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Liver Cirrhosis: Not always due to alcohol abuse.
Cirrhosis represents a late stage of progressive hepatic fibrosis characterized
by distortion of the hepatic architecture and the formation of regenerative
nodules. It is generally considered to be irreversible in its advanced
stages at which point the only option may be liver transplantation. Patients
with cirrhosis are susceptible to a variety of complications and their
life expectancy is markedly reduced.
CLINICAL MANIFESTATIONS — Patients with cirrhosis may present in
a variety of ways. They may have stigmata of chronic liver
disease discovered on routine physical examination. They may have undergone
laboratory or radiologic testing or an unrelated surgical procedure that
incidentally uncovered the presence of cirrhosis. They may present with
decompensated cirrhosis, which is characterized by the presence of dramatic
and life-threatening complications, such as variceal hemorrhage, ascites,
spontaneous bacterial peritonitis (SBP), or hepatic encephalopathy.Some
patients never come to clinical attention. In older reviews, cirrhosis
was diagnosed at autopsy in up to 30 to 40 percent of patients.
All patients with cirrhosis should undergo diagnostic endoscopy to document
the presence of varices and to determine their risk for variceal hemorrhage.
Patients at high risk for development of variceal hemorrhage should be
considered for primary prophylaxis. I like Variceal
Banding.
Much to often Liver Cirrhosis is being related to excessive alcohol ingestion.
Even though alcoholism is a common cause of this entity, it is by far
not the only cause of this often deadly disease. Liver cirrhoses is not
curable, but it is definitely preventable in many instances, if one recognizes
and minimizes certain risk factors.
The liver, the largest of our organs, is a real laboratory. With many
many functions, essential for the human body. Within the liver cell carry
out, complex enzymatic processes take place and many essential nutrients
as well as glycogen, proteins, fat and vitamins are stored. Generally
speaking, it is a very resistant organ that rarely ever fails to perform,
contrary to the teachings and beliefs of many naturist, quacks and charlatans,
who readily blame the liver for many diverse symptoms, without any scientific
basis.
HOW IS CIRRHOSIS DIAGNOSED?
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| Physical Examination . A physical examination may
reveal the following findings in a patient with cirrhosis: |
A liver biopsy is the only definite method for
diagnosing cirrhosis. It also helps determine its cause, treatment
possibilities, the extent of damage, and the long-term outlook. For
example, hepatitis C patients who show no significant liver scarring
when biopsied appear to have a low risk for cirrhosis.
Percutaneous Liver Biopsy. This approach uses a needle inserted
through the abdomen to obtain a tissue sample from the liver. Various
forms of needles are used, including those that use suction or those
that cut out the tissue. If cirrhosis is suspected, a cutting needle
is the better tool. This approach should not be used in patients with
bleeding problems, and it must be used with caution in patients with
ascites or severe obesity.
Laparoscopy. This procedure employs small abdominal incision
through which the physician inserts a thin tube that contains small
surgical instruments and a tiny camera to view the surface of the
liver. This is generally reserved for staging cancer or for ascites
with unknown causes. Biopsies can be dangerous, so they cannot be
performed on patients who have test results that indicate clotting
problems, on those who have had previous liver biopsies, or who have
ascites . |
Imaging Tests. Ultrasound examination of the abdomen
is useful to confirm hepatosplenomegaly and may also reveal enlargement
or venous obstruction of the portal or splenic veins with portal hypertension.
Cavernous transformation of the portal vein can also be identified
and the presence of esophageal varices is suggested. New ultrasound
modalities are beginning to estimate portal vein flow.
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| Blood Tests .
Routine tests of liver function may be quite normal in cirrhosis.
A decreased serum albumin and a prolonged prothrombin time directly
reflect impaired hepatic function in the truest sense. An increased
serum gamma globulin accompanies many forms of chronic liver disease.
AST and ALT are often moderately elevated, while alkaline phosphatase
may be normal or increased, particularly with biliary obstruction.
Bilirubin is usually normal. Increased total serum globulin is common.
A normochromic normocytic (occasionally macrocytic) anemia, thrombocytopenia,
and leukopenia may be present. With alcohol-related liver disease,
the anemia is occasionally macrocytic.
