Table 2

Statistical comparison between the four molecular groups, overall and individually, for the six factors of the NH-CSS

11p15
ICR1 hypomethylation
mUPD7*L-SRS-dblnegUL-SRS*p Value
Mean number of factors positive†,‡5.86 (n=29)4.73 (n=11)4.42 (n=12)2.88 (n=8)0.000
 SGA (birth weight and/or length)§35 of 35 (100%)8 of 11 (72.7%)12 of 14 (85.7%)7 of 8 (87.5%)0.034
 Postnatal growth failure§34 of 34 (100%)10 of 11 (90.9%)11 of 13 (84.6%)6 of 8 (75%)0.060
 Relative macrocephaly at birth§31 of 32 (96.9%)9 of 11 (81.8%)6 of 13 (46.2%)2 of 8 (25%)0.000
 Protruding forehead§32 of 33 (97.0%)11 of 11 (100%)12 of 14 (85.7%)4 of 8 (50%)0.001
 Body asymmetry§33 of 35 (94.3%)3 of 11 (27.3%)8 of 14 (57.1%)1 of 8 (12.5%)0.000
 Feeding difficulties and/or BMI <−2SDS§35 of 35 (100%)11 of 11 (100.0%)12 of 13 (92.3%)3 of 8 (37.5%)0.000
  • †One-way ANOVA.

  • §χ2 test.

  • *The ‘mUPD7 Unlikely SRS’ subject was removed from this analysis. Even if this subject was included in the ‘Unlikely SRS’ group, the significance level of the overall system remained at 0.000 and the level of significance of the factors remained within their overall significance level categories (<0.001, 0.05 or 0.1).

  • ‡Only subjects with data for all 6 factors were included in this ‘overall’ top-level analysis, to ensure that group mean factor numbers were comparable.

  • ANOVA, analysis of variance; L-SRS-dblneg, Likely SRS double-negative; mUPD, maternal UPD of chromosome 7; NH-CSS, Netchine-Harbison clinical scoring system; SGA, small for gestational age; SRS, Silver-Russell syndrome; UL-SRS, Unlikely-SRS.