Scoliosis Thoracic cage dystrophy Pulmonary osteoarthropathy Cirrhosis Portal hypertension Fatty infiltration of liver Gall stones Malnutrition Vitamin A deficiency Growth retardation Hyponatremia Hypoprothrombinemia Meconium ileus

T his article presents our experience with bronchoscopy as a therapeutic and diagnostic tool in the management of patients with cystic fibrosis. It is well known that patients with cystic fibrosis present a wide variety of disorders in many organ systems ( Table 1 ) . Some of these disorders are related to the enzymochemical defect and some occur secondarily as a result of the pathophysiology of the disease. We will confine our discussion exclusively to the problem of the pulmonary complications ( Table 2). Following the initial diagnosis of cystic fibrosis, a strict plan of meticulous management is instituted which covers the dietary, pulmonary, social, family and psychologic aspects. After the institution of these measures the disease enters a period of relative quiescence and the patient lives a full and active life until a critical pulmonary event occurs when for some inexplicable reason the patient starts a downhill course. This course is reflected in many of the parameters of pulmonary function as illustrated in the case report summarized in Figure 1. Some patients will survive with a steadily worsening pulmonary problem for many years, while others may succumb within a few months, as is shown by the patient, in Figure 1. The main thrust of our efforts with bronchoscopy oven recent years has been to try to alter the rate of deterioration. Over 100 bronchoscopic procedures in cystic fibrosis patients have been performed at the University of Minnesota. Various techniques with the rigid bronchoscope and flexible bronchoscope have been employed. The flexible bronchoscope is reserved for patients whose pulmonary status is not severely compromised and is used mainly for diagnostic purposes. In the majority of our patients we use the rigid bronchoscope, as we consider this to be much safer in these high risk individuals. Bronchoscopy is performed in three basic situations, as follows. Emergency Bronchoscopy in Acute Respiratory Distress