Background: The largest age group among children and adolescents referred for lung transplantation for cystic fibrosis (CF) have been those who in the pubertal or post pubertal age range. However, over 100 younger patients with CF have undergone lung transplantation over the last three decades in the USA. Methods: We performed a retrospective review of our experience with 18 children with CF who underwent lung transplantation in our center before the age of 11 years. Results: Notable findings were a high prevalence of methicillin-resistant Staphylococcus aureus, a high prevalence of diabetes mellitus and an impressive prevalence of consolidated lobar or whole lung disease. Post-transplant outcomes, however, were comparable to those older than 10 years of age in our center. Conclusions: In an era of increasingly effective medications modifying the natural history of CF, identification of risk factors for early severe lung disease in CF remains relevant and potentially more important for effective intervention in order to extend life.

Pulmonary hypertension represents an increasingly important group of pediatric patients which commonly come to the attention, if not the primary care of pediatric pulmonologists around the world. There have been major advances in diagnosis and therapy over the past 25 years. To address potential gaps in knowledge, the authors were invited by the Editor of Pediatric Pulmonology to organize a series of manuscripts in a special supplement of the journal. Our authors include pulmonologists, pharmacists, intensivists, mid-level practitioners, neonatologists and cardiologists. We believe that this issue will be of great interest to most of the readership community that the Journal addresses.