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202 Chapter 9 TABLE 2. Comparison of colon diameter and SCS ratio between segments with and without HAPCs HAPC+ HAPC- P -value a median % difference (IQR) median absolute difference (IQR) Overall ( n =30) Colonic diameter in cm (median, IQR) 4.08 (3.20-4.99) 5.48 (4.40-6.60) <.001 40 (18-57) 1.32 (0.81-2.12) SCS ratio b (median, IQR) 1.14 (0.97-1.38) 1.66 (1.34-1.91) .001 41 (20-57) 0.41 (0.27-0.65) Patients with prematurely terminating HAPCs ( n =16) Colonic diameter (cm) (median, IQR) 4.25 (3.21-5.06) 5.48 (4.71-6.78) <.001 34 (19-54) 1.29 (0.82-1.71) SCS ratio b (median, IQR) 1.19 (1 .00-1.55) 1.66 (1.41-1.87 ) .001 34 (20-60) 0.41 (0.27-0.57) a Wilcoxon signed rank test (paired measurements). b one missing value because distance between pedicles L2 could not be determined in one patient with prematurely terminating HAPCs. HAPC, high-amplitude propagating contraction; HAPC+, colonic segment where HAPCs were observed; HAPC-, colonic segment where HAPCs were not observed; SCS, standardized colonic size. TABLE 3. Comparison of colon diameter and SCS ratio (groups based on colonic manometry findings) Variable Fully propagating HAPCs ( n =12) Prematurely terminating HAPCs ( n =16) No HAPCs ( n =2) P -value a Colonic diameter (cm) (median, IQR) 4.21 (3.29-4.50) 4.95 (4.02-5.59) 3.97 (3.65-4.29) .080 SCS ratio (median, IQR) 1.16 (1.01-1.35) 1.42 b (1.22-1.70) 1.18 (0.99-1.37) .026 c a Kruskal-Wallis test. b one missing value because distance between pedicles L2 could not be determined in one patient with prematurely terminating HAPCs. c Wilcoxon rank-sum test, post-hoc analyses: Bonferroni correction for multiple testing: P < .025 considered significant. SCS ratios fully propagating group versus prematurely terminating group:.008. There is evidence to suggest that the median SCS ratios of patients with fully propagating HAPCs and patients with prematurely terminating HAPCs are different. Comparisons for SCS ratios between the fully propagating group versus no HAPCs group and prematurely propagating group versus no HAPCs group: ns. Bold values indicate significant results ( P < .05). Finally, we compared colonic diameter measurements in our sample with the predefined cutoff value of 6.5 cm, which is commonly used to define megacolon and megarectum in adults. 13 In total, 15 children had colonic and or rectal diameters >6.5 cm in at least one of the measurements. In all of these children, the colonic diameter exceeded the cut-off value of 6.5 cm at least in the most distal 5-10 cm of the colorectum (the most distal 1-2 measurement points).Therewas nodifference in theprevalence ofmegacolon/megarectum among children with fully propagating HAPCs ( n =5, 42%), prematurely terminating HAPCs

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