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non cirrhosis portal hypertension

by Mr. Declan Jaskolski V Published 1 year ago Updated 4 months ago
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Table 1

PSVD EHPVO Cirrhosis
Small portal vein obliteration (“hepato- ... ++ +/- -
Periportal shunt vessel ++ + -
Fibrous septa +/-; without bridging 1 - ++; with bridging delimitating nodules
May 5 2022

Full Answer

What is idiopathic non-cirrhotic portal hypertension?

Idiopathic non-cirrhotic portal hypertension: a review Idiopathic non-cirrhotic portal hypertension (INCPH) is a rare disease characterized of intrahepatic portal hypertension in the absence of cirrhosis or other causes of liver disease and splanchnic venous thrombosis.

What is the pathophysiology of portal hypertension?

No drug references linked in this topic. Portal hypertension is defined by a pathologic increase in the pressure of the portal venous system. Cirrhosis is the most common cause of portal hypertension, but it can also be present in the absence of cirrhosis, a condition referred to as "noncirrhotic portal hypertension."

What causes portal hypertension and cirrhosis?

It can result from several conditions such as hepatitis (an inflammatory disease) or alcohol abuse. Autoimmune diseases of the liver such as autoimmune hepatitis, primary sclerosing cholangitis, and primary biliary cholangitis also are causes of cirrhosis and portal hypertension.

What is non-cirrhotic portal fibrosis?

Non-cirrhotic Portal Fibrosis (NCPF) variously called as Idiopathic PHT (IPH), hepatoportal sclerosis and obliterative venopathy, is a disorder of unknown etiology, clinically characterized by features of PHT; moderate to massive splenomegaly, with or without hypersplenism, preserved liver functions, and patent hepatic and portal veins, (Table 2).

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Can portal hypertension occur without cirrhosis?

Portal hypertension is defined by a pathologic increase in the pressure of the portal venous system. Cirrhosis is the most common cause of portal hypertension, but it can also be present in the absence of cirrhosis, a condition referred to as "noncirrhotic portal hypertension."

What is non-cirrhotic liver disease?

Idiopathic non-cirrhotic portal hypertension (INCPH) is a rare disease characterized of intrahepatic portal hypertension in the absence of cirrhosis or other causes of liver disease and splanchnic venous thrombosis.

Does portal hypertension mean you have cirrhosis?

The most common cause of portal hypertension is cirrhosis, or scarring of the liver. Cirrhosis results from the healing of a liver injury caused by hepatitis, alcohol abuse or other causes of liver damage. In cirrhosis, the scar tissue blocks the flow of blood through the liver and slows its processing functions.

What is non-cirrhotic portal fibrosis?

Non-cirrhotic portal fibrosis is a syndrome of obscure etiology, characterized by 'obliterative portovenopathy' leading to PHT, massive splenomegaly and well-tolerated episodes of variceal bleeding in young adults from low socioeconomic backgrounds, having near normal hepatic functions.

How long can you live with nonalcoholic cirrhosis of the liver?

Survival and mortality The median survival was 24.2 (range 0.2-26.1) years in the NAFLD group and 19.5 (range 0.2-24.2) years in the AFLD group (p = 0.0007). Median follow-up time for the non-alcoholic group was 9.9 years (range 0.2-26 years) and 9.2 years (0.2-25 years) for the alcoholic group.

What are the 3 categories of portal hypertension?

With regard to the liver itself, causes of portal hypertension usually are classified as prehepatic, intrahepatic, and posthepatic.

Will portal hypertension go away?

Outlook. While cirrhosis cannot be cured, portal hypertension can be managed. After treatment, patients should schedule regular ultrasounds to monitor the health of their liver. In addition, they should make healthy lifestyle changes, such as limiting alcohol, eating a well-rounded diet, and getting regular exercise.

What stage of liver disease is portal hypertension?

Portal hypertension is defined as the pathological increase of portal venous pressure, mainly due to chronic end-stage liver disease, leading to augmented hepatic vascular resistance and congestion of the blood in the portal venous system.

Is portal hypertension life threatening?

Portal hypertension is a dangerous condition with severe, life-threatening complications. Call your healthcare provider right away if you notice any of these symptoms: Yellowing of the skin.

How long can you live with portal hypertension?

