Figure 6

Survival and occurrence of fibrosis. All curves were performed using Kaplan-Meier method. A. Overall survival from birth showing a 5-year survival rate of 65%. B. Overall survival from birth according to gender. Five-year survival from birth was significantly lower in boys than in girls (54% in boys versus 83% in girls, logrank test, p = 0.04). C. Occurrence of fibrosis according to the age at which lung biopsy was performed showing a probability of significant pulmonary fibrosis of almost 50% by the age of 14 years old. D. Overall survival from birth with or without WLL. WLL did not significantly change global survival rates (logrank test, p = 0.46). E. Overall survival from birth with or without WLL in patients diagnosed before the age of one year with a larger number of WLL carried out earlier (WLL+, n = 14 / WLL-, n = 5). In this group, this treatment did not significantly change global survival rates (logrank test, p = 0.39).