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Background: Hepatoportal sclerosis (HPS) is a cause of noncirrhotic portal hypertension, with patients typically presenting with variceal bleeding. It is idiopathic in nature but is felt to be due to an abnormality of the intrahepatic vasculature. HPS is characterized by varying degrees of portal fibrosis, sclerosis of portal vein branches and dilatation of sinusoidal spaces. Nodular regenerative hyperplasia (NRH), another cause of noncirrhotic portal hypertension, has also been recently described in HIV patients initially diagnosed as having cryptogenic liver disease., Methods/results: We describe four cases of HIV+ patients presenting with noncirrhotic portal hypertension; liver biopsies were reviewed by an experienced liver pathologist and found to be consistent with HPS. No other etiologies for their liver disease were found., Conclusions: HPS has been recently identified as a cause of noncirrhotic portal hypertension in patients with HIV. It should be considered in the differential diagnosis of HIV patients presenting with variceal bleeding. We postulate that it may be due to intrahepatic microthrombosis or an altered hepatic fibrogenesis related to highly active antiretroviral therapy or due to HIV itself.

Background: In patients with idiopathic myelofibrosis (IM), portal hypertension (PHT) without thrombosis of the hepatic or splenoportal veins is infrequent., Objective: To ascertain the mechanisms responsible for the development of PHT in IM and their therapeutic implications., Patients and Methods: Color Doppler ultrasound with portal flow quantification, hepatic hemodynamic studies, and histological examinations of the liver biopsies were performed in three IM patients with PHT in whom hepatic and splenoportal thrombosis were ruled out., Results: Two patients showed sinusoidal PHT (increased hepatic venous pressure gradient), normal portal flow, and massive myeloid metaplasia of the liver. Transjugular intrahepatic portosystemic shunt (TIPS) was indicated for variceal bleeding and ascites unresponsive to medical therapy, and resulted in an adequate control of these PHT complications. At the time of TIPS placement, direct portal pressure measurement showed a marked presinusoidal component in the PHT. A third patient died as a consequence of the IM before treatment of PHT could be instituted. This patient showed an extremely increased portal flow and lesser hepatic infiltration., Conclusions: IM patients with PHT might have a marked presinusoidal component contributing to PHT that is underestimated by hepatic vein catheterization. Treatment of the complications of PHT might not differ from that of patients with cirrhosis.

Objective: Portal lymphoid aggregates/follicles (lymphoid A/F) is a characteristically histological finding in patients with chronic hepatitis C. We assessed the prevalence of lymphoid A/F in Chinese patients with chronic hepatitis C and evaluated the correlation of this phenomenon with clinical, biochemical, immunological, virological, and other histological features of these patients., Methods: Eighty-nine Chinese patients with chronic hepatitis C were enrolled and portal lymphoid A/F was evaluated in liver biopsy. Clinical, biochemical, immunological, histological, and virological data, including serum HCV RNA titer and HCV genotype and the response to interferon therapy, were compared between patients with and without portal lymphoid A/F., Results: Twenty-nine (33%) of 89 patients with chronic hepatitis C had portal lymphoid A/F. Patients with lymphoid A/F had a significantly higher frequency of HCV genotype 1b infection (p = 0.039) and had a significantly higher mean score of bile duct damage, periportal necroinflammation, and portal inflammation in liver histologies when compared with patients without lymphoid A/F. No significant difference in sex distribution, mean age, history of blood transfusion, serum liver biochemistry, presence of serum autoantibodies/cryoglobulinemia, serum viral titer, and response to interferon therapy was noted between the two groups. Multivariate logistic regression analysis showed HCV genotype 1b infection and periportal necroinflammation were significant independent predictors associated with portal lymphoid A/F., Conclusions: The presence of portal lymphoid A/F in Chinese patients with chronic hepatitis C was significantly correlated with HCV genotype 1b infection and periportal necroinflammation.

Objectives: Although recent reports demonstrate that eosinophilia is a distinctive feature of early stage primary biliary cirrhosis (PBC), the pathogenesis of this eosinophilia remains unknown. Clinical and experimental data suggest potential mast cell activation in cholestatic liver diseases. Because mast cell-derived mediators could induce eosinophil chemotaxis and activation, we hypothesized that mast cell activation may play a role in the pathogenesis of eosinophilia in PBC. Thus, as the first step in examining a possible link between mast cell activation and eosinophilia in PBC, we quantified the infiltration of mast cells and eosinophils in the livers of patients with PBC., Methods: The study population consisted of 11 patients with stage I or stage II PBC and 11 patients with chronic viral hepatitis. Mast cells and eosinophils were identified by immunohistochemistry using monoclonal antibodies against mast cell tryptase (AA1) and eosinophilic cationic protein (EG2), respectively. Cell infiltration in the portal tract was quantitated morphometrically., Results: When compared with patients with chronic viral hepatitis, patients with PBC showed significantly increased portal infiltration with mast cells (140 +/- 25 vs 72 +/- 10 cells/mm2 [mean +/- SEM, p < 0.05]) and eosinophils (76 +/- 19 vs 32 +/- 9 cells/mm2 [p < 0.05]). Numbers of portal mast cells correlated with numbers of eosinophils in patients with PBC (r = 0.60, p < 0.05) but not in patients with chronic hepatitis (r = 0.34, p = 0.47)., Conclusion: Concomitant increases in mast cell and eosinophil infiltration in the portal tract suggest a role for mast cell activation in the pathogenesis of eosinophilia in PBC.

Objective: We examined the usefulness of imaging portosystemic collaterals accompanying liver cirrhosis by magnetic resonance angiography (MRA), which facilitates imaging of the vascular system without contrast medium., Methods: MRA was performed in 30 patients with liver cirrhosis. Of the 30 patients, percutaneous transhepatic portography (PTP) was performed in 10 patients, and conventional arterial portography (CAP) was performed in 20 patients. The three-dimensional (3D) phase contrast method was used for MRA. Study 1: The ability to image portosystemic collaterals was compared between PTP or CAP and MRA. Study 2: The usefulness of MRA for evaluating the effect of treatment on gastric and esophageal varices was examined., Results: Study 1: In comparing PTP and MRA (n = 10), the left gastric vein (n = 10), splenorenal (gastrorenal) shunt (n = 5), and paraumbilical vein (n = 2) were imaged similarly. However, MRA did not reveal any esophageal varices (n = 10). In comparing CAP and MRA (n = 20), the left gastric vein (n = 17), splenorenal (gastrorenal) shunt (n = 10), and paraumbilical vein (n = 4) were imaged similarly. Whereas CAP revealed esophageal varices (n = 4) in four patients, MRA revealed esophageal varices in only one patient. Study 2: When the effect of treatment for varices was evaluated, MRA 3 wk after embolization therapy for gastric varices (n = 4) confirmed the disappearance of gastrorenal shunt. However, it was impossible to evaluate esophageal varices by MRA., Conclusions: It was possible to image portosystemic collaterals accompanying liver cirrhosis by MRA. MRA was useful as a routine examination. Furthermore, it was useful for evaluating the effect of embolization therapy on gastric varices.

Hepatic involvement in a patient with secondary syphilis was documented by demonstrating spirochetes in the portal tracts. Raised alkaline phosphatase levels in early hepatic syphilis may be explained by involvement of bile ducts and vascular structures.