[Cystic fibrosis: long-term treatment at a specialized centre, and its prognostic value (author's transl)]

Abstract

Data are presented on morbidity, mortality, and age at diagnosis of 90 patients with cystic fibrosis who were seen at the Medical School of Hannover from 1975 to 1981. The status of patients was characterized by the clinical score and the score of chest roentgenogram. Course and prognosis in groups of patients defined by the items "false diagnosis", "false negative sweat test", "meconium ileus", "late onset of clinical symptoms after age 5", and "comprehensive long-term treatment at a centre for cystic fibrosis" were compared. In patients with intestinal or pulmonary symptoms a delay in diagnosis for patients, who died, died in early childhood. However, for those patients who receive proper medical care at a specialized CF-centre a clinical course can be achieved which may resemble the course of disease in patients with late onset of symptoms.