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| This picture displays a Liver with Hepatic Cirrhosis, Macronudular tipe. |
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COMPLICATIONS |
| Ascites is the accumulation of fluid within the
peritoneal cavity. It is the most common complication of cirrhosis.
Nearly 60 percent of all patients with compensated cirrhosis will
develop ascites in 10 years . The two-year survival of patients with
ascites is approximately 50 percent . |
| Spontaneous bacterial peritonitis Spontaneous bacterial
peritonitis (SBP) is an infection of preexisting ascitic fluid without
evidence for an intraabdominal secondary source such as a perforated
viscus . SBP is almost always seen in the setting of end-stage liver
disease . Manifestations of SBP include fever, abdominal pain, abdominal
tenderness, and altered mental status. Some patients are asymptomatic
and present with only mild laboratory abnormalities. |
Hepatorenal Syndrome.The hepatorenal syndrome refers
to the development of acute renal failure in a patient who usually
has advanced hepatic disease, due to cirrhosis or less often metastatic
tumor or severe alcoholic hepatitis. Rather than being a new disease,
the hepatorenal syndrome usually represents the end-stage of a sequence
of reductions in renal perfusion induced by increasingly severe hepatic
injury. The initial reductions in glomerular filtration rate are often
masked clinically since associated decreases in muscle mass and hepatic
urea production minimize elevations in the plasma creatinine concentration
and blood urea nitrogen. Hepatorenal syndrome (HRS) is the development
of renal failure in patients with advanced chronic liver disease,
occasionally fulminant hepatitis, who have portal hypertension and
ascites. Estimates indicate that at least 40% of patients with cirrhosis
and ascites will develop HRS during the natural history of their disease.
Causes: Risk factors for developing HRS have been reported based on
a large series of patients with cirrhosis and ascites. Patients with
marked sodium and water retention, characterized by a low urinary
sodium excretion (<5 mEq/L) and dilutional hyponatremia, have a
higher probability of developing HRS compared to patients with less
sodium and water retention. Another important risk factor is the presence
of severe disturbances in the systemic circulation (mean arterial
pressure <80 mm Hg) associated with marked activation of the RAAS
and SRS. Surprisingly, patients with advanced liver disease, defined
by a high Child-Pugh score or worsening albumin, bilirubin, and prothrombin
levels, are not at higher risk of developing HRS.
Mortality/Morbidity: Importantly, be aware that 2 different
forms of HRS are described. Although their pathophysiology is similar,
their manifestations and outcomes are quite different.
Type 1 HRS is characterized by rapid and progressive renal impairment
and is precipitated most commonly by SBP. Type 1 HRS occurs in approximately
25% of patients with SBP, despite rapid resolution of the infection
with antibiotics. Without treatment, median survival of patients
with type 1 HRS is less than 2 weeks and virtually all patients
die within 10 weeks after the onset of renal failure.
Type 2 HRS is characterized by a moderate and stable reduction in
the GFR and commonly occurs in patients with relatively preserved
hepatic function. Median survival is 3-6 months. Although this is
markedly longer than type 1 HRS, it is still shorter compared to
patients with cirrhosis and ascites who do not have renal failure.
The following is a list of risk factors associated with the development
of HRS in patients with cirrhosis who are nonazotemic. All measurements
were obtained after a minimum of 5 days on a low-salt diet and without
diuretics.
Low urinary sodium excretion (<5 mEq/L)
Low serum sodium (dilutional hyponatremia)
Reduced free-water excretion after water load
Low mean arterial pressure.
High plasma renin activity
Increased plasma norepinephrine
Low plasma osmolality
High urine osmolality
High serum potassium
Previous episodes of ascites
Absence of hepatomegaly
Presence of esophageal varices
Poor nutritional status
Moderately increased serum urea (>30 mg/dL)
Moderately increased serum creatinine (>1.5 mg/dL)
Moderately reduced GFR (<50 mL/min)
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Variceal hemorrhage.Variceal
hemorrhage is a devastating complication that occurs in 25 to 40 percent
of patients with cirrhosis . Prior to the widespread use of current
therapies for acute variceal hemorrhage, the mortality rate of a single
variceal hemorrhage was 30 percent and only one-third of patients
survived for one year . Although survival has improved with modern
techniques for controlling variceal hemorrhage, mortality rates remain
high.
