Liver disease portal hypertension

Portal hypertension most commonly occurs as a consequence of chronic liver disease. Portal hypertension Is caused by Increased blood flow within the portal venous system and increased resistance to portal flow within the liver. Splanchnic blood flow is increased in patients with cirrhosis due to low arteriolar resistance that is mediated by increased circulating vasodilators and decreased vascular sensitivity to vasoconstrictors. Intrahepatic vascular resistance is increased in cirrhosis due to fixed fibrosis within the spaces of Disse and hepatic veins as well as reversible vasoconstriction of hepatic sinusoids and venules. Among the consequences of portal hypertension are ascites, hepatic encephalopathy, and the development of portosystemic collaterals—especially gastric or esophageal varices. Varices can rupture, leading to massive upper gastrointestinal bleeding. 

A 70-year-old woman with a 2-year history of primary biliary cirrhosis confirmed by histological and immunological criteria took colestyramine sachets twice daily for 2 months and developed lethargy, confusion, and drowsiness (3). She had signs of chronic liver disease, portal hypertension, and hepatic encephalopathy. Laboratory investigations confirmed a metabolic acidosis (pH 7.15) and hyperchloremia. Multiple cultures failed to reveal sepsis, and a urinary pH of 4.85 together with tests of renal acidification excluded renal tubular acidosis. No other cause was found and she responded to 600 mmol of sodium bicarbonate intravenously over 36 hours. 

O Portal hypertension is the precipitating factor for the complications of cirrhotic liver disease—ascites, spontaneous bacterial peritonitis (SBP), variceal bleeding, and hepatic encephalopathy. Lowering portal pressure can reduce the complications of cirrhosis and decrease morbidity and mortality. 

Cirrhosis is the progressive replacement of normal hepatic cells by fibrous scar tissue. This scarring is accompanied by the loss of viable hepatocytes, which are the functional cells of the liver. Progressive cirrhosis is irreversible and leads to portal hypertension that is in turn responsible for many of the complications of advanced liver disease. These consequences include (but are not limited to) spontaneous bacterial peritonitis (SBP), hepatic encephalopathy, and variceal bleeding.1... 

FIGURE 19-4. Treatment algorithm for active gastrointestinal bleeding resulting from portal hypertension. (From Schianotd, Bodenheimer HC. Complications of Chronic Liver Disease. In Friedman SL, McQuaid KR, Grendell JH (eds.)... 

CIRRHOSIS A chronic liver disease that is caused by alcohol abuse, toxins, nutritional deficiency, or infection. A main symptom of cirrhosis is portal hypertension. 

Alcoholics with chronic liver disease may have disorders of fluid and electrolyte balance, including ascites, edema, and effusions. These factors may be related to decreased protein synthesis and portal hypertension. Alterations of whole body potassium induced by vomiting and diarrhea, as well as severe secondary aldosteronism, may contribute to muscle weakness and can be worsened by diuretic therapy. Some alcoholic patients develop hypoglycemia, probably as a result of impaired hepatic gluconeogenesis. Some alcoholics also develop ketosis, caused by excessive lipolytic factors, especially increased cortisol and growth hormone. 

Liver cirrhosis is among the top 10 causes of death in the Western world. The disease occurs after chronic damage to hepatic cells, mainly hepatocytes, which can be caused by viral hepatitis, chronic alcohol abuse or toxic injury, biliary disease, and metabolic liver disorders [64], Liver cirrhosis is characterized by an abnormal deposition of connective tissue in the liver, which hampers the normal functions of the liver. Other features of the disease are general tissue damage, chronic inflammation, and the conversion of normal liver architecture into structurally abnormal nodules. Secondary to these anatomical changes are disturbances in the liver function and in the hemodynamics leading to portal hypertension and intrahepatic shunting [39, 64, 103],... 

The patient was a 35-year-old white male with a x-antitrypsin deficiency. He received a combined liver-kidney transplant for cirrhosis complicated by portal hypertension, renal insufficiency secondary to membranoproliferative glomerulonephritis, and combined restrictive and obstructive pulmonary disease at age 18 years. 

