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 LIVER DISEASES
There are over 100 types of liver diseases, and because of more than 200 functions that the Liver plays, many can be life-threatening unless treated.
The Liver diseases have been listed down in alphabetical order. Click on the alphabet to read more about the diseases.
 
 A B C D E F G H I J K L M
N O P Q R S T U V W X Y Z
 
Autoimmune Hepatitis Alcoholic liver disease
Alagille Syndrome

Alpha-1-Antitrypsin Deficiency

 

Autoimmune Hepatitis

Disease condition

It is a disease in which the body's immune system attacks liver cells. This causes the liver to become inflamed (hepatitis). About 70 percent of those with autoimmune hepatitis are women, most between the ages of 15 and 40. The disease may start at any age, but is most common in adolescence or early adulthood.

Autoimmune hepatitis or autoimmune chronic hepatitis is a progressive inflammation of the liver that has been identified by a number of different names, including autoimmune chronic active hepatitis (CAH), idiopathic chronic active hepatitis, and lupoid hepatitis.

Autoimmune hepatitis was first described in 1950 as a disease of young women, associated with increased gamma globulin in the blood and chronic hepatitis on liver biopsy. The presence of antinuclear antibodies (ANA) and the resemblance of some symptoms to "systemic lupus erythematosus" (SLE) led to the label "lupoid hepatitis." It later became evident that this disease was not related to SLE. The disease is now called autoimmune hepatitis.

The disease is usually quite serious and, if not treated, gets worse over time. It's usually chronic, meaning it can last for years, and can lead to cirrhosis (scarring and hardening) of the liver and eventually liver failure.

Autoimmune hepatitis is classified as either type I or II. It occurs at any age and is more common among women than men. About half of those with type I have other autoimmune disorders, such as type 1 diabetes, proliferative glomerulonephritis, thyroiditis, Graves' disease, Sjögren's syndrome, autoimmune anemia, and ulcerative colitis. Type II autoimmune hepatitis is less common, typically affecting girls ages 2 to 14, although adults can have it too.

Disease Etiology

The reason for this inflammation is not certain, but it is associated with an abnormality of the body's immune system and is often related to the production of antibodies that can be detected by blood tests.

Researchers think a genetic factor may predispose some people to autoimmune diseases.

Symptoms

The most common symptoms of autoimmune hepatitis are fatigue, abdominal discomfort, aching joints, itching, jaundice, enlarged liver, and spider angiomas (tumors) on the skin. Patients may also have complications of more advanced chronic hepatitis with cirrhosis, such as ascites (abdominal fluid) or mental confusion called encephalopathy.


Diagnosis

Blood tests identify antinuclear antibodies (ANA) or smooth muscle antibodies (SMA) in the majority of patients (60 percent). More than 80 percent of affected individuals have increased gamma globulin in the blood. Some patients have other autoimmune disorders such as thyroiditis, ulcerative colitis, diabetes mellitus, vitiligo (patchy loss of skin pigmentation), or Sjogren's syndrome (a syndrome that causes dry eyes and dry mouth).

A liver biopsy is important to confirm the diagnosis and provide a prognosis. Liver biopsy may show mild chronic active hepatitis, more advanced chronic active hepatitis with scarring (fibrosis), or a fully developed cirrhosis.

Treatment – Medical, Surgical

The treatment of autoimmune hepatitis is immunosuppression with prednisone alone or prednisone and azathioprine. This medical therapy has been shown to decrease symptoms, improve liver tests, and prolong survival in the majority of patients. Therapy is usually begun with prednisone 30 to 40 mg per day and then this dosage is reduced after a response is achieved.

The standard dosage used in the majority of patients is prednisone 10-15 mg per day, either alone or with azathioprine 50 mg per day. Higher doses of prednisone given long-term are associated with an increase in serious side effects including hypertension, diabetes, peptic ulcer, bone thinning, and cataracts. Lower doses of prednisone may be used when combined with azathioprine.

The goal of treatment of autoimmune hepatitis is to cure or control the disease. In two thirds to three quarters of the patients, liver tests fall to within the normal range. Long-term follow-up studies show that autoimmune hepatitis appears more often to be a controllable rather than a curable disease, because the majority of patients relapse within six months after therapy is ended. Therefore, most patients need long-term maintenance therapy.

