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Patients With Decompensated Cirrhosis
AASLD Practice Guidelines
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Decompensated Cirrhosis
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ASCITES
An accumulation of excess fluid in the abdomen. Causes abdominal distention. Can be treated with a low sodium diet, and the use of diuretics, i.e. water pills.
"Ascites" is fluid that accumulates in the abdominal (peritoneal) cavity. It is a complication of cirrhosis and appears as an abdominal bulge. The peritoneum is the inner lining of the abdominal cavity, which also folds over to cover the organs inside the abdomen such as the liver, gallbladder, spleen, pancreas, and intestines.
Ascites develops because of a combination of two factors:
(1) increased pressure in the vein system that carries blood from the stomach, intestines, and spleen to the liver (portal hypertension)
(2) a low level of albumin in the blood (hypoalbuminemia). Albumin, which is the predominant protein in the blood and which helps maintain blood volume, is reduced in cirrhosis primarily because the damaged liver is not able to produce enough albumin.
2017
Evaluation of Ascites
Analysis of Ascitic Fluid
Basic Management of Ascites
Management of Refractory Ascites
Complications Associated with Ascites
ASTERIXIS
An uncontrollable flapping of the hands that becomes noticeable when patients stretch out their arms, palms out, as if stopping traffic. Is associated with mental confusion, i.e. encephalopathy.
Testing For Asterixis
Extend the arms, spread the fingers, dorsiflex the wrist and observe for the abnormal “flapping” tremor at the wrist. If not immediately apparent, this tremor may be accentuated by asking the patient to keep the arms straight while the examiner gently hyperextends the patient’s wrist with a sweeping motion
An alternate method of testing for asterixis involves having the patient relax his legs while he lies supine with his knees bent. The feet should be kept flat on the table and as the legs fall to the sides, watch for flapping of the legs at the hip joint. This repetitively brings the knees back together
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"Bilateral Asterixis": Metabolic encephalopathies, especially hepatic and renal, are the most common causes of bilateral asterixis. Those caused specifically by hepatic failure are known as “liver (or hepatic) flap”.
Other causes of asterixis include cardiac and respiratory disease, electrolyte abnormalities and drug intoxication. Electrolyte abnormalities known to cause asterixis include hypoglycaemia, hypokalaemia and hypomagnesaemia. Drug intoxications include barbiturate intoxication, alcoholism, phenytoin intoxication (“phenytoin flap”) and primidone intoxication. Wilson’s disease and focal brain lesions in the rostral midbrain tegmentum may also cause asterixis.
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CAPUT MEDUSAE.
Enlarged blood vessels that snake out from the belly button in a patient with ascites. The term Caput Medusae describes the appearance of distended and engorged umbilical veins which are seen radiating from the umbilicus across the abdomen to join systemic veins. It is a sign of severe portal hypertension with portal-systemic shunting through the umbilical veins. The name originates from the apparent similarity to Medusa's hair once Minerva had turned it into snakes.
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Caput Medusae is distinguished from inferior vena cava obstruction by determining the direction of flow in the veins below the umbilicus; it is towards the legs in the former, and towards the head in the latter (as abdominal collaterals develop to bypass the blocked inferior vena cava and permit venous return from the legs).
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EDEMA
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Edema occurs when tiny blood vessels in your body (capillaries) leak fluid. This leakage can result from damage to or increased pressure in the capillaries, or from lowered levels of serum albumin, a protein in your blood. When your body senses the capillaries are leaking, your kidneys begin to retain more sodium and water than normal to compensate for the lost fluid from your blood vessels. This increases the amount of fluid circulating through your body, which causes the capillaries to leak more. The fluid from the capillaries leaks into the surrounding tissue, causing the tissue to swell.
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Edema is caused by either systemic diseases, that is, diseases that affect the various organ systems of the body, or by local conditions involving just the affected extremities. The most common systemic diseases associated with edema involve the heart, liver, and kidneys. In these diseases, edema occurs primarily because of the body's retention of too much salt (sodium chloride). The excess salt causes the body to retain water. This water then leaks into the interstitial tissue spaces, where it appears as edema.
