[2014] Krystina Glasner: Anatomy Biliary Atresia

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Human Anatomy
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[2014] Krystina Glasner: Anatomy Biliary Atresia

BILIARY ATRESIA

What are the symptoms?First is jaundice. Jaundice occurs when the liver does not remove bilirubin. Blockage of the bile ducts forces bilirubin to build up in the blood.

What is biliary atresia?-is a life threatening condition in infants in which the bile ducts inside or outside the liver do not have normal openings. Bile ducts in the liver, called hepatic ducts, are tubes that carry bile from the liver to the gallbladder for storage and to the small intestine for use in digestion. Bile is a fluid made by the liver that serves two main functions; carrying toxins and waste products out of the body and helping the body digest fats and absorb the fat soluble vitamins A, D, E, and K.

Bile becomes trapped, builds up, and damages the liver. Damage leads to scarring, loss of liver tissue, and cirrhosis.

Two Types:Fetal biliary atresia appears while the baby is in the womb.Perinatal biliary atresia is more common and does not become evident until 2 to 4 weeks after birth.

Who is at risk?-is rare and only affects ~one in 18,000 infants; females, premature babies, and children of Asia or African American heritage.

Jaundice is when the skin and whites of the eyes turn yellow.

What causes it?Not an inherited disease and is most likely caused by an even in the womb or around the time of birth.

Some are:-a viral or bacterial infection after birth, such as cytomegalovirus, reovirus, or rotavirus-an immune system problem, such as when the immune system attacks the liver or bile ducts for unknown reasons-a genetic mutation-a problem during liver and bile duct development in the womb-exposure to toxic substances

Additional Symptoms-dark urine, from high levels of bilirubin in the blood spilling over into the urine-gray or white stools, from lack of bilirubin reaching the intestines-slow weight gain and growth

What's the diagnosis?No test can diagnose, so a series of tests are needed. These tests include a blood test, abdominal x rays, ultrasound, liver scans, liver biopsy, and/or diagnostic surgery.

Usually referred to a pediatric gastroenterologist, hepatologist, and/or surgeon.

Treatment?Usually with surgery, called the Kasai procedure, or eventually a liver transplant.

The Kasai procedure is usually the first treatment during which the pediatric surgeon removes the infant's damaged bile ducts and brings up a loop of intestine to replace them. As a result, bile forms straight to the small intestine.

If the Kasai procedure fails then the infant will recieve a liver transplant withing the next 1 to 2 years. After the transplant, medications are used to prevent the immune system from rejecting the liver. Blood pressure medications and antibiotics along with special diets and vitamin supplements may be perscribed.

Eating, Diet, and NutritionInfants often have nutritional deficiencies and require special diets as they grow up. They may need a higher calorie diet, beacuse biliary atresia leads to a faster metabolism. The disease also prevents them from digesting fats and can lead to protein and vitamin deficiencies.


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