Depending on the underlying genetic variations, patients have a poor prognosis with substantial mortality. However, the group of children and adolescents with chILD who reach adulthood is growing. How are these patients doing? What are the problems in this orphaned group and how are the patients coping with their disease?
How successful is the transition from care in pediatric clinics into adult services? Over the last decade, the spectrum of conditions within chILD has widened substantially. However, most evidence is often limited to small case series, with reports disseminated across an array of severe subspecialties, clinical and molecular journals.
In particular, the frequency, management and outcome of pediatric pulmonary fibrosis is not well characterized, unlike in adults, where clear diagnosis and treatment guidelines are available.
The paper in Thorax assessed the current understanding of pulmonary fibrosis in chILD. Based on registry data, the occurrence of pulmonary fibrosis in was identified in 47 different disease entities. The team's findings: When patients transitioned from pediatric to adult specialty care, diagnoses corresponded mostly, but not always to similar ILD categories in the adult classification systems.
This may lead to potentially inappropriate diagnostic labeling. The researchers are convinced that the effects of this suboptimal transition to adult ILD need to be further investigated. Re-specification of diagnosis and targeted treatments could contribute to precision and personalization.
