Pulmonary hypertension and the common organisms present in the sputum culture in children with cystic fibrosis

Background: Cystic fibrosis is an autosomal recessive genetic defect that affects multiple organs in the body. These patients present with progressive obstructive lung disease, sinusitis, exocrine pancreatic insufficiency and male infertility. The respiratory tract of these patients is more vulnerable for bacterial colonization, most commonly Pseudomonas aeruginosa, Hemophilus influenza and Staphylococcus aureus. There is a possibility of pulmonary hypertension development in these patients due to alveolar hypoxia alongside chronic systemic inflammatory response causing endothelial dysfunction. Methods: This was a descriptive cross-sectional study which studied 109 cystic fibrosis patients admitted to CMC, Tehran in 2 years. Results: In this study 109 patients were studied. 35 patients (32.1%) were <1 year of age, 11 patients (10.1%) were 1-5 years of age, 26 patients (23.9%) were 5-10 years of age and 37 patients (33.9%) were >10 years of age. 61 patients (56%) were females and 48 patients (44%) were males. The most common organisms encountered in sputum culture were Staphylococcus aureus (23.9%), Pseudomonas aeruginosa (18.3%) and Candida albicans (11.9%). Pulmonary arterial hypertension was present in 24.7% of the target population, out of which 22.9% were having mildly elevated PAH and 1.8% had severely high PAH. Conclusions: The most common organism seen is S. aureus followed by P. aeruginosa. A significant number of patients presented with mildly elevated PAH and some with severely high PAH.