2.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
;
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
3.Recent Research Advance to Differentiate Portal Hypertension Associated with Primary Myelofibrosis and Cirrhosis --Review.
Rui LI ; Hua-Sheng LIU ; Ying CHEN
Journal of Experimental Hematology 2023;31(2):598-601
Primary myelofibrosis (PMF) is easily confused with cirrhosis, due to its main clinical manifestations of splenomegaly and the blood cytopenia. This review focuses on clinical studies to identify primary myelofibrosis and cirrhosis related portal hypertension, to analyze the differences between the two diseases, in order to distinguish PMF and cirrhosis from the pathogenesis, clinical manifestations, laboratory examinations and treatment principles, and simultaneously improve clinicians' understanding of PMF, which is a reference for exploring the early screening or diagnostic indicators of PMF, also provides a clinical basis for the application of new targeted drugs such as ruxolitinib.
Humans
;
Primary Myelofibrosis/drug therapy*
;
Hypertension, Portal/complications*
;
Liver Cirrhosis/pathology*
;
Splenomegaly/pathology*
;
Anemia
4.The lymphatic vascular system in liver diseases: its role in ascites formation.
Clinical and Molecular Hepatology 2013;19(2):99-104
The lymphatic system is part of the circulatory system and plays a key role in normal vascular function. Its failure plays a crucial role in the development and maintenance of various diseases including liver diseases. Lymphangiogenesis (the growth of lymphatic vessels) and changes in the properties of lymphatic vessels are associated with pathogenesis of tumor metastases, ascites formation, liver fibrosis/cirrhosis and portal hypertension. Despite its significant role in liver diseases and its importance as a potential therapeutic target for those diseases, the lymphatic vascular system of the liver is poorly understood. Therefore, how the lymphatic vascular system in general and lymphangiogenesis in particular are mechanistically related to the pathogenesis and maintenance of liver diseases are largely unknown. This article summarizes: 1) the lymphatic vascular system; 2) its role in liver tumors, liver fibrosis/cirrhosis and portal hypertension; and 3) its role in ascites formation.
Ascites/*etiology
;
Humans
;
Hypertension, Portal/complications/pathology
;
Liver Cirrhosis/complications/pathology
;
Liver Diseases/complications/*pathology
;
Liver Neoplasms/complications/pathology
;
Lymphangiogenesis
;
Lymphatic Vessels/metabolism/physiopathology
5.Gastric polyposis associated with portal hypertension.
Tae Hee LEE ; Jae Young JANG ; Soung Won JEONG ; So Young JIN
The Korean Journal of Internal Medicine 2013;28(2):261-261
No abstract available.
Aged
;
Biopsy
;
Endoscopy, Digestive System
;
Female
;
Humans
;
Hypertension, Portal/*complications
;
Polyps/*etiology/pathology
;
Stomach Diseases/*etiology/pathology
6.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
;
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
7.Management of portal hypertensive gastropathy and other bleeding.
Clinical and Molecular Hepatology 2014;20(1):1-5
A major cause of cirrhosis related morbidity and mortality is the development of variceal bleeding, a direct consequence of portal hypertension. Less common causes of gastrointestinal bleeding are peptic ulcers, malignancy, angiodysplasia, etc. Upper gastrointestinal bleeding has been classified according to the presence of a variceal or non-variceal bleeding. Although non-variceal gastrointestinal bleeding is not common in cirrhotic patients, gastroduodenal ulcers may develop as often as non-cirrhotic patients. Ulcers in cirrhotic patients may be more severe and less frequently associated with chronic intake of non-steroidal anti-inflammatory drugs, and may require more frequently endoscopic treatment. Portal hypertensive gastropathy (PHG) refers to changes in the mucosa of the stomach in patients with portal hypertension. Patients with portal hypertension may experience bleeding from the stomach, and pharmacologic or radiologic interventional procedure may be useful in preventing re-bleeding from PHG. Gastric antral vascular ectasia (GAVE) seems to be different disease entity from PHG, and endoscopic ablation can be the first-line treatment.
Gastric Antral Vascular Ectasia/complications
;
Gastric Mucosa/pathology
;
Gastrointestinal Hemorrhage/*etiology
;
Humans
;
Hypertension, Portal/*complications/prevention & control
;
Liver Cirrhosis/complications
;
Peptic Ulcer/complications
8.The role of hepatic sinusoid capillarization during the formation of portal hypertension in fibrotic rats induced by dimethylnitrosamine.
Xiong LU ; Ping LIU ; Guang-fu XU ; Cheng-hai LIU ; Feng-hua LI ; Cheng LIU
Chinese Journal of Hepatology 2003;11(10):595-598
OBJECTIVESTo study the role of hepatic sinusoid capillarization during the formation of portal hypertension in fibrotic rats induced by dimethylnitrosamine (DMN).
METHODSHepatic fibrotic rats were induced by administration of DMN intraperitoneally three times a week for 4 weeks. The rats were harvested on day 2 and weeks 1, 2, 3, 4, 5, 6, 8, 12 and 24. The formation of liver fibrosis and hepatic sinusoid capillarization were observed by morphologic methods. Pressure of portal vein (Ppv) was directed measured with intubation tube method by mesentry anterior vein.
RESULTSThe Ppv was getting higher and higher with the administration of DMN. After four weeks, the Ppv was higher than that of control [(1.10+/-0.18)kPa vs (0.52+/-0.04)kPa, t=6.41, P<0.01]. The dynamic change of hepatic sinusoid capillarization was in accordance with that of Ppv, which normalized gradually after the DMN was stopped. Significant positive correlation existed between the dynamic change of Ppv and the expression of vWF, laminin and alpha-SMA in sinus (r=0.833, P<0.01; r=0.953, P<0.01; r=0.919, P<0.01).
CONCLUSIONHepatic sinusoid capillarization is the vital cause for portal hypertension in fibrotic rats induced by DMN.
Animals ; Capillaries ; pathology ; Dimethylnitrosamine ; Hypertension, Portal ; chemically induced ; etiology ; pathology ; Liver ; blood supply ; pathology ; Liver Cirrhosis, Experimental ; chemically induced ; complications ; pathology ; Male ; Rats ; Rats, Wistar
9.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
;
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
10.What we need to know when performing and interpreting US elastography.
So Hyun PARK ; So Yeon KIM ; Chong Hyun SUH ; Seung Soo LEE ; Kyoung Won KIM ; So Jung LEE ; Moon Gyu LEE
Clinical and Molecular Hepatology 2016;22(3):406-414
According to the increasing need for accurate staging of hepatic fibrosis, the ultrasound (US) elastography techniques have evolved significantly over the past two decades. Currently, US elastography is increasingly used in clinical practice. Previously published studies have demonstrated the excellent diagnostic performance of US elastography for the detection and staging of liver fibrosis. Although US elastography may seem easy to perform and interpret, there are many technical and clinical factors which can affect the results of US elastography. Therefore, clinicians who are involved with US elastography should be aware of these factors. The purpose of this article is to present a brief overview of US techniques with the relevant technology, the clinical indications, diagnostic performance, and technical and biological factors which should be considered in order to avoid misinterpretation of US elastography results.
Disease Progression
;
Elasticity Imaging Techniques/instrumentation/*methods
;
Fatty Liver/complications/diagnostic imaging
;
Humans
;
Hypertension, Portal/complications
;
Liver/*diagnostic imaging/physiopathology
;
Liver Cirrhosis/diagnostic imaging/pathology