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. Author manuscript; available in PMC: 2020 Jan 1.
Published in final edited form as: Eur J Neurol. 2018 Sep 16;26(1):58–65. doi: 10.1111/ene.13763

Table 2:

Standardized Mortality Ratios (SMR) for DM1-affected Relatives by their Reported DM1-status

DM1-Unaffected DM1-Affected DM1-Unknown
O E SMR (95% CI) O E SMR (95% CI) O E SMR (95% CI)
Parents US 29 38.27 0.76 (0.56, 1.02) 55 32.32 1.70 (1.47, 1.97) 85 64.47 1.32 (1.17, 1.48)
UK 25 23.02 1.09 (0.81, 1.46) 57 21.05 2.71 (2.38, 3.08) 45 31.59 1.42 (1.20, 1.69)
Total 54 61.29 0.88 (0.71, 1.09) 112 53.36 2.10 (1.90, 2.31) 130 96.06 1.35 (1.23, 1.49)
Siblings US 16 21.03 0.76 (0.46, 1.26) 32 12.14 2.64 (2.16, 3.22) 15 3.88 3.87 (3.06, 4.90)
UK 4 4.64 0.86 (0.32, 2.34) 20 4.58 4.37 (3.58, 5.34) 18 2.95 6.11 (5.18, 7.21)
Total 20 25.67 0.78 (0.50, 1.22) 52 16.72 3.11 (2.69, 3.59) 33 6.82 4.84 (4.21, 5.56)
Offspring US 0 2.27 - 6 2.22 2.70 (1.67, 4.36) 1 1.01 0.99 (0.14, 6.90)
UK 0 0.59 - 11 0.85 12.93 (10.98, 15.22) 3 0.38 7.87 (5.27, 11.76)
Total 0 2.86 - 17 3.07 5.53 (4.53, 6.75) 4 1.39 2.87 (1.62, 5.08)
Total 74 89.82 0.82 (0.67, 1.01) 181 73.16 2.47 (2.29, 2.67) 167 104.27 1.60 (1.48, 1.74)

Abbreviations: O (observed), E (expected), SMR (standardized mortality ratio), CI (confidence interval)