Document Type : Case Report
Children Medical Center Hospital, Tehran University School of Medical Sciences, Tehran, Iran
Children’s Hospital, Tabriz University of Medical Sciences, Tabriz, Iran
Cystic fibrosis (CF), the most common life-shortening, hereditary disease in whites, manifests itself principally in childhood. Patients presenting with CF as adults appear to be different when compared to patients diagnosed with CF during childhood. Often these patients have been previously diagnosed with asthma, chronic bronchitis or emphysema. We present a case of a woman diagnosed with CF at age 37 years. We noticed her finger clubbing during her son’s hospital admission for CF decompensation. Taking a thorough history, she complained of chronic productive cough and was treated for hyper reactive airway disease for many years. A Computed Tomography scan was performed which showed bronchiectasis, atelectasis and the presence of a honey comb pattern in her lung fields. Two sweat tests were performed, both of which were strongly positive. Her CF diagnosis was confirmed. The clinical course of patients receiving a diagnosis of CF in adulthood is largely unknown, but frequently they have milder disease and a more favorable prognosis. The proportion and number of patients with CF diagnosed in adulthood has increased. A large number of these patients present with subtle symptoms or single-organ disease. Since the majority have pulmonary disease CF should be included in the differential diagnosis of chronic respiratory symptoms in adults.