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Pitting edema can be demonstrated by applying pressure to the swollen area by depressing the skin with a finger. If the pressing causes an indentation that persists for some time after the release of the pressure, the edema is referred to as pitting edema. Any form of pressure, such as from the elastic in socks, can induce pitting with this type of edema.
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Peripheral edema, which is usually seen as pitting edema of the legs and feet, also occurs in cirrhosis. The edema is a consequence of the hypoalbuminemia and activation of the renin-angiotensin- aldosterone hormonal system, which prompt the kidneys to retain salt and water. The presence or absence of edema in patients with cirrhosis and ascites is an important consideration in the treatment of the ascites.
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In patients with ascites without edema, diuretics must be given with extra caution. The reason for this is that a diuresis (induced increased volume of urine) that is too depleting or rapid in these patients can lead to a low blood volume (hypovolemia), which can possibly be followed by kidney and liver failure.
In contrast, when patients who have both edema and ascites undergo diuresis, the edema fluid in the interstitial space serves as somewhat of a buffer against the development of low blood volume. The excess interstitial fluid moves into the blood vessel saces to rapidly replenish the depleted blood volume
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ENCEPHALOPATHY
An altered mental status leading to coma. Can be treated with animal protein restriction and a poorly absorbed sugar called Lactulose.
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Hepatic encephalopathy is caused by disorders that affect the liver. These include disorders that reduce liver function (such as cirrhosis or hepatitis) and conditions in which blood circulation does not enter the liver. The exact cause of hepatic encephalopathy is unknown. An important job of the liver is to change toxic substances that are either made by the body or taken into the body (such as medicines) and make them harmless. However, when the liver is damaged, these "poisons" may build up in the bloodstream.
Ammonia, which is produced by the body when proteins are digested, is one of the harmful substances that is normally made harmless by the liver. Many other substances may also build up in the body if the liver is not working well. They can cause damage to the nervous system.
Patients with very mild hepatic encephalopathy may have normal memory, language and motor skills, but may have impairment of attention and decision-making, and may have impaired fitness to drive.
These patients usually have normal function on standard mental state testing but abnormal psychometric testing.
Patients with mild and moderate hepatic encephalopathy show decreased short-term memory and concentration with testing of mental state. They may also have a flapping tremor, fetor hepaticus (a sweet musty aroma of the breath), hyperventilation and hypothermia.
First Principles of Gastroenterology GI Textbook Hepatic Encephalopathy
Hepatic encephalopathy (HE) is a complex, potentially reversible neuropsychiatric condition that occurs as a consequence of acute or chronic liver disease. It is characterized by changes of personality, consciousness, behavior and neuromuscular function
Early features include reversal of sleep pattern, apathy, hypersomnia, irritability and personal neglect. In later stages, delirium and coma may occur. Neurologic signs may include hyperreflexia, rigidity, myoclonus and asterixis. Asterixis is not specific to hepatic encephalopathy and may be present in other causes of metabolic encephalopathy. Seizures and lateralizing signs are uncommon and are
more commonly seen in acute than chronic liver failure.
Grading of hepatic encephalopathy
Grade 0: subclinical; normal mental status, but minimal changes in memory, concentration, intellectual function, coordination.
Grade 1: mild confusion, euphoria or depression, decreased attention, slowing of ability to perform mental tasks, irritability, disorder of sleep pattern such as inverted sleep cycle.
Grade 2: drowsiness, lethargy, gross deficits in ability to perform mental tasks, obvious personality changes, inappropriate behavior, intermittent disorientation.
Grade 3: somnolent but rousable, unable to perform mental tasks, disorientation to time and place, marked confusion, amnesia, occasional fits of rage, speech is present but incomprehensible.
Grade 4: coma, with or without response to painful stimuli
2017
Clinical Features
Diagnostic Tests
General Approach to the Management of HE
Medical Therapy for Hepatic Encephalopathy
2017
Minimal Hepatic Encephalopathy in Cirrhosis- How Long to Treat?
