Determination of mortality from cystic fibrosis

Assessing the prognosis of cystic fibrosis (CF) and evaluating the effect of indicators of mortality is very important in predicting the life expectancy of the CF patients.

Determining the effect of seven variables including sex, Forced Expiratory Volume in one second (FEV1), Body Mass Index (BMI), bacteriology, hemoglobin (Hb), pulmonary arterial pressure (PAP) and the number of previous admissions on the survival of 27 patients admitted in Pediatric Pulmonary Ward of Masih Daneshvari Hospital in 2007-2009.

27 CF patients were enrolled in a retrospective cross-sectional study. Patients data were collected during 2 years of study. Data of patients who died and those who remained alive were compared by independent samples t-tests and Chi-square.

Twenty seven CF patients (11 female, 10 male) with age range of 5-19 years and mean age of 13.11 +/- 4.69 were studied. There was no difference in age, sex, FEV1, BMI, Hb between the deceased and alive group (p > 0.05). Mean PAP for expired patients and alive patients was 40 +/- 15.1 and 68 +/- 11.5 respectively. The number of admissions during last 6 months was dominant in those patients who died. 50% of the alive patients were colonized with Pseudomonas. This is compared to deceased patients which 100% were colonized with Pseudomonas. There was a strong correlation between death and number of previous admissions, PAP and Pseudomonas infection (p < 0.05).

Pseudomonas infection, number of previous admissions and the severity of pulmonary hypertension has shown to be the major predictors of mortality in our study.