These complications result from portal hypertension and/or from liver insufficiency. The survival of both stages is markedly different with compensated patients having a median survival time of over 12 years compared to decompensated patients who survive less than 2 years (1, 3).

What does non-cirrhotic liver morphology mean?

Conclusion: Non-cirrhotic portal fibrosis is a justifiable name for this disease that can progress to end stage liver disease. It represents a single entity that has been considered as different diseases and given various names on the basis of the dominant element in its heterogeneous morphological manifestation.

What can cause portal hypertension?

Portal hypertension is a term used to describe elevated pressures in the portal venous system (a major vein that leads to the liver). Portal hypertension may be caused by intrinsic liver disease, obstruction, or structural changes that result in increased portal venous flow or increased hepatic resistance.

Is chronic liver disease and cirrhosis the same?

Key points about cirrhosis Cirrhosis is a long-term (chronic) liver disease. The most common causes are hepatitis and other viruses, and alcohol abuse. Other medical problems can also cause it. The damage to the liver usually can't be reversed.

What is the most common complication of portal hypertension?

Variceal hemorrhage is the most common complication associated with portal hypertension. Almost 90% of patients with cirrhosis develop varices, and approximately 30% of varices bleed. The estimated mortality rate for the first episode of variceal hemorrhage is 30-50%.

What are the 4 stages of cirrhosis of the liver?

Cirrhosis is classified into four stages that include:Stage I: Steatosis. The first stage of liver disease is characterized by inflammation of the bile duct or liver. ... Stage II: Scarring (fibrosis) of the liver due to inflammation. ... Stage III: Cirrhosis. ... Stage IV: Liver failure or advanced liver disease or hepatic failure.

What stage of cirrhosis does varices occur?

Cirrhosis can be divided into 4 stages: stage 1, no varices, no ascites; stage 2, varices without ascites and without bleeding; stage 3, ascites+/-varices; stage 4, bleeding+/-ascites.

What are the criteria for portal hypertension?

A formal diagnosis is based on the following criteria: 1) presence of unequivocal signs of portal hypertension, 2) absence of cirrhosis, advanced fibrosis or other causes of chronic liver diseases , and 3) absence of thrombosis of the hepatic veins or of the portal vein at imaging.

What is INCPH in a patient?

Idiopathic non-cirrhotic portal hypertension (INCPH) is a rare disease characterized of intrahepatic portal hypertension in the absence of cirrhosis or other causes of liver disease and splanchnic venous thrombosis. The etiology of INCPH can be classified in five categories: 1) immunological disorders (i.e. association with common variable immunodeficiency syndrome, connective tissue diseases, Crohn's disease, etc.), 2) chronic infections, 3) exposure to medications or toxins (e.g. azathioprine, 6- thioguanine, arsenic), 4) genetic predisposition (i.e. familial aggregation and association with Adams-Oliver syndrome and Turner disease) and 5) prothrombotic conditions (e.g. inherited thrombophilias myeloproliferative neoplasm antiphospholipid syndrome). Roughly, INCPH diagnosis is based on clinical criteria and the formal exclusion of any other causes of portal hypertension. A formal diagnosis is based on the following criteria: 1) presence of unequivocal signs of portal hypertension, 2) absence of cirrhosis, advanced fibrosis or other causes of chronic liver diseases, and 3) absence of thrombosis of the hepatic veins or of the portal vein at imaging. Patients with INCPH usually present with signs or symptoms of portal hypertension such as gastro-esophageal varices, variceal bleeding or splenomegaly. Ascites and/or liver failure can occur in the context of precipitating factors. The development of portal vein thrombosis is common. Survival is manly limited by concomitant disorders. Currently, treatment of INCPH relies on the prevention of complications related to portal hypertension, following current guidelines of cirrhotic portal hypertension. No treatment has been studied aimed to modify the natural history of the disease. Anticoagulation therapy can be considered in patients who develop portal vein thrombosis.

What causes portal hypertension?

The main cause of portal hypertension is cirrhosis. This is a scarring of the liver. It can result from several conditions such as hepatitis (an inflammatory disease) or alcohol abuse. Autoimmune diseases of the liver such as autoimmune hepatitis, primary sclerosing cholangitis, and primary biliary cholangitis also are causes ...