Omesh Goyal, Sandeep S. Sidhu, Harsh Kishore
Minimal hepatic encephalopathy (MHE) can reverse after short-term treatment. However, relapse rate of MHE after stopping treatment has not been studied so far. We aimed to evaluate long-term (9 months) efficacy of a short-term (3 months) treatment of MHE with lactulose/rifaximin, for maintenance of remission from MHE. Material and methods.
A particularly foul "dead mouse" smell found on the breath. Frequently precedes coma. A distinctive musty, sweet breath odor characterizes hepatic encephalopathy, a life-threatening complication of severe liver disease. The odor results from the damaged liver’s inability to metabolize and detoxify mercaptans produced by bacterial degradation of methionine, a sulfurous amino acid. These substances circulate in the blood, are expelled by the lungs, and flavor the breath.
GYNECOMASTIA
Gynecomastia due to the increased estrogen levels, which is due to the inability of the diseased liver to detoxify estrogen but may also be due to Aldactone, a drug used to control ascites
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Enlarged, tender breasts in men.
HAIR LOSS
Hair becomes sparse in men from the face, chest and pubis, and under the arms in women.
JAUNDICE
A yellow discoloration of the skin due to an elevated bilirubin level.
The normal level of bilirubin in the blood is 1 mg/dL. If the concentration increases to 2 mg/dL, yellowing of the skin, sclera, and mucous membranes will become evident. Yellowing due to increased levels of bilirubin in the blood (hyperbilirubinemia), which then deposits in the tissues of the body, is called jaundice or icterus. Depending on the site of the problem, hyperbilirubinemia is usually dominated by either the unconjugated or conjugated type.
Jaundice can be classified according to the mechanism that produced it:
1. Hemolytic (prehepatic) jaundice
occurs when the liver cells are normal, but so much bilirubin is present that the capacity of the liver to take it up and/or conjugate it is exceeded. A typical cause is excessive breakdown of red blood cells (i.e., hemolytic anemia). In prehepatic jaundice, the type of bilirubin seen in the blood is largely unconjugated.
2. Hepatocellular jaundice
usually occurs when production of bilirubin is normal, but damage to the liver cells interferes with uptake, conjugation, or excretion of bilirubin. Depending on which factors predominate, bilirubin may be conjugated or unconjugated.
3. Posthepatic (obstructive)
is due to obstruction in the biliary drainage system, rather than to any problem with the hepatocytes themselves. Strictures or stones in the extrahepatic biliary tract, as well as tumors, may produce this type of jaundice. This "backed-up" bilirubin, which is conjugated, can get into the blood. .
SCLERAL ICTERUS 
Yellow discoloration of the whites of the eyes [sclera], due to an elevated bilirubin level.
Low Blood Pressure
Many patients with cirrhosis or scarring of the liver develop abnormalities in their cardiovascular system as a result of their liver disease. All diseases that result in cirrhosis can lead to these cardiovascular changes but they occur more frequently in patients with more advanced disease.
It is believed that substances circulating in the blood that are normally cleared by a healthy liver can cause a generalized dilatation of the blood vessels throughout the body (except in the kidney where they are intensely constricted).
The generalized vasodilatation then results in a low blood pressure. Most patients with this form of low blood pressure are asymptomatic because of its very gradual onset and because of a compensatory increased blood flow from the heart. There is no specific treatment for the development of low blood pressure in patients with cirrhosis other than general, supportive care. The blood pressure will normalize after removal of the diseased liver and transplantation of a healthy liver. .
Articles in Press - Muscle Cramps in Liver Disease
PALMAR ERYTHEMA
Bright red coloring of the palms, particularly at the base of the thumb and little finger. May be due to excess estrogen or liver disease.
Palmar erythema is reddening of the palms at the thenar and hypothenar eminences. It is associated with various physiological as well as pathological changes, the principal one of which is portal hypertension. It is also seen in patients with liver dysfunction
PAPER MONEY SKIN
Numerous small blood vessels that resemble the silk threads in a U.S. dollar bill. Commonly cover the upper body, often in association with spider naevi.
PAROTID GLAND ENLARGEMENT
Enlargement of a gland on the face located under the ear. Causes an unusual appearance of the protrusion of the earlobes straight out from the jaw.
