Idiopathic Noncirrhotic Portal Hypertension

Portal hypertension without known risk factors for hypertension, e.g., HEPATIC CIRRHOSIS and SCHISTOSOMIASIS. Idiopathic Noncirrhotic Portal Hypertension is most often associated with pathology in the PORTAL SYSTEM vasculature.

Also Known As:

Banti Disease; Banti Syndrome; Congestive Splenomegaly, Idiopathic; Disease, Banti's; Disease, Porto-Sinusoidal Vascular; Diseases, Porto-Sinusoidal Vascular; Fibrosis, Noncirrhotic Portal; Hypertension, Idiopathic Portal; Idiopathic Congestive Splenomegalies; Idiopathic Non Cirrhotic Portal Hypertension; Idiopathic Portal Hypertensions; Noncirrhotic Portal Fibroses; Portal Fibrosis, Noncirrhotic; Portal Hypertension, Idiopathic; Porto Sinusoidal Vascular Diseases; Porto-Sinusoidal Vascular Disease; Splenomegaly, Idiopathic Congestive; Syndrome, Banti's; Vascular Disease, Porto-Sinusoidal; Banti's Disease; Banti's Syndrome; INCPH; Idiopathic Congestive Splenomegaly; Idiopathic Non-Cirrhotic Portal Hypertension; Idiopathic Portal Hypertension; Noncirrhotic Portal Fibrosis; Porto-Sinusoidal Vascular Diseases

Networked: 208 relevant articles (12 outcomes, 8 trials/studies)

Relationship Network

Disease Context: Research Results

Digestive System Diseases: 1042
- Liver Diseases: 41660
  - Portal Hypertension: 7261
    - Idiopathic Noncirrhotic Portal Hypertension: 208

Related Diseases

1.	Idiopathic Noncirrhotic Portal Hypertension
2.	Liver Cirrhosis (Hepatic Cirrhosis)
3.	Fibrosis (Cirrhosis)
4.	Hemorrhage
5.	Esophageal and Gastric Varices (Esophageal Varices)

Experts

1.	Shiomi, Susumu: 4 articles (01/2015 - 09/2003)
2.	Ikegami, Toru: 3 articles (04/2022 - 11/2013)
3.	Seijo, Susana: 3 articles (01/2021 - 09/2012)
4.	Harada, Kenichi: 3 articles (12/2015 - 08/2007)
5.	Nakanuma, Yasuni: 3 articles (12/2015 - 08/2007)
6.	Sasaki, Motoko: 3 articles (12/2015 - 08/2007)
7.	Sato, Yasunori: 3 articles (12/2015 - 08/2007)
8.	Morikawa, Hiroyasu: 3 articles (01/2015 - 05/2007)
9.	Garcia-Pagan, Juan Carlos: 2 articles (01/2021 - 06/2013)
10.	Hernández-Gea, Virginia: 2 articles (01/2021 - 01/2019)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Idiopathic Noncirrhotic Portal Hypertension:

1.	Streptomycin (Streptomycin Sulfate)FDA Link 05/01/1953 - "Banti's disease; with two illustrative case reports, treated successfully with streptomycin."
2.	Sclerosing Solutions (Sclerosing Agents)IBA 02/01/1949 - "Esophageal varices in Banti's disease; report of two cases in which the condition was improved with injection of sclerosing solutions."
3.	eculizumab FDA Link 05/27/2019 - "Eculizumab improved intravascular hemolysis and reversed clinical manifestations of INCPH in a patient with paroxysmal nocturnal hemoglobinuria."
4.	SonazoidIBA 05/01/2012 - "The aim of this prospective study was to elucidate the efficacy of contrast-enhanced three-dimensional (3D) ultrasound with Sonazoid(®) (GF Healthcare, Oslo, Norway) as a non-invasive tool to discriminate idiopathic portal hypertension (IPH) from cirrhosis by demonstration of portal vein structure. "
5.	Endothelin-1 (Endothelin 1)IBA 07/01/2007 - "This study partially supports the hypothesis that idiopathic portal hypertension might be promoted by endothelin-1 overproduction from splenic B lymphocytes in response to certain substances." 07/01/2007 - "The aim of this study was to experimentally identify how endothelin-1 is involved in the development of idiopathic portal hypertension and which cells produce endothelin-1 in the spleen. " 07/01/2007 - "Experimental investigation of the role of endothelin-1 in idiopathic portal hypertension." 09/01/2000 - "Hepatic localization of endothelin-1 in patients with idiopathic portal hypertension and cirrhosis of the liver." 10/01/1993 - "We measured plasma endothelin-1 (ET-1) concentrations in 20 healthy controls and 63 patients with liver diseases including 9 cases of acute hepatitis (AH), 14 cases of chronic hepatitis (CH), 24 cases of liver cirrhosis (LC), 11 cases of hepatocellular carcinoma with LC (HCC), 3 of primary biliary cirrhosis and 2 of idiopathic portal hypertension. "
6.	AntigensIBA 01/01/2018 - "This report indicates that, in coeliac disease, repetitive stimulation by antigens along the portal vein-and immune responses to them-can result in the development of idiopathic portal hypertension." 10/01/1991 - "We recently reported that HLA-DR antigen was expressed on the microvasculature of portal tracts more frequently in idiopathic portal hypertension (IPH) than in normal livers or in other hepatic diseases, and that this HLA-DR expression may be involved in the development of the portal venopathy characteristic of IPH. " 05/01/1991 - "Endothelial cells of the main portal vein, within the small and medium-sized portal tract, did not express HLA-DR antigen in idiopathic portal hypertension and controls. " 05/01/1991 - "Endothelial cells of hepatic veins and hepatic arteries occasionally expressed HLA-DR antigen, and there was no difference in the expression between idiopathic portal hypertension and controls. " 05/01/1991 - "Expression of HLA-DR antigen on hepatic vascular endothelial cells in idiopathic portal hypertension."
7.	HLA-DR Antigens (HLA-DR)IBA 10/01/1991 - "HLA-DR expression on the microvasculature of portal tracts in idiopathic portal hypertension. " 10/01/1991 - "We recently reported that HLA-DR antigen was expressed on the microvasculature of portal tracts more frequently in idiopathic portal hypertension (IPH) than in normal livers or in other hepatic diseases, and that this HLA-DR expression may be involved in the development of the portal venopathy characteristic of IPH. " 05/01/1991 - "Endothelial cells of the main portal vein, within the small and medium-sized portal tract, did not express HLA-DR antigen in idiopathic portal hypertension and controls. " 05/01/1991 - "Endothelial cells of hepatic veins and hepatic arteries occasionally expressed HLA-DR antigen, and there was no difference in the expression between idiopathic portal hypertension and controls. " 05/01/1991 - "Expression of HLA-DR antigen on hepatic vascular endothelial cells in idiopathic portal hypertension."
8.	Monoclonal AntibodiesIBA 03/01/1989 - "Lymphocyte subsets in the peripheral blood, liver and spleen of patients with idiopathic portal hypertension (IPH) were examined by means of flow cytometry and immunohistochemical analysis using monoclonal antibodies. " 11/01/1987 - "[A study of the T-lymphocytes and their subsets in the peripheral and spleen using OK and Leu series monoclonal antibodies--especially patients with idiopathic portal hypertension (IPH) and liver cirrhosis]." 11/01/1987 - "The T lymphocytes and their subsets in the peripheral blood and spleen of the patients with idiopathic portal hypertension (IPH) and liver cirrhosis were analysed using OK and Leu series monoclonal antibodies. "
9.	Thrombopoietin (c-mpl Ligand)IBA 08/01/2002 - "The aim of this study was to evaluate the relation between thrombopoietin (TPO) and thrombocytopenia in patients with liver cirrhosis and those with idiopathic portal hypertension (IPH) before and after partial splenic embolization (PSE). "
10.	ArsenicIBA 07/01/2007 - "Other investigators have shown that persistent exposure to environmental contaminants including arsenic might induce idiopathic portal hypertension. " 04/01/2005 - "Noncirrhotic portal fibrosis has been reported to occur in humans due to prolonged intake of arsenic contaminated water. " 05/01/1979 - "Though the aetiology of idiopathic portal hypertension is not known, it is possible that arsenic intake may be one of the factors." 05/01/1979 - "Estimates were made of the arsenic concentration in liver specimens from nine patients having idiopathic portal hypertension (IP), and in four livers these were found to be significantly higher than those in patients with cirrhosis and in control subjects. " 05/01/1979 - "Chronic oral arsenic intoxication as a possible aetiological factor in idiopathic portal hypertension (non-cirrhotic portal fibrosis) in India."

