Pharmacotherapy challenges of Fontan-associated plastic bronchitis: a rare pediatric disease.

摘要: Pediatric pharmacotherapy is often challenging due to the paucity of available clinical data on safety and efficacy drugs that are commonly used in children. This quandary even more prevalent children with rare diseases. Although extrapolations for dosing administration made from adult similar disease states, this translation becomes problematic pediatric Understanding pathophysiology typically poor, few, if any, effective therapies have been studied identified. One condition illustrates these issues plastic bronchitis, a rare, most characterized by production obstructive bronchial airway casts. illness primarily occurs congenital heart disease, after palliative surgery. Plastic bronchitis highly clinically relevant therapeutically problem high mortality rate, and, generally accepted regimen has yet be Furthermore, ill defined, which makes timely identification treatment difficult. The pharmacotherapies currently manage largely anecdotal vary between use macrolide antibiotics, mucolytics, bronchodilators, inhaled fibrinolytics myriad combinations. purpose review 2-fold: first, highlight dilemma treating second, bring attention continuing need studies drug so safe regimens can established, particularly