Liver have complex functions with a high workload. Various liver diseases are the result of the interaction of diverse genetic and environmental factors. Moreover, other systemic diseases may also affect liver, producing corresponding manifestations, such as abnormal liver function tests, portal vein or hepatic vein thrombosis, portal hypertension, hepatosplenomegaly and liver space-occupying lesions. Therefore, it is extremely important for hepatologists to have an in-depth understanding of other systemic diseases of hepatic manifestations, especially hematologic, connective tissue, endocrine, and circulatory, in order to improve the level of clinical diagnosis and treatment.
Humans ; Hypertension, Portal ; Portal Vein/pathology*

Cholecystolithiasis ; complications ; Humans ; Hypertension, Portal ; Portal Vein ; pathology

No abstract availble.
Blood Flow Velocity ; Humans ; Hypertension, Portal/*ultrasonography ; Liver Cirrhosis/pathology ; Portal Vein/ultrasonography ; *Ultrasonography, Doppler

Liver involvement is often observed in hematological disorders, resulting in liver abnormality, including unconjugated hyperbilirubinemia, monoclonal hyperglobulinemia, portal vein, or hepatic vein thrombosis or portal hypertension, hepatosplenomegaly, or iron accumulation in the liver. Here we summarize the major hematological diseases that often affect the liver: hemolytic anemia, defect in coagulation or anti-coagulation factors, myeloproliferative neoplasm, hemophagocytic lymphohistiocytosis, multiple myeloma, leukemia, and lymphoma. We hope this review will help clinicians diagnose and manage the patients with liver involvement by hematological disorders.
Hematologic Diseases ; Humans ; Hypertension, Portal ; Myeloproliferative Disorders/diagnosis* ; Portal Vein/pathology*

OBJECTIVETo review the outcomes of living-related liver transplantation (LRLT) in treating 3 cases of cavernous transformation of portal vein (CTPV) with severe portal hypertension.

METHODSThree children (two boys and one girl) were presented to our hospital with recurring esophageal variceal bleeding, decompensating ascites, splenomegaly and refractory anemia. CTPV was confirmed by intravenous computed tomographic portography using a helical computed tomography scanner and 3-dimensional image reconstruction. LRLT were performed in these 3 patients from July 2006 to January 2007. The evaluation of the outcomes was made by referring to their clinical features and laboratory and imaging examination findings.

RESULTSAlthough one patient died from early graft thrombosis, the other two patients showed excellent prognoses. They lived and stayed well during a follow-up period of 12-14 months. Following the transplantations, there had been no esophageal variceal hemorrhage, the ascites disappeared and the portal hypertension vanished. Their hemoglobin, blood platelets count, and serum albumin reached normal values.

CONCLUSIONLRLT is an effective procedure in treating CTPV with severe portal hypertension. The reconstruction of the portal vein is the difficult part of the LRLT procedure.

Child ; Female ; Humans ; Hypertension, Portal ; pathology ; surgery ; Liver Transplantation ; Living Donors ; Male ; Parents ; Portal Vein ; pathology ; Treatment Outcome

OBJECTIVETo study the histopathological changes of livers in idiopathic portal hypertension (IPH).

METHODSLiver specimens from 29 cases with idiopathic portal hypertension were studied. Histological preparations of the livers were stained with haematoxylin eosin and Masson's trichrome; reticular fibers in the liver tissues were demonstrated. The slides were also stained using some immunohistochemistry methods, and the pathological changes of the livers were analyzed.

RESULTSThe characteristic changes found in these IPH livers were dense portal fibrosis; obliteration, with or without phlebitis, of the branches of the portal vein; dilatation of the sinusoids; atrophy and nodular hyperplasia of liver cells.

CONCLUSIONSHistopathological changes of the livers in IPH are dense portal fibrosis, portal vein branch obliteration and nodular hyperplasia of liver cells. These are the main features for a histopathological diagnosis of IPH.

Adolescent ; Adult ; Female ; Fibrosis ; pathology ; Humans ; Hypertension, Portal ; pathology ; Liver ; pathology ; Male ; Middle Aged ; Young Adult

A normal liver can develop cirrhosis through long-term and repeated stimulation from various etiologies. Histological manifestations like the collapse of hepatic lobular structure (including microvascular structure) and the formation of pseudolobules can lead to portal hypertension and even decompensated cirrhosis. More and more evidence suggests that effective etiological treatment can not only delay but also reverse the progression of cirrhosis. The mechanism of cirrhosis reversal mainly includes the degradation of extracellular matrix, hepatocyte regeneration, and hepatic lobular remodeling. The "gold standard" for the evaluation of cirrhosis reversal at present is still a liver biopsy. Therefore, the histopathological evaluation of cirrhosis reversal is very important for determining the disease's prognosis, efficacy, and mechanism of exploration.
Humans ; Liver Cirrhosis/pathology* ; Liver/pathology* ; Hypertension, Portal ; Hepatocytes/pathology* ; Prognosis

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

OBJECTIVETo compare the characteristics of esophageal gastric varices in portal hypertension patients with and without spontaneous shunts.

METHODSClinical data of 118 patients with esophageal gastric varices undergoing portal vein computed tomographic angiography (CTA) and gastroscopy between January 2012 and August 2015 was retrospectively reviewed.

RESULTSPortal vein CTA results showed that spleno-renal or gastro-renal shunts were detected in 24 out of 118 cases. The average portal vein diameters (PVD) of patients with and without spontaneous shunt were (12.48±2.79) mm and (13.58±3.46) mm, respectively (P>0.05). The average area of gastric veins in patients with spontaneous shunt was significantly larger than that of patients without shunt [294.00 (0.00~2400.00) mm2 vs. 26.00 (0.00~1620.00) mm2, respectively, (P<0.001]. Compared with patients without spontaneous shunt, the location of esophageal varices was lower and the degree was less serious in patients with spontaneous shunt (P<0.05). No matter with history of uppergastrointestinal bleeding, the average area of gastric veins in patients with spontaneous shunt was significantly larger than that of patients without shunt (P<0.05). For patients having no history of splenectomy, the average portal vein diameter (PVD) in those with spontaneous shunt was significantly smaller than that in those without shunt (P<0.05).

CONCLUSIONThe portal vein diameter of patients without splenectomy and with spontaneous shunts is shorter and their esophageal varices are less serious; the gastric veins are large and wriggly in patients with spontaneous shunts.

Angiography ; Esophageal and Gastric Varices ; physiopathology ; Gastroscopy ; Humans ; Hypertension, Portal ; physiopathology ; Portal Vein ; pathology ; Retrospective Studies ; Spleen ; Tomography, X-Ray Computed

Child ; Child, Preschool ; Female ; Humans ; Hypertension, Portal ; diagnosis ; etiology ; therapy ; Infant ; Male ; Portal Vein ; pathology ; Venous Thrombosis ; diagnosis ; etiology ; therapy