A review of 151 cases of pediatric noncystic fibrosis bronchiectasis in a tertiary care center

OBJECTIVE: This study was conducted to review the etiological factors and diseases associated with pediatric noncystic fibrosis bronchiectasis in a tertiary care center in Saudi Arabia. MATERIALS AND METHODS: A retrospective review of all patients with confirmed noncystic fibrosis (Non-CF) bronchiectasis by chest X-ray and/or CT chest in a pulmonary clinic during the period 1993-2005 at a tertiary care center in Riyadh. RESULTS: A total of 151 cases were diagnosed as Non-CF bronchiectasis. Seventy-five (49.7%) were male, 76 (50.3%) were female; 148 (98%) are alive and 3 (2%) died. The southwestern regions constituted 72 (50%) of the cases. There was a period of (5 +/- 3.2) years between the start of symptoms and diagnosis of bronchiectasis. More than two-thirds of the patients had cough, tachypnea, wheezing, sputum production and failure to thrive. Ninety-one (60%) had associated diseases: Pulmonary diseases in 48 (32%), immunodeficiency in 27 (18%), central nervous system anomalies in 10 (7%), cardiac in 10 (7%) and asthma in 103 (68%) of the patients. Left lower lobe was commonly involved in 114 (76%) patients. Sixty-eight (67%) were found to have sinusitis. More than two-thirds of patients had two or more associated diseases. Forty-nine (32%) developed gastroesophageal reflux. Hemophilus influenza was cultured in 56 (37%), strep pneumoniae in 25 (17%) and pseudomonas aeruginosa in 24 (16%) of the patients. Eighty percent of the patients who had pulmonary function test had abnormal changes. Disease progression was related to development of symptoms before 5 years of age, persistent atelectasis and right lower lobe involvement (P< 0.05). CONCLUSION: Non-CF bronchiectasis should be included in the differential diagnosis of recurrent chest infection in Saudi Arabia. Early diagnosis and identification of associated diseases is needed to prevent progression of the disease.