Table 3.
PEBD Outcomes
| Feature |
FIC1 (n = 22) |
BSEP |
P*,
Common BSEP vs. Other BSEP |
P*, FIC1 vs. Other BSEP |
|||
|---|---|---|---|---|---|---|---|
|
All BSEP (n = 35) |
Common |
Other (n = 10) |
|||||
|
D482G (n = 11) |
E297G (n = 14) |
||||||
| Progressed to cirrhosis‡, | 0/20 (0%) | 5/30 (17%) | 0/10 (0%) | 2/11 (18%) | 3/9 (33%) | 0.143 | 0.023 |
| LTX or listing | 6/22 (27%) | 11/35 (31%) | 1/11 (9%) | 3/14 (21%) | 7/10 (70%) | 0.0039 | 0.049 |
| Deceased | 0/22 (0%) | 3/35 (9%) | 1/11 (9%) | 2/14 (14%) | 0/10 (0%) | 0.542 | † |
| Poor outcome§ | 6/22 (27%) | 14/35 (40%) | 2/11 (18%) | 5/14 (36%) | 7/10 (70%) | 0.053 | 0.049 |
Each cell shows total n (denominators). P values <0.05 are noted in bold. For all features in this table, P values for all BSEP versus FIC1 and for common BSEP versus FIC1 did not reach significance. *Two‐tailed Fisher's exact tests; † P value not calculable. ‡,Progression to cirrhosis excludes patients who had cirrhosis at the time of diversion. Six patients (1 D482G carrier, 3 E297G carriers, and 2 FIC1 patients) had been diagnosed with cirrhosis prior to diversion. §Poor outcome was defined as one or more of the following: progression to cirrhosis, listing for or performance of LTX, or death. Three patients, all with one or two copies of E297G, developed HCC after PEBD; of these patients, 2 died and 1 had LTX. A third patient who was homozygous for D482G died due to end‐stage liver disease approximately 5 years after PEBD.