Idiopathic Portal Hypertension Research Articles

"Endemic" idiopathic portal hypertension: report on 32 patients with non-cirrhotic portal fibrosis.

The clinical features, surgical management, and long term follow up of 32 patients from Iran with idiopathic portal hypertension are reported. Many features of the disease are similar to those reported from India and Japan. The unsuspected finding was a 46% history of marked pica of clay (geophagia) in a subset of 26 patients. In addition, 81% of our patients had a prolonged prothrombin time, despite otherwise normal to minimally abnormal liver function tests. Liver biopsies revealed intrahepatic periportal fibrosis with subintimal thickening of terminal branches, and in many specimens a striking peri-ductular fibrosis was seen in the adjacent bile ducts. The spleen was very large with a dilated artery (external diameter: 11 mm to 15 mm). Portal venous pressure (PVP) was measured intra-operatively before and after clamping the splenic artery (SA). Clamping the SA consistently caused a decreased in PVP which ranged from 2.0 to 18.2 cm water with the mean +/- SEM of 9.7 +/- 1.5 cm water (p < 0.001, paired t-test). It was equivalent to 32.3 +/- 3.6% decrease in PVP. Fifteen selected patients (Group I) were managed with splenectomy with excellent short and long term results. The selection criteria for splenectomy included a decrease in PVP to < 24 cm of water after clamping the SA. Three patients from this group were re-examined 10 to 12 years following splenectomy. Cirrhosis had not developed, but the minimal abnormalities in the liver function tests had persisted.(ABSTRACT TRUNCATED AT 250 WORDS)

A MULTIFOCAL GASTRIC LEIOMYOSARCOMA WITH LIVER AND LYMPH NODE METASTASES

Multifocal leiomyosarcoma of the stomach is very rare. In Japan, only one case was reported. A multifocal gastric leiomyosarcoma with liver and lymph node metastases in a 47-year-old woman is discribed. Because of associated idiopathic portal hypertension, total gastrectomy with pancreatosplenectomy was performed in a two-step approach. The resected specimen showed two tumors in an upper portion of the stomach and a tumor in the body. Histologic examination revealed similar findings in all the tumors, which were composed of a differentiated portion with abundant smooth muscle fibers and a portion with epithelioid pattern. Three positive lymph nodes and two metastatic lesions of the liver were found histologically. After resection, chemotherapy with cisplatin, etoposide and mitomycin C through an intra-hepatic arterial catheter was performed. The patient have no recurrence over 1 year after operation.

Thioguanine used in maintenance therapy of chronic myeloid leukaemia causes non‐cirrhotic portal hypertension. RESULTS FROM MRC CML II TRIAL COMPARING BUSULPHAN WITH BUSULPHAN AND THIOGUANINE

Portal hypertension with varices developed in 18/675 patients with chronic myeloid leukaemia (CML) in a randomized trial comparing busulphan with busulphan and thioguanine. All 18 had received the drug combination and none busulphan alone (P less than 0.0001). Ascites was also seen significantly more often in the combination arm (P less than 0.05). These results strongly suggest that the addition of thioguanine was responsible for the development of portal hypertension. The histological features were predominantly those of non-cirrhotic portal hypertension--either idiopathic portal hypertension with minimal morphological abnormalities, nodular regenerative hyperplasia or in two cases leukaemic infiltration only was noted. Cirrhosis was present in 3/16 cases studied. Both treatment groups developed abnormal liver function tests during the chronic phase, but particularly with progression of the disease. During chronic phase abnormalities were significantly more frequent in those receiving busulphan and thioguanine-alkaline phosphatase (P less than 0.02), transaminases (P less than 0.04), bilirubin (P less than 0.05), multiple abnormalities (P less than 0.01). The development of portal hypertension was often associated with abnormalities of these tests; however, lack of specificity precludes their use as a predictor of subsequent clinical problems. Thioguanine confers no survival advantage in this disease. In view of its hepatotoxicity it should not be used routinely for maintenance of control in chronic phase CML.

