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Journal of Veterinary Internal Medicine 2011Portal hypertension (PH) is the result of increased vascular resistance in the portal circulation, increased portal venous blood flow, or both. In veterinary medicine,... (Review)
Review
Portal hypertension (PH) is the result of increased vascular resistance in the portal circulation, increased portal venous blood flow, or both. In veterinary medicine, where portal pressure is seldom measured directly, the diagnosis of PH often is inferred from identification of associated complications including multiple acquired portosystemic shunts, ascites, and hepatic encephalopathy. Likewise, treatment of PH primarily is aimed at controlling these complications. The goal of this review is to provide an update on the pathophysiology, diagnosis, and treatment of PH. The review draws from information in the veterinary hepatology literature, reviews, and consensus statements in human hepatology and the literature on experimental models of PH.
Topics: Animals; Cat Diseases; Cats; Dog Diseases; Dogs; Hypertension, Portal; Liver; Liver Circulation; Portal System
PubMed: 21382073
DOI: 10.1111/j.1939-1676.2011.00691.x -
Mayo Clinic Proceedings Apr 2019Portal hypertension is a major complication of cirrhosis, and its consequences, including ascites, esophageal varices, hepatic encephalopathy, and hepatorenal syndrome,... (Review)
Review
Portal hypertension is a major complication of cirrhosis, and its consequences, including ascites, esophageal varices, hepatic encephalopathy, and hepatorenal syndrome, lead to substantial morbidity and mortality. The past several decades have seen major improvements in the clinical management of complications of portal hypertension, resulting in substantial gains in patient outcomes. However, important challenges remain. This review focuses on the pathophysiology and diagnosis of portal hypertension and discusses general approaches in the management of patients with ascites as a result of portal hypertension.
Topics: Ascites; Disease Management; Gastrointestinal Hemorrhage; Hepatic Encephalopathy; Humans; Hypertension, Portal; Liver Cirrhosis
PubMed: 30947834
DOI: 10.1016/j.mayocp.2018.12.020 -
Clinics in Liver Disease Nov 2019
Topics: Ascites; Esophageal and Gastric Varices; Frailty; Gastrointestinal Hemorrhage; Hepatic Encephalopathy; Hepatopulmonary Syndrome; Hepatorenal Syndrome; Humans; Hypertension, Portal; Liver Diseases; Portasystemic Shunt, Transjugular Intrahepatic; Sarcopenia
PubMed: 31563223
DOI: 10.1016/j.cld.2019.08.001 -
Current Gastroenterology Reports Sep 2020Non-cirrhotic portal hypertension (NCPH) includes a heterogeneous group of conditions. The aim of this paper is to make an overview on the denominations, diagnostical... (Review)
Review
PURPOSE OF THE REVIEW
Non-cirrhotic portal hypertension (NCPH) includes a heterogeneous group of conditions. The aim of this paper is to make an overview on the denominations, diagnostical features and management of porto-sinusoidal vascular disease (PSVD) and chronic portal vein thrombosis (PVT) being the main causes of NCPH in the Western world.
RECENT FINDINGS
The management of NCPH consists in the treatment of associated diseases and of portal hypertension (PH). PH due to PSVD or PVT is managed similarly to PH due to cirrhosis. TIPS placement and liver transplantation are considerable options in patients with refractory variceal bleeding/ascites and with progressive liver failure. Anticoagulation is a cornerstone both in the treatment of thrombosis in PSVD and in the prevention of thrombosis recurrence in patients with portal cavernoma. Physicians should be aware of the existence of PSVD and chronic PVT and actively search them in particular settings. To now, the management of portal hypertension-related complications in NCPH is the same of those of cirrhosis. Large cooperative studies on the natural history of NCPH are necessary to better define its management.
Topics: Chronic Disease; Disease Progression; Humans; Hypertension, Portal; Liver; Liver Cirrhosis; Liver Diseases; Portal Vein; Vascular Diseases
PubMed: 32940785
DOI: 10.1007/s11894-020-00792-0 -
Journal of Hepatology Feb 2014NCPH is a heterogeneous group of liver disorders of vascular origin, leading to PHT with near normal HVPG. NCPF/IPH is a disorder of young adults or middle aged women,... (Review)
Review
NCPH is a heterogeneous group of liver disorders of vascular origin, leading to PHT with near normal HVPG. NCPF/IPH is a disorder of young adults or middle aged women, whereas EHPVO is a disorder of childhood. Early age acute or recurrent infections in an individual with thrombotic predisposition constitute the likely pathogenesis. Both disorders present with clinically significant PHT with preserved liver functions. Diagnosis is easy and can often be made clinically with support from imaging modalities. Management centers on control and prophylaxis of variceal bleeding. In EHPVO, there are additional concerns of growth faltering, portal biliopathy, MHE and parenchymal dysfunction. Surgical shunts are indicated in patients with failure of endotherapy, bleeding from sites not amenable to endotherapy, symptomatic hypersplenism or symptomatic biliopathy. Persistent growth failure, symptomatic and recurrent hepatic encephalopathy, impaired quality of life or massive splenomegaly that interferes with daily activities are other surgical indications. Rex-shunt or MLPVB is the recommended shunt for EHPVO, but needs proper pre-operative radiological assessment and surgical expertise. Both disorders have otherwise a fairly good prognosis, but need regular and careful surveillance. Hepatic schistosomiasis, CHF and NRH have similar presentation and comparable prognosis.
