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Table 4 Mortality of 99 Dutch Pompe patients compared to general Dutch population

From: Survival and associated factors in 268 adults with Pompe disease prior to treatment with enzyme replacement therapy

Analysis Median follow-up time (range) Observed no. deaths (O) Expected no. deaths (E) * Ratio (O/E) P -value
1 2.3 (<1 month-7 years) 5 2.3 2.2 0.09
2 3.3 (<2 months-7 years) 9 2.8 3.2 0.002
  1. Analysis 1: 5 deceased patients before start of ERT Analysis 2: including 4 patients who died after start of ERT with follow-up of every patient on ERT extended with 1 year after start of ERT. * According to death probabilities derived from Dutch Central Bureau of Statistics.
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Orphanet Journal of Rare Diseases

ISSN: 1750-1172