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Friday, September 17, 2010

A Family Doctor's Tale - CIRRHOSIS

DOC I HAVE LIVER CIRRHOSIS

Most cases of Liver cirrhosis is related to alcoholism.
Rarely is it due to Hepatitis B and C viruses. Sometimes it is caused by toxic drugs or traditional medications.The liver is usually damaged with high liver enzymes. Once damaged there is no cure for liver cirrhosis except for liver transplant. Most cases of liver cirrhosis are treated at the hospital.

Liver Cirrhosis is a chronic liver disease where the normal liver tissue is replaced by bands of fibrous scar tissue separating nodules of regenerated liver cells resulting in gradual loss of liver function.


The main causes of Liver Cirrhosis are:

1.chronic alcoholism:

Alcohol tends to block the normal metabolism of protein, fats, and carbohydrates leaving toxic material behind which can injure the liver and ends in liver cirrhosis.


2.hepatitis C virus infection:

Chronic hepatitis c viral infection causes inflammation of the liver which over the years can  damage the liver and lead to cirrhosis.


3.Primary biliary and secondary cirrhosis:

Primary biliary cirrhosis cause is unknown.
Genetic causes of Biliary Obstruction such as biliary atresia


Secondary biliary cirrhosis results from chronic obstruction of the biliary tract resulting in chronic inflammation of the liver cells and fibrosis of the liver.
Nodular regeneration of the liver cells occurs subsequently.
Obstruction to the bile ducts can be caused by:
Cholecystitis
Gallstones
Biliary cholangitis


4.Obese -fatty liver can end in liver cirrhosis


5.Others:
Exposure to chemicals and medications


Metabolic diseases:
 Hemachromatosis
 Wilson Disease


Parasitic Disease:
 Schistosomiasis


Cardiac cirrhosis
 Liver congestion results from right sided heart failure


Autoimmune Diseases:
 Autoimmune hepatitis


Glycogen storage disease


Common symptoms of Liver Cirrhosis are:

1.Anorexia


2.fatigue and weakness


3.Gradual onset of jaundice(Yellow discoloring of the skin, eye, and mucus membranes due to increased bilirubin)


4.weight loss.


5.Fetor hepaticus -  odor in breath due to increased dimethyl sulfide


Signs:

1.Spider nevi- central arteriole surrounded by many smaller vessels due to an increase in estradiol .


2.Palmar erythema -redness of the palms due to impaired sex hormone metabolism.


3.Liver size. Can be enlarged, normal, or shrunken.


4.Splenomegaly  - Due to congestion of the spleen as a result of portal hypertension.


5.Ascites . Accumulation of fluid in the peritoneal cavity


6.hydrocele and penile flomation (swelling of the penile shaft)


7.Hypogonadism - impotence, infertility, poor sexual drive, and testicular atrophy due to primary gonadal injury or suppression of hypothalamic or pituitary function.


8.Gynecomastia - This is due to increased estradiol in male patients.


9.Hypertrophic osteoarthropathy - Chronic proliferative periostitis of the ribs can be very painful.


10.Dupuytren's contracture -palmar fascia tightens to cause flexion deformities of the fingers.


11.Nail changes:

Muehrcke's nails - paired horizontal bands  due to hypoalbuminemia 
Terry's nails    - proximal 2/3 of the nail  appears white with distal one-third red due to hypoalbuminemia
Clubbing         - angle between the nail plate and proximal nail fold - 180 degrees


12.Caput medusa -In portal hypertension, the umbilical vein may be distended.


13.Cruveilhier-Baumgarten murmur. Venous blood flow hum heard in epigastric region due to portal hypertension


14.Asterixis - Bilateral asynchronous flapping of outstretched, dorsiflexed hands seen in patients with hepatic encephalopathy.


Diagnosis of Liver Cirrhosis:
1. Liver biopsy will confirm liver cirrhosis through a percutaneous  laparoscopic , or fine-needle approach but may not necessary if the clinical, laboratory, and radiologic data suggests cirrhosis.

There is a small but significant risk to liver biopsy, and cirrhosis itself predisposes for complications due to liver biopsy


2.Laboratory findings
The following findings are typical in cirrhosis:

a.Aminotransferases  - AST and ALT are moderately elevated, with AST and ALT.
b.Alkaline phosphatase  - usually slightly elevated.
c.GGT  - correlates with AP levels- much higher in chronic liver disease from alcohol.
d.Bilirubin  - may elevate as cirrhosis progresses.
e.Albumin  - levels fall as the synthetic function of the liver declines with worsening cirrhosis
f.Prothrombin time  - increases since the liver synthesizes clotting factors.
g.Globulins  - increased due to shunting of bacterial antigens away from the liver to lymphoid tissue.
h.Serum sodium is low due to inability to excrete free water resulting from high levels of ADH and aldosterone
i.Thrombocytopenia - due to both congestive splenomegaly as well as decreased thrombopoietin  from the liver. Platelet count is rarely < 50,000/mL.
j.Leukopenia and neutropenia  - due to splenomegaly with splenic margination.
k.Coagulation  defects - the liver produces most of the coagulation factors and thus coagulopathy correlates with worsening liver disease.


l.Serology for hepatitis viruses, autoantibodies  (ANA or Anti-nuclear antibody, anti-smooth muscle, anti-mitochondria antibody, anti-LKM)
n.Ferritin and transferrin saturation  (markers of iron overload),
o.copper and ceruloplasmin:
Ceruloplasmin (markers of copper overload)
p.Immunoglobulin levels (IgG, IgM, IgA) - high

q.Cholesterol and glucose
r.Alpha 1-antitrypsin


3.Imaging
Ultrasound  is used to assess the degree of cirrhosis:

a.small and nodular liver in advanced cirrhosis
b.increased echogenicity with irregular appearing areas.


