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Hepatol Res. 2012 Dec;42(12):1211-20. doi: 10.1111/j.1872-034X.2012.01033.x. Epub 2012 May 14.

Prognostic factors in patients with idiopathic portal hypertension: Two Japanese nationwide epidemiological surveys in 1999 and 2005.

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Department of Public Health, Osaka City University Graduate School of Medicine, Osaka Department of Public Health, Aichi Medical University School of Medicine, Aichi Department of Disaster and Emergency Medicine, Kyushu University Graduate School of Medical Sciences, Fukuoka The 4th Department of Internal Medicine, Tokyo Medical University, Tokyo, Japan.



  To investigate factors affecting disease prognosis among patients with idiopathic portal hypertension (IPH).


  Two Japanese nationwide epidemiological surveys on IPH were conducted in 1999 and 2005. Analyzing data from each cross-sectional survey separately, we examined the consistent prognostic factors detected from each analysis and assessed the effect of treatments for varices on IPH prognosis. Outcome was the disease condition at last observation compared with that at diagnosis, categorized into two levels (recovered/improved and unchanged/aggravated/deceased). To evaluate the association with each characteristic, we calculated adjusted odds ratio (OR) and 95% confidence interval (CI) using a binary model for logistic regression.


  Among 160 patients with IPH in 1999 and 85 in 2005, 10% had aggravated disease at a mean of 6-7 years after the diagnosis. Statistically significant ORs were observed for two factors: esophageal varices, especially of F(2-3) stage, at diagnosis (OR = 6.83, 95% CI = 2.10-22.1 in 1999; OR = 10.7, 95% CI = 1.08-105 in 2005) ameliorated IPH; and hepatic encephalopathy at diagnosis (OR = 0.19, 95% CI = 0.03-1.04 in 1999; crude OR = 0.05, 95% CI = 0.01-0.60 in 2005 using a proportional odds model) worsened IPH. Further analyses explained that the apparent ameliorating effect of esophageal varices was due to the effect of subsequent treatment (OR = 4.59, 95% CI = 0.89-23.7 in 2005).


  The prognosis of IPH would be better if varices were adequately controlled. Patients with liver failure at diagnosis would be at high risk for subsequent disease aggravation.

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