This patient illustrates a complicated clinical course of oq-antitrypsin deficiency. Our patient had liver disease that presented during infancy and developed into hepatic cirrhosis. He exhibited most of the complications of cirrhosis, including portal hypertension with ascites, hyperammonemia, malnutrition, and variceal hemorrhage. These complications of cirrhosis are not unique to a,-antitrypsin deficiency, but it is important to note the potential severity of the liver disease associated with this condition. 

Liver disease is managed conventionally, maintaining appropriate nutritional support and managing the complications of portal hypertension and hepatic failure as they occur. We counsel heterozygote parents of a PIZZ... 

An enlargement of the spleen can be associated with many conditions, including infections, anaemias, malignancies and liver disease. In portal hypertension the back pressure in the splenic vein causes the spleen to enlarge. 

Many centres prefer to avoid using NSAIDs in any patient with liver disease because of their side-effect profile. However, if the liver disorder is purely cholestatic in origin and the disease has not progressed to cirrhosis and portal hypertension, NSAIDs may be an option. Any risk-benefit assessment should consider the potential risk of hepatotox-icity, albeit rare. There are no specific contraindications in this patient because they are not cirrhotic, do not have deranged clotting, and are unlikely to be at increased risk of deteriorating renal function. If deemed necessary an NSAID could be used cautiously. 

The first-pass effect may be reduced in cirrhosis and portal hypertension owing to reduced blood flow through the liver. Additionally, in cirrhosis and acute hepatitis the number of functional hepatocytes may be reduced. Drugs with a high first-pass extraction ratio and a narrow therapeutic window, such as the statins, are more likely to be toxic in patients with liver dysfunction [20]. In liver disease, an increase in the percentage of drug reaching the systemic circulation is an important factor in statin toxicity. 

Consensus guidelines on the management of osteoporosis associated with chronic liver disease recommend oral calcium and vitamin D supplementation plus transdermal HRT as first-line therapy for women with established osteoporosis. Transdermal oestradiol should be used at a dose of 50 pg/day (equivalent to 2 mg daily of oral oestradiol). This should be given in combination with a progestogen in women with an intact uterus [4]. Oral bisphosphonates should be avoided in cirrhotic patients who may have portal hypertension and oesophageal varices because of their potential to precipitate a variceal bleed [4, 27],... 

No obvious signs of chronic liver disease, e.g. portal hypertension, varices, ascites... 

Hepatic Fibrosis/Cirrhosis Fibrosis usually results from chronic inflammation which can be the result of continuous exposure to a variety of hepatotoxic chemicals such as organic arscnicals, vinyl chloride, or high doses of vitamin A (Zimmerman, 1999), chronic ethanol ingestion and nonalcoholic fatty liver disease. Fibrosis usually occurs around the portal area, in the space of Disse, and around the central veins. This results in loss of liver architecture and function. The hepatocytes are replaced with fibrous material and thus there is hepatocyte loss. Periportal fibrosis may lead to portal hypertension. 

Boyer TD, Henderson JM. Portal hypertension and bleeding esophageal varices. In Zakim D, Boyer TD, eds. Hepatology A textbook of liver disease. 4th ed. Philadelphia WB Saunders 2003. p.581-629. 

Saverymuttu, S.H., Grammatopoulos, A., Meanock, CJ., Maxwell, JJ)., Joseph, A.E. Gallbladder wall thickening (congestive cholecystopathy) in chronic liver disease a sign of portal hypertension. Brit. J. Radiol. 1990 63 922-925... 

Differentiation of vascular-related liver diseases Detection of alterations regarding the bile ducts Differential diagnosis of mechanical jaundice Assessment of portal hypertension Extent of collateral vessels Unclarified diffuse alterations of the liver parenchyma... 

The presinusoidal block can be classified under non-parenchymatous portal hypertension. The WHVP is normal. There is no impairment of liver function until a later stage of the underlying disease has been reached. This type of block is caused by a congenital or acquired constriction of the lumen or by a reduction in portal venous branches within the periportal triangles, (s. tab. 14.3)... 

Both liver adenomas and nodular regenerative hyperplasia can cause a presinusoidal block due to distortion of the portal veins. Wilson s disease leads to an overlapping of presinusoidal and sinusoidal portal hypertension. In alcoholic cirrhosis, there is an overlap of the sinusoidal and postsinusoidal forms. 

