Table 1.
Complications reported in CPSS.
| Complication | Range | References |
|---|---|---|
| Pulmonary vascular complications | ||
| PoPH | 7 to 14% (67%∗) | [1], [2], [3], [4], [5], [6], [7], [8], [9] |
| HPS | 3 to 12% | 1,[4], [5], [6], [7], [8], [9] |
| Unspecified | 2 to 28% | 1,10 |
| Liver nodule - any type of which % malignant (HB and HCC) | 0 to 73% (of which 0 to 83% malignant and 6 to 63% premalignant) | 1,[3], [4], [5], [6],[8], [9], [10], [11], [12], [13] |
| Neurological complications | 14% to 73% | [1], [2], [3], [4], [5], [6],[8], [9], [10], [11], [12] |
| Endocrine/metabolic/growth | 14 to 67% | 2,3,8,10 |
| Haematology | 9 to 33% | 2,12,13 |
| Cholestasis/Hyperbilirubinemia | 9 to 73% | 3,4,8,9,12,13 |
| Other∗∗ | Sporadic | 2,3,7,8,10,12,13 |
HB, hepatoblastoma; HCC, hepatocellular carcinoma; HPS, hepatopulmonary syndrome; PoPH portopulmonary hypertension.
This table combines reports of complications in extrahepatic and intrahepatic cases. The following references include patients aged 18 and over at diagnosis:1,2,5,6,8,11 Percentages (%) express fraction of patients in a given series.
∗
Overestimated prevalence due to selection bias.
∗∗
Pancreatitis, microangiopathic haemolytic anaemia, glomerulonephritis, vaginal bleeding, protein losing gastropathy, coagulopathy, gastrointestinal bleeding, intrauterine growth retardation, abdominal symptoms, isolated neonatal respiratory distress.