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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

From: A retrospective review of the contribution of rare diseases to paediatric mortality in Ireland

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

  1. *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