Terminale Lungeninsuffizienz bei milder Mukoviszidose

We present the case of a female CF-patient with primary mild disease and pancreatic sufficiency. She did not attend any CF centres for long periods of time. With progression of the disease, she was referred to our thoracic surgery centre for lung transplantation (LTX) at the age of 16 years. 2? years previously, she had been diagnosed with CF in another CF centre. Follow-up in specialised centres was recommended, but was not followed. Nevertheless, the indication for LTX was questioned by the thoracic surgeons and she was referred to our CF centre. With CF-specific therapy according to the current standards, pulmonary function improved from initially FEV1 = 1.2 L (38 %) to 3.6 L (122 %). In parallel, the patient gained 13.1 kg in body weight. According to our case report and to recent data a significantly better pulmonary function and nutritional status is found in CF patients who had attended specialised centres. We discuss the special features of CF treatment. Our report underlines the necessity for CF patients to be treated in specialised centres.