By far, jaundice is the most common presenting sign in BA. Unfortunately there are a vast number of conditions that can cause jaundice. As explained earlier, bilirubin has a retrograde (backwards) flow into the blood. Bilirubin is then deposited in the superficial tissues causing the yellow/orange skin color. Scleral icterus (yellowing of the eyes) results from the same process and can be seen when plasma bilirubin levels are 3-5 mg/dL. Bilirubin also plays a role in the pale color stool that is most often seen in BA. Bilirubin has a yellow color to it and when deposited into the duodenum causes the brown color of stool. Other common signs include hepatomegaly (enlarged liver) measuring >4cm, ascites, abdominal distention, splenomegaly (enlarged spleen) and low weight/failure to thrive or significant drop on growth charts. Decreases in levels of fat soluble vitamins A, D, E, and K lead to blindness, osteomalacia/rickets, neurological issues, and prolonged clotting time, respectively, which may all be seen to various degrees.
Bruising may be another indication of worsening liver disease. The liver produces all the clotting factors that help to stop bleeding. If the liver is damaged than the clotting cascade is hindered causing bruises and bleeding that are difficult to control.
If BA continues untreated or initial treatments fail additional signs of hepatic encephalopathy(1) due to increases in ammonia, esophageal varices (varicose veins of the esophagus) due to portal hypertension,(2) caput medusa (varicose veins of the stomach) due to portal hypertension, and developmental delays from inadequate fat absorption/toxic levels of ammonia can be seen. The triad of varices, encephalopathy, and ascites is considered progression from cirrhosis/compensated liver failure to decompensated liver disease. Untreated, individuals are expected to live an average of 1-2 years.
1. Hepatic Encephalopathy is the occurrence of confusion, altered mental status, and possible coma as a result of liver failure.
2. Portal hypertension is high blood pressure in the venous system that receives blood from the spleen, kidneys and gastrointestinal tract for filtration in the liver.
There are many other signs/symptoms that may be present all of which differ from patient to patient.
Bruising may be another indication of worsening liver disease. The liver produces all the clotting factors that help to stop bleeding. If the liver is damaged than the clotting cascade is hindered causing bruises and bleeding that are difficult to control.
If BA continues untreated or initial treatments fail additional signs of hepatic encephalopathy(1) due to increases in ammonia, esophageal varices (varicose veins of the esophagus) due to portal hypertension,(2) caput medusa (varicose veins of the stomach) due to portal hypertension, and developmental delays from inadequate fat absorption/toxic levels of ammonia can be seen. The triad of varices, encephalopathy, and ascites is considered progression from cirrhosis/compensated liver failure to decompensated liver disease. Untreated, individuals are expected to live an average of 1-2 years.
1. Hepatic Encephalopathy is the occurrence of confusion, altered mental status, and possible coma as a result of liver failure.
2. Portal hypertension is high blood pressure in the venous system that receives blood from the spleen, kidneys and gastrointestinal tract for filtration in the liver.
There are many other signs/symptoms that may be present all of which differ from patient to patient.