Novel ATP8B1 mutation in an adult male with progressive familial intrahepatic cholestasis

Table 1 Serum biochemistry values throughout the course of the disease

Variables	Reference range	Age 10 yr	On admission	2 wk	1 mo	2 mo
Bilirubin, total (μmol/L)	3.4-20.5	213.5	250.1	303.7	180.5	420.1
BiLirubin, direct (μmoL/L)	0.0-6.8	158.6	188.6	213.1	115.6	286.7
GGT (U/L)	10-47	8	7	31	26	NA
ALP (U/L)	40-129	237	345	322	300	NA
AST (U/L)	8-40	256	202	222	156	466
ALT (U/L)	5-40	375	196	183	130	148
ALbumin (g/L)	40-50	NA	35	33.3	NA	24.7
PT (s)	11.5-13.5	NA	14.9	NA	NA	28.5
PTA (%)	80-120	NA	80.6	NA	NA	24.7
BiLe acids (μmoL/L)	0-10	NA	296	239	218	NA
PLasma ammonia (μmoL/L)	0-23	NA	15	NA	NA	61.5
CHE (U/L)	NA	NA	1519	1720	NA	NA

ALP: Alkaline phosphatase; AST: Aspartate aminotransferase; ALT: Alanine aminotransferase; PT: Prothrombin time; PTA: Prothrombin activity; NA: Not available; GGT: γ-glutamyl transpeptidase; CHE: Cholinesterase.