Modern ideas about obliterating bronchiolitis in children

The literature review presents modern information on obliterating bronchiolitis (OB) in childhood. It consistently provides information on deficiencies, morphology, coding of the disease according to ICD10, history of study, etiology, epidemiology, classification and pathogenesis of OB. Biomarkers, clinical, radiological picture and functional diagnosis of OB are separately characterized. Based on a generalization of literature and authors' own data, the diagnostic criteria for post-infectious OB as the most common version of OB in children are provided. The approaches to therapy and disease prognosis are described. © 2020, Pediatria Ltd.. All rights reserved

Issues of diagnosis and management of patients with neuroendocrine cell hyperplasia of infancy

Neuroendocrine hyperplasia of infancy (NEHI) is an interstitial lung disease (ILD) of unknown etiology, which develops in infancy and manifests as persistent tachypnea. Objective of the research: to summarize results of observations of patients with NEHI, including during the pandemic of a new coronavirus infectious disease (COVID-19). Materials and methods: clinical and follow-up observation, laboratory and instrumental examination, including lung biopsy, 52 children with NEHI aged from 21 days to 10 years. Results: NEHI more often affects boys in the first 6 months of life; can be diagnosed on the basis of pediatric ILD-syndrome detection; specific CT signs, lung biopsy. It is characterized by undulating course with increased respiratory failurec aused by acute respiratory viral infections, COVID-19; half of the patients need oxygen therapy. © 2020, Pediatria Ltd.. All rights reserved

The structure of interstitial lung diseases in children of the first two years of life

For the first time in Russia, the article provides data on interstitial lung diseases structure in children of the first two years of life, based on a series of observations of 68 patients with these rare diseases, as a part of multi-center ambispective study. Interstitial lung diseases in observed children included: Wilson-Mikity syndrome (23,4%), neuroendocrine hyperplasia of infancy (22%), bronchiolitis obliterans with organizing pneumonia (7,4%), primary pulmonary hypoplasia (1,5%), secondary pulmonary hypoplasia with Jeune syndrome (10,3%), secondary pulmonary hypoplasia with Edwards syndrome (2,9%), secondary pulmonary hypoplasia with other associated pathology (omphalocele - 1,5%, non-immune fetal hydrops - 1,5%), subpleural cysts in patients with Down syndrome (5,9%), congenital deficiency of surfactant protein B (1,5%), brain-lung- thyroid syndrome (2,9%), congenital alveolar-capillary dysplasia (1,5%), interstitial lung diseases with systemic diseases (Langerhans cell histiocytosis - 16,2%, Niemann-Pick disease - 1,5%). The article summarizes clinical features, the results of image diagnosis and disease outcomes. © 2015, Pediatria Ltd. All rights reserved

The structure of interstitial lung diseases in children of the first two years of life

For the first time in Russia, the article provides data on interstitial lung diseases structure in children of the first two years of life, based on a series of observations of 68 patients with these rare diseases, as a part of multi-center ambispective study. Interstitial lung diseases in observed children included: Wilson-Mikity syndrome (23,4%), neuroendocrine hyperplasia of infancy (22%), bronchiolitis obliterans with organizing pneumonia (7,4%), primary pulmonary hypoplasia (1,5%), secondary pulmonary hypoplasia with Jeune syndrome (10,3%), secondary pulmonary hypoplasia with Edwards syndrome (2,9%), secondary pulmonary hypoplasia with other associated pathology (omphalocele - 1,5%, non-immune fetal hydrops - 1,5%), subpleural cysts in patients with Down syndrome (5,9%), congenital deficiency of surfactant protein B (1,5%), brain-lung- thyroid syndrome (2,9%), congenital alveolar-capillary dysplasia (1,5%), interstitial lung diseases with systemic diseases (Langerhans cell histiocytosis - 16,2%, Niemann-Pick disease - 1,5%). The article summarizes clinical features, the results of image diagnosis and disease outcomes. © 2015, Pediatria Ltd. All rights reserved