TY - JOUR T1 - Community-acquired pneumonia in pediatric patients with heritable connective tissue disorders: Novel phenotypic variants JF - European Respiratory Journal JO - Eur Respir J VL - 40 IS - Suppl 56 SP - P2957 AU - Zoia Nesterenko AU - Olena Ivanina Y1 - 2012/09/01 UR - http://erj.ersjournals.com/content/40/Suppl_56/P2957.abstract N2 - Background: The prevalence of heritable connective tissue disorders (HCTD) changed manifestations of community-acquired pneumonia (CAP) in pediatric patients, leading to diagnostic and therapeutic errors.Objective: to study clinical features of CAP in children with HCTD.Material and methods: 105 patients aged 3-18 years, with clinically, serologically and radiographically diagnosed CAP, were followed up for two years. 60(57.4%) patients had moderate persistent asthma (A). All pediatric patients had HCTD manifestations.Results: CAP caused by Mycoplasma pneumonia (Mp) was in 89.1% of patients, by Cytomegalovirus (Cmv) - in 26.7%, by Chlamydophilla pneumonia (Cp) - in 21.9%. CAP was caused by association of Mp, Cp and Cmv in 38.1% of children. CAP in asthmatic patients occurred with longer duration of respiratory symptoms and bronchial obstruction (11.5±2 days). Pulmonary hypertension (PH) was in 51,7% of patients with CAP and A, with pulmonary fibrosis (PF) in 48.3% of this group of patients. 26.7% of children with PH and PF had CT evidence of emphysematous bullae (EB). Patients with CAP and A received antibiotic treatment (macrolides) (ABT) besides controller therapy for achievement of A control.Conclusions: 1. Close relationship between CAP, caused by intracellular pathogens, and HCTD is revealed. 2. CAP provoked A exacerbation in 57.4% of patients. 3. CAP in asthmatic patients had more long-term and severe clinical course. 4. PH was diagnosed in 51.7% of patients with CAP, A and HCTD, 48.3% of them had PF. 5. 26.7% of patients with PH and PF had EB. 6. It is advisable to consider CAP associated with HCTD and A as clinical phenotypes of CAP. ER -