PubMedDate not supplied
PURPOSE: Primary immunodeficiencies (PI) are a heterogeneous and relatively uncommon group of conditions. As a result of one or more immune system abnormalities, PI decrease the body resistance to infections. The respiratory tract is affected in most cases as a result of its natural exposure to pathogenic agents; repeated infections can lead to pulmonary alterations. We investigated the yield of High Resolution CT (HRCT) and Helical CT in pediatric patients with different PI and then correlated the CT patterns with the patients' clinical history and physical status. MATERIAL AND METHODS: We examined 19 pediatric patients with different types of PI. All patients presented recurrent infections (rhinosinusitis, bronchitis, bronchopneumonia) with cough and chronic catarrh for at least 3 months a year. Fifteen patients were examined with HRCT and 4 with Helical CT. Images were evaluated for presence and severity of alveolitis, bronchiectasis, peribronchial thickening, mucous plugs, air trapping, bronchiolitis, consolidation, abscesses, bullae, emphysema and fibrotic changes. All parameters were given a score and a partial and an overall score calculated for each parameter. All scores were compared to study the correlations between CT patterns, clinical history and patients' status and for possible characterization of the different groups by CT patterns. Finally, all alterations were classified by their anatomical distribution in each pulmonary lobe. RESULTS: Scores ranged 0 to 18, with a mean of 8.1 points. There were no correlations between CT patterns, patients' history and clinical status and any disease type. The midlobe was the preferential site for bronchiectasis while lower lobes were more involved by other conditions, such as consolidation, air trapping and alveolitis. CONCLUSIONS: Both HRCT and Helical CT proved to be useful tools for monitoring PI patients. The two techniques can be especially valuable in symptomatic patients with negative radiographic findings because they can show (non-)reversible damage, which helps improve planning of drug and/or physical therapy. The scoring system, even if not closely correlated with clinical signs, could be a major tool for PI follow-up and for monitoring treatment success.