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Gastrointestinal hemorrhage due to esofageal variceal
bleeding.
This endoscopic image displays the exactly site of the bleeding
at the cardias, actve variceal bleeding is appreciated at the
esophageal cardias.
DIAGNOSIS OF THE BLEEDING SOURCE, Endoscopy is an essential
step in the diagnosis and treatment of acute variceal bleeding.
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The endoscopic image display 2 esofageal
varices that have been ligated. see
more examples. |
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Hepatopulmonary Syndrome The
hepatopulmonary syndrome (HPS) is considered to be present in patients
with the following triad:
1. Liver disease.
2 .Increased alveolar-arterial gradient while breathing room air
3.Evidence for intrapulmonary vascular abnormalities, referred to
as intrapulmonary vascular dilatations (IPVDs). Estimates of the
prevalence of HPS among patients with chronic liver disease range
from 4 to 47 percent, depending upon the diagnostic criteria and
methods used. Even in cirrhotic patients lacking HPS, mild hypoxemia
is common and is presumably caused by ascites, with resulting diaphragmatic
elevation and ventilation/perfusion mismatch.
Hypoxemia is seen in one-third of decompensated cirrhotic patients.
Hepatopulmonary syndrome (HPS) is the triad of chronic liver disease,
increased alveolar-arterial oxygen gradient on room air, and intrapulmonary
arteriovenous shunting (due to subpleural arteriovenous microshunts
that resemble spider angiomas . Patients present with the gradual
onset of hypoxemia that is commonly more severe in the upright position.
Laboratory abnormalities include hypoxemia, and decreased diffusing
capacity for CO2. The majority of patients with HPS demonstrate
marked improvement in symptoms when given 100% oxygen. HPS is a
relative contraindication to liver transplant surgery, however,
some patients with HPS may actually improve following transplant.
Chest radiograph abnormalities are detected in 46-100%
of patients with HPS . Medium sized (1.5 to 3 mm), bilateral, basilar,
nodular or reticulonodular opacities, with normal lung volumes,
are characteristic of HPS and represent dilated subpleural lung
vessels.
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Hepatic Encephalopathy Hepatic encephalopathy describes
the spectrum of potentially reversible neuropsychiatric abnormalities
seen in patients with liver dysfunction. Disturbance in the diurnal
sleep pattern (insomnia and hypersomnia) is a common early feature
that typically precedes overt neurologic signs. More advanced neurologic
features include the presence of asterixis, hyperactive deep tendon
reflexes, and less commonly, transient decerebrate posturing.
| Hepatocellular carcinoma Patients with cirrhosis
have a markedly increased risk of developing hepatocellular
carcinoma (HCC). The incidence in well compensated cirrhosis
is approximately 3 percent per year . Patients with most forms
of chronic hepatitis are not at an increased risk until cirrhosis
develops. Exceptions to this rule are patients with chronic
hepatitis B virus infection who can develop HCC in the absence
of cirrhosis Certain causes of cirrhosis appear to have a relatively
increased risk for HCC. Patients with cirrhosis from hepatitis
B, hepatitis C, and hemochromatosis are at the highest risk,
while those with cirrhosis from autoimmune hepatitis, nonalcoholic
steatohepatitis, and Wilson's disease appear to have a lower
risk.
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| The image displays a person with advanced liver cirrhosis
with bulking abdomen due to ascites. |
Ascites is the accumulation of fluid in the peritoneal cavity.
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Another image of the same patient. |
Grading — A grading system for ascites has been proposed by
the International Ascites Club.
Grade 1 — mild ascites detectable only by ultrasound examination
Grade 2 — moderate ascites manifested by moderate symmetrical
distension of the abdomen
Grade 3 — large or gross asites with marked abdominal distension.
