The prognosis of idiopathic portal hypertension in Japan.

To clarify the factors relating to the prognosis of patients with idiopathic portal hypertension (IPH), we followed 171 patients with IPH until the end of 1990, who were registered in the database among those with abnormal portal circulation as of 1985 in hospitals of Japan. During the follow-up period, twenty patients died; 6 from gastro-intestinal tract bleeding, 5 from hepatic insufficiency and 9 from other causes. Cox's proportional hazard model suggested that male patients (hazard ratio 4.85, 95% confidence interval 1.82-12.94), with a disease onset at less than 40 years of age (H.R.3.94, 95% C.I. 1.31-11.57), and/or with varices (H.R.2.86, 95% C.I. 1.05-7.77) generally had poorer prognoses.