EPIDEMIOLOGICAL STUDY OF CYSTIC FIBROSIS OF THE PANCREAS

Abstract

414 patients seen at Hospital de Niños Ricardo Gutiérrez between 1968 and 1988 with the diagnosis of Cystic Fibrosis of the Pancreas (CFP) were studied. The purpose was to find risk factors during follow up. The items investigated were age at diagnosis and at admission, sex, year and severity at admission, total number of siblings and of affected siblings and follow up. The mean age at diagnosis, admission and death were 3.28 ± 4.2; 3.76 ± 4.55 years; and 8.55 ± 7.06, respectively. The mortality rate was 8.1 deaths per 100 year/patients. This rate was 10.2 for 1968-74, 9.5 for 1975-81 and 6.0 for 1982-88 (p < 0.05). Severity on admission, monitored by the Shwachman score, was decreasing. In the 1968-74 period half of the patients had severe forms of the disease while in 1982-88 only 26.3% were severe (p < 0.05). This was inversely correlated with patient survival. Sex wasn't significant for prognosis. Patients who were older on admission had higher mortality rates. Because of the high rate (74.8%) of patients with a severe forms without follow up, we couldn's demonstrate significant differences in patient survival according to age at admission. Patients who had one or more siblings with CFP had lower mortality rates. After 3 years of follow up survival of patients reached 68.5%, after 6 years this was 50% and after 10 years; 34.8%. During 1982-88 3 year survival was (82.4%) more frequent than in 1968-74 (67.5%) and 1974-81 (71.8%). These significant differences disappeared at 6 and 10 years of follow up.