meaning of Arteriohepatic dysplasia

Arteriohepatic dysplasia meaning in Medical Dictionary

Also known as Alagille problem, this ia an inherited condition described as jaundice in the newborn period, liver disease with cholestasis, peripheral pulmonic stenosis and unusual face. Children with Alagille syndrome generally current with jaundice (yellowing of the skin and whites of the eyes) in the newborn period. Cholestasis (stagnant movement of bile through the liver) then develops with puritis (irritation), stools with no typical yellowing brown color, and growth of this liver and spleen. Peripheral pulmonic stenosis is a kind of congenital heart disease (CHD). Other styles of CHD in addition occur. The face has actually deep-set eyes, wide forehead, long nostrils with level tip, importance of the chin, and low-set or malformed ears. The outlook is determined by the amount of extent regarding the CHD therefore the liver infection (it can cause liver failure). The disorder is an autosomal principal characteristic which means that the gene for this is on a non-sex chromosome (an autosome) and just one edition of the Alagille gene is sufficient to make the condition. The gene is found on chromosome 20 in musical organization 20p12. The problem was initially described by D. Alagille into the French medical literary works in 1969.