Table 1.
Baseline demographic, laboratory and instrumental imaging characteristics of 154 hereditary hemorrhagic telangiectasia (HHT) patients with liver vascular malformations (VMs)
| Demographics | 13 patients symptomatic at baseline | 141 patients asymptomatic at baseline | All 154 patients | |||
|---|---|---|---|---|---|---|
| Mean ± SD | Range | Mean ± SD | Range | Mean ± SD | Range | |
| Age (y) | 51 ± 16 | 21–64 | 47 ± 15 | 7–82 | 48 ± 16 | 7–82 |
| Sex | 6 male, 7 female | 41 male, 100 female | 47 male, 107 female | |||
| Laboratory findingsa | ||||||
| Hb, g/dl | 10.2 ± 1.8 | 7.0–13.1 | 12.23 ± 2.9 | 5.6–19.2 | 12.13 ± 2.1 | 5.6–19.2 |
| AST (IU/l × ULN) | 1.1 ± 0.3 | 1–2 | 1.0 ± 0.3 | 1–4 | 1.1 ± 0.3 | 1–4 |
| ALT (IU/l × ULN) | 1.2 ± 0.4 | 1–2 | 1.37 ± 0.4 | 1–4 | 1.3 ± 0.4 | 1–4 |
| GGT (IU/l × ULN) | 3.46 ± 3.1 | 1–10 | 1.32 ± 1.2 | 1–8 | 1.52 ± 1.2 | 1–10 |
| Alk phosphatase (IU/l × ULN) | 1.28 ± 0.4 | 1–2 | 1.0 ± 0.2 | 1–3 | 1.08 ± 0.2 | 1–3 |
| Bilirubin (mg/dl; ×ULN) | 1.15 ± 0.5 | 1–3 | 1.0 ± 0.1 | 1–3 | 1.02 ± 0.1 | 1–3 |
| INR | 1.03 ± 0.07 | 1–1.2 | 1.0 ± 0.1 | 1–2 | 1.03 ± 0.1 | 1–2 |
| Albumin (g/dl) | 3.98 ± 0.1 | 3.8–4.2 | 4.33 ± 0.2 | 2.9–4.8 | 4.30 ± 0.2 | 2.9–4.8 |
| Doppler US and echocardiographyb | ||||||
| Hepatic artery | ||||||
| Inner diameter (mm) | 11.3 ± 4.5 | 6.3–22 | 7.78 ± 3.2 | 5.1–17 | 8.18 ± 2.7 | 5.1–22 |
| Resistance index | 0.53 ± 0.08 | 0.40–0.68 | 0.55 ± 0.1 | 0.34–0.75 | 0.54 ± 0.1 | 0.34–0.75 |
| Peak flow velocity (cm/sec) | 127.6 ± 35.1 | 77–210 | 106.0 ± 25.4 | 75–212 | 108.04 25.4 | 75–212 |
| Portal vein | ||||||
| Inner diameter (mm) | 11.4 ± 3.0 | 8–16 | 8.78 ± 2.7 | 6–21 | 8.9 ± 2.7 | 6–21 |
| Mean flow velocity (cm/sec) | 17.0 ± 5.0 | 11–26 | 19.45 ± 4.6 | 8–33 | 18.90 ± 3.6 | 8–33 |
| Hepatic vein | ||||||
| Inner diameter (mm) | 10.3 ± 3.7 | 7–18 | 7.89 ± 2.2 | 5–17 | 8.16 ± 2.2 | 5–18 |
| Peak flow velocity (cm/sec) | 37.0 ± 11.5 | 22–57 | 34.7 ± 7.4 | 20–76 | 35.2 ± 8.4 | 20–76 |
| Focal nodular hyperplasia | ||||||
| Number | 0.38 ± 0.6 | 0–2 | 0.15 ± 0.7 | 1–5 | 0.23 ± 0.6 | 0–5 |
| Diameter (cm) | 1.0 ± 1.7 | 0–5.2 | 0.76 ± 1.9 | 2.2–20.7 | 0.79 ± 2.5 | 0–20.7 |
| Cardiac Index (l/min/m2)c | 4.3 ± 1.0 | 2.9–10.7 | 3. 2 ± 0.2 | 2.7–10.1 | 3.3 ± 0.4 | 2.7–10.7 |
| Pulmonary artery pressure (mmHg) | 34.2 ± 9.2 | 24–50 | 27.10 ± 4.0 | 21–48 | 28.14 ± 6.0 | 21–50 |
Hb hemoglobin, GGT gammaglutamyltranspeptidase
aEight patients were anti-HCV positive (5/8 HCV RNA positive); two patients were HBsAg positive and HBV DNA negative
bNodularity of the liver surface was found on Doppler US in five patients; focal or diffuse dilatation of intrahepatic bile ducts was found in two patients
cMeasured in 145/154 at baseline