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

Murai, Y; Ohfuji, Satoko; Fukushima, Wakaba; Tamakoshi, Akiko; Yamaguchi, Shohei; Hashizume, Makoto; Hirota, Yoshio

doi:10.1111/j.1872-034x.2012.01033.x

Cited by 22 publications

(27 citation statements)

References 24 publications

(78 reference statements)

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“…The mean age at onset in our study (35.1 ± 16.5 years) was much younger than that reported in most of the other studies . In addition, there was a remarkable difference between male and female patients in this respect; male patients were on average 12 years younger than female patients in our cohort.…”

Section: Resultscontrasting

confidence: 68%

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Clinical features of idiopathic portal hypertension in China: A retrospective study of 338 patients and literature review

Sun

Shao

et al. 2018

J of Gastro and Hepatol

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Background and Aim Idiopathic portal hypertension (IPH) refers to a relatively rare condition characterized by intrahepatic portal hypertension in the absence of underlying disease such as liver cirrhosis. Methods We retrospectively reviewed 338 patients with IPH that were diagnosed at the pathological consultation center of our hospital. Results The ratio of male to female patients was 1:1. Mean age at onset was 35.1 ± 16.5 years; male patients on average were 12 years younger than female patients at onset. The median duration from onset to IPH diagnosis was 12 months. In 50 patients, medication use may have been an etiological factor. The most common clinical manifestations were splenomegaly (91.3%) and hypersplenism (68.9%); 57.0% patients presented varicosis, while 25.1% patients had a history of variceal bleeding. Nodular regenerative hyperplasia was found in 22.2% liver biopsies. Among patients for whom laboratory data were available, 65.0%, 50.3%, and 71.4% patients presented leukopenia, anemia, and thrombocytopenia due to hypersplenism. Liver function was mostly in the compensated stage. Female patients showed worse leukopenia and anemia, while male patients were more likely to have abnormal serum transaminase and bilirubin levels. Sixty‐seven patients received surgical or interventional treatment. Conclusions High‐quality liver biopsy, detailed clinical information, and expert pathologist are necessary for diagnosis of IPH. IPH can occur concurrently with other liver disease such as hepatitis and drug‐induced liver injury. Medication appears to be an important etiological factor for IPH in China. Management approach was largely focused on treatment of portal hypertension and its complications.

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Section: Resultscontrasting

confidence: 68%

“…Although IPH is a worldwide disease, it is particularly prevalent in Asia, especially in Japan and India . The etiology of IPH is still not clear.…”

Section: Introductionmentioning

confidence: 99%

Clinical features of idiopathic portal hypertension in China: A retrospective study of 338 patients and literature review

Sun

Shao

et al. 2018

J of Gastro and Hepatol

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“…The male to female patient ratio was approximately 1:2.7. The age of confirmed diagnosis peaked in the 40–50‐year‐old age band, and the average age was approximately 49 years …”

Section: Guidelines For Diagnosis Of Aberrant Portal Hemodynamicsmentioning

confidence: 99%

“…When symptoms associated with splenomegaly (abdominal pain and compression) or a tendency to bleed and severely decreased blood cells (at least one of the following: platelets, ≤5 × 10 4 ; white blood cells, ≤3000; and red blood cells, ≤300 × 10 4 ) associated with splenomegaly, partial splenic embolization or splenectomy should be considered …”

Section: Guidelines For Treatment Of Aberrant Portal Hemodynamicsmentioning

confidence: 99%

Diagnosis and treatment guidelines for aberrant portal hemodynamics

Furuichi

Tanaka

Takikawa

et al. 2017

Hepatology Research

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Idiopathic portal hypertension (IPH), causing aberrant portal hemodynamics, is a disease with an as yet unidentified cause and no established treatment protocol. The Japanese research group on IPH in Japan was set up in 1975 by the Ministry of Health, Labor and Welfare. Extrahepatic portal obstruction and Budd-Chiari syndrome (BCS) have since been added to the group's research subjects. The aims of the research group are to accurately evaluate the current status of the three diseases in Japan, elucidate their etiology and pathogenesis, and develop new treatments. Due to the long-term efforts of the Japanese research group, aberrant portal hemodynamics has been investigated in a variety of aspects, from epidemiological and pathological studies to molecular biology analyses. As a result, it has been shown that there are abnormal genes in the liver, specific for IPH. In addition, pathological findings of BCS were internationally compared and the difference in findings between Japan and Europe (or North America) has been clarified. Furthermore, it was found that complication rates of hepatocellular carcinoma in BCS were higher in Japan. Based on the research, "Diagnosis and treatment of aberrant portal hemodynamics (2001)", including diagnostic criteria for aberrant portal hemodynamics, was published in 2001. In 2013, it was revised to "Diagnosis and treatment guidelines for aberrant portal hemodynamics (2013)" after the incorporation of diagnosis and treatment in accordance with its current status.

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“…Therefore, nationwide epidemiologic surveys on IPH, EHO, and BCS were carried out in 1999, 2005, and 2015 using the same study protocol . The aims of these surveys were to estimate the annual number of patients seeking medical care in Japan and to explore the demographic and clinical characteristics, including prognoses, of these diseases.…”

Section: Introductionmentioning

confidence: 99%

Japanese periodical nationwide epidemiologic survey of aberrant portal hemodynamics

Ohfuji

Furuichi

Akahoshi

et al. 2019

Hepatology Research

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AimIdiopathic portal hypertension (IPH), extrahepatic portal obstruction (EHO), and Budd–Chiari syndrome (BCS) are characterized by aberrant portal hemodynamics of unknown etiology. The aim of this study was to explore trends in the descriptive epidemiology of these diseases through periodical nationwide surveys.MethodsNationwide epidemiologic surveys were undertaken in 1999, 2005, and 2015 using the same protocol. The survey targets were selected from all departments of gastrointestinal medicine, surgery, pediatrics, and pediatric surgery in Japan by stratified random sampling according to the number of beds. We asked each department to complete a mail‐back questionnaire on the annual numbers of patients with IPH, EHO, and BCS during the preceding year.ResultsThe estimated number of BCS patients increased from 280 (95% confidence interval, 200–360) in 1999 survey to 410 (300–530) in 2015 survey, whereas the number of IPH and EHO patients has remained largely unchanged during the 15 years (IPH was approximately 1000; EHO was approximately 770 in 2015 survey). The mean age at symptom onset was approximately 45 years for IPH, 30 years for EHO, and 40 years for BCS over the past 15 years. Those who described disease aggravation from the time of diagnosis accounted for approximately 10% of IPH, 15% of EHO, and 20% of BCS patients in each of the three surveys.ConclusionsIn Japan, the prevalence of BCS is increasing, while those of IPH and EHO appear to be stable. Clinical characteristics, including prognoses, have remained largely unchanged in the past 15 years.

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

Abstract: 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.

Cited by 22 publications

References 24 publications

Clinical features of idiopathic portal hypertension in China: A retrospective study of 338 patients and literature review

Clinical features of idiopathic portal hypertension in China: A retrospective study of 338 patients and literature review

Diagnosis and treatment guidelines for aberrant portal hemodynamics

Japanese periodical nationwide epidemiologic survey of aberrant portal hemodynamics

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