SPIDER ANGIOMATA
Enlarged blood vessels that resemble little spiders. Usually found on the upper chest,back, face, and arms. Turn white when their center is touched [blanches].
Spider angioma is so named because of its appearance: a central, red, elevated area with surrounding broken blood vessels radiating outward like a spider's legs. Many children and adults have a spider angioma.
Causes Spider angiomas are associated with childhood, pregnancy, liver disease, birth control pills, estrogen treatment, and they develop for unknown reasons as well.
Signs & Symptoms
Spider angiomas classically appear as small, centrally raised bumps (papules)caused by a dilated arteriole (small artery). A network of dilated capillaries (tiny blood vessels) radiate from the arteriole. Pressing on the lesion causes the redness to disappear briefly, and there is a rapid return of redness once the pressure is lifted.
SPONTANEOUS BACTERIAL PERITONITIS
Fever, and abdominal pain in a patient with ascites.
Spontaneous bacterial peritonitis (inflammation and infection of the membrane that is lining the abdominal cavity) is a complication of cirrhotic ascites that occurs in the absence of any intra-abdominal, surgically treatable source of infection.
TERRY'S NAILS ..
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The normal pinkish color of the nails turns completely white, with the disappearance of the half-moon circles at the base of the nails. Clinical findings of liver diseases White nails. White nails are commonly seen in patients with cirrhosis and rarely in other diseases. They are due to opacity of the nail bed. Nails appear white and a pink zone is seen only at the tip of the nail. The lunula may be not distinguished.
Thrombocytopenia
Definition
Thrombocytopenia is an abnormal drop in the number of blood cells involved in forming blood clots. These cells are called platelets.
Description
The normal amount of platelets is usually between 150,000 and 450,000 cells per microliter of blood. A microliter is an amount equal to one one-millionth of a liter (a liter is almost equal to a quart). Platelet numbers are counted by having a blood sample collected and placing a measured amount of blood in a machine called a cell counter. When the platelet number drops below 150,000 cells per microliter of blood, this person is said to be thrombocytopenic.
Causes & symptoms
Abnormal reductions in the number of platelets are caused when abnormalities occur in any of the following three processes: decreased platelet production by the bone marrow; increased trapping of platelets by the spleen; or a more rapid than normal destruction of platelets. Persons with this condition easily bruise and can have episodes of excess bleeding (a hemorrhage).
Platelets come from megakaryocytes, which are produced in the material located within the center cavity of the bones (bone marrow). When abnormalities develop in the marrow, the marrow cells can lose their ability to produce platelets in correct amounts. The result is a lower than normal level of platelets in the blood. Drugs used in cancer chemotherapy can cause the marrow to malfunction in this way, as can the presence of tumor cells in the marrow itself. Normally, the spleen holds about one-third of the body's platelets as part of this organ's function to recycle aging or damaged red blood cells (the cells that carry oxygen in the blood). When liver disease or cancer of the spleen is present, the spleen can enlarge, resulting in a greater number of platelets staying in the organ. This condition results in abnormally low numbers of platelets in the blood.
Platelets can breakdown in unusually high amounts in persons with abnormalities in their blood vessel walls, with blood clots, or with man-made replacement heart valves. Devices placed inside blood vessels to keep them from closing (stents) due to weakened walls or fat build-up can also cause platelets to breakdown. In addition, infections and other changes in the immune system can speed up the removal of platelets from the circulation.
Diagnosis
Thrombocytopenia is diagnosed by having a blood sample taken and counting the platelets present in the sample. However, accurately determining the medical reason for this conditions is complex.
Once a low platelet count is verified, a careful evaluation of the function of the bone marrow and spleen are necessary. Improper functioning of either or both of these organs can cause thrombocytopenia. In addition, the causes for the abnormal spleen or marrow function must be investigated since different cancers, blood disorders, or liver disease can be the true cause for the drop in platelets found in the blood.
Treatment
If low platelet counts are caused by an enlarged spleen, removal of the spleen can help raise the platelet level, since the spleen is no longer there to capture the platelets. However, proper treatment for what causes the enlarged spleen is necessary as well. Low platelet counts can indicate more serious conditions. If a dysfunctional immune system is found to be the cause for this condition, drugs like steroids or gamma globulin can be used to help maintain platelet levels in certain cases. If low platelet levels are due to an abnormally low level of platelet production, transfusions of platelets can be given as well.
