Biliary atresia

Biliary atresia. It is a chronic and progressive liver problem that manifests itself shortly after birth. The ducts inside and outside the liver , called bile ducts, normally allow a liquid produced by the liver to drain bile , which aids digestion, into the intestines.

Summary

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  • 1 Causes
  • 2 Symptoms
    • 1 Other symptoms
  • 3 Tests and reviews
  • 4 Treatment
  • 5 Forecast
  • 6 When to contact a medical professional
  • 7 Frequency in our environment
  • 8 Assessment and diagnosis
  • 9 Possible complications
  • 10 In children
  • 11 Sources

Causes

Biliary atresia occurs when the bile ducts inside and outside the liver do not develop normally. However, the reason why the biliary system fails to develop normally is unknown.

The bile ducts help remove waste from the liver and carry salts that help the small intestine break down (digest) fats.

In babies with biliary atresia, obstruction of the flow of bile from the liver to the gallbladder occurs, which can lead to liver damage and cirrhosis of the liver, which can be fatal if left untreated.

Symptoms

Newborns with this condition may appear normal at birth; however, jaundice (a yellowish discoloration of the skin and mucous membranes) occurs by the second or third week of life. The baby may gain weight during the first month, but then loses it, becomes irritable, and has worsening jaundice.

Other symptoms

  • Cloudy urine
  • Splenomegaly
  • Floating stool
  • Stools with foul odor
  • Pale or clay-colored stools
  • Slow growth
  • Little or no weight gain

Tests and exams

The doctor will perform a physical exam, which includes palpating the patient’s ventral area. The doctor may also feel an enlarged liver.

Tests to diagnose biliary atresia include, but are not limited to: Abdominal X-ray Abdominal ultrasound Blood tests to check total and direct bilirubin levels A hepatobiliary iminodiacetic acid (HIDA) scan, also called cholescintigraphy, to help determine if the bile ducts and the gallbladder is working properly Biopsy of the liver to determine the severity of cirrhosis or to rule out other causes of jaundice Radiography of the bile ducts (cholangiography).

Treatment

An operation called the Kasai procedure is done to connect the liver to the small intestine, passing around the abnormal ducts. This surgery is most effective if it is done before the baby is 8 weeks old, however, a liver transplant may still be necessary.

Forecast

Early surgery improves the survival of more than a third of babies with this problem. The long-term benefits of a liver transplant are not yet known, but it is expected to increase patient survival.

When to contact a medical professional

Call your health care provider if your child develops jaundice or if other symptoms of biliary atresia occur.

Frequency in our environment

It cannot be said that it is frequent but neither is it an unusual disease. The global incidence is 1 for every 8,000 to 15,000 live births. In Cuba it is around 1 for every 13,000 live births.

Assessment and diagnosis

Blood tests are performed to know the general condition and functioning of the liver and also ultrasound of the abdomen . In our country, we make the diagnosis by radiographic exploration of the bile ducts with the use of laparoscopy .

Possible complications

  • Infection
  • Irreversible cirrhosis
  • Liver failure
  • Surgical complications, including failure of the Kasai procedure.
  • When to contact a medical professional
  • Call your health care provider if your child develops jaundice or if other symptoms of biliary atresia occur.

In the kids

The appearance of the newborn is completely normal, and even this disorder is more frequent in children born at term and with good weight.

 

 

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