Alagille syndrome, also referred to as Alagille disease, is a condition characterized by abnormal bile ducts that lead to chronic cholestasis. This condition translates to less production of bile
passed to the intestine from the liver. When bile is not able to drain out of the liver, it causes liver damage because of the bile piling up in the liver. When there is extreme liver damage, it
can cause cirrhosis.
Alagille syndrome is generally a hereditary syndrome handed down from parent to child. The siblings of a child who has Alagille’s syndrome have a one in thirty chance of developing it if both parents do not have any symptoms. Now, if one parent has the symptoms, it raises the chances to one in ten.
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Individuals who have Alagille syndrome have the potential of developing jaundice and cholestasis because the liver of the patient has few bile ducts compared to an average person. This can happen within the first four months after birth. Cholestasis refers to the obstruction or reduced flow of bile out of the liver. Due to the occurrence of jaundice, you can easily see the yellowing of the skin, whitening of the eyes, intense itching (pruritus), pale stools, dark urine, fatty bumps (xanthomas) or sores under the skin’s surface, and an abnormally large liver (hepatomegaly), or enlarged spleen (splenomegaly). Since the body will not be in a position to properly absorb fat soluble vitamins and fats. Children affected by Alagille syndrome may experience growth deficiencies.
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