Symptoms Of Alagille Syndrome

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.

Reveal the symptoms of Alagille syndrome now.

Jaundice

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.

Continue reading to learn about more symptoms of Alagille syndrome now.

Heart Murmurs

Individuals with this condition have heart abnormalities that can start from unnoticeable heart murmurs to serious defects in the heart's structure. With heart murmurs, you will hear an additional sound when the heart is beating. In children who have Alagille syndrome, heart murmurs are caused by the narrowing of the pulmonary artery. You will find children with Alagille syndrome have complex heart defects, such as tetralogy of Fallot, which is a rare version of cyanotic heart disease. Cyanosis is characterized by a bluish discoloration of the mucous membranes and the skin because of the lack of the right amount of oxygenated blood.

There are four heart defects in the tetralogy of Fallot: a massive enlargement of the right atrium, displacement of the largest artery in the body (aorta) that causes blood-flow from the left and right ventricles to the aorta, ventricular heart defect, and obstruction of the blood flow from the right atrium of the heart to the lungs because of pulmonary stenosis. Pulmonary stenosis is an abnormally narrow opening that occurs between the pulmonary artery and the right atrium. Other defects include atrial septal defects, ventricular septal defects, aorta coarctation, and patent ductus arteriosus.

Some studies have indicated in rare cases, Wolff-Parkinson-White syndrome can be associated with this condition and symptoms. In Wolff-Parkinson-White syndrome, electrical disturbances are felt in the heart.

Keep on reading to learn more details about the various symptoms of Alagille syndrome now.