High Rate of Portal Thrombosis After Splenectomy in Patients With Esophageal Varices and Idiopathic Portal Hypertension

Data on 126 consecutive patients who were admitted to our clinics from January 1979 to May 1989 were scrutinized to assess changes in portal hemodynamics following splenectomy. Two groups were classified: (1) a group of 106 patients with cirrhosis of the liver and (2) a group of 20 patients with idiopathic portal hypertension (IPH). Portal thrombosis was present in five (25.0%) of the 20 patients with IPH and in two (1.8%) of the 106 patients with cirrhosis of the liver. As seen on celiac arteriography, the mean (+/- SD) diameter of the trunk of the splenic artery and vein was 8.99 +/- 1.55 and 16.2 +/- 3.6 mm, respectively, in patients with IPH, while it was 7.94 +/- 1.28 and 14.2 +/- 3.1 mm, respectively, in patients with cirrhosis of the liver. Changes in portal venous pressure were 78.4 +/- 59.4 mm H2O in patients with IPH and 43.5 +/- 38.7 mm H2O in patients with cirrhosis of the liver. There were no significant differences in the maximum level of thrombocytes in patients with IPH or in those patients with cirrhosis of the liver. These events suggest that portal thrombosis can occur with a significantly higher incidence in patients with IPH than in those patients with cirrhosis of the liver after splenectomy, and a decrease in blood flow in the portal vein may be closely linked to the formation of portal thrombosis after splenectomy in patients with IPH. Preoperative examination of portal hemodynamics must be thorough.

MR Imaging in Idiopathic Portal Hypertension

Magnetic resonance imaging was performed in four patients with biopsy proven idiopathic portal hypertension (IPH). The MR images show proximity of medium-sized intrahepatic vessels to each other and to the liver surface in all patients. Small vessels running parallel to the second order branches of the intrahepatic portal vein are commonly seen as collateral pathways of portal flow in IPH and were seen in two patients. These findings were clearly demonstrated on gradient-recalled echo images. Intrahepatic periportal abnormal high intensity was seen in all patients on T2-weighted images and may reflect abnormalities in the portal tracts such as fibrous enlargement and increase in the number of vascular channels. Tiny low-intensity nodules sometimes observed in liver cirrhosis were not seen in any patient. Magnetic resonance was a useful noninvasive method in the differentiation of IPH from liver cirrhosis.

特発性門脈圧こう進症におけるひ静脈洞の走査電顕による観察

Based on analysis of hemodynamic state, it has been assumed that splenic blood flow in idiopathic portal hypertension (IPH) is elevated. The author examined by scanning electron microscopy the sinuses of spleens resected from 3 patients with IPH, as well as from 10 patients with liver cirrhosis and from 3 patients with gastric cancer as controls. In IPH, the arrangement of rod cells of the splenic sinuses was severely disordered, and the slits between rod cells were irregularly enlarged. Rod cells were also deformed and accompanied branching or process formation. The bridges, rod cells which traversed the sinusal lumen, were frequently observed. Decreased resistance in the intrasplenic circulation arising from enlargement of the slits was considered to produce increased splenic blood flow. In liver cirrhosis, the slits tended to close. Slight deformity of rod cells and some bridges were found.

Idiopathic portal hypertension associated with intrahepatic portal aneurysm and splenic artery aneurysm

Idiopathic portal hypertension associated with intrahepatic portal aneurysm and splenic artery aneurysm

A case of idiopathic portal hypertension after renal transplantation

A case of idiopathic portal hypertension (IPH) developing after renal transplantation is reported. A 33-year-old Japanese male who had undergone renal transplantation 8 years previously was transferred to our hospital because of hematemesis from ruptured esophageal varices. He had no history of any liver disease before the renal transplantation, but had a history of receiving blood transfusion. Abdominal computed tomography (CT) and ultrasonography revealed marked splenomegaly and collateral channels, but no obliteration which might cause portal hypertension in the hepatic or portal vein. No findings suggestive of hepatitis or liver cirrhosis were found either macroscopically on laparoscopy or by liver biopsy. Light microscopic study of the liver biopsy specimen showed mild periportal fibrosis, inconspicuous portal branches in the most peripheral tracts, but no pseudolobule formation or piecemeal necrosis. However collagen deposition was found in the perisinusoidal space and partly in intercellular space on electron microscopy. We consider that the development of portal hypertension in this case is responsible for the collagen deposition, which may be related to the administration of azathioprine after renal transplantation. There are few reports on IPH after renal transplantation, and it is stressed that a lower amount of azathioprine than previously reported may induce IPH under such conditions.

Transient Mitral Regurgitation Due to Mitral Valve Prolapse Accompanied by Systolic Anterior Motion of the Mitral Valve

A grade 4/6 systolic murmur, systolic anterior motion of the mitral valve (SAM), and severe mitral regurgitation (MR) documented by two-dimensional Doppler echocardiography developed suddenly on the structurally normal heart of a patient with idiopathic portal hypertension. The patient did not have signs of congestive heart failure and the aforementioned phenomenon disappeared completely when the patient was in hepatic failure. This could be explained by a change in circulating blood volume either by gastrointestinal hemorrhage or hepatic failure.