Prognosis
Thrombocytopenia can result in fatal bleeding, but it also can indicate various other, more serious, cancers and disorders that affect the blood cells. This condition requires thorough medical evaluation.
Prevention
There is no known way to prevent thrombocytopenia. ,
Key Terms to know: Gamma globulin: One of a group of proteins found in the blood that is involved in helping the body fight infections. Stent: A man-made surgical device, usually tube-shaped, that is placed into a blood vessel to keep it from closing. Transfusion: The transfer of blood from one person to another. Transfusions can be direct, in which blood is transferred from the donor to the recipient; or indirect, in which the blood is taken from the donor, stored in a container, and then given to the recipient.
UMBILICAL HERNIA
Patients with massive ascites may experience abdominal discomfort, depressed appetite, and decreased oral intake. Diaphragmatic elevation may lead to symptoms of dyspnea. Pleural effusions may result from the passage of ascitic fluid across channels in the diaphragm. Umbilical and inguinal hernias are common in patients with moderate and massive ascites. The use of an elastic abdominal binder may protect the skin overlying a protruding umbilical hernia from maceration and may help prevent rupture and subsequent infection. Timely large-volume paracentesis also may help to prevent this disastrous complication.
Umbilical hernias should not undergo elective repair unless patients are significantly symptomatic or their hernias are irreducible. As with all other surgeries in patients with cirrhosis, herniorrhaphy carries multiple potential risks such as intraoperative bleeding, postoperative infection, and liver failure because of anesthesia-induced reductions in hepatic blood flow. However, these risks become acceptable in patients with severe symptoms from their hernia. Urgent surgery is necessary in the patient whose hernia has been complicated by bowel incarceration.
Esophageal Varices
Esophageal varices are swollen veins in the lining of the lower esophagus near the stomach. Gastric varices are swollen veins in the lining of the stomach. The swelling of these veins is caused by liver disease. Swollen veins in the esophagus or stomach resemble the varicose veins that some people have in their legs. Because the veins in the esophagus are so close to the surface of the esophagus, swollen veins in this location can rupture and cause dangerous bleeding.
Esophageal varices almost always occur in people who have cirrhosis of the liver. Cirrhosis causes scarring of the liver, which slows the flow of blood through the liver. Scarring causes blood to back up in the portal vein, the main vein that delivers blood from the stomach and intestines to the liver. This "back up" causes high blood pressure in the portal vein and other nearby veins, and this is called portal hypertension. The backup of blood forces veins to enlarge in the vicinity of the stomach and esophagus. Esophageal varices usually have enlarged, irregularly shaped bulbous regions (varicosities) that are interrupted by narrower regions. Because the blood pressure inside the varices is higher than inside normal blood vessels, and the walls of the vessels (and esophagus) are thin, the veins rupture easily and can bleed profusely.
Symptoms
Portal hypertension often does not cause any symptoms, and sometimes is discovered only when the varices bleed. When significant bleeding occurs, a person will vomit blood, often in large amounts. People with massive bleeding feel dizzy and may lose consciousness. Some people bleed in smaller amounts over a longer period, and they swallow the blood rather than vomit. Their stools may contain red or tarry-black blood. People with bleeding from the esophagus usually also have symptoms of cirrhosis of the liver.
Diagnosis
To diagnose esophageal varices, a doctor will use an instrument called an endoscope, a thin, flexible tube with a camera at its tip that is inserted through the mouth so the doctor can see the walls of the esophagus and search for the source of bleeding. If bleeding is occurring in the esophagus, this procedure will be done as an emergency. Tiny instruments may be attached to the endoscope to provide treatment at the same time.
Expected
Duration Bleeding from esophageal varices can stop on its own or with treatment. However, bleeding esophageal varices can be fatal, particularly in people with severe liver disease. Half or more of people who survive episodes of bleeding from esophageal varices will have the problem return during the first one to two years. The risk of recurrence can be reduced with treatment.
