A population-based study to estimate survival and standardized mortality of tuberous sclerosis complex (TSC) in Taiwan

Table 3 Life tables and standardized mortality ratio (SMR) chart for patients with Tuberous Sclerosis Complex (TSC) in Taiwan, 1997–2010. (a) Life table of 100,000 hypothetical TSC patients, based on age at endpoint (death or 2010) and mortality rate (MR). (b) Life table of 100,000 hypothetical TSC patients, based on enrollment age groups and MR. (c) MR and SMR as compared to the general population in the same period

Age at endpoint	TSC patients	Deaths	Probability of death	Expected survivals in hypothetical group	Expected deaths in hypothetical group	Person-years lived between this age group until the next	Total number of person-years lived above age of age group	ARL
(a)
< 1	2	1	0.50	100,000	50,000	57,500	1,794,804	17.95
1–18	235	2	0.16	50,000	7,843	921,569	1,737,304	34.75
19–40	196	6	0.47	42,157	19,761	645,527	815,735	19.35
> 40	38	5	1.00	22,396	22,396	170,208	170,208	7.60
(b)
< 1	36	1	0.03	100,000	2,778	97,639	3,169,221	31.69
1–18	279	3	0.19	97,222	18,878	1,755,663	3,071,582	31.59
19–40	129	5	0.56	78,344	43,768	1,129,206	1,315,919	16.80
> 40	27	5	1.00	34,576	34,576	186,713	186,713	5.40

Year	TSC patients	Number of deaths in Taiwan	Population of Taiwan	MR in the general population	Expected deaths (TSC x MR)	Observed deaths in TSC patients
1997	8	121,000	21,742,815	0.0056	0.04	0
1998	20	123,180	21,928,591	0.0056	0.11	0
1999	21	126,113	22,092,387	0.0057	0.12	0
2000	19	125,958	22,276,672	0.0057	0.11	0
2001	23	127,647	22,405,568	0.0057	0.13	0
2002	36	128,636	22,520,776	0.0057	0.21	0
2003	57	130,801	22,604,550	0.0058	0.33	2
2004	23	135,092	22,689,122	0.0060	0.14	0
2005	65	139,398	22,770,383	0.0061	0.40	2
2006	50	135,839	22,876,527	0.0059	0.30	2
2007	44	141,111	22,958,360	0.0061	0.27	2
2008	43	143,624	23,037,031	0.0062	0.27	2
2009	39	143,582	23,119,772	0.0062	0.24	3
2010	23	145,772	23,162,123	0.0063	0.14	1
Total	471		2.97%/14 years 0.21%/years		2.81/14 years	14/14 years
		MR	2.97%/14 years 0.21%/years	SMR (95% CI)	4.99 (2.34–7.60) P = 0.0028

SMR was calculated by the equation \(\frac{\text{Observed no. of deaths per year}}{\text{Expected no. of deaths per year}}\); because TSC mortality numbers are often at zero for a given year, an average mortality of across 14 years was used instead of year-to-year mortality. ARL, average remaining lifetime; CI, confidence interval

ISSN: 1750-1172