required in severe cases

Left upper lobe atelectasis due to plastic bronchitis

Plastic bronchitis is a rare condition in children, characterized by expectoration of branching bronchial casts. It can cause atelectasis in the lung. Herein we reported a 4.5-year-old boy with left upper lobe atelectasis due to plastic bronchitis. Although his chest X-ray is specific for left upper left atelectasis, thoracic computerized tomography had been performed and was compatible with obliterated left upper lobe bronchus. Typical radiological appearance of the left upper lobe atelectasis is not well known by clinicians which results unnecessary further examinations such as computerized tomography which exposes high dose radiation. We want to emphasize the long-term side effects of radiation and avoid unnecessary examinations in children

Respiratory Bronchiolitis-Associated Interstitial Lung Disease in Childhood: New Sequela of Smoking

Childhood interstitial lung diseases are rare disorders of largely unknown etiology characterized by variable types and degrees of parenchymal inflammation. Disease spectrum and prognosis considerably from those in adults. Respiratory bronchiolitis-associated interstitial lung disease (RB-ILD) is a well-described entity occurring almost exclusively in adults who are current heavy cigarette smokers. We describe an 11-year-old boy with failure to thrive, dry cough, and exertional dyspnea for 1 year who was diagnosed with RB-ILD due to heavy passive smoking exposure. Although RB-ILD is well defined in smoking adults, there are no reports in the English literature in nonactive smokers, especially in childhood