Causes of death and life span in Finnish gelsolin amyloidosis

Figures & data

Table 1. Characte ristics of AGel amyloidosis patients with death certificate available.

	Patients n = 272
	Underlying CoDavailable n = 267	Immediate CoDavailable n = 203
Female	141 (52.8%)	108 (53.2%)
Male	126 (47.2%)	95 (46.8%)
Age at death
30–59-year-olds	27 (10.1%)	15 (7.4%)
60–69-year-olds	49 (18.4%)	32 (15.8%)
70–79-year-olds	102 (38.2%)	78 (38.4%)
80–99-year-olds	89 (33.3%)	78 (38.4%)
Year of death
1969–1974	14 (5.2%)	n/a
1975–1984	40 (15.0%)	n/a
1985–1994	68 (25.5%)	58 (28.6%)
1995–2004	68 (25.5%)	68 (33.5%)
2005–2014	77 (28.8%)	77 (37.9%)

CoD: cause of death; n/a: information not available.

Table 2. Proportional mortality ratios (PMR) for selected medical conditions for AGel amyloidosis patients, underlying causes of death.

		All n = 267			Male n = 126			Female n = 141
Disease category	ICD-10	Observedn (%)	Expectedn (%)	PMR(95% CI)	Observedn (%)	Expectedn (%)	PMR (95% CI)	Observedn (%)	Expectedn (%)	PMR (95% CI)
Malignant neoplasms	C00–C97	26 (9.7%)	55.4 (20.7%)	0.47 (0.31–0.69)	13 (10.3%)	28.0 (22.2%)	0.46 (0.25–0.79)	13 (9.2%)	29.6 (21.0%)	0.44 (0.23–0.75)
Endocrine, nutritional and metabolic diseases	E00–E90	53 (19.9%)	0.5 (0.2%)	114.2 (85.6–149.4)	26 (20.6%)	0.2 (0.1%)	156.8 (102.5–229.8)	27 (19.1%)	0.30 (0.2%)	91.3 (60.2–132.8)
Ischaemic heart diseases	I20–I25	67 (25.1%)	73.1 (27.4%)	0.92 (0.71–1.16)	33 (26.2%)	38.4 (30.5%)	0.86 (0.59–1.21)	34 (24.1%)	36.0 (25.5%)	0.94 (0.65–1.32)
Other heart diseases (excl. rheumatic heart diseases)	I30–I425, I427–I52	9 (3.4%)	13.8 (5.2%)	0.65 (0.30–1.24)	4 (3.2%)	5.3 (4.2%)	0.76 (0.21–1.94)	5 (3.5%)	8.4 (6.0%)	0.59 (0.19–1.38)
Cerebrovascular diseases	I60–I69	24 (9.0%)	30.2 (11.3%)	0.79 (0.51-1.18)	11 (8.7%)	11.6 (9.2%)	0.95 (0.47–1.70)	13 (9.2%)	19.3 (13.7%)	0.67 (0.36–1.15)
Other diseases of the circulatory system	I00–I15, I26–I28, I70–I99	16 (6.0%)	12.6 (4.7%)	1.27 (0.73–2.06)	6 (4.8%)	5.2 (4.1%)	1.16 (0.43–2.53)	10 (7.1%)	7.6 (5.4%)	1.31 (0.63–2.41)
Pneumonia	J12–J18, J849	9 (3.4%)	10.1 (3.8%)	0.89 (0.41–1.69)	6 (4.8%)	4.6 (3.6%)	1.31 (0.48–2.85)	3 (2.1%)	5.5 (3.9%)	0.55 (0.11–1.60)
Diseases of the genitourinary system	N00–N99	5 (1.9%)	3.2 (1.2%)	1.54 (0.50–3.59)	2 (1.6%)	1.2 (1.0%)	1.65 (0.20–5.95)	3 (2.1%)	2.1 (1.5%)	1.43 (0.30–4.18)
Other accidents and sequelae of accidents	W20-W64, W75–X39,X50–X59, Y85–Y86	2 (0.7%)	2.0 (0.7%)	1.01 (0.12–3.64)	2 (1.6%)	1.1 (0.9%)	1.76 (0.21–6.37)	0 (0.0%)	0.56 (0.4%)	0.00 (0.00–6.55)
Suicides and sequelae of intentional self-harm	X60–X84, Y870	4 (1.5%)	5.1 (1.9%)	0.78 (0.21–1.99)	3 (2.4%)	2.9 (2.3%)	1.05 (0.22–3.06)	1 (0.7%)	1.1 (0.8%)	0.91 (0.02–5.08)

CI: confidence interval.

