1.Deficiencies in proteins C and S in a patient with idiopathic portal hypertension accompanied by portal vein thrombosis.
Sena HWANG ; Do Young KIM ; Minju KIM ; Young Eun CHON ; Hyun Jung LEE ; Young Nyun PARK ; Jun Yong PARK ; Sang Hoon AHN ; Kwang Hyub HAN ; Chae Yoon CHON
The Korean Journal of Hepatology 2010;16(2):176-181
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
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Hypertension, Portal/complications/*diagnosis/pathology
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Male
;
*Portal Vein
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Protein C Deficiency/*complications
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Protein S Deficiency/*complications
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Tomography, X-Ray Computed
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Venous Thrombosis/complications/*diagnosis/pathology
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Young Adult
2.Hepatoportal Sclerosis in Childhood: Descriptive Analysis of 12 Patients.
Mustafa Serdar CANTEZ ; Nelgin GERENLI ; Vildan ERTEKIN ; Mine GULLUOGLU ; Ozlem DURMAZ
Journal of Korean Medical Science 2013;28(10):1507-1511
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
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Child
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Child, Preschool
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Female
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Follow-Up Studies
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Gastroenterology
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Humans
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Hypertension, Portal/complications/*diagnosis/pathology
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Liver/pathology
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Liver Diseases/complications/*diagnosis/pathology
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Male
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Portal Vein/pathology
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Retrospective Studies
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Sclerosis/*diagnosis/*pathology
3.An Analysis of Extravariceal Collaterals of Gastric Varices Using Magnetic Resonance Angiography in Portal Hypertensive Patients.
Chul Hee PARK ; Hoon Jai CHUN ; Rok Sun JEONG ; Kyung Ho KIM ; Yong Sik KIM ; Young Sun KIM ; Yoon Tae JEEN ; Hong Sik LEE ; Soon Ho UM ; Sang Woo LEE ; Jai Hyun CHOI ; Chang Duck KIM ; Ho Sang RYU ; Jin Hai HYUN
The Korean Journal of Gastroenterology 2003;42(4):313-321
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
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Aged
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*Collateral Circulation
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Esophageal and Gastric Varices/complications/diagnosis/*pathology
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Female
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Humans
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Hypertension, Portal/complications/*pathology
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*Magnetic Resonance Angiography
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Male
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Middle Aged
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Stomach/*blood supply
4.The usefulness of non-invasive liver stiffness measurements in predicting clinically significant portal hypertension in cirrhotic patients: Korean data.
Won Ki HONG ; Moon Young KIM ; Soon Koo BAIK ; Seung Yong SHIN ; Jung Min KIM ; Yong Seok KANG ; Yoo Li LIM ; Young Ju KIM ; Youn Zoo CHO ; Hye Won HWANG ; Jin Hyung LEE ; Myeong Hun CHAE ; Hyoun A KIM ; Hye Won KANG ; Sang Ok KWON
Clinical and Molecular Hepatology 2013;19(4):370-375
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
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Aged
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Alcohol-Related Disorders/complications
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Area Under Curve
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*Elasticity Imaging Techniques
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Female
;
Hepatitis B/complications
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Hepatitis C/complications
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Humans
;
Hypertension, Portal/*complications/*diagnosis
;
Linear Models
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Liver Cirrhosis/*complications/*diagnosis/pathology
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Male
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Middle Aged
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ROC Curve
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Republic of Korea
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Sensitivity and Specificity
5.Portal hypertensive gastropathy.
Chinese Journal of Hepatology 2009;17(4):254-256
Animals
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Diagnosis, Differential
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Disease Models, Animal
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Gastric Antral Vascular Ectasia
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diagnosis
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pathology
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Gastric Mucosa
;
pathology
;
Gastrointestinal Hemorrhage
;
etiology
;
pathology
;
therapy
;
Gastroscopy
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Humans
;
Hypertension, Portal
;
complications
;
epidemiology
;
pathology
;
Intestinal Mucosa
;
pathology
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Liver Cirrhosis
;
complications
;
pathology
;
Liver Cirrhosis, Experimental
;
complications
;
pathology
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Rats
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Stomach Diseases
;
epidemiology
;
etiology
;
pathology
;
therapy
6.Portal Hypertensive Gastropathy and Gastric Antral Vascular Ectasia.
The Korean Journal of Gastroenterology 2010;56(3):186-191
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
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Gastric Antral Vascular Ectasia/*diagnosis/etiology/therapy
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Gastric Mucosa/metabolism/pathology
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Humans
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Hypertension, Portal/*complications
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Portasystemic Shunt, Transjugular Intrahepatic
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Vasodilator Agents/therapeutic use
7.Relationship between Tetrahydrobiopterin and Portal Hypertension in Patients with Chronic Liver Disease.
Won Ki HONG ; Kwang Yong SHIM ; Soon Koo BAIK ; Moon Young KIM ; Mee Yon CHO ; Yoon Ok JANG ; Young Shik PARK ; Jin HAN ; Gaeun KIM ; Youn Zoo CHO ; Hye Won HWANG ; Jin Hyung LEE ; Myeong Hun CHAE ; Sang Ok KWON
Journal of Korean Medical Science 2014;29(3):392-399
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
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Aged
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Biopterin/*analogs & derivatives/analysis
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*Chromatography, High Pressure Liquid
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Chronic Disease
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Elasticity Imaging Techniques
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Female
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Hepatic Veins/physiology
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Humans
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Hypertension, Portal/complications/*diagnosis/metabolism
;
Liver/pathology
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Liver Cirrhosis/ultrasonography
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Liver Diseases/complications/*diagnosis/metabolism
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Male
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Middle Aged
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Nitric Oxide/metabolism
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Portal Pressure
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Regression Analysis
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Severity of Illness Index