Pediatric Idiopathic Pulmonary Fibrosis: A Case Series report

Idiopathic Pulmonary Fibrosis (IPF) is a chronic rare disease with unknown etiology which is usually fatal and occurs mostly in adults. This disease is extremely rare in children and infants. Eight children with IPF who were examined in the pediatric department of Masih Daneshvari Hospital during 2001-2004 have been reported.There were 5 boys and 3 girls at the age range of 7 to 13 years (mean age 10.2 yrs). Clinical symptoms were cough and dyspnea in all patients which were progressive and exacerbated by recurrent respiratory infections. In clinical examination, crackles were found in both lungs of all patients while cyanosis and clubbing were detected in 5 subjects. Chest x ray showed reticulonodular and bullocystic patterns in 6 and 2 cases respectively. Pulmonary function tests in all patients were in concord with those of pulmonary restrictive diseases.To make a definite diagnosis, lung biopsy was performed in all patients which showed histologic changes in accord with IPF. Patients were mostly treated with steroids. In the present study, the prognosis of disease has been good in our patients and only one death occurred due to respiratory failure. To our knowledge, this study is the first report of P.I.P.F (Pediatric Idiopathic Pulmonary Fibrosis) in Iran. Other cases of this disease may be found in other medical centers which should be collected and evaluated by the researchers.