Pediatric pulmonology 2021 year in review: Rare and diffuse lung disease

Abstract

The field of rare and diffuse pediatric lung disease is experiencing rapid progress as diagnostic and therapeutic options continue to expand. In this annual review, we discuss manuscripts published in Pediatric Pulmonology in 2021 in (1) children's interstitial and diffuse lung disease, (2) congenital airway and lung malformations, and (3) noncystic fibrosis bronchiectasis including primary ciliary dyskinesia. These include case reports, descriptive cohorts, trials of therapies, animal model studies, and review articles.The results are put into the context of other literature in the field. Each furthers the field in important ways, while also highlighting the continued need for further studies.

Incidence, prevalence, and pattern of medical service utilization of children's rare lung diseases in South Korea

“…Main characteristics of children's rare lung diseases are their great heterogeneity among all entities they encompass and their low prevalence. We classified children's rare lung diseases into five different groups based on a previous paper 6 . When analyzing frequencies by age group, we found more than 65% of cases were diagnosed less than 2 years of age, with a mean prevalence rate of 131.4 cases per million in infants compared with 8.6 cases per million in those greater than 2 years of age.…”

“…Rare lung diseases were classified into the following groups: (1) chILD including idiopathic pulmonary fibrosis, alveolar proteinosis, microscopic polyarteritis, Wegener granulomatosis, necrotizing respiratory granulomatosis, Goodpasture's syndrome, polyarteritis with lung involvement, sarcoidosis of lung; (2) noncystic fibrosis bronchiectasis, including primary ciliary dyskinesia; (3) congenital airway and lung anomaly, including congenital malformation of diaphragm and congenital diaphragmatic hernia; (4) cystic fibrosis; and (5) others, including primary pulmonary hypertension and congenital central hypoventilation syndrome 6 …”