How to reduce blood pressure in portal vein?

improving your diet. avoiding alcohol consumption. exercising regularly. quitting smoking if you smoke. Medications such as beta-blockers are also important to help reduce your blood pressure and relax your blood vessels. Other medications, such as propranolol and isosorbide, may help lower the pressure in the portal vein, too.

What causes a blood clot in the portal vein?

liver infections. reaction to certain medications, such as methotrexate. Cirrhosis can cause the normally smooth inner walls of the portal vein to become irregular. This can increase resistance to blood flow. As a result, blood pressure in the portal vein increases. A blood clot can also form in the portal vein.

What is the best way to check for portal veins?

Screenings such as a doppler ultrasound are helpful. An ultrasound can reveal the condition of the portal vein and how blood is flowing through it. If an ultrasound is inconclusive, a CT scan may be helpful.

What does it mean when your blood pressure is too high?

When the blood pressure in the portal vein is too high, you have portal hypertension. Portal hypertension can be quite serious, though it’s treatable if diagnosed in time. It’s not always easy to diagnose, however. Typically, you become alerted to the condition when you start experiencing symptoms.

Why does my liver have scar tissue?

Whenever your liver is harmed, it attempts to heal itself. This causes scar tissue to form. Too much scarring makes it harder for your liver to do its job. Other cirrhosis causes include: nonalcoholic fatty liver disease. iron buildup in your body. cystic fibrosis. poorly developed bile ducts. liver infections.

What is the role of the liver in the heart?

The liver plays an important role in your circulation. It filters out toxins and other waste matter that the digestive organs have deposited in your blood stream. When the blood pressure in the portal vein is too high, you have portal hypertension.

What is a non-cirrhotic portal fibrosis?

Non-cirrhotic Portal Fibrosis (NCPF) variously called as Idiopathic PHT (IPH), hepatoportal sclerosis and obliterative venopathy, is a disorder of unknown etiology, clinically characterized by features of PHT; moderate to massive splenomegaly, with or without hypersplenism, preserved liver functions , and patent hepatic and portal veins [2], [3] ( Table 2 ).

What is portal hypertension?

Portal hypertension (PHT) is a clinical syndrome defined by a portal venous pressure gradient between the portal vein (PV) and inferior vena cava exceeding 5 mmHg [1]. Cirrhotic PHT is associated with an elevated hepatic venous pressure gradient (HVPG) predominantly due to raised sinusoidal resistance, while in the non-cirrhotic PHT (NCPH), HVPG is normal or only mildly elevated and is significantly lower than PV pressure. The diseases leading to NCPH are primarily vascular in nature and classified anatomically on the basis of site of resistance to blood flow, as prehepatic, hepatic, and post-hepatic – hepatic causes are further subdivided into pre-sinusoidal, sinusoidal and post-sinusoidal ( Table 1) [2], [3]. Most of the times, PHT is a late manifestation of the primary disease. However, non-cirrhotic portal fibrosis (NCPF) and extra-hepatic PV obstruction (EHPVO) are two disorders, which present only with features of PHT without any evidence of significant parenchymal dysfunction [2], [3], [4], [5]. In this review, an updated account of these two clinical entities along with some of the other causes will be presented.

What is portal biliopathy?

Portal biliopathy refers to biliary ductal (extra- and intra-hepatic) and gall-bladder wall abnormalities in patients with PHT , which take the form of intrahepatic biliary radicles dilatation, indentations, caliber irregularities, displacements, angulations, ectasias, strictures, common bile duct stones, filling defects, compressions, gall-bladder, and pericholedochal varices or a mass (pseudocholangiocarcinoma sign). Frequency of these changes in patients with EHPVO, cirrhosis and NCPF is 80–100%, 0–33%, and 9–40%, respectively. Increased prevalence of biliopathy in EHPVO is related to long standing portal cavernoma in the biliary and peribiliary region, causing compressive and ischemic changes on the biliary tree, the later ones may remain irreversible even after shunt surgery [109]. The left hepatic duct is involved more commonly (38–100%) and severely. Liver histology is essentially normal. Portal biliopathy usually remains asymptomatic (62–95%). Common symptoms are jaundice, biliary colic, abdominal pain and recurrent cholangitis and are seen with old age, long-standing disease, presence of stones and abnormal liver function tests [109], [110], [111], [112], [113]. ERCP is the diagnostic gold standard, but, being invasive is indicated in symptomatic cases requiring endotherapy. A classification based on ERCP has been proposed ( Fig. 6 A) [109]. MRCP with portography has equal efficacy and is also helpful in differentiating choledochal varices from stones. Radiologically, biliopathy commonly occurs in those EHPVO cases, where PVT extends into mesenteric veins or bile duct is more acutely angulated (median 110° vs. 128°) [114]. Natural history of biliopathy is ill-defined and varies from asymptomatic state to development of various sequelae like choledocholithiasis, cholangitis, and secondary biliary cirrhosis. About 4–10% of portal biliopathy cases may succumb to these sequelae despite endoscopic treatments [109], [111].