Alcoholic hepatitis regularly causes ascites with or without cirrhosis.
| Diuretic-resistant cirrhotic ascites
is considered to be present when one or both of the following two
criteria is present in the absence of therapy with a nonsteroidal
antiinflammatory drug (NSAID), which can induce renal vasoconstriction
and diminish diuretic responsiveness |
| An inability to mobilize ascites despite compliance with
dietary sodium restriction (as confirmed by a 24-hour urine collection
containing less than 78 meq of sodium) and the administration of maximum
tolerable doses of oral diuretics (400 mg/day of spironolactone and
160 mg/day of furosemide). The 78 meq of sodium represents the recommended
88 meq intake minus 10 meq in nonurinary losses. Patients who gain
weight despite excreting more than 78 meq of sodium per day are not
compliant with the diet. The development of
prohibitive diuretic-related complications, such as progressive
azotemia, hepatic encephalopathy or progressive electrolyte imbalance.
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Causes of Cirrhosis |
Although most often associated with alcohol abuse, cirrhosis of
the liver can result from many causes. Almost any chronic liver disease
can lead to cirrhosis. This list gives some of the many causes:
Alcoholic liver disease most common cause.
Chronic viral hepatitis B, C and D Postnecrotic cirrhosis:
Hepatitis, a viral infection of the liver, usually causes this disease,
although poisonous substances may also cause it. Two types of hepatitis,
hepatitis B or hepatitis C, cause 25-75% of these cases. Large areas
of scar tissue mix with large areas of healing nodules.
Chronic autoimmune hepatitis
Non-alcoholic Steatohepatitis,
Malnutrition and Diabetes: Steatohepatitis is the medical term for
an enlarged, fatty liver. The condition is usually caused by alcoholism,
but can also be the result of malnutrition, obesity and diabetes.
Nonalcoholic steatohepatitis (liver inflammation that can be caused
by fatty liver)
Inherited metabolic diseases (e. g. hemochromatosis,
Wilson disease, Galactosemia)
Chronic bile duct diseases (e. g. primary biliary
cirrhosis) When small tubes that help you digest food become blocked,
your body mistakenly turns on itself and reacts against these bile
tubes. Gallstones often block tubes and cause this type of cirrhosis.
The disease usually affects women aged 35-60 years
Chronic congestive heart failure Your heart is a
pump that pushes blood throughout your body. When your heart doesn't
pump well, blood "backs up" into the liver. This congestion
causes damage to your liver. It may become swollen and painful. Later
it becomes hard and less painful. The cause of the heart failure may
be from heart valve problems, smoking, or infection of the heart muscle
or the sac around the heart
Parasitic infections (e. g. schistosomiasis)
Long term exposure to toxins or drugs.
Alpha-1-antitrypsin Deficiency: This is a hereditary
disorder that prevents the body from properly utilizing the alpha-1-antitrypsin
protein. In some cases, alpha-1-antitrypsin builds up in the liver,
where excess amounts can lead to tissue scarring.
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Liver Cirrhosis is a disease of this noble organ, which is not easily
understood by the lay person. This disease comprises the destruction (necrosis)
of the individual liver cells and its substitution for scar tissue, a
process which is irreversible. Often this is consequence of chronic alcohol
abuse, but may also be the result of previous infection with hepatitis
B,C,D . Specially hepatitis C which can develop in chronic viral hepatitis
is frequently related to liver cirrhosis.
Other causes of liver cirrhosis are diseases of metabolic origin. Metabolic
defects may cause the deposition of cirrhosis inducing substances into
the liver cells. Wilson´s disease as well as Galactosemia are such entities.
In Wilson´s disease, a specific enzyme deficiency causes the deposition
of copper particles in liver tissue. In Galactosemia , the deficiency
of the enzyme Galactose 1 Uridiltransferase , necessary for metabolism
of galactose, is responsible for the abnormal deposition of galactose
in the tissues, leading to mental retardation, cataracts and liver cirrhosis.Liver
injury that results in cirrhosis also may be caused by a number of inherited
diseases such as cystic fibrosis, alpha-1 antitrypsin deficiency, hemochromatosis
and glycogen storage diseases others causes of liver cirrhosis are prolonged
exposure to environmental toxins, and repeated bouts of heart failure
with liver congestion.
Prescription Drug abuse or even the prolonged or simultaneous ingestion
of some Over the Counter remedies, may cause liver cirrhosis. Special
attention should be given to the abuse of acetaminophen and paracetamol,
which is widespread here in El Salvador as well as in many other countries,
and is being self-medicated for a diverse variety of symptoms including
"hangovers", since liver cirrhosis may be a consequence of this abuse.
