AtoZ Diseases

What is this condition?

Cirrhosis is a chronic liver disease marked by the widespread destruction of liver cells and their replacement by fibrous cells. This disease alters the liver’s structure and its blood vessels. The fibrous cells interfere with blood and lymph flow and ultimately cause liver failure.

Cirrhosis is twice as common in men as in women and is especially prevalent among malnourished, chronic alcoholics over age 50. The death rate is high: Many persons die within 5 years of its start. A person with the first signs of cirrhosis will benefit from diet and life­style changes.
What causes it?

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There are many types of cirrhosis, linked to various causes:
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Portal, nutritional, or alcoholic cirrhosis, called Laennec’s cirrhosis, is the most common type. It affects 30% to 50% of people with the disorder, and 90% of them have a history of alcoholism. Liver damage results from malnutrition, a lack of dietary protein, and heavy drinking for a long period.
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Biliary cirrhosis (15% to 20% of cases) is caused by bile duct diseases that suppress bile flow.
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Postnecrotic cirrhosis (10% to 30% of cases) is caused by various types of hepatitis.
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Pigment cirrhosis (5% to 10% of cases) may be caused by disorders such as abnormal iron metabolism.
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In about 10% of people with cirrhosis, there is no known cause.

What are the symptoms of Cirrhosis of The Liver ?

The symptoms are similar for all types, regardless of the cause. At first, symptoms are vague, but the person with cirrhosis usually has stomach troubles, including loss of appetite, indigestion, nausea, vomiting, constipation or diarrhea, and a dull ache in the abdomen. Additional symptoms develop as the liver deteriorates:

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fluid in the lungs, reduced chest expansion, and light-headedness
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lethargy, mental changes, slurred speech, a flapping tremor, neuritis, paranoia, hallucinations, and coma
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nosebleeds, easy bruising, bleeding gums, and anemia
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testicular atrophy and chest-hair loss in men and menstrual irregularities in women
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severe itching, extreme dryness, patches on the skin and, possibly, jaundice
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fluid accumulation in the stomach, swollen legs, and other symptoms of full-fledged cirrhosis
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musty breath odor, loss of muscle from disuse, a fever, stomach pain that worsens when the person sits up or leans forward, bleeding from the throat, and an enlarged liver or spleen.

How is it diagnosed?

The doctor will perform a liver biopsy (obtain a tissue specimen for study) to confirm cirrhosis and may order X-ray scans to check the gallbladder and bile duct for gallstones. To learn more about the extent of the cirrhosis and its complications, the doctor may order lab tests of blood, feces, and utine.
How is it treated?

Treatment aims to remove or alleviate the cause of cirrhosis, prevent further liver damage, and prevent or treat complications. Salt and fluids are usually restricted. The person may benefit from a high-calorie and moderate- to high-protein diet (unless he or she also has hepatic encephalopathy, a condition that requires less protein).

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If the person’s condition continues to deteriorate, he or she may need tube feedings or vitamin supplements. Recovery depends on a regimen of rest, moderate exercise, and avoidance of infections and whatever substances caused the disorder.
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Drugs must be given cautiously, because the diseased liver can’t detoxifY harmful substances efficiently. If possible, the doctor will avoid prescribing sedatives but may prescribe an antiemetic for nausea, a vasoconstrictor to stop disease-related bleeding in the throat and, possibly, diuretics to reduce swelling.
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The doctor may recommend surgery to repair disease-related bleeding in the throat and to remove portions of damaged organs such as the spleen. Finally, prevention and control of cirrhosis depends on the person avoiding alcohol.

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What is this condition?

Galactosemia is the name for any disorder that interferes with the body’s ability to metabolize galactose, the sugar found in milk. This disorder produces symptoms ranging from cataracts and liver damage to mental retardation. It occurs in two forms: classic galactosemia and galactokinase-deficiency galactosemia.

Although a galactose-free diet relieves most symptoms, galactosemia-induced mental impairment is irreversible, and the person may have some vision loss. Caught early, the condition can be stopped by changing from milk-based to meat- or soy-based formulas.

What causes Galactosemia?

Both forms of galactosemia are inherited as recessive traits and occur in about 1 in 60,000 births in North America. However, up to 1.25% of the population has the gene that may lead to classic galactosemia. The second form, galactokinase-denciency galactosemia, is rarer. In both forms, inability to normally metabolize the sugar causes galactose accumulation and damage. The mechanism of this defect is still unknown.

What are its symptoms?

Children who inherit the classic galactosemia gene show symptoms at birth or a few days after they start nursing. The symptoms are a failure to thrive, vomiting, and diarrhea. Other effects include yellow skin and eyes, protein in the urine, and cataracts.

If the child continues to drink milk or eat galactose-containing foods, he or she may develop mental retardation, malnourishment, and progressive liver failure and may eventually die. Although treatment may prevent mental impairment, the condition can produce a short attention span, cataracts, difficulty with spatial and mathematical relationships, and apathetic, withdrawn behavior.

How is it diagnosed?

The doctor will order blood tests and urine tests to check for evidence of both types of galactosemia, but the measurements must be precise. Some infants who consume large amounts of milk have elevated blood galactose levels but don’t have the condition. Also, new­borns excrete galactose in their urine for about a week after birth; premature infants, even longer. Other tests include biopsy of liver tissue samples and enzyme tests. Amniocentesis, the sampling of the amniotic fluid that’s done before the infant is born, is recommended when parents are known to have the gene.

How is it treated?

If galactose and lactose are eliminated from the infant’s diet, most of the symptoms disappear. The infant gains weight and the cataracts shrink. If a child has the disorder, the doctor will advise replacing cow’s milk formula or breast milk with a meat-based or soy-based formula. As the child grows, a balanced, galactose-free diet must be maintained.

A pregnant woman who carries the gene should also follow a galactose-restricted diet. Such a diet supports normal growth and development and may delay symptoms in the infant. If the parents of a child with galactosemia want to have another child, the doctor will recommend genetic counseling. In some states, screening of all new­borns for galactosemia is required by law.

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