Not all patients with autoimmune hepatitis respond to prednisone treatment. Approximately 15-20 percent of patients with severe disease continue to deteriorate despite initiation of appropriate therapy. This is most common in patients with advanced cirrhosis on initial liver biopsy. Such patients are unlikely to respond to further medical therapy, and liver transplantation should be considered.

Life expectancy and Quality of Life

The 10-year survival rate in untreated patients is approximately 10 percent.

 

Alcoholic liver disease

This is the commonest liver disease in India. It is caused by excessive consumption of alcohol generally beyond safe dose. It is a preventable disease. The three primary types of alcohol-induced liver injuries are fatty liver, alcoholic hepatitis and liver cirrhosis.

Fatty liver

This is the commonest alcohol induced liver injury. Fatty liver is excessive accumulation of fat inside the liver cells. The liver is enlarged, causing upper abdominal discomfort on the right side. Liver tests are generally normal. This can be observed on ultrasonography of abdomen

Alcoholic hepatitis

Alcoholic hepatitis is an inflammation of the liver, accompanied by the destruction of individual liver cells and scarring. Symptoms may include fever, jaundice, and enlarged, tender liver and spider-like veins in the skin. If consumption is not controlled it leads to cirrhosis which is end-stage liver disease. Severe alcoholic is associated with significant mortality.

Alcoholic cirrhosis

Alcoholic cirrhosis is the permanent destruction of normal liver tissue, leaving non-functioning scar tissue. The severity of liver disease depends on duration and quantity of alcohol the patient consumes. Pain in right upper side of abdomen, fever, yellowing of the skin and eyes, spider-like veins in the skin are common features of alcohol related liver damage.

Some other serious symptoms may include hemetemesis i.e. vomiting of blood along with passage of tarry black stools; lump or heaviness in left upper side of abdomen because of enlarged spleen, ascites i.e. fluid build-up in the abdominal cavity leading to abdominal distension along with swelling on feet, kidney failure and in more severe situation confusion, drowsiness, stupor, coma can be seen.

Consumption of alcohol also accelerates preexisting damage due to other causes like hepatitis C infection.

Diagnosis

A complete medical history and physical examination are required along with few other tests.

Liver function tests include a series of special blood tests that can determine liver function. Abdominal sonography is useful to detect changes in liver, spleen, fluid in abdomen. Gastroscopy is an endoscopic procedure to look for enlarged veins in esophagus and stomach due liver damage and it also helps to treat in the form of injection or rubber-band application on bleeding vessels.

If patient has never bled, some medicines can be given to reduce the pressure and there by prevent the bleeding from these enlarged vessels. This procedure is life saving when there is severe hemetemesis i.e. passage of blood in vomiting or in stool due to bleeding from these esophageal vessels.

Treatment

Complete abstinence from alcohol.
Adequate nutrition including anti-oxidants to reduce liver damage.

Diuretics, medicines to reduce the fluid in abdomen by increasing the urine output.

Pentoxiphylline, another drug to reduce effects on to kidneys in patients with alcoholic hepatitis.

Pentoxiphylline, another drug to reduce effects on to kidneys in patients with alcoholic hepatitis.

Endoscopic intervention for bloody vomiting.

Drugs to prevent bleeding from esophageal vessels.

Differentiation between alcoholic hepatitis and alcoholic cirrhosis

HOW TO DIFFERENTIATE ALCOHOLIC HEPATITIS FROM ALCOHOLIC CIRRHOSIS AND IS THE DIFFERENTIATION IMPORTANT?

