STUDIES IN CYSTIC FIBROSIS

Report of 130 Patients Diagnosed Under 3 Months of Age Over a 20-Year Period

¹ Division of Clinical Laboratories and the Department of Medicine, Children's Hospital Medical Center, the Department of Pediatrics, Harvard Medical School, and the Children's Cancer Research Foundation, Boston

Early diagnosis and treatment can alter the morbidity and mortality of patients with cystic fibrosis (CF). This conclusion is based on a study of 130 patients in whom the diagnosis was made under the age of 3 months during the 20year period from 1949 to 1969. These patients were observed for a total of 912 patient-years.

The patients were segregated into three groups as follows: Group A (63) were diagnosed prior to the development of symptoms; Group B (13) had mild symptoms; Group C (54) were diagnosed during hospitalization. Immediately following the diagnosis all patients were started on a therapeutic program and followed at regular intervals. There were 29 deaths, 11 in Groups A and B, and 18 in Group C, 8 of which occurred under 1 year of age.

The present clinical status of the 101 surviving children is an follows: 14 are in excellent condition, 71 have mild disease or are in good health, and 12 are moderately or severely ill and have a poor prognosis. For all the 130 patients the calculated survival rate at age 20 is 77%.

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