Table 1.
Laboratory and histological features at diagnosis of 75 children with autoimmune liver disease
|
|
All patients, n = 75
|
AIH, n = 54
|
ASC, n = 21
|
P
value
|
| Biochemical profile | ||||
| AST U/L (NV ≤ 45) | 438 (129-982) | 678 (204-1200) | 150 (94-333) | < 0.001 |
| GGT U/L (NV ≤ 50) | 116 (60-296) | 107 (54-196) | 360 (68-607) | < 0.001 |
| Total bilirubin (NV ≤ 1 mg/dL) | 1.7 (0.6-4.5) | 2.7 (0.6-5.3) | 1.2 (0.7-2.5) | 0.05 |
| ALP (NV ≤ 350 U/L) | 296 (204-469) | 283 (199-462) | 301 (242-494) | 0.328 |
| ALP/AST ratio | 0.7 (0.3-2.2) | 0.4 (0.2-1.6) | 2.3 (0.7-3.5) | 0.002 |
| Albumin (NV: 30-50 g/dL) | 42 (38-44) | 42 (37-44) | 42 (40-46) | 0.082 |
| INR (NV: 0.9-1.2) | 1.2 (1.1-1.5) | 1.2 (1.1-1.6) | 1.1 (1.0-1.2) | < 0.05 |
| Platelet (109/L) | 252 (180-350) | 234 (167-314) | 319 (251-393) | < 0.05 |
| Autoimmune profile | ||||
| ANA (≥ 1:20): n (%) | 55 (73) | 38 (70) | 17 (81) | 0.777 |
| SMA (≥ 1:20): n (%) | 53 (71) | 38 (70) | 15 (71) | 1 |
| Anti-LKM-1 (≥ 1:10): n (%) | 12 (16) | 12 (22) | 0 (0) | < 0.05 |
| Anti-LC1: n (%) | 9 (12) | 9 (17) | 0 (0) | < 0.05 |
| ANCA: n (%) | 37 (49) | 22 (41) | 15 (71) | < 0.05 |
| IgG g/dL (NV: 0.5-1.8 g/dL) | 2.0 (1.4-3.2) | 2.3 (1.4-3.3) | 1.7 (1.5-2.2) | 0.325 |
| IgG > ULN: n (%) | 51 (68) | 37 (69) | 14 (67) | 1 |
| Histology, n (%) | ||||
| Interface hepatitis | 51 (68) | 42 (78) | 12 (57) | 0.09 |
| Fibrosis | 61 (81) | 42 (78) | 19 (90) | 0.324 |
| Cirrhosis | 17 (23) | 15 (28) | 2 (10) | 0.127 |
| Features of biliopathy1 | 62 (83) | 37 (68) | 17 (81) | 0.764 |
It includes at least one of the following: inflammatory injury of the bile duct, ductular reaction, periductular fibrosis, biliary metaplasia, granulomatous cholangitis[16]. Values are expressed as median and interquartile ranges.
AIH: Autoimmune hepatitis; ASC: Autoimmune sclerosing cholangitis; AST: Aspartate aminotransferase; GGT: Gamma-glutamyl transferase; ALP: Alkaline phosphatase; INR: International normalized ratio; ANA: Antinuclear antibody; SMA: Smooth muscle antibody; LKM-1: Liver-kidney microsome antibody type 1; LC1: Liver cytosol antibody type 1; SLA: Liver soluble antigen; ANCA: Anti-neutrophil cytoplasmic LT antibodies; ULN: Upper limit of normal; NV: Normal value.