Survival of patients with rare diseases: a population-based study in Tuscany (Italy)

Table 3 Effect of sex, age at diagnosis, and period of diagnosis on mortality risk for total cases and by nosological group

	Sex		Age at diagnosis		Period of diagnosis
	HR^a	95% CI	HR^b	95% CI	HR^c	95% CI
Neoplasms	1.43	0.90–2.29	1.04	1.03–1.06	0.79	0.45–1.36
Endocrine diseases	2.01	1.02–3.94	1.09	1.06–1.13	1.16	0.41–3.26
Metabolic diseases	1.08	0.82–1.42	1.05	1.04–1.06	1.20	0.88–1.62
Immune system disorders	2.13	1.35–3.35	1.06	1.05–1.08	0.48	0.26–0.87
Diseases of the blood and blood-forming organs	0.99	0.52–1.88	1.07	1.05–1.09	0.93	0.47–1.86
Peripheral and central nervous system disorders	1.08	0.98–1.20	1.07	1.06–1.07	0.96	0.86–1.08
Disorders of the eye and adnexa	1.83	1.04–3.19	1.11	1.09–1.14	0.81	0.33–1.96
Circulatory system diseases	1.50	1.20–1.88	1.09	1.08–1.10	0.61	0.45–0.84
Respiratory diseases	1.79	1.43–2.23	1.08	1.07–1.09	0.80	0.62–1.03
Digestive disorders	0.62	0.30–1.31	1.12	1,07–1,16	0.95	0.33–2.76
Diseases of the genitourinary system	2.48	0.84–7.29	1.12	1.06–1.18	0.34	0.06–1.90
Diseases of the skin and subcutaneous tissue	1.53	1.22–1.93	1.12	1.10–1.14	0.84	0.64–1.10
Diseases of the musculoskeletal system and connective tissue	1.63	1.24–2.14	1.10	1.09–1.12	0.64	0.49–0.84
Congenital anomalies, chromosomal aberrations and genetic syndromes^a	1.63	1.03–2.58	1.05	1,04–1,06	0.75	0.40–1.41
Total	1.48	1.38–1.58	1.07	1.07–1.08	0.89	0.82–0.96

^aOnly cases diagnosed beyond 1 month. For the nosological group of infectious and parasitic diseases the small number of cases did not allow to calculate HRs. Females were the reference group in the analysis by sex; the 2000–2009 period was the reference group and was compared to 2010–2018 period in the analysis by period of diagnosis. As for congenital anomalies, chromosomal aberrations and genetic syndromes, cases diagnosed by one month of age were excluded. HR hazard ratio, 95% CI 95% confidence interval
^aHRs were adjusted for age and period of diagnosis
^bHRs were adjusted for sex and period of diagnosis
^cHRs were adjusted for sex and age at diagnosis. Statistically significant differences are reported in bold

ISSN: 1750-1172