TABLE 1.
Overview of results by telomere length (TL) group
| Total n = 22 | NTel n = 6 | STel n = 9 | VSTel n = 7 | |
|---|---|---|---|---|
| Women (n, %) | 8 (36) | 4 (67) | 4 (44) | 0 (0%) |
| Age at time of analysis (y, median, range) | 54 (27–71) | 42 (27–59) | 47 (35–64) | 57 (28–71) |
| Age at documentation of liver disease (y, median, range) | 36 (16–63) | 38 (16–56) | 34 (25–58) | 51 (21–63) |
| PSVD classification | ||||
| Nodular regenerative hyperplasia (n, %) | 5 (23) | 1 (17) | 2 (22) | 2 (29) |
| Portal vein stenosis (n, %) | 16 (73) | 5 (83) | 7 (78) | 4 (57) |
| Noncirrhotic portal hypertension (n, %) | 1 (5) | 0 (0) | 0 (0) | 1 (14) |
| Signs of portal hypertension (n, %) | 14 (64) | 2 (33) | 5 (56) | 7 (100) |
| Ascites (n, %) | 7 (32) | 0 (0) | 3 (33) | 4 (57) |
| Esophageal varices (n, %) | 11 (50) | 1 (17) | 5 (56) | 5 (71) |
| Splenomegaly (n, %) | 8 (36) | 1 (17) | 5 (56) | 2 (29) |
| Platelet count (G/l, median, range) | 182 (25–526) | 219 (165–526) | 284 (25–500) | 106 (40–189) |
| Available HVPG measurement (n) | 11/22 | 2/6 | 3/9 | 6/7 |
| Gradient (mm Hg, median, range) | 11 (2–16) | 4 (2–6) | 12 (3–15) | 12 (2–16) |
| Other conditions | ||||
| Interstitial lung disease (n, %) | 6 (27) | 0 (0) | 0 (0) | 6 (86) |
| Hematological manifestations (n, %)a | 13 (59) | 4 (67) | 4 (44) | 5 (71) |
| Chronic kidney disease (n, %)b | 4 (18) | 0 (0) | 0 (0) | 4 (57) |
| Laboratory analyses | ||||
| Liver function tests | ||||
| ALT (U/l, median, range) | 40 (12–495) | 44 (12–495) | 55 (16–289) | 38 (22–56) |
| ALP (U/l, median, range) | 125 (40–239) | 109 (40–239) | 125 (63–185) | 117 (72–160) |
| R-value (median, range)c | 1.1 (0.3–6.9) | 1.0 (0.4–6.9) | 1.6 (0.3–6.5) | 1.1 (0.4–2.3) |
| Liver function | ||||
| Total bilirubin (µmol/l, median, range) | 10 (3–43) | 9 (5–16) | 10 (3–35) | 15 (5–43) |
| Albumin (g/l, median, range) | 45 (27–48) | 47 (46–48) | 45 (39–47) | 33 (27–41) |
| INR (median, range) | 1.0 (1.0–3.4) | 1.0 (1.0–1.2) | 1.0 (1.0–3.4)d | 1.1 (1.0–1.4) |
| Imaging analyses | ||||
| Dysmorphic liver (n, %) | 10/21 (48) | 0/6 (0) | 4/8 (50) | 6/7 (86) |
| Portal venous flow < 15 cm/s (n, %) | 9/20 (45) | 1/6 (17) | 3/8 (38) | 5/6 (83) |
Note: Results of the cohort stratified by TL classification (cf. Methods section).
Hematological manifestations included unexplained cytopenia, myelodysplastic syndrome, monoclonal gammopathy, iron-deficiency anemia, and unexplained thrombocytosis.
Chronic kidney disease patients had an estimated glomerular filtration rate inferior to 60 mL/min/1.73 m2.
([ALT/ULN] / [ALP/ULN]); ULN for ALT, 40 U/l; ULN for ALP, 120 U/l.
Two patients taking vitamin K inhibitors, both presented normal factor V levels.
Abbreviations: ALP, alkaline phosphatase; ALT, alanine aminotransferase; INR, international normalized ratio; NRH, nodular regenerative hyperplasia; NTel, normal telomere length; PVS, portal vein stenosis; PSVD, porto-sinusoidal vascular disorder; STel, short telomere length; ULN, upper limit of the norm; VSTel, very short telomere length.