Topics: Animals; Disease Models, Animal; Esophageal and Gastric Varices; Female; Hemodynamics; Humans; Hypertension, Portal; Liver Cirrhosis; Male; Pancytopenia; Portal Vein; Splenomegaly; Idiopathic Noncirrhotic Portal Hypertension
PubMed: 23978714
DOI: 10.1016/j.jhep.2013.08.013 -
Internal Medicine Journal Jan 2015Portal hypertension is an important complication of liver disease. As a result of elevated pressures within the portal vein several complications can arise, including... (Review)
Review
Portal hypertension is an important complication of liver disease. As a result of elevated pressures within the portal vein several complications can arise, including the development of oesophageal and gastric varices, ascites, hepatic encephalopathy as well as complications secondary to circulatory dysfunction, such as hepatorenal syndrome, portopulmonary syndrome and hepatopulmonary syndrome. This review outlines the pathogenesis and diagnosis of portal hypertension and outlines the management of these various important clinical sequelae. The management of oesophageal and gastric varices is particularly important, and both the emergency management together with prophylactic management of this condition are described.
Topics: Diagnostic Imaging; Disease Management; Humans; Hypertension, Portal; Portal Vein; Tomography, X-Ray Computed; Ultrasonography, Doppler; Vascular Resistance; Venous Pressure
PubMed: 25230084
DOI: 10.1111/imj.12590 -
Nature Reviews. Gastroenterology &... Apr 2019The liver microcirculatory milieu, mainly composed of liver sinusoidal endothelial cells (LSECs), hepatic stellate cells (HSCs) and hepatic macrophages, has an essential... (Review)
Review
The liver microcirculatory milieu, mainly composed of liver sinusoidal endothelial cells (LSECs), hepatic stellate cells (HSCs) and hepatic macrophages, has an essential role in liver homeostasis, including in preserving hepatocyte function, regulating the vascular tone and controlling inflammation. Liver microcirculatory dysfunction is one of the key mechanisms that promotes the progression of chronic liver disease (also termed cirrhosis) and the development of its major clinical complication, portal hypertension. In the present Review, we describe the current knowledge of liver microcirculatory dysfunction in cirrhotic portal hypertension and appraise the preclinical models used to study the liver circulation. We also provide a comprehensive summary of the promising therapeutic options to target the liver microvasculature in cirrhosis.
Topics: Combined Modality Therapy; Humans; Hypertension, Portal; Liver Circulation; Microcirculation; Vascular Resistance
PubMed: 30568278
DOI: 10.1038/s41575-018-0097-3 -
Current Opinion in Gastroenterology May 2018Noncirrhotic portal hypertension represents a heterogeneous group of liver disorders that is characterized by portal hypertension in the absence of cirrhosis. The... (Review)
Review
PURPOSE OF REVIEW
Noncirrhotic portal hypertension represents a heterogeneous group of liver disorders that is characterized by portal hypertension in the absence of cirrhosis. The purpose of this review is to serve as a guide on how to approach a patient with noncirrhotic portal hypertension with a focus on recent developments.
RECENT FINDINGS
Recent studies pertaining to noncirrhotic portal hypertension have investigated aetiological causes, mechanisms of disease, noninvasive diagnostic modalities, clinical characteristics in the paediatric population and novel treatment targets.
SUMMARY
Noncirrhotic portal hypertension is an underappreciated clinical entity that can be difficult to diagnosis without a healthy suspicion. Diagnosis then relies on a comprehensive understanding of the causes and clinical manifestations of this disease, as well as a careful interpretation of the liver biopsy. Noninvasive approaches to diagnosis may play a significant role moving forward in this disease. Treatment in NCPH remains largely targeted at the individual sequalae of portal hypertension.
Topics: Adult; Child; Humans; Hypertension, Portal; Liver Cirrhosis
PubMed: 29465431
DOI: 10.1097/MOG.0000000000000433 -
Clinics in Liver Disease May 2014
Topics: Humans; Hypertension, Portal
PubMed: 24679508
DOI: 10.1016/j.cld.2014.02.001 -
Hepatology (Baltimore, Md.) Dec 2018Idiopathic portal hypertension (IPH) is a rare disorder characterized by clinical portal hypertension in the absence of a recognizable cause such as cirrhosis.... (Review)
Review
Idiopathic portal hypertension (IPH) is a rare disorder characterized by clinical portal hypertension in the absence of a recognizable cause such as cirrhosis. Laboratory tests often reveal a preserved liver function with anemia, leukopenia, and thrombocytopenia due to splenomegaly. Imaging studies reveal signs of portal hypertension, whereas liver stiffness and portal pressure values are usually normal or slightly elevated. Liver biopsy is considered mandatory in order to rule out other causes of portal hypertension, mainly cirrhosis. Liver histology may only show subtle or mild changes, and the definite diagnosis of IPH often requires an expert pathologist and a high-quality specimen. The most frequent clinical presentation is variceal bleeding. Ascites is rarely observed initially, although it may occasionally appear during follow-up. Typical histological findings associated with IPH have been described in patients without portal hypertension, probably representing early stages of the disease. Although the pathophysiology of this entity remains largely unknown, it is frequently associated with underlying immunological disorders, bacterial infections, trace metal poisoning, medications, liver circulatory disturbances, and thrombotic events. The long-term prognosis of patients with IPH, where ascites and the underlying condition are important prognostic factors, is better than in patients with cirrhosis. Treatments that modify the natural history of the disease remain an unmet need, and management of IPH is frequently restricted to control of portal hypertension-related complications.
Topics: Animals; Humans; Hypertension, Portal; Liver Cirrhosis; Pancytopenia; Splenomegaly; Idiopathic Noncirrhotic Portal Hypertension
PubMed: 30066417
DOI: 10.1002/hep.30132