What is the prevalence of NCPF/IPH?

NCPF/IPH in the setting of HIV and AIDS needs special mention [21], [95], [99]. The prevalence of NCPF/IPH in HIV is around 0.45–1% and is rapidly increasing. This is due to prolonged survival of HIV infected patients following usage of highly active antiretroviral therapy (HAART) and is related to either one or a combination of the following factors – recurrent opportunistic gut infections, usage of HAART especially didanosine, hypercoagulability, direct effect of HIV – but the exact mechanism still remains unclear [21], [95], [99]. The role of the underlying prothrombotic state is controversial [21], [96], [97]. HIV virus itself may be implicated in the disease pathogenesis as indicated by its propensity to infect hepatic stellate cells and cause endothelial injury via cytokines like endothelin-1, inerleukins-1 and 6 and platelet derived growth factor [99]. HIV related NCPF occurs predominantly in males (50–100%), homosexuals (50–75%), prolonged infection (median 11.5 years, range 7–15 years) and is associated with immune reconstitution. Patients with HIV who develop NCPF are older with reduced platelets and CD4 counts, elevated liver enzymes, and have longer exposure to didanosine or concomitant exposure to stavudine or tenofovir [21], [95], [96], [97], [98]. Presentation is with features of PHT. Median liver stiffness is 7.8–10.2 kPa. Median HVPG is 8 mmHg. PVT has been observed in 25–75% [95], [96]. Liver decompensation requiring liver transplantation (LTx) has been reported [100].

What are the factors that cause EHPVO?

Like other states of venous thrombosis, the factors leading to EHPVO can be grouped as those within the vessel lumen, within the wall and outside the vessel; and also as prothrombotic states (inherited or acquired) and local factors (trauma, injury, inflammatory conditions). The most common prothrombotic states seen in children are methylene tetrahydrofolate reductase (MTHFR) deficiency (C677T) and prothrombin gene mutations (G20201A), whereas in adults, primary myeloproliferative disorders (MPD) (with or without janus kinase 2, JAK2 mutation V617F) are the commonest. Overall, a single or more prothrombotic states are seen in 28–62% of cases, but none of the studies has screened for all known prothrombotic states [35], [36], [37], [38], [39], [40], [41], [42], [43], [44], [45], [46], [47], [48], [49], [50], [51], [52], [53], [54], [55], [56], [57], [58], [59] ( Table 4 ). In a recent meta-analysis, the prevalence of MPD and JAK2 mutations in PVT was found to be 31.5% and 27.7%, respectively [60]. On the other hand, in a patient with non-malignant non-cirrhotic PVT, the odds ratios of usage of oral contraceptives, or presence of prothrombin gene mutation, factor-V Leiden, or deficiencies of protein-C, protein-S and antithrombin-III are 50, 7, 1.5, 5, 3, and 1, respectively [61]. But, direct extrapolation of these results for EHPVO is unjust. Other conditions leading to EHPVO are local abdominal inflammatory and neoplastic conditions and direct or indirect PV injury secondary to accidental or non-accidental trauma or iatrogenic causes subsequent to development of PVT. Lastly, rare congenital and developmental anomalies like PV stenosis, atresia or agenesis can lead to EHPVO, which are usually associated with other malformations, particularly cardiac [4].

Is NCPH a heterogeneous group?

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.

Is NCPH a heterogeneous group of liver disorders of vascular origin, leading to PHT with

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.

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