Table 3.
Characteristics of patients with extrahepatic portal hypertension over the past 15 years in Japan
| Characteristics | 1999 survey (n = 97) | 2005 survey (n = 70) | 2015 survey (n = 211) | |
|---|---|---|---|---|
| Sex | Male | 52 (54) | 45 (51) | 113 (54) |
| Confirmation of diagnosis | Confirmed | 82 (85) | 67 (96) | 193/207 (93) |
| Potential causative factors | ||||
| Family history | Present | 3/88 (3) | 4/66 (6) | 3/193 (2) |
| Smoking habit | Ever | 21/92 (23) | 11/66 (17) | 35/192 (18) |
| Drinking habit | Ever | 35/92 (38) | 25/67 (37) | 58/193 (30) |
| Oral contraceptives | Ever | 0/38 (0) | 0/63 (0) | 0/188 (0) |
| Underlying illnesses | ||||
| Neonatal omphalitis | Present | 1/54 (2) | 0/51 (0) | 4/162 (2) |
| Cholecystitis/cholangitis | Present | NA | 10/64 (16) | 17/194 (9) |
| Hepatitis | Present | 10/88 (11) | 4/69 (6) | 16/207 (8) |
| Phlebothrombosis | Present | NA | 2/58 (3) | 5/191 (3) |
| Collagen diseases | Present | 1 (1) | 0/70 (0) | 2/206 (1) |
| Blood diseases | Present | 4 (4) | 6/70 (9) | 13/207 (6) |
| Cancer | Present | 9 (9) | 5/70 (7) | 25/206 (12) |
| History of splenectomy | Present | 12/92 (13) | NA | 12/205 (6) |
| Age at symptom onset, years† | Mean ± SD | 30.7 ± 26.5 | 32.0 ± 27.4 | 25.2 ± 25.3 |
| At time of diagnosis | ||||
| Age, years‡ | Mean ± SD | 37.6 ± 23.9 | 32.7 ± 26.2 | 33.1 ± 24.9 |
| Duration from symptom onset, years§ | Mean ± SD | 2.4 ± 6.2 | 1.4 ± 3.0 | 1.8 ± 5.7 |
| Clinical symptoms | ||||
| Esophageal varices | Present | 69/92 (75) | 43/65 (66) | 110/164 (67) |
| Gastric varices | Present | 37/90 (41) | 31/64 (48) | 78/155 (50) |
| Ascites | Present | 18 (19) | 17/67 (25) | 33/178 (19) |
| Splenomegaly | Present | 56 (58) | 42/68 (62) | 107/170 (63) |
| Anemia | Present | 33 (34) | 37/62 (60) | 90/159 (57) |
| Gastrointestinal bleeding | Present | 42/87 (48) | 30/69 (43) | 67/191 (35) |
| Hepatic encephalopathy | Present | 4 (4) | 2/67 (3) | 6/179 (3) |
| Laboratory data | ||||
| Platelet count, ×104/μL | <15.8 | 48/91 (53) | 39/61 (64) | 92/160 (58) |
| Albumin, g/dL | <4.1 | 35/91 (38) | 35/52 (67) | 90/156 (58) |
| Total bilirubin, mg/dL | ≥1.6 | 28/91 (31) | 12/61 (20) | 26/158 (16) |
| At time of last observation | ||||
| Age, years⁋ | Mean ± SD | 42.1 ± 22.8 | 38.7 ± 24.3 | 43.9 ± 23.1 |
| Duration from diagnosis, years‡ | Mean ± SD | 4.2 ± 5.5 | 6.2 ± 7.2 | 9.8 ± 9.6 |
| Disease condition | Recovered | 2 (2) | 1 (1) | 5 (2) |
| Improved | 40 (42) | 38 (57) | 60 (29) | |
| Unchanged | 38 (40) | 21 (31) | 115 (55) | |
| Aggravated | 4 (4) | 3 (4) | 19 (9) | |
| Deceased | 12 (13) | 4 (6) | 11 (5) | |
| Cause of death | Gastrointestinal bleeding | 1 | 1 | 1 |
| Hepatic failure | 7 | 1 | 3 | |
| Hepatocellular carcinoma | 0 | 0 | 3 | |
| Others†† | 4 | 2 | 4 | |
Percentages are shown in parentheses.
Based on 53, 55, and 122 patients in the 1999, 2005, and 2015 surveys, respectively.
Based on 92, 61, and 189 patients in the 1999, 2005, and 2015 surveys, respectively.
Based on 52, 53, and 114 patients in the 1999, 2005, and 2015 surveys, respectively.
Based on 97, 65, and 210 patients in the 1999, 2005, and 2015 surveys, respectively.
In the 1999 survey, the causes of four deaths were as follows: cancer (n = 1), stroke (n = 1), and pulmonary hypertension (n = 2). In the 2005 survey, one patient died from pancreatic cancer and one from thrombotic thrombocytopenic purpura and sepsis. In the 2015 survey, the causes of four deaths were as follows: cholangiocarcinoma (n = 2), pancreatic cancer (n = 1), and unknown cause (n = 1).
NA, not applicable; SD, standard deviation.