Skip to main content
. 2022 Nov 16;5(2):100626. doi: 10.1016/j.jhepr.2022.100626

Table 1.

Patient baseline characteristics of the selected BSEP genotypes.

BSEP1/1 (n = 31) p value BSEP1/1 vs. BSEP1/3∗∗ BSEP1/3 (n = 30) p value BSEP1/3 vs. BSEP3/3∗∗ BSEP3/3 (n = 77) p value Overall
Demographics, median
Age first visit, y [IQR] 0.8 [0.3-1.9] 1.3 [0.5-4.4] 0.7 [0.3-1.9] 0.07
 Available, n (%) 30 (97) 30 (100) 77 (100)
Year of birth, [IQR] 1992 [1987-2009] 0.006 2001 [1995-2009] 0.008 2009 [2002-2012] <0.001
 Available, n (%) 31 (100) 30 (100) 77 (100)
Females, n (%) 16 (52) 19 (63) 40 (52) 0.56
 Available, n (%) 31 (100) 30 (100) 76 (99)

Laboratory data at presentation to the tertiary referral center, median [IQR]
Serum bile acids (μmol/L) 247 [153-378] 0.009 459 [354-539] <0.001 209 [151-309] 0.002
 Available n (%) 18 (58) 11 (37) 44 (57)
Total serum bilirubin (μmol/L) 95 [44-180] 110 [57-150] 104 [53-145] 0.99
 Available n (%) 26 (84) 20 (67) 67 (87)
ALT (IU/L) 126 [63-251] 0.26 148 [92-437] 0.19 293 [138-502] 0.01
 Available n (%) 28 (90) 18 (60) 64 (83)
AST (IU/L) 246 [102-475] 257 [128-648] 359 [157-591] 0.22
 Available n (%) 22 (71) 18 (60) 64 (83)
GGT (IU/L) 15 [10-29] 0.07 22 [18-35] 0.37 27 [18-38] 0.01
 Available n (%) 26 (84) 18 (60) 65 (84)
Platelet count, (109/L) 397 [341-561] 321 [192-468] 395 [265-539] 0.16
 Available n (%) 25 (81) 18 (60) 54 (70)

Medication prior to or at moment of first presentation at the tertiary referral center, n (%)
UDCA 15/31 (48) 11/30 (37) 30/77 (39) 0.59
Rifampicin 4/31 (13) 6/30 (20) 19/77 (25) 0.39
Phenobarbital 4/31 (13) 2/30 (7) 3/77 (4) 0.10
Cholestyramine 7/31 (23) 2/30 (7) 8/77 (10) 0.18
Antihistamines 4/31 (13) 2/30 (7) 9/77 (12) 0.94

ALT, alanine aminotransferase; AST, aspartate aminotransferase; BSEP, bile salt export pump; GGT, gamma-glutamyltransferase; UDCA, ursodeoxycholic acid. Genotypic categorization clarified in Methods.

Mann-Whitney U, Kruskal-Wallis test, Chi-squared test or Fisher’s exact test as appropriate, to test differences between the three groups.

A p value of <0.05 is considered statistically significant.

∗∗

Subgroup statistics only performed at overall p value <0.05.