Specially the ingestion of acetaminophen during "hangovers" should be
avoided since the alcohol toxicity and the acetaminophen liver toxicity
are a dangerous combination for the liver. In normal persons large doses
of acetaminophen are needed to damage liver cells while patients with
chronic alcoholism may suffer massive liver damage when exposed to small
therapeutic doses of this drug. For this reason acetaminophen should be
avoided in chronic alcoholic patients.
What Are the Symptoms of Cirrhosis?
People with cirrhosis often have few symptoms at first. At the beginning
The person may experience fatigue, weakness, and exhaustion. Loss of appetite
is usual, often with nausea and weight loss. As liver function declines,
less protein is made by the organ. For example, less of the protein albumin
is made, which results in water accumulating in the legs (edema) or abdomen
(ascites). A decrease in proteins needed for blood clotting makes it easy
for the person to bruise or to bleed.
With respect to the clinical signs and symptoms of liver cirrhosis In
the later stages this entity may present with uncharacteristic clinical
features over a long period of time and go undiagnosed over many years.
Symptoms and observable symptoms may appear late in the disease and may
include loss of libido, nausea, anorexia, vomiting, ocular or total body
jaundice, itching, reddening (erythema) of the palms, spider naevi on
the chest, gynaecomastia, abdominal swelling, hepatomegaly, splenomegaly,
pubic and axillary hair loss, swelling of ankles, abdominal pain, or mores
severe manifestations such as encephalopathy, upper G.I. Tract bleeding
due to esophageal varices or gastric ulcers or patient may even fall into
a comatose state. Due to this diversity of manifestations, special classifications
exist that classify a patient according to his or hers clinical features,
which range from an asymptomatic state to an advanced life threatening
condition.
The definite diagnosis of liver cirrhosis can only be obtained through
liver biopsy. Other diagnostic methods such as ultrasound, CT Scan, MRI
can detect certain morphologic abnormalities associated with cirrhosis,
although these changes are usually seen in advanced disease only.
Liver cirrhosis in its initial stages can most often not be diagnosed
by diagnostic imaging methods. Endoscopy is useful to document the presence
of esophageal varices, as well as gastric or duodenal ulcers, often associated
with cirrhosis.
What Are the Treatments for Cirrhosis?
As Liver cirrhoses is not curable the Treatment of cirrhosis is aimed
at stopping or delaying its progress, minimizing the damage to liver cells,
and reducing complications.
The major goals of treating the cirrhotic patient include:
1. Slowing or reversing the progression of liver disease.
2. Preventing superimposed insults to the liver.
3. Preventing and treating the complications
4. Determining the appropriateness and optimal timing for liver transplantation
Slowing or reversing the progression of liver disease Although
cirrhosis is generally considered to be irreversible in its advanced stages,
the exact point at which it becomes irreversible is unclear . Some chronic
liver diseases respond to treatment even when the liver disease has progressed
to cirrhosis. Thus, specific therapies directed against the underlying
cause of the cirrhosis should be instituted. As examples:
The 10-year survival rate in patients with cirrhosis from autoimmune hepatitis
who are treated with steroids or immunosuppressive agents approaches 90
percent. This number is similar to survival rates of treated patients
with autoimmune hepatitis who do not have cirrhosis.
Abstinence from alcohol improves survival in alcoholic cirrhosis.
Interferon therapy slows the progression of cirrhosis in patients with
chronic hepatitis C virus infection, and may also decrease fibrosis and
the risk of hepatocellular carcinoma
Treatment of patients with chronic hepatitis B with lamivudine may result
in significant improvement in liver function and histology.
Cirrhosis of the liver is irreversible but treatment of the underlying
liver disease may slow or stop the progression. Such treatment depends
upon the underlying etiology. Termination of alcohol intake will stop
the progression in alcoholic cirrhosis and for this reason, it is important
to make the diagnosis early in a chronic alcohol abuser.
Risk Factors for Developing Cirrhosis
from Hepatitis C. |
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Between 20% and 30% of people with Hepatitis C
develop cirrhosis after twenty years. (It should be noted that even
in patients with cirrhosis, survival rates in one study were nearly
80% at 10 years in these patients.) The following conditions put
people with hepatitis C at higher risk for liver damage:
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