Postoperative Portal Vein Obstruction in Patients with Idiopathic Portal Hypertension

Postoperative Portal Vein Obstruction in Patients with Idiopathic Portal Hypertension

Impairment of autologous mixed lymphocyte reaction in the spleen and peripheral blood lymphocytes of patients with idiopathic portal hypertension

The etiology of idiopathic portal hypertension (IPH) is unknown, although many studies have suggested that it might be an autoimmune disease. The autologous mixed lymphocyte reaction (AMLR) involves the proliferation of T lymphocytes when co-cultured with autologous non-T cells and may reflect immune control mechanisms in vivo. The AMLRs in the spleen and peripheral blood of three patients with IPH were measured and it was shown that the AMLRs both in the spleen and peripheral blood were significantly suppressed compared to those of normal healthy subjects. By allogeneic MLR, there was a tendency that the disturbance of non-T cells was more intensive than that of T cells. The AMLR of peripheral blood did not improve by splenectomy. Thus, the depressed cause of AMLR in patients with IPH was suggested mainly to disturbance of the antigen-presenting ability of non-T cells, and it was suggested that not only the spleen cells, but systemic immune disturbance caused the impairment of AMLR in IPH.

Pathomorphologic study on the extrahepatic portal vein in idiopathic portal hypertension

Patients with idiopathic portal hypertension (IPH) are known to have sclerotic changes of the intrahepatic portal vein radicles. In order to elucidate the pathological changes in the extrahepatic portal venous system in IPH, studies were carried out on the portal trunk in 12 patients with IPH, 59 patients with liver cirrhosis including some with associated hepatocellular carcinoma, and 12 normal matched control subjects. Histological examinations including histomorphometry were performed on the transverse sections of the portal trunk taken at autopsy. Most of the patients with IPH showed severe phlebosclerosis which was more pronounced than seen in liver cirrhosis. Thrombosis was also frequently observed in IPH. In IPH, the portal trunk was characterized by fibrous thickening of the intima and media with a prominent increase of elastic fibers. The mean area and thickness of the intima and media were significantly greater than in patients with liver cirrhosis. Sclerosis extensively involving both the extrahepatic and intrahepatic ramifications of the portal vein appeared to be characteristic of IPH.

Improved Survival After Prophylactic Portal Nondecompression Surgery for Esophageal Varices: A Randomized Clinical Trial

To evaluate prophylactic surgery for esophageal varices, a prospective randomized controlled trial was begun in 1980 by the Japanese Research Society for Portal Hypertension. Methods of operation included selective shunts and nonshunting interruption procedures. One hundred and twelve Japanese patients, in whom endoscopic findings suggested risk of bleeding but who had no bleeding episode, were randomly allocated to the operated group of 60 patients or nonoperated group of 52 patients. Nine patients with idiopathic portal hypertension, histologically proven noncirrhotic disease, which all fell in the operated group, were excluded from the study and the remaining 103 patients (51 operated and 52 nonoperated) were analyzed. Long-term follow-up of patients for a median of 49 mo with a maximum of 73 mo showed a total of 11 (22%) deaths, including 2 operative deaths, in the operated group compared with 23 (49%) deaths in the nonoperated group. The cumulative survival rate at 5 yr in the operated group was 72%, which was significantly higher than the 45% of the nonoperated group (p less than 0.05). The cumulative variceal bleeding rate at 5 yr was 7% in the operated group, which was significantly lower than that of the nonoperated group of 46% (p less than 0.001). It was concluded that portal nondecompression surgery was effective in preventing the variceal bleeding and in improving survival.

Idiopathic portal hypertension associated with cytotoxic drugs.

Four patients developed clinically important portal hypertension with histological features of idiopathic portal hypertension while they were receiving cytotoxic drugs for chronic myeloid leukaemia and Hodgkin's disease. Mild sclerosis of some small portal triads was the only abnormality seen at light microscopical examination in three of the four cases. In the remaining case light microscopical findings seemed to be normal. Two cases examined by electron microscopy showed perisinusoidal fibrosis; in one case this was the only abnormality detected. There is an association between idiopathic portal hypertension and the use of chemotherapeutic agents, particularly thioguanine. Adequate histological examination of liver tissue, including electron microscopic studies, is recommended for patients who develop hepatic problems while receiving cytotoxic treatment to elucidate this problem.

