Categorizing diffuse parenchymal lung disease in children.

BACKGROUND: Aim of this study was to verify a systematic and practical categorization system that allows dynamic classification of pediatric DPLD irrespective of completeness of patient data. METHODS: The study was based on 2322 children submitted to the kids-lung-register between 1997 and 2012. Of these children 791 were assigned to 12 DPLD categories, more than 2/3 belonged to categories manifesting primarily in infancy. The work-flow of the pediatric DPLD categorization system included (i) the generation of a final working diagnosis, decision on the presence or absence of (ii) DPLD and (iii) a systemic or lung only condition, and (iv) the allocation to a category and subcategory. The validity and inter-observer dependency of this workflow was re-tested using a systematic sample of 100 cases. RESULTS: Two blinded raters allocated more than 80% of the re-categorized cases identically. Non-identical allocation was due to lack of appreciation of all available details, insufficient knowledge of the classification rules by the raters, incomplete patient data, and shortcomings of the classification system itself. CONCLUSIONS: This study provides a suitable workflow and hand-on rules for the categorization of pediatric DPLD. Potential pitfalls were identified and a foundation was laid for the development of consensus-based, international categorization guidelines.

Predictive values of antibodies against Pseudomonas aeruginosa in patients with cystic fibrosis one year after early eradication treatment.

BACKGROUND: Patient dependent parameters to predict the long-term success of early eradication treatment of Pseudomonas aeruginosa have not yet been defined. For this purpose we assessed serum antibodies against P. aeruginosa in CF patients after early eradication treatment. METHODS: Retrospective analyses of all consecutive patients with first P. aeruginosa detection 2005 to 2008. Absence of P. aeruginosa in the third year was defined as successful long-term eradication. Main outcome was to determine the predictive value of P. aeruginosa antibody results one year after initiation of early eradication treatment using antibodies against alkaline protease, elastase, and exotoxin A with regard to long-term success of eradication treatment. RESULTS: Antibodies against P. aeruginosa correlated well with success of eradication; positive and negative predictive values after one year were 75% and 82% respectively. The incidence of new detection of P. aeruginosa was 8.5%. Long-term eradication was successful in 32 of 53 patients (60%). CONCLUSIONS: Determination of serum antibodies against P. aeruginosa one year after first detection of P. aeruginosa and early eradication treatment can predict success of long-term eradication.