Congenital bile stenosis

Congenital bile stenosis's disease overview

congenital biliary tract stenosis is a disease of biliary tract characterized by partial narrowing or completely at one or more positions of the liver's bile duct system. This is a disease that starts in the newborn stage, the rate is often low.

Bile is a digestive liquid produced in the liver, then passed through the bile ducts, part of the reserves in the remaining gallbladder goes into the small intestine, helping to digest fats. In children with congenital bile stenosis, the biliary tract is inflamed and narrows immediately after birth. This causes clogged production to destroy the bile path inside and outside the liver, causing biliary obstruction in the liver and damage the liver. If there is no timely biliary surgery, it can cause extremely serious complications, then the liver failure then the liver function may be completely destroyed, the risk of death from liver failure is very high, which often leads to death before. 2 years old.

The classification of congenital biliary tract stenosis is often based on narrow location in the bile path system divided into the following three categories:

  • Type I: Bile stenosis
    • Type II: Narrow up to the common liver tube, divided into two small groups: IIA narrowing of the common gallbladder and the common liver and IIB are the common liver pipe.

    Type III: Narrow to the liver navel (this is the most common type that accounts for 90% of cases).

    Causes of Congenital bile stenosis's disease

    Causes of bile stenosis in most newborns has not been clearly defined. Some factors have been given but have no specific evidence and research. Some may be due to defects in the development of early bile ducts (especially those who have more abnormalities other than congenital bile narrow) and some may arise during the period of birth Outdoors such as Hepatotropic virus infection, reovirus 3 in the liver, congenital cytomegalovirus or autoimmune cause. Consider on two reasons:

    Genetics

    A relationship between biliary and genes for the first time was discovered in China through a combined study across the entire genome, and has been confirmed in Asians and humans. Thai white skin. A relationship that can occur with GPC1 gene removal, Glypican encryption 1 - a heparan sulfate proteoglycan, has been reported. This gene is located on the long branch of chromosomes No. 2 (2Q37). Babies with biliary stenosis are found to have a GSTM1 genotype while all their mothers are heterosexual to GSTM1. 

    Poisoning

    Some cases of biliary tract stenosis may occur due to contact with aflatoxin B1 and at a lower level than aflatoxin B2 in the last months of pregnancy. Thanks to the liver function of the mother, the fetus is almost protected during pregnancy, but after giving birth, these babies will be affected by Aflatoxin available in the blood and in the liver, which can cause disease. 

    In addition, there are studies on biliary tract narrowing in animals. For example, the sheep born to graze sheep on the Red Crumbweed has developed cholecitis at certain times. These factories were later found to contain a toxin, now called Biliatresone. Studies are being conducted to determine whether there is any connection between cases of human tract infections and toxins like biliatresone. There are several signs that a metabolic substance of some human intestinal bacteria can be similar to Biliatresone.

    Symptoms of Congenital bile stenosis's disease

    Babies and children with the first congenital bile stenosis often show signs of bile stasis. Bile stasis is a condition where the bile is accumulated in the liver because the bile path is clogged while the bile continues to be produced, resulting in more and more bile accumulated inside the liver. In bile contains bilirubin so when the liver bile is not able to eliminate bilirubin through bile ducts, bilirubin starts to accumulate in the blood, causing clinical symptoms.

    Symptoms of congenital bile stenosis usually appear in the middle of the second to the sixth week after birth. Children may appear as follows:

  • jaundice
  • Itching, rash on the skin;

    • Ability to absorb poor nutrients (causing slow growth);

  • light feces, dark urine and finally abdominal distention;
  • cirrhosis

    • Hypertension

    If not treated, congenital bile stenosis can lead to liver failure. Unlike the manifestations of other jaundice diseases, congenital stenosis does not lead to cerebral cerebral syndrome (a form of brain damage due to liver dysfunction). Because the liver has biliary stenosis can still resolve bilirubin and bilirubin that cannot be resolved by the blood barrier into the brain.

    People at risk for Congenital bile stenosis's disease

    Some factors are determined to increase the risk of congenital biliary stenosis, namely:

  • Geography: The highest percentage of biliary tract narrow in Asian countries, according to congenital biliary narrow statistics, is more common in Chinese children than Japanese children;
    