Therapies and Procedures

1.	Splenectomy 01/01/1913 - ""Banti's Disease" cured by Splenectomy." 06/21/1954 - "[Studies on Banti's disease; case controlled seven years following splenectomy]." 12/12/2017 - "Eleven years earlier, he underwent a splenectomy due to idiopathic portal hypertension. " 12/01/2016 - "The patient had undergone splenectomy for idiopathic portal hypertension 1 year ago and vaccination against Streptococcus pneumoniae immediately post operation. " 06/01/2015 - "As an important background the patient underwent splenectomy owing to Banti syndrome 60 years earlier. "
2.	Surgical Portasystemic Shunt (Portosystemic Shunt) 05/19/2016 - "CONCLUSIONS Portosystemic shunt surgery is a safe and effective procedure for the treatment of patients with INCPH. " 05/19/2016 - "Patients with INCPH who had undergone portosystemic shunt procedure were included in the study. " 05/19/2016 - "The aim of the present study was to present our results in using portosystemic shunt surgery in patients with INCPH. " 05/19/2016 - "Portosystemic Shunt Surgery in Patients with Idiopathic Noncirrhotic Portal Hypertension." 05/01/2006 - "Magnetic resonance images of the globus pallidus in patients with idiopathic portal hypertension: a quantitative analysis of the relationship between signal intensity and the grade of portosystemic shunt."
3.	Transjugular Intrahepatic Portasystemic Shunt 05/26/2020 - "In patients with INCPH, TIPS and SED were more effective in controlling rebleeding than ET + NSBB, but survival rates were not significantly different among the three groups. " 05/01/2022 - "Results: All 13 patients with INCPH completed TIPS successfully with an average age of 45±8 (33 to 59) years. " 05/26/2020 - "To compare the outcomes of TIPS, SED and ET + NSBB in the control of variceal rebleeding in patients with INCPH. " 01/01/2020 - "Letter to the Editor: Abdominal Surgery in Idiopathic Noncirrhotic Portal Hypertension: Is Preemptive TIPS Reducing Postoperative Complications?" 01/01/2016 - "The charts of patients with idiopathic noncirrhotic portal hypertension undergoing TIPS in seven centers between 2000 and 2014 were retrospectively reviewed. "
4.	Therapeutics 01/01/2001 - "The authors consider that indications for shunting operations for idiopathic portal hypertension, especially when using renal veins, should be determined more carefully, phlebosclerotic therapy and transsection of the esophagus being recommended as alternative interventions." 04/01/1986 - "This pattern of noncirrhotic portal fibrosis has not been described following antitumor therapy." 06/01/1964 - "[ON THE PATHOGENESIS, CLINICAL ASPECTS AND THERAPY OF "BANTI'S SYNDROME" IN CHILDHOOD]." 09/25/1953 - "[Therapy of dynamic spleen decompensation; pseudo-Banti's syndrome, hypersplenia]." 01/01/1953 - "[Dynamic splenic decompensation (Banti's syndrome) in hepato-lenticular degeneration, and modern surgical therapy]."
5.	Surgical Splenorenal Shunt (Splenorenal Shunt) 01/01/1987 - "Portal vein pressure was elevated in one patient with idiopathic portal hypertension in whom portal venous flow was increased postoperatively as a result of resection of a large gastro- and splenorenal shunt conducted additionally." 01/01/2019 - "In patients with noncirrhotic portal fibrosis and symptomatic portal hypertensive biliopathy, portal decompression surgery by proximal splenorenal shunt is one successful treatment option." 08/01/2005 - "Long-term efficacy of distal splenorenal shunt with splenopancreatic and gastric disconnection for esophagogastric varices in patients with idiopathic portal hypertension." 09/01/2001 - "In a 59-year-old man with recurrent hepatic encephalopathy for one year, a large splenorenal shunt was detected in the computed tomography and angiography, and liver biopsy revealed a portal fibrosis consistent with idiopathic portal hypertension. " 12/01/1989 - "From December 1973 to December 1987, we performed a distal splenorenal shunt (DSRS) in 112 cases of portal hypertension, including 107 with postnecrotic liver cirrhosis and 5 with idiopathic portal hypertension (IPH). "