Table 3. Proportional mortality ratios (PMR) for selected medical conditions for AGel amyloidosis patients, immediate causes of death.

		All n = 203			Male n = 95			Female n = 108
Disease category	ICD-10	Observedn (%)	Expectedn (%)	PMR (95% CI)	Observedn (%)	Expectedn (%)	PMR (95% CI)	Observedn (%)	Expected n (%)	PMR (95% CI)
Malignant neoplasms	C00–C97	18 (8.9%)	37.4 (18.4%)	0.48 (0.29–0.76)	8 (8.4%)	17.7 (18.6%)	0.45 (0.20–0.89)	10 (9.3%)	19.7 (18.3%)	0.51 (0.24–0.93)
Endocrine, nutritional and metabolic diseases	E00–E90	16 (7.9%)	2.1 (1.0%)	7.76 (4.43–12.6)	7 (7.4%)	1.2 (1.3%)	5.64 (2.27–11.6)	9 (8.3%)	0.8 (0.8%)	11.0 (5.02–20.8)
Ischaemic heart diseases	I20–I25	47 (23.2%)	45.7 (22.5%)	1.03 (0.76–1.37)	23 (24.2%)	23.4 (24.7%)	0.98 (0.62–1.47)	24 (22.2%)	22.3 (20.6%)	1.08 (0.69–1.60)
Other heart diseases (excl. rheumatic heart diseases)	I30–I425, I427–I52	14 (6.9%)	11.6 (5.7%)	1.21 (0.66–2.03)	7 (7.4%)	5.4 (5.7%)	1.30 (0.52–2.69)	7 (6.5%)	6.2 (5.8%)	1.13 (0.45–2.32)
Cerebrovascular diseases	I60–I69	13 (6.4%)	17.8 (8.8%)	0.73 (0.39–1.25)	2 (2.1%)	5.8 (6.1%)	0.34 (0.04–1.24)	11 (10.2%)	12.0 (11.1%)	0.92 (0.46–1.64)
Other diseases of the circulatory system	I00–I15, I26–I28, I70–I99	10 (4.9%)	10.1 (5.0%)	0.99 (0.47–1.82)	6 (6.3%)	4.4 (4.6%)	1.36 (0.50–2.97)	4 (3.7%)	5.7 (5.3%)	0.70 (0.19–1.79)
Pneumonia	J12–J18, J849	36 (17.7%)	34.6 (17.1%)	1.04 (0.73–1.44)	22 (23.2%)	16.7 (17.6%)	1.32 (0.83–1.99)	14 (13.0%)	17.9 (16.6%)	0.78 (0.43–1.31)
Diseases of the genitourinary system	N00–N99	8 (3.9%)	3.5 (1.7%)	2.29 (0.99–4.52)	1 (1.1%)	1.0 (1.1%)	1.00 (0.03–5.55)	7 (6.5%)	2.5 (2.3%)	2.82 (1.13–5.81)
Other accidents and sequelae of accidents	W20–W64, W75–X39,X50–X59, Y85–Y86	1 (0.5%)	1.0 (0.5%)	0.96 (0.02–5.33)	1 (1.1%)	0.71 (0.7%)	1.42 (0.04–7.89)	0 (0.0%)	0.3 (0.3%)	0.00 (0.00–10.9)
Suicides and sequelae of intentional self-harm	X60–X84, Y870	2 (1.0%)	2.6 (1.3%)	0.76 (0.09–2.75)	1 (1.1%)	1.9 (2.1%)	0.51 (0.01–2.86)	1 (0.9%)	0.7 (0.6%)	1.47 (0.04–8.20)

CI:  confidence interval.