Clinical Study of Eighty-six Cases of Idiopathic Portal Hypertension and Comparison With Cirrhosis With Splenomegaly

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.

Section: Discussioncontrasting

confidence: 61%

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

Sun

Shao

et al. 2018

“…These findings could be related to increased venous flow or immunological abnormalities. 4 In our animal model of NCPF, despite a sustained rise in portal pressure and splenomegaly, we could not detect any collaterals in the portal circulation. This was not unexpected, as the experimental period was of only 6 months.…”

Section: Discussioncontrasting

confidence: 53%

“…These etiological agents include chronic exposure to arsenic, 5 vinyl chloride, 6 bordeaux mixture, 7 ingestion of indigenous drugs, adulterated food, herbal and fungal toxin or parasitic infections, 8 repeated subclinical or clinical attacks of malaria, 9 immunological 10,11 or immunogenetic factors 12 and intra-abdominal infections. 4 Portal fibrosis and PHT have also been reported to develop after repeated intraportal injection of killed Escherichia coli 12 or a mixture of E. coli and anti-E. coli into rabbits in a study from Chiba, Japan. 13,14 In contrast, Suzuki 15 and Okabayashi 16 used an approach of repeated immunosensitization of rabbits by injecting egg albumin into them.…”

Section: Introductionmentioning

confidence: 99%

“…Non-cirrhotic portal fibrosis (NCPF) or idiopathic portal hypertension (IPH) is a common cause of portal hypertension (PHT) in India and Japan. [1][2][3][4] While the two diseases resemble each other closely, certain distinct differences between NCPF and IPH have been highlighted; namely, the former is predominantly a disease of men in the second or third decade of life with very few markers of autoimmune liver disease. 1,2 Noncirrhotic portal fibrosis is a distinct clinical entity, characterized by increased portal pressure, patent splenic and portal veins, and pathologically by portal fibrosis and phlebosclerosis.…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Rabbit model of non‐cirrhotic portal fibrosis with repeated immunosensitization by rabbit splenic extract

Kathayat

Pandey²,

Malhotra

et al. 2002

“…7 In 1969, a workshop organized by the Indian Council of Medical Research (ICMR) reviewed all available information on this condition and christened this distinct clinicopathological entity as 'non-cirrhotic portal fibrosis'. 8 This condition was simultaneously reported from Japan, where the condition is known as IPH, 9,10 and subsequently from Iran 11 and Pakistan. 12…”

Section: Historical Perspectivementioning

confidence: 93%

Non‐cirrhotic portal fibrosis: Current concepts and management

Sarin

Kapoor

2002

150

183

Non-cirrhotic portal hypertension (NCPH) comprises diseases having an increase in portal pressure (PP) due to intraheptic or prehepatic lesions, in the absence of cirrhosis. The lesions are generally vascular, either in the portal vein, its branches or in the perisinusoidal area. Because the wedged hepatic venous pressure is near normal, measurement of intravariceal or intrasplenic pressure is needed to assess PP. The majority of diseases included in the category of NCPH are well-characterized disease entities where portal hypertension (PHT) is a late manifestation and, hence, these are not discussed. Two diseases that present only with features of PHT and are common in developing countries are non-cirrhotic portal fibrosis (NCPF) and extrahepatic portal vein obstruction (EHPVO). Non-cirrhotic portal fibrosis is a syndrome of obscure etiology, characterized by 'obliterative portovenopathy' leading to PHT, massive splenomegaly and well-tolerated episodes of variceal bleeding in young adults from low socioeconomic backgrounds, having near normal hepatic functions. In some parts of the world, NCPF is called idiopathic portal hypertension (IPH) or 'hepatoportal sclerosis'. Because 85-95% of patients with NCPF and EHPVO present with variceal bleeding, treatment involves management with endoscopic sclerotherapy (EST) or variceal ligation (EVL). These therapies are effective in approximately 90-95% of patients. Gastric varices are another common cause of upper gastrointestinal bleeding in these patients and these can be managed with cyanoacrylate glue injection or surgery. Other indications for surgery include failure of EST/EVL, and symptomatic hypersplenism. The prognosis of patients with NCPF is good and 5 years survival in patients in whom variceal bleeding can be controlled has been reported to be approximately 95-100%.