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    (Eng) 2.4.1

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    Biliary atresia is a rare condition affecting 1 in 15,000 births where the bile ducts outside and inside the liver are abnormally narrowed or blocked. Boys are affected twice as often as girls. The exact cause is unknown but genetic and infectious factors may play a role. Diagnosis involves physical exam findings of jaundice and liver enlargement as well as imaging and liver biopsy. Treatment involves surgery to reconstruct bile ducts along with nutritional support and medications to manage symptoms. Prognosis depends on age at surgery, with greater success rates if surgery occurs before 8 weeks of age. Complications include liver damage and infections if not treated.

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    (Eng) 2.4.1

    Uploaded by

    karyndp
    12 views7 pages
    Biliary atresia is a rare condition affecting 1 in 15,000 births where the bile ducts outside and inside the liver are abnormally narrowed or blocked. Boys are affected twice as often as girls. The exact cause is unknown but genetic and infectious factors may play a role. Diagnosis involves physical exam findings of jaundice and liver enlargement as well as imaging and liver biopsy. Treatment involves surgery to reconstruct bile ducts along with nutritional support and medications to manage symptoms. Prognosis depends on age at surgery, with greater success rates if surgery occurs before 8 weeks of age. Complications include liver damage and infections if not treated.

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    Biliary atresia is a rare condition affecting 1 in 15,000 births where the bile ducts outside and inside the liver are abnormally narrowed or blocked. Boys are affected twice as often as girls. The exact cause is unknown but genetic and infectious factors may play a role. Diagnosis involves physical exam findings of jaundice and liver enlargement as well as imaging and liver biopsy. Treatment involves surgery to reconstruct bile ducts along with nutritional support and medications to manage symptoms. Prognosis depends on age at surgery, with greater success rates if surgery occurs before 8 weeks of age. Complications include liver damage and infections if not treated.

    Copyright:

    © All Rights Reserved
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    12 views7 pages

    (Eng) 2.4.1

    Uploaded by

    karyndp
    Biliary atresia is a rare condition affecting 1 in 15,000 births where the bile ducts outside and inside the liver are abnormally narrowed or blocked. Boys are affected twice as often as girls. The exact cause is unknown but genetic and infectious factors may play a role. Diagnosis involves physical exam findings of jaundice and liver enlargement as well as imaging and liver biopsy. Treatment involves surgery to reconstruct bile ducts along with nutritional support and medications to manage symptoms. Prognosis depends on age at surgery, with greater success rates if surgery occurs before 8 weeks of age. Complications include liver damage and infections if not treated.

    Copyright:

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    of 7

    BILIARY ATRESIA

    Epidemiology
    • Incidence 1 in 15,000 births
    • Boys : girls 2:1
    Etiology
    • Idiopathic
    • Genetic factors: trisomy chromosomes 17, 18, 21
    • Organ anomaly (30% of cases)
    • Infection
    Pathophysiology
    • The problem of biliary atresia arising in the fetal form is related to
    congenital anomalies
    • However, in a more general form, the neonatal type is characterized
    by progressive inflammatory lesions, which are caused by infections /
    toxins that cause damage to the bile ducts
    Diagnosis and Differential Diagnosis
    Physical examination : 4 diagnostic criteria:
    - Hepatomegaly - Birth weight
    - Splenomegaly shows progressive - Feces color
    cirrhosis with portal hypertension - The age of the patient when the feces
    - Cardiac murmurs show abnormalities begins alcoholics
    in the heart - Liver state
    Supporting investigation : Differential diagnosis:
    - Regular and special lab (blood, urine, - Biliary Hypoplasia
    feces)
    - Imaging - Spontaneous perforation of bile duct
    - Liver biopsy - Idiopathic neonatal hepatitis
    - Arteriopathic dysplasia
    Management
    • Surgical therapy • Medikamentosa:
    • Nutritional therapy: - Phenobarbital
    - Feeding contains a medium - Cholestyramine
    chain triglyceride to treat fat - Ursodeoxicolic acid
    malabsorption
    - Management of fat-soluble
    vitamin deficiency
    Complications and Prognosis
    Complications: Prognosis:
    - Cholangitis - Success is determined by the age
    - Portal hypertension of the child during surgery,
    hepatic portion hystitis
    - Hepatopulmonary syndrome &
    pulmonary hypertension - When done at age <8 weeks, the
    success is 71.86% If> 8 weeks, its
    - Malignancy success 34,43%
    - If not done surgery 3-year
    survival rate is only 10% and will
    die on average at age 12 months

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