Ultrasound may also screen for
a.hepatocellular carcinoma
b.portal hypertension
c.Budd-Chiari syndrome by checking the blood flow in the hepatic vein


FibroScan (transient elastography) uses elastic waves to assess liver stiffness which can grade the severity of cirrhosis.


Abdominal CT and liverand bile duct MRI - may show the the degree of liver cirrhosis


4.Imaging of the bile ducts, such as
ERCP Endoscopic_retrograde_cholangiopancreatography or
MRCP Magnetic_resonance_cholangiopancreatography (MRI of biliary tract and pancreas)
can show abnormalities in the liver


5.Endoscopy
Gastroscopy is performed in patients with liver cirrhosis to exclude the possibility of esophageal varices.


Complications may develop with progression of the cirrhosis.

1.Bruising  and bleeding  due to decreased production of coagulation  factors.


2.Jaundice due to decreased processing of bilirubin


3.Itching  (pruritus due to bile products deposited in the skin).


4.Hepatic encephalopathy  - the liver does not clear ammonia  and related nitrogenous substances from the blood, which are carried to the brain, affecting cerebral functioning:
 a.neglect of personal appearance,
 b.unresponsiveness,
 c.forgetfulness,
 d.trouble concentrating,
 e.changes in sleep habits.
 f.Sensitivity to medication due to decreased metabolism of the active compounds.


5.Hepatocellular carcinoma is primary liver cancer one of the common complication of cirrhosis and has a high mortality rate.


6.Portal hypertension  - blood normally carried from the intestines and spleen through the hepatic portal vein  flows more slowly and the pressure increases causing :


a.Ascites - fluid leaks through the vasculature into the abdominal cavity.
b.Esophageal varices  - collateral portal blood flow through vessels in the stomach and esophagus. These blood vessels may become enlarged and are more likely to burst.


7.Immune system dysfunction, leading to

a.nonspecific infection in the body organs
b..Spontaneous bacterial peritonitis -fluid in the abdomen may become infected with bacteria normally present in the intestines 


8.Hepatorenal syndrome  - insufficient blood supply to the kidneys, causing acute renal failure and a very high mortality (over 50%).


9.Hepatopulmonary syndrome  - blood bypassing the normal lung circulation  leading to cyanosis  and dyspnea  (shortness of breath) characteristically worse on sitting up


10.Portopulmonary hypertension  - increased blood pressure over the lungs as a consequence of portal hypertension.



In all cases of liver cirrhosis,  the liver damage from cirrhosis cannot be repaired so treatment is aimed at reducing progression and complications of the disease.

1. A healthy fat free diet is needed as energy use in cirrhosis is high


2.Close monitoring of the liver function is important.


3.Antibiotics will be prescribed for infections,


4.Antihistamines can help with itching.


5.Laxatives such as lactulose decrease risk of constipation


6.Treating alcoholism
Alcoholic cirrhosis caused by alcoholism is treated by abstaining from alcohol.


7.Treatment for hepatitis-related cirrhosis involves medications to treat the different types of hepatitis, such as

a.interferon for viral hepatitis
b.corticosteroids for autoimmune hepatitis.


8.Cirrhosis caused by Wilson's disease in which copper builds up in organs is treated with chelation therapy  (e.g. penicillamine ) to remove the copper.


9.Preventing complications:


a. Ascites
Salt restriction
Diuretics may be necessary to reduce fluid in abdomen.


b. Esophageal variceal bleeding -
Vasopressin
surgical portacaval shunting


c. portal hypertension
propranolol lower blood pressure over the portal system.
Transjugular intrahepatic portosystemic shunting will relieve pressure on the portal vein as a temporary measure .


c. Spontaneous bacterial peritonitis
antibiotics.


10. Decompensated cirrhosis
Decompensation in previously stable patients may occur due to various causes:


a.constipation
b.infection
c.increased alcohol intake,
d.medication
e.bleeding from esophageal varices
f.dehydration.


Patients with decompensated cirrhosis has to be admitted to hospital for
1.close monitoring of the:

fluid balance
mental status,


2.emphasis on adequate nutrition


3.medical treatment -

a.diuretics
b.antibiotics
c.laxatives
d.enemas
e.thiamine
f.steroids
g.acetylcysteine
h.pentoxifylline


Surgical Liver Transplantation:


Liver transplantation is necessary if:

liver stop functioning
severe complications cannot be controlled


Survival rate from liver transplantation has improved to 80% for five year postoperative period.


Transplantation necessitates the use of immune suppressants for life.


Prognosis is generally not good unless detected at very early stage.


Most liver cirrhosis patient are in the moderate to advanced stage of the disease when detected.


If there are complications, the prognosis is poor.


Prevention:
1. Alcohol- stop the abuse of alcohol


2. Vaccination against hepatitis B


3. Reduce fat in obese -fat cells in liver can lead to cirrhosis

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