The association of alcohol abuse and liver damage is known since the times of ancient Greeks and is also recognised in Ayurveda. The clinical spectrum of alcoholic liver injury varies from asymptomatic hepatomegaly to profound hepatocellular failure with portal hypertension. The clinical picture tends to be more florid in individuals with more advanced liver injury. Alcoholic liver injury appears to progress from fatty changes through alcoholic hepatitis to cirrhosis. Majority of the individuals who abuse alcohol will develop fatty changes in their liver at some stage of their drinking career. However only 20% of such individuals will develop cirrhosis. The apparent predisposition of certain people to develop alcoholic cirrhosis is unknown. Fatty liver, though indicating a profound metabolic disturbance within the liver, is not necessarily harmful. Certainly, cirrhosis may develop in an alcoholic who has never had fatty change and isolated fatty change has not been shown to proceed directly to cirrhosis. Alcoholic hepatitis develops in only a proportion of drinkers even after decades of abuse and is assumed to be a precirrhotic lesion, although its natural history is not well understood. Thus in approximately 50% of individuals alcoholic hepatitis may persist for several years and in 10% of individuals the lesion may heal despite continued alcohol abuse. It has therefore been suggested that although alcoholic hepatitis may contribute, when present, to the evolution towards cirrhosis, it is not a sine qua non of such progression. Though most of the alcoholics may have a combination of alcoholic hepatitis and cirrhosis on biopsy and more or less similar clinical and biochemical features, there are certain features which may help in differentiating the two conditions as given in the Table 1 below.

Since alcoholic hepatitis is reversible and hepatic function improves over a period of time with abstinence, management consists predominantly of abstinence from alcohol and supportive care; whereas alcoholic cirrhosis once established is irreversible and hepatic function may not improve over time, management consists of abstinence from alcohol, treatment of complications and liver transplantation may be a viable option in carefully selected patients. Liver transplantation should not be done in patients with pure alcoholic hepatitis. Hence it is very essential to differentiate a patient having alcoholic from the one having alcoholic cirrhosis as the management and prognosis is different.

Differentiation between alcoholic hepatitis and alcoholic cirrhosis
Alcoholic hepatitis Alcoholic cirrhosis
Symptomatology

Symptomatology
Patients with alcoholic hepatitis have been abusing alcohol till the time of presentation; they look more ill and being symptomatic present to a physician. Patients with alcoholic cirrhosis may not have abused alcohol for many years prior to presentation; most of them are well compensated, with only one-third being symptomatic.
Jaundice is usually one of the most common symptom. Some common mode of presentations are: jaundice - 50% of the patients, ascites in 30 - 60% and splenomegaly - 15% of the patients. Ascites is usually the common symptom. Some common mode of presentations are: 40%, dilated abdominal wall veins - 60% and splenomegaly - 25% of the patients.
Fever (even high grade) is seen in upto 50% of the subjects. High grade fever is not seen (unless there is superadded infection).
Symptoms of variceal bleeding and hepatic encephalopathy are uncommon. Variceal bleeding and hepatic encephalopathy are quite common.
Clinical Signs Clinical Signs
Spider naevi and palmar erythema may be florid.8 Spider naevi and palmar erythema though seen, may not be florid.
Features of portal hypertension - Ascites, dilated abdominal wall veins, splenomegaly and oesophageal varices are not a prominent features of pure alcoholic hepatitis. Features of portal hypertension are a prominent feature of alcoholic cirrhosis
Liver is very large and tender on palpation; its surface is smooth and consistency is soft to firm. Liver is mild to moderately enlarged or may not be palpable in advance cirrhosis and when palpable it is nontender, irregular with palpable nodules and firm in consistency.
Arterial bruit may be heard over the liver area. Unless there is superadded hepatocellular Unless there is superadded hepatocellular
Investigations Investigations
Polymorphonuclear leucocytosis (upto) 20,000/mm3) is quite common. Polymorphonuclear leucocytosis though seen may not be as high as in alcoholic hepatitis.
Platelet function is depressed, but there may not be thrombocytopenia. There is no evidence of hypersplenism. Both platelet function and number are reduced and there is evidence of hypersplenism.
SGOT and SGPT are elevated upto 300 to 400 IU with SGOT/SGPT ratio > 2. SGOT and SGPT are usually normal.
Highest levels of rise of gamma glutamyl transpeptidase, glutamate dehydrogenase and tumour necrosis factor are seen in alcoholic hepatitis. There is mild to moderate rise of gamma glutamyl transpeptidase and glutamate dehydrogenase in alcoholic cirrhosis.
Most of the elevated enzymes fall back to normal level within 1 week of abstinence. No significant fall in enzyme levels are seen over a period of time, even if abstinent.
Isotope liver scan may show total absence of radiotracer uptake by the hepatic parenchyma ("Medical hepatectomy") with avid uptake by the spleen and the bone marrow of vertebrae and the ribs. After a period of recovery, the liver scan may show normal tracer uptake. Isotope liver scan show inhomogeneous tracer distribution in the liver, with left lobe uptake greater than the right lobe, colloid shift to the spleen ("Hot spleen") and visualisation of the bone marrow of the vertebrae. Liver scan picture does not show improvement over time.
Liver biopsy histology Liver biopsy histology
Three obligatory features for the histologic diagnosis are - On liver biopsy the following features are seen-
ballooning degeneration of hepatocytes, with areas of necrosis.
parenchymal necrosis
inflammatory cell infiltrates, predominantly
regeneration
 
