Table 1.
Classification of all registered deaths in children < 15 years in Ireland for the period 2006–2016 by age group and within age groups those identified as having a rare disease
| Age group | Total number of cases n (%) | Rare disease cases n (%) | Rare disease cases as a % of total n (%) | Crude mortality rate per 100,000 [7] | |
|---|---|---|---|---|---|
| 2006–2016 | 2006–2016 | 2006–2016 | 2006 | 2016 | |
| Neonatal | 2050 (51%) | 1140 (41%) | 1140/2050 (55.6%) | – | – |
| Post-neonatal | 778 (19%) | 450 (19%) | 450/778 (57.8%) | – | – |
| Age 0–1 years | 2828 (70%) | 1590 (60%) | 1590/2828 (55.2%) | 389.8 | 311.9 |
| Age 1–4 years | 527 (13%) | 337 (14.2%) | 337/527 (63.9%) | 15.8 | 11.5 |
| Age 5–9 years | 316 (8%) | 219 (9.2%) | 219/316 (69.3%) | 11.1 | 3.7 |
| Age 10–14 years | 373 (9%) | 222 (9.3%) | 222/373 (59.5%) | 12.8 | 9.4 |
| Total | 4044 (100%) | 2368 (100%) | 2368/4044 (58.6%)* | 41.1 | 26.7 |
*p < 0.005 for the Z score testing the hypothesis that there is a difference in the proportion of children with a RD in the mortality register compared to the expected proportion from children with RD in the general population