What is Alagille Syndrome?

Alagille syndrome

is especially observed in infants and children as this disorder is an inherited one. This syndrome is a disease of the liver that affects the bile ducts in infants.

Alagille Syndrome:

This syndrome decreases the bile ducts in the livers of children. This disease may be inherited from either of the parents and almost 50 percent children who inherit it develop the syndrome.

  • Children are known to lose these bile ducts within the liver (which are very useful for digestion) progressively over the first year. At the same time, the structures those outside the liver are narrowed considerably.
  • Losing or narrowing of the bile ducts would lead to an accumulation of bile within the liver leading to liver cells’ damage. A damage to the liver cells would cause scars in the liver thus leading to cirrhosis (a chronic liver disease) in almost half the children affected.


Let us look at the symptoms of this syndrome.

  • During the first three months of growth, the child can develop jaundice and grows poorly. Also, the stool of the child is pale and loose.
  • As the child grows, the child can suffer from severe jaundice attacks, stunted growth, deposits of fat in the skin and itching.
  • After the age of four, the problem usually stabilises and the symptoms start to recede.

Other Recognisable Features:

Some other features that can help with diagnosing this syndrome are-

  • cardiovascular abnormalities
  • spinal column abnormalities
  • kidneys and eye abnormalities


Since the disease is mainly caused due to the accumulation of bile in the liver, it is important that the bile flow be increased in order to assist in the normal development of the child.

  • Children who suffer from this syndrome have a decreased flow of the bile into the intestines. Medicines like Colistipol, Questran and Phenobarbital can improve the flow of bile there by reducing liver damage and improving fat digestion in children.

The life expectancy in children who suffer from

Alagille syndrome

is difficult to predict. It would depend on what other systems might be affected.

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