scarring
polymorphonuclear leucocytes  
fibrosis, both pericellular (producing a lattice-like or chicken wire appearance) and perivenular (centrolobular).  
20% of alcoholics show features of hepatitis on 18.3% of alcoholics show features of cirrhosis (6.7%) or in combination with cirrhosis (13.4%).8 Alcoholic hepatitis rarely is seen as an isolated pathology on liver biopsy. On most occasions it is seen in combination with either fatty liver or cirrhosis. 18.3% of alcoholics show features of cirrhosis on biopsy; 5% as only cirrhosis and 13.4% in combination with alcoholic hepatitis. Thus alcoholic cirrhosis may be the only pathology alcoholic cirrhosis may be the only pathology
Management Management
Alcoholic hepatitis is usually reversible on Alcoholic cirrhosis is generally considered to Alcoholic cirrhosis is generally considered to be an irreversible lesion once it is established
Treatment consists of abstinence and proper nutritional support. Liver transplantation is not recommended at this stage. Apart from abstinence and treatment of complications, liver transplantation may be a viable option.
Prognosis Prognosis
Though high initial in hospital mortality of about 50%, long term prognosis of those who abstain from alcohol is very good. Though initial in hospital mortality may not be high (but depends on the mode of decompensation), long term prognosis is presentation and degree of hepatic dismal with nearly 50% 5 year mortality
The Maddrey discrimination function is a simple equation in which the serum bilirubin and prothrombin time are used to indicate the presence of severe alcoholic hepatitis (4.6 x (PT in secs - control time) + serum bilirubin in mg/dl). A score greater than 32 indicates severe alcoholic hepatitis with poor prognosis. Child-Pugh score is used to determine the prognosis using the following parameters - ascites, encephalopathy, bilirubin, albumin and prothrombin time. Child class A has the best prognosis and child class C has the worst

Summary

Alcoholic hepatitis Alcoholic cirrhosis

Acute debauch, continued alcohol consumption

Probably a past drinker

Ill patient (may be febrile)

Relatively well preserved (unless severely decompensated)

Presents usually with jaundice which may be deep (Cholestatic!)

Presents usually with ascites or GI bleeding

Tender, large and smooth hepatomegaly

Non tender, firm, irregular and nodular liver

Florid spider angioma and palmar erythema Spider angioma and palmar erythema present

No major signs of portal hypertension

Signs of portal hypertension present

Arterial bruit over the liver

Arterial bruit over liver only with hepatoma

Polymorphonuclear leucocytosis

Minimal polymorphonuclear leucocytosis

GGT, SGOT and SGPT elevated - usually upto 300 IU/L

Liver enzymes usually in normal range

Gold standard for diagnosis is liver biopsy

Gold standard for diagnosis is liver biopsy

Poor prognosis for Maddrey’s score > 32. Poor prognosis for Child class C.



Alagille syndrome

Disease condition

Alagille syndrome is an inherited disorder that mimics other forms of prolonged liver disease seen in infants and young children. However, a group of unusual features in other organ systems distinguishes Alagille syndrome from other liver and bile duct diseases in infants.