RUPTURE OF DUODENAL VARICES WITH RESULTING MASSIVE HEMATEMESIS AND MELENA

A 59-year-old woman, who suddenly developed massive hematemesis and melena, was admitted. Because of shock despite humoral and blood transfusion, celiotomy was performed on a suspicion of hemorrhage from gastric or duodenal ulcer. Duodenal varix measuring 3×1cm was detected posteriorly to the horizontal section of the duodenum. There was no evidence of liver cirrhosis but splenomegaly was found. After distal partial gastrectomy, hemorrhage was ceased by ligation and resection of the varix. Platelet decrease persisted postoperatively. Idiopathic portal hypertension was diagnosed by postoperative abdominal CT, angiography and perioperative abdominal examination. Idiopathic portal hypertension seemed to be responsible for the duodenel varix in this case. We have sometimes difficulties in diagnosing a bleeding from the digestive organ, due to a rupture of mesenteric varices, in which even endoscopy and angiography fail to indicate the lesion. When massive hemorrhage from the digestive tract is encountered, close examination and appropriate surgical management should be planned before it is late, always bearing in mind a possible occurrence of this disease.

肝のｐａｒｔｉａｌ ｎｏｄｕｌａｒ ｔｒａｎｓｆｏｒｍａｔｉｏｎの１例 特発性門脈圧こう進症との異同について

肝のpartial nodular transformation (PNT)が特発性門脈圧亢進症(IPH)と同一疾患である可能性を示唆する症例を報告した.症例は55歳女性で,脾腫と門脈圧亢進症を呈し,食道静脈瘤の破綻で死亡した.肝は,肝門部を中心に粗大な結節性過形成性変化が存在し,PNTと考えられた.IPHとの異同を検討した結果,肝病理組織所見は,IPHに類似し,肝被膜下領域の萎縮,門脈域の円形の線維化,末梢門脈枝の潰れや狭小化,中等大および門脈主幹の系統的な門脈硬化が存在し,かつ,両者の肝組織計測の比較でも,形態学的類似性が見られた.本症例の過形成性変化の原因は不明であるが,末梢門脈枝の潰れや狭小化は,肝被膜下領域のみならず,肝門部にも存在し,一方,肝動脈枝は肝門部では正常に開存していた.肝門部では,肝動脈血流が維持され,門脈血流の減少を代償し,ひいては被膜下領域の萎縮に対する代償性の過形成変化が生じた可能性が示唆された.

A case of idiopathic portal hypertension (IPH) with positive anti mitochondrial antibody

A case of idiopathic portal hypertension (IPH) with positive anti mitochondrial antibody

A case of idiopathic portal hypertension with "weeping willow"like appearance of intrahepatic bile ducts

A case of idiopathic portal hypertension with "weeping willow"like appearance of intrahepatic bile ducts

エンドトキシン門脈内注入による特発性門脈圧こう進症（ＩＰＨ）類似肝病変の作製

エンドトキシン門脈内注入による特発性門脈圧こう進症（ＩＰＨ）類似肝病変の作製

Case of the day. Noncirrhotic idiopathic portal hypertension (Banti syndrome).

HomeRadioGraphicsVol. 10, No. 1 PreviousNext Case of the day. Noncirrhotic idiopathic portal hypertension (Banti syndrome).M P Cordeau, O Prosmanne, P RobillardM P Cordeau, O Prosmanne, P RobillardM P CordeauO ProsmanneP RobillardPublished Online:Jan 1 1990https://doi.org/10.1148/radiographics.10.1.2296683MoreSectionsPDF ToolsImage ViewerAdd to favoritesCiteTrack CitationsPermissionsReprints ShareShare onFacebookTwitterLinked In Article HistoryPublished in print: 1990 FiguresReferencesRelatedDetailsCited ByRadiopaedia.orgFrancisDeng, RajalakshmiRamesh2015Splenomegaly, hypersplenism, and hereditary disorders with splenomegalyNeal J.Weinreb, Barry E.Rosenbloom2013 | Open Journal of Genetics, Vol. 03, No. 01Hematologic and Oncologic Diseases and the LiverMarvin M.Singh, Paul J.Pockros2011 | Clinics in Liver Disease, Vol. 15, No. 1Variceal Bleeding in an Adolescent With HIV Diagnosed With Hepatoportal Sclerosis and Nodular Regenerative HyperplasiaIsraelKochin, MargretMagid, RonenArnon, AngelaGlasscock, NandaKerkar, TamirMiloh2010 | Journal of Pediatric Gastroenterology & Nutrition, Vol. 50, No. 3Taybi and Lachman's Radiology of Syndromes, Metabolic Disorders and Skeletal DysplasiasRalph S.Lachman2007Recommended Articles RSNA Education Exhibits RSNA Case Collection Vol. 10, No. 1 Metrics Altmetric Score PDF download