Table 1 Survival of the cohort at 1, 5 and 10 years from diagnosis for the total number of cases and for each nosological group
From: Survival of patients with rare diseases: a population-based study in Tuscany (Italy)
CASES (n) | Age at diagnosis (mean ± standard deviation) | % males | 1 year | 95% CI | 5 years | 95% CI | 10 years | 95% CI | |
|---|---|---|---|---|---|---|---|---|---|
Peripheral and central nervous system disorders | 5928 | 53.0 ± 21.3 | 51.6 | 0.953 | 0.948–0.959 | 0.796 | 0.785–0.807 | 0.679 | 0.664–0.694 |
Congenital anomalies, chromosomal aberrations and genetic syndromesa | 2377 | 17.4 ± 18.2 | 51.5 | 0.998 | 0.995–1.000 | 0.984 | 0.977–0.989 | 0.961 | 0.949–0.970 |
Circulatory system diseases | 2177 | 55.4 ± 19.7 | 35.2 | 0.982 | 0.975–0.987 | 0.917 | 0.903–0.929 | 0.800 | 0.776–0.821 |
Diseases of the musculoskeletal system and connective tissue | 1826 | 57.3 ± 15.5 | 18.9 | 0.973 | 0.964–0.980 | 0.892 | 0.875–0.907 | 0.768 | 0.740–0.794 |
Metabolic diseases | 1773 | 36.7 ± 28.0 | 58.2 | 0.962 | 0.951–0.963 | 0.899 | 0.883–0.914 | 0.845 | 0.822–0.865 |
Respiratory diseases | 1639 | 60.1 ± 16.4 | 57.3 | 0.947 | 0.934–0.957 | 0.719 | 0.687–0.749 | 0.565 | 0.520–0.608 |
Diseases of the skin and subcutaneous tissue | 1413 | 58.9 ± 22.3 | 43.9 | 0.949 | 0.936–0.960 | 0.811 | 0.786–0.833 | 0.715 | 0.677–0.748 |
Diseases of the blood and blood-forming organs | 1361 | 27.9 ± 22.3 | 48.6 | 0.992 | 0.986–0.996 | 0.972 | 0.960–0.981 | 0.962 | 0.947–0.973 |
Immune system disorders | 1331 | 38.5 ± 23.0 | 38.8 | 0.998 | 0.994–1.000 | 0.969 | 0.956–0.979 | 0.903 | 0.875–0.925 |
Disorders of the eye and adnexa | 1301 | 37.5 ± 18.6 | 57.3 | 0.998 | 0.993–0.999 | 0.989 | 0.981–0.994 | 0.964 | 0.947–0.975 |
Endocrine diseases | 1036 | 24.1 ± 20.5 | 26.7 | 1.000 | – | 0.988 | 0.976–0.994 | 0.957 | 0.933–0.973 |
Neoplasms | 987 | 31.5 ± 22.7 | 45.2 | 0.986 | 0.977–0.992 | 0.944 | 0.926–0.958 | 0.923 | 0.899–0.941 |
Diseases of the genitourinary system | 489 | 43.0 ± 20.5 | 47.8 | 0.996 | 0.983–0.999 | 0.989 | 0.971–0.996 | 0.954 | 0.907–0.977 |
Digestive disorders | 257 | 51.1 ± 21.4 | 53.7 | 0.968 | 0.936–0.984 | 0.896 | 0.841–0.933 | 0.818 | 0.720–0.884 |
1infectious and parasitic diseases | 13 | 47.2 ± 19.7 | 53.8 | 1.000 | – | 0.909 | 0.508–0.987 | 0.909 | 0.508–0.987 |
Total | 23,671 | 44.3 ± 25.0 | 46.0 | 0.973 | 0.971–0.975 | 0.888 | 0.884–0.893 | 0.808 | 0.801–0.815 |