Research Article
Progressive Familial Intrahepatic Cholestasis: A Descriptive Study in a Tertiary Care Center
Table 1
Baseline patients’ characteristics.
| | Overall () |
| | Gender | | Female | 36 (45.6%) | | Male | 43 (54.4%) | | Age at diagnosis of PFIC (months) | | Female mean (SD) | 56.8 (53.1) | | Male mean (SD) | 68.3 (66.2) | | Overall mean (SD) | 63.1 (60.5) | | Consanguinity between parents | | Missing data () | 25 | | Yes | 47 (87.0%) | | Any other family members affected | | Missing data() | 11 | | Yes | 42 (61.8%) | | Jaundice | 39 (49.4%) | | Pruritus | 38 (48.1%) | | Failure to thrive | 8 (10.1%) | | Bilirubin (μmol/L) | | Mean (SD) | 114.2 (152.2) | | Range | 3.0-811.0 | | ALT (U/L) | | Mean (SD) | 189.0 (197.6) | | Range | 21.5-826.0 | | Alkaline phosphatase (U/L) | | | Mean (SD) | 531.2 (484.6) | | Range | 86.2-2577.0 | | GGT (IU/L) | | Mean (SD) | 131.2 (146.7) | | Range | 8.0-691.0 | | Bile acid level (μmol/L) | | Mean (SD) | 204.4 (151.6) | | Range | 9.6-599.4 | | AFP (μg/L) | | Mean (SD) | 4268.8 (16324.5) | | Range | 0.6-100000.0 | | HCC | 4 (5.1%) | | Liver transplant rate (%) | 51 (64.6%) | | Duration of disease before liver transplant (months) | | Mean (SD) | 53.9 (67.0) | | Range | 0.0-277.0 | | Death after liver transplant (%) | | | Yes | 2 (3.9%) | | Recurrence after liver transplant (%) | | Yes | 4 (7.8%) | | Appearance of symptoms after liver transplant (months) | | Mean (SD) | 22.5 (23.0) | | Range | 1.0-55.0 | | Survival after liver transplant (months) | | Mean (SD) | 69.1 (44.1) | | Range | 0.0-199.0 | | Genetic alleles mutation | | Heterozygous | 6 (16.2%) | | Homozygous | 31 (83.8%) |
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