Biliary Atresia Disease

http://medicastore.com/penyakit/906/Atresia_Bilier.html
Biliary atresia is a condition where the bile ducts are not formed or does not develop
normally. The function of the bile system is to remove metabolic wastes from the liver
and bile salt transport needed to digest fat in the intestine. In biliary atresia there
blockage of bile flow from the liver to the gallbladder. This can cause liver damage
and liver cirrhosis, which if untreated can be fatal.

CAUSE
Biliary atresia is due to abnormal development of the bile ducts inside and outside the
liver. But the cause of the biliary tract developmental disorder is unknown.
Biliary atresia was found in 1 of 15,000 births.

SYMPTOMS
Symptoms usually appear within two weeks after birth, namely in the form:
- Dark urine baby
- Pale stool
- Yellow skin
- Does not increase weight or slow weight gain
- Enlarged liver.

At the time the baby reaches the age of 2-3 months, the following symptoms occur:
- Growth retardation
- Itching
- Fussy
- High blood pressure in porta vein (blood vessels that carry blood from the stomach,
intestines and spleen to the liver).

DIAGNOSIS
Diagnosis based on symptoms and physical examination.
On examination the abdomen, enlarged liver palpable.
# Check the usual place: Blood tests (there are elevated levels of bilirubin)
# USG abdomen
# X-ray abdomen (liver appeared enlarged)
# Kolangiogram
# Liver biopsies
# Laparotomy (usually done before the baby is 2 months old).
TREATMENT
The best procedure is to replace the bile ducts that drain bile into the intestine. But
this procedure may be performed only at 50-10% sufferer. To skip biliary atresia and
directly connects the liver with the intestine, surgery is called the Kasai procedure.
Surgery will be successful if done before the baby is 8 weeks old. Usually surgery is
only a temporary treatment and eventually need a liver transplant performed.