Hypertension of the portal vein, with its numerous intrinsic or acquired causes, may not display any symptoms for several years. Portal hypertension itself is very often a concomitant symptom in a number of liver diseases. (65) It can lead to severe or even fatal complications. For this reason, hepatological investigation frequently needs to explore (7.) the presence of portal hypertension, (2.) its aetiology, (i.) its severity, and (4.) potentially successful treatment of the underlying causes - in order to produce a favourable effect on portal hypertension. (22, 23, 33, 37, 38, 69, 158)... 

Liver transplantation not only removes the continued risk of variceal bleeding, but also eliminates the underlying liver disease causing portal hypertension. However, due to the scarcity of liver donors, limited financial resources and the life-long immunosuppression required, this major surgical intervention can only rarely be considered - perhaps in cases where a previous shunt operation or the creation of a TIPS was not possible. The survival rate for transplantation is higher than when recurrent bleeding is treated by repeated sclerotherapy (73% versus 17% after 4 years). The indication for transplantation (e. g. cirrhosis Child B or C) should be set as early as possible, (s. p. 872)... 

Tekeste, H., Latour, F., Levitt, R.E. Portal hypertension complicating sarcoid liver disease case report and review of the literature. Amer. J. Gastroenterol. 1984 79 389 - 396... 

In liver diseases involving elevated hydrostatic pressure (e.g. as a result of portal hypertension), the inflow of fluid into the interstitium is increased, whereas the return of fluid into the vascular bed is decreased due to the depressed colloidosmotic pressure (e.g. as a result of hypalbuminaemia). Likewise, a boost in capillary permeability leads to an outflow of fluid into the interstitial tissue. (2, 5, 8, 12)... 

An increase in the retention of sodium occurs in the early stages of severe liver disease, particularly in liver cirrhosis, without any disruption of the water balance. This early tendency towards sodium retention can be detected using the NaCl-tolerance test. The retention of sodium reduces the sodium excretion rate in the urine to < 10 mval/day (normal rate 120 to 220 mval/day). Diuresis is not primarily restricted patients with ascites and oedema react to an excessive intake of water with an adequate excretion of diluted urine, albeit in the virtual absence of sodium excretion. The limited sodium excretion derives from increased, mainly proximal tubular reabsorption of sodium and not from diminished glomerular filtration. Overall maintenance of the liver architecture is usually accompanied by undisturbed sodium excretion, despite existing portal hypertension (such as in primary biliary cirrhosis). Marked sodium retention is, however, usually found in alcoholic-toxic cirrhosis. For this reason, such patients are not only the ones most frequently affected by ascites and oedema, but as a rule they display the most serious forms. This is probably also due to additional biochemical and hormonal factors which are present to a greater degree in patients with alcohol-related liver disease. 

It is not clearly understood why in some cases oedema without ascites and in other cases ascites without oedema as well as ascites together with oedema or even pleural effusion without ascites occur. Ascites develops most frequently during the course of liver disease (= hepatogenic ascites), in particular in chronic liver diseases with portal hypertension (= portal ascites), (s. tab. 16.7) Various mechanical, biochemical and neural disorders overlap in their effects and pathways, depending on the underlying liver disease. Only rarely is ascites found in diseases with presinusoidal localization of portal hypertension (such as portal vein thrombosis) or with minor restrictions in the synthesis of albumin (as in biliary cirrhosis). Formation of ascites occurs in about 50% of all cirrhotic patients within 10 years of... 

The hepatorenal syndrome (HRS) is a functional, oliguric, progressive, in principle reversible, circulation-related kidney failure occurring in severe liver disease and portal hypertension and increasing liver insufficiency - assuming there are indeed no other causes of the renal insufficiency. 

Tab. 18.1 Liver diseases associated with hepatopulmonary syndrome. Portal hypertension is considered to be an essential factor in the pathogenesis of HPS (with some references)...

Liver transplantation is the only way of ensuring the elimination of the underlying liver disease with portal hypertension - and thus also the eradication of the pre-... 

Lower gastrointestinal bleeding due to liver disease is very rare. The sources of bleeding develop in the course of portal hypertension. Intestinal erosions constitute the predominant mucosal changes in portal hypertensive vasculopathy in the gastrointestinal tract. They are usually responsible for the frequency of occult intestinal bleeding in cirrhosis patients. (232, 245)... 

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