Children with Alagille Syndrome usually have a liver disease characterized by a progressive loss of the bile ducts within the liver over the first year of life and narrowing of bile ducts outside the liver. This leads to a buildup of bile in the liver, causing damage to liver cells. Scarring may occur and lead to cirrhosis in about 30 to 50 percent of affected children.

Although Alagille Syndrome was first described in the English medical literature in 1975, it is now becoming recognized more frequently among children with chronic forms of liver disease.

Disease Etiology

Genetics

Alagille Syndrome is generally inherited only from one parent and there is a 50 percent chance that each child will develop the syndrome. Each affected adult or child may have all or only a few of the features of the syndrome. Frequently a parent or brother or sister of the affected child will share the facial appearance, heart murmur or butterfly vertebrae, but have a completely normal liver and bile ducts.

Symptoms

Symptoms of the illness are jaundice, pale, loose stools and poor growth within the first three months of life. Later there is persistent jaundice, itching, fatty deposits in the skin and stunted growth and development during early childhood. Frequently, the disease stabilizes between ages 4 and 10 with an improvement in symptoms.

Other features which help establish the diagnosis include abnormalities in the cardiovascular system, the spinal column, the eye and the kidneys. Narrowing of the blood vessel connecting the heart to the lungs (pulmonary artery) leads to extra heart sounds but rarely to problems in heart function. The shape of the bones of the spinal column may look like a butterfly's wings on X-ray but almost never cause problems with function of the nerves in the spinal cord.

More than 90 percent of children with Alagille Syndrome have an unusual abnormality of the eyes. An extra, circular line on the surface of the eye requires specialized eye examination to detect and does not lead to any disturbances in vision. In addition, some children have various abnormalities in their kidneys that may lead to minor changes in kidney function.

Many physicians believe that there is a specific facial appearance shared by most of the children with Alagille Syndrome that makes them easily recognizable. The features include a prominent, broad forehead, deep-set eyes, a straight nose and a small pointed chin.

Diagnosis

Diagnosis can be established by microscopic examination of liver biopsy specimens, a stethoscope examination of the child's heart and chest, a slip-lamp eye examination, an X-ray of the spinal column and an ultrasound (sonogram) examination of the abdomen.


Treatment:

Medical

Treatment of Alagille Syndrome is based on trying to increase the flow of bile from the liver, maintain normal growth and development and prevent or correct any of the specific nutritional deficiencies that often develop.

Because bile flow from the liver to the intestine is slow in Alagille Syndrome, medications designed to increase the flow of bile are frequently prescribed, including Phenobarbital and Cholestyramine or Colistipol. This may decrease the damage in the liver and improve the digestion of fat in foods that are eaten.

Also, itching caused by the buildup of bile in the blood and skin may be relieved. Other drugs are also used to relieve itching, like Diphenhydramine hydrochloride or Hydroxyzine.

Elevations in blood cholesterol also respond to the medications used to increase bile flow. Elevated blood cholesterol levels can lead to small yellow deposits of cholesterol on the skin of knees, elbows, palms, eyelids and other surfaces that are frequently rubbed. Lowering blood cholesterol usually causes the cholesterol skin deposits to improve; although these are unsightly, they are almost never associated with any dangerous symptoms.

Although reduced flow of bile into the intestine leads to poor digestion of dietary fat, a specific type of fat can still be well digested and therefore infant formulas containing high levels of medium-chain triglycerides (MCT) are usually substituted for conventional formulas.

Problems with fat digestion and absorption may lead to deficiency of fat-soluble vitamins--A, D, E and K. Vitamin A deficiency causes night blindness and red eyes. Vitamin D deficiency causes softening and fractures of the bones and teeth (rickets). Vitamin E deficiency causes a disabling disease of the nervous system and muscles, and vitamin K deficiency causes bleeding problems. Deficiencies of these vitamins can be diagnosed by blood tests and usually can be corrected by large oral doses. If the child's system cannot absorb vitamins given by mouth, vitamin injections into the muscle are necessary.

Surgical

Sometimes surgery is necessary during infancy to help establish the diagnosis of Alagille Syndrome by direct examination of the bile duct system. However, surgical reconstruction of the bile duct system is not recommended because bile can still flow from the liver and there is presently no procedure that can correct for the loss of the bile ducts within the liver. Occasionally liver cirrhosis advances to a stage where the liver fails to perform its functions. Liver transplantation is then considered.


Life expectancy and Quality of Life

The overall life expectancy for children with Alagille Syndrome is unknown, but depends on several factors: the severity of scarring in the liver, whether heart or lung problems develop because of the narrowing in the pulmonary artery, and the presence of infections or other problems related to poor nutrition. Many adults with Alagille Syndrome lead normal lives.

Treatment is primarily medical and not surgical. Patients generally have a much better outcome than children with some of the other liver diseases that may present at the same age.

 

Alpha-1-Antitrypsin Deficiency

Disease condition

Alpha-1 - antitrypsin deficiency is a hereditary disease that may lead to hepatitis and cirrhosis. It is the most common genetic cause of liver disease in children. Adults are also affected and may have lung involvement with emphysema as well as liver disease. The protein alpha-1 - antitrypsin is a substance made in the liver. It plays an important role preventing the breakdown of enzymes in various organs of the body.

Disease Etiology

A child must inherit the tendency from both parents to develop the disease, alpha-1 -antitrypsin deficiency. The incidence of the disease in the United States is approximately 1:2000 live births. Fortunately, for reasons that are not understood, only 10-20 percent of the babies born with the deficiency will have liver disease. Decreased levels of the serum protein, alpha-1 - antitrypsin, lead to liver damage with scarring and abnormal liver function.

Symptoms

The disease most often appears in the newborn period with jaundice, swelling of the abdomen, and poor feeding. It may also appear in late childhood or adulthood and be detected because of fatigue, poor appetite, swelling of the abdomen and legs or abnormal liver tests.

Patients who develop cirrhosis (scarring of the liver) have changes in blood flow through the liver which produce other complications: nosebleeds, bruising, excess body fluid, enlarged veins in the inside of the stomach and esophagus (varices). Occasionally, increases in pressure in these veins make them leak, and internal bleeding may result. Increased sleepiness after eating protein (due to increased blood ammonia levels) and increased risk of infection may be late complications.

Diagnosis

The diagnosis is made by blood tests when the serum level of alpha-1 - antitrypsin is low and standard liver function tests are abnormal. Other tests such as urine collection, ultrasound examination, or tests using specialized X-ray techniques may be necessary. A biopsy of the liver (sampling liver tissue with a needle or by operation) is usually performed to look for liver injury. Relatives who are carriers but do not have the disease can also be diagnosed by blood tests.

The Diagnosis of Alpha 1 - Antitrypsin Deficiency is also carried out by using Arterial blood gases.
Blood gases measure the pH (acidity), oxygen content, and carbon dioxide content of the blood. Usually, blood gases are used to analyze the arterial blood. In rarer cases, venous blood may be used.
The test is performed by collecting a sample of blood from an artery. Using a small needle, the sample may be collected from the radial artery in the wrist, the femoral artery in the groin, or the brachial artery in the arm.
Before blood is drawn, the circulation to the hand may be tested (if the wrist is the site). After the blood is drawn, pressure is applied to the puncture site for at least 5 minutes to completely stop the bleeding.
The test must be sent to the laboratory for analysis immediately, or the accuracy of the results cannot be guaranteed.

Treatment – Medical, Surgical

Currently, there is no cure for this disease. However, certain abnormalities can be treated or controlled. Treatment is designed to maintain normal nutrition, to provide the liver and the body with essential nutrients, and to identify complications early in order to treat them better. Multiple vitamins and vitamins E, D and K are often given.
When jaundice is severe or itching appears, phenobarbital or cholestyramine may be used. If the disease progresses, excess body fluid may occur and can be treated with diuretics.

Liver transplantation can be done when liver failure develops and interferes with normal functioning at school, work or in the home.

Life expectancy and Quality of Life

The long-term outcome of the disease is variable. Approximately 25 percent of affected patients develop cirrhosis and its complications, but 75 percent of individuals will not have any significant liver disease after the newborn period. Some patients with cirrhosis lead relatively normal lives for relatively long periods of time. The reason for this difference is not known.

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