Table 1.

IBD clinical, endoscopic, and laboratory characteristics of patients at the diagnosis of IBD.

IBD CharacteristicsVEO-PSC-IBD (n = 28)PSC-IBD (n = 41)P
Age, median (IQR)5.2 (3.7-5.9)15.7 (13.3-16.9)<0.0001
Sex F, n (%)10 (36%)13 (32%)0.79
Family history (IBD), n (%)4 (14%)3 (7%)0.4
Family history (PSC), n (%)1 (3%)1 (2%)1
UC, n (%)23 (82%)37 (90%)0.35
CD, n (%)3 (11%)1 (2.5%)0.29
IBDU, n (%)2 (7%)3 (7.5%)1
Disease location CD, n (%)
L2
3 (100%)1 (100%)1
Disease location UC,n (%)
 E11 (4%)01
 E21 (4%)2 (5%)1
 E304 (10%)0.13
 E423 (92%)34 (85%)0.24
PUCAI34 ± 1630.5 ± 90.11
PUCAI, n (%)
 remission < 105 (20%)8 (20%)1
 mild 10-345 (20%)19 (47.5%)0.03
 moderate 35-6415 (60%)9 (22.5%)0.003
 severe 65-8504 (10%)0.15
MAYO, n (%)
 111 (44%)13 (32.5%)0.4
 211 (44%)20 (50%)0.7
 33 (12%)7 (17.5%)0.7
IBD diagnosed, n (%)
 Prior to PSC13 (46%)15 (36.5%)0.61
 Concomitantly11 (39%)19 (46.5%)0.62
 After PSC4 (14%)7 (17%)1
CRP (mg/L)6.5 ± 7.28.7 ± 140.48
ESR (mm/hr)45.7 ± 2642.5 ± 340.71
Albumin (g/dL)4.18 ± 0.484.11 ± 0.410.56
Fecal calprotectin (mcg/g)667 ± 734530 ± 4800.48
IBD CharacteristicsVEO-PSC-IBD (n = 28)PSC-IBD (n = 41)P
Age, median (IQR)5.2 (3.7-5.9)15.7 (13.3-16.9)<0.0001
Sex F, n (%)10 (36%)13 (32%)0.79
Family history (IBD), n (%)4 (14%)3 (7%)0.4
Family history (PSC), n (%)1 (3%)1 (2%)1
UC, n (%)23 (82%)37 (90%)0.35
CD, n (%)3 (11%)1 (2.5%)0.29
IBDU, n (%)2 (7%)3 (7.5%)1
Disease location CD, n (%)
L2
3 (100%)1 (100%)1
Disease location UC,n (%)
 E11 (4%)01
 E21 (4%)2 (5%)1
 E304 (10%)0.13
 E423 (92%)34 (85%)0.24
PUCAI34 ± 1630.5 ± 90.11
PUCAI, n (%)
 remission < 105 (20%)8 (20%)1
 mild 10-345 (20%)19 (47.5%)0.03
 moderate 35-6415 (60%)9 (22.5%)0.003
 severe 65-8504 (10%)0.15
MAYO, n (%)
 111 (44%)13 (32.5%)0.4
 211 (44%)20 (50%)0.7
 33 (12%)7 (17.5%)0.7
IBD diagnosed, n (%)
 Prior to PSC13 (46%)15 (36.5%)0.61
 Concomitantly11 (39%)19 (46.5%)0.62
 After PSC4 (14%)7 (17%)1
CRP (mg/L)6.5 ± 7.28.7 ± 140.48
ESR (mm/hr)45.7 ± 2642.5 ± 340.71
Albumin (g/dL)4.18 ± 0.484.11 ± 0.410.56
Fecal calprotectin (mcg/g)667 ± 734530 ± 4800.48

Continuous data are expressed as mean ± standard deviation unless specified otherwise.

Abbreviations: VEO-PSC-IBD, children with both PSC and IBD diagnosed before age 6; PSC-IBD, children with both PSC and IBD diagnosed after age 6.

Abbreviations: VEO, very early onset; PSC, primary sclerosing cholangitis; IBD, inflammatory bowel disease; UC, ulcerative colitis; CD, Crohn disease; IBDU, undetermined inflammatory bowel disease; PUCAI, pediatric ulcerative colitis activity index; ESR, erythrocyte sedimentation rate; CRP, c-reactive protein

Table 1.

IBD clinical, endoscopic, and laboratory characteristics of patients at the diagnosis of IBD.

IBD CharacteristicsVEO-PSC-IBD (n = 28)PSC-IBD (n = 41)P
Age, median (IQR)5.2 (3.7-5.9)15.7 (13.3-16.9)<0.0001
Sex F, n (%)10 (36%)13 (32%)0.79
Family history (IBD), n (%)4 (14%)3 (7%)0.4
Family history (PSC), n (%)1 (3%)1 (2%)1
UC, n (%)23 (82%)37 (90%)0.35
CD, n (%)3 (11%)1 (2.5%)0.29
IBDU, n (%)2 (7%)3 (7.5%)1
Disease location CD, n (%)
L2
3 (100%)1 (100%)1
Disease location UC,n (%)
 E11 (4%)01
 E21 (4%)2 (5%)1
 E304 (10%)0.13
 E423 (92%)34 (85%)0.24
PUCAI34 ± 1630.5 ± 90.11
PUCAI, n (%)
 remission < 105 (20%)8 (20%)1
 mild 10-345 (20%)19 (47.5%)0.03
 moderate 35-6415 (60%)9 (22.5%)0.003
 severe 65-8504 (10%)0.15
MAYO, n (%)
 111 (44%)13 (32.5%)0.4
 211 (44%)20 (50%)0.7
 33 (12%)7 (17.5%)0.7
IBD diagnosed, n (%)
 Prior to PSC13 (46%)15 (36.5%)0.61
 Concomitantly11 (39%)19 (46.5%)0.62
 After PSC4 (14%)7 (17%)1
CRP (mg/L)6.5 ± 7.28.7 ± 140.48
ESR (mm/hr)45.7 ± 2642.5 ± 340.71
Albumin (g/dL)4.18 ± 0.484.11 ± 0.410.56
Fecal calprotectin (mcg/g)667 ± 734530 ± 4800.48
IBD CharacteristicsVEO-PSC-IBD (n = 28)PSC-IBD (n = 41)P
Age, median (IQR)5.2 (3.7-5.9)15.7 (13.3-16.9)<0.0001
Sex F, n (%)10 (36%)13 (32%)0.79
Family history (IBD), n (%)4 (14%)3 (7%)0.4
Family history (PSC), n (%)1 (3%)1 (2%)1
UC, n (%)23 (82%)37 (90%)0.35
CD, n (%)3 (11%)1 (2.5%)0.29
IBDU, n (%)2 (7%)3 (7.5%)1
Disease location CD, n (%)
L2
3 (100%)1 (100%)1
Disease location UC,n (%)
 E11 (4%)01
 E21 (4%)2 (5%)1
 E304 (10%)0.13
 E423 (92%)34 (85%)0.24
PUCAI34 ± 1630.5 ± 90.11
PUCAI, n (%)
 remission < 105 (20%)8 (20%)1
 mild 10-345 (20%)19 (47.5%)0.03
 moderate 35-6415 (60%)9 (22.5%)0.003
 severe 65-8504 (10%)0.15
MAYO, n (%)
 111 (44%)13 (32.5%)0.4
 211 (44%)20 (50%)0.7
 33 (12%)7 (17.5%)0.7
IBD diagnosed, n (%)
 Prior to PSC13 (46%)15 (36.5%)0.61
 Concomitantly11 (39%)19 (46.5%)0.62
 After PSC4 (14%)7 (17%)1
CRP (mg/L)6.5 ± 7.28.7 ± 140.48
ESR (mm/hr)45.7 ± 2642.5 ± 340.71
Albumin (g/dL)4.18 ± 0.484.11 ± 0.410.56
Fecal calprotectin (mcg/g)667 ± 734530 ± 4800.48

Continuous data are expressed as mean ± standard deviation unless specified otherwise.

Abbreviations: VEO-PSC-IBD, children with both PSC and IBD diagnosed before age 6; PSC-IBD, children with both PSC and IBD diagnosed after age 6.

Abbreviations: VEO, very early onset; PSC, primary sclerosing cholangitis; IBD, inflammatory bowel disease; UC, ulcerative colitis; CD, Crohn disease; IBDU, undetermined inflammatory bowel disease; PUCAI, pediatric ulcerative colitis activity index; ESR, erythrocyte sedimentation rate; CRP, c-reactive protein

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