Portal vein thrombosis (PVT) is an uncommon cause of presinusoidal portal hypertension. Among various hepatoportal disorders, noncirrhotic portal hypertension conditions such as idiopathic portal hypertension (IPH) are considered to have a close relation with PVT. PVT is known to have several predisposing conditions, including infection, malignancies, and coagulation disorders. There is growing interest and recognition that deficiencies in proteins C and S are associated with a hypercoagulable state. These deficiencies are regarded as key factors of systemic hypercoagulability and recurrent venous thromboembolism. We report the case of a 19-year-old male diagnosed as IPH with PVT and combined deficiencies in proteins C and S.
Humans ; Hypertension, Portal/complications/*diagnosis/pathology ; Male ; *Portal Vein ; Protein C Deficiency/*complications ; Protein S Deficiency/*complications ; Tomography, X-Ray Computed ; Venous Thrombosis/complications/*diagnosis/pathology ; Young Adult

Hepatoportal sclerosis (HPS) is defined as sclerosis of portal areas in the absence of cirrhosis. There is little information about HPS in children in the literature. The aim of this study was to describe the clinical presentation, associated disorders, laboratory characteristics and outcome of children who were diagnosed as HPS. This study included 12 children diagnosed as HPS by the Pathology Department between 2005 and 2011. Data were collected from the gastroenterology clinic charts retrospectively, including demographics, presentation characteristics, laboratory data and recent status of patients. Twelve patients were enrolled (6 girls, 6 boys). The median age of patients was 13.5 yr. Median age at the time of biopsy was 11 yr. Four patients had splenomegaly, 3 had esophageal varices, one had hepatopulmonary syndrome and had been transplanted. Smooth muscle antibody was found positive in 4 patients, without autoimmune hepatitis findings in liver biopsy. One patient had celiac disease and another patient had positive celiac disease serology but pathology findings. Another patient had Turner's syndrome. Mean follow-up time was 39 months (3.3 yr) after biopsy. Hepatoportal sclerosis does not necessarily present with portal hypertension in children.
Adolescent ; Child ; Child, Preschool ; Female ; Follow-Up Studies ; Gastroenterology ; Humans ; Hypertension, Portal/complications/*diagnosis/pathology ; Liver/pathology ; Liver Diseases/complications/*diagnosis/pathology ; Male ; Portal Vein/pathology ; Retrospective Studies ; Sclerosis/*diagnosis/*pathology

BACKGROUND/AIMS: This study was aimed to analyze the relationship between gastric varices and its collaterals using magnetic resonance angiography (MRA) and to assess the usefulness of MRA in studies of portosystemic circulation. METHODS: Eighty-one patients who had portal hypertension with gastric varices took MRA before the therapy for gastric varices. RESULTS: The types of collaterals observed by MRA were left gastric vein in 67 patients (83%), short gastric vein in 28 (35%), gastrorenal shunt in 25 (31%), and splenorenal shunt in 14 (17%). In most of patients with advanced gastric varices, the size of left gastric vein was larger than others. In most cases of large gastric varices, the short gastric vein ranged between 5 to 10 mm. Gastrorenal shunt was also correlated with the size of gastric varices. The types of more prominent esophageal varices showed a right type (left gastric vein predominance), but the types of more prominent gastric varices or only the gastric varices showed a left type (posterior or short gastric vein predominance) (p<0.05). CONCLUSIONS: Gadolinium enhanced 3D-MRA can contribute to the study of the hemodynamic relationships between gastric vein and the collateral circulations by presenting more clear images for patients with portal hypertension.
Adult ; Aged ; *Collateral Circulation ; Esophageal and Gastric Varices/complications/diagnosis/*pathology ; Female ; Humans ; Hypertension, Portal/complications/*pathology ; *Magnetic Resonance Angiography ; Male ; Middle Aged ; Stomach/*blood supply

BACKGROUND/AIMS: Liver stiffness measurement (LSM) has been proposed as a non-invasive method for estimating the severity of fibrosis and the complications of cirrhosis. Measurement of the hepatic venous pressure gradient (HVPG) is the gold standard for assessing the presence of portal hypertension, but its invasiveness limits its clinical application. In this study we evaluated the relationship between LSM and HVPG, and the predictive value of LSM for clinically significant portal hypertension (CSPH) and severe portal hypertension in cirrhosis. METHODS: LSM was performed with transient elastography in 59 consecutive cirrhotic patients who underwent hemodynamic HVPG investigations. CSPH and severe portal hypertension were defined as HVPG > or =10 and > or =12 mmHg, respectively. Linear regression analysis was performed to evaluate the relationship between LSM and HVPG. Diagnostic values were analyzed based on receiver operating characteristic (ROC) curves. RESULTS: A strong positive correlation between LSM and HVPG was observed in the overall population (r2=0.496, P<0.0001). The area under the ROC curve (AUROC) for the prediction of CSPH (HVPG > or =10 mmHg) was 0.851, and the sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) for an LSM cutoff value of 21.95 kPa were 82.5%, 73.7%, 86.8%, and 66.7%, respectively. The AUROC at prediction of severe portal hypertension (HVPG > or =12 mmHg) was 0.877, and the sensitivity, specificity, PPV, and NPV at LSM cutoff value of 24.25 kPa were 82.9%, 70.8%, 80.6%, and 73.9%, respectively. CONCLUSIONS: LSM exhibited a significant correlation with HVPG in patients with cirrhosis. LSM could be a non-invasive method for predicting CSPH and severe portal hypertension in Korean patients with liver cirrhosis.
Adult ; Aged ; Alcohol-Related Disorders/complications ; Area Under Curve ; *Elasticity Imaging Techniques ; Female ; Hepatitis B/complications ; Hepatitis C/complications ; Humans ; Hypertension, Portal/*complications/*diagnosis ; Linear Models ; Liver Cirrhosis/*complications/*diagnosis/pathology ; Male ; Middle Aged ; ROC Curve ; Republic of Korea ; Sensitivity and Specificity

Animals ; Diagnosis, Differential ; Disease Models, Animal ; Gastric Antral Vascular Ectasia ; diagnosis ; pathology ; Gastric Mucosa ; pathology ; Gastrointestinal Hemorrhage ; etiology ; pathology ; therapy ; Gastroscopy ; Humans ; Hypertension, Portal ; complications ; epidemiology ; pathology ; Intestinal Mucosa ; pathology ; Liver Cirrhosis ; complications ; pathology ; Liver Cirrhosis, Experimental ; complications ; pathology ; Rats ; Stomach Diseases ; epidemiology ; etiology ; pathology ; therapy

Portal hypertensive gastropathy (PHG) is a term used to define the endoscopic findings of gastric mucosa with a characteristic mosaic-like pattern with or without red spots, and a common finding in patients with portal hypertension. These endoscopic findings correspond to dilated mucosal capillaries without inflammation. The pathogenesis of PHG in not well known, but portal hypertension and some humoral factors seem to be crucial factors for its development. Pharmacological (e.g. propranolol), or interventional radiological (such as transjugular intrahepatic portosystemic shunt) procedures may be useful in preventing re-bleeding from PHG. The classic features of gastric antral vascular ectasia (GAVE) syndrome include red, often haemorrhagic lesions predominantly located in the gastric antrum which can result in significant blood loss. Although the pathogenesis of GAVE is not clearly defined, it seems to be a separate disease entity from PHG, because GAVE generally does not respond to a reduction of portal pressures. Endoscopic ablation (such as argon plasma coagulation) is the first-line treatment of choice. This review will focus on the incidence, clinical importance, etiology, pathophysiology, and treatment of PHG and GAVE syndrome in the setting of portal hypertension.
Esophageal and Gastric Varices/*diagnosis/etiology/therapy ; Gastric Antral Vascular Ectasia/*diagnosis/etiology/therapy ; Gastric Mucosa/metabolism/pathology ; Humans ; Hypertension, Portal/*complications ; Portasystemic Shunt, Transjugular Intrahepatic ; Vasodilator Agents/therapeutic use

Tetrahydrobiopterin (BH4) is an essential cofactor in NO synthesis by endothelial nitric oxide synthase (eNOS) enzymes. It has been previously suggested that reduced intrahepatic BH4 results in a decrease in intrahepatic NO and contributes to increased hepatic vascular resistance and portal pressure in animal models of cirrhosis. The main aim of the present study was to evaluate the relationship between BH4 and portal hypertension (PHT). One hundred ninety-three consecutive patients with chronic liver disease were included in the study. Liver biopsy, measurement of BH4 and hepatic venous pressure gradient (HVPG) were performed. Hepatic fibrosis was classified using the Laennec fibrosis scoring system. BH4 levels were determined in homogenized liver tissues of patients using a high performance liquid chromatography (HPLC) system. Statistical analysis was performed to evaluate the relationship between BH4 and HVPG, grade of hepatic fibrosis, clinical stage of cirrhosis, Child-Pugh class. A positive relationship between HVPG and hepatic fibrosis grade, clinical stage of cirrhosis and Child-Pugh class was observed. However, the BH4 level showed no significant correlation with HVPG or clinical features of cirrhosis. BH4 concentration in liver tissue has little relation to the severity of portal hypertension in patients with chronic liver disease.
Adult ; Aged ; Biopterin/*analogs & derivatives/analysis ; *Chromatography, High Pressure Liquid ; Chronic Disease ; Elasticity Imaging Techniques ; Female ; Hepatic Veins/physiology ; Humans ; Hypertension, Portal/complications/*diagnosis/metabolism ; Liver/pathology ; Liver Cirrhosis/ultrasonography ; Liver Diseases/complications/*diagnosis/metabolism ; Male ; Middle Aged ; Nitric Oxide/metabolism ; Portal Pressure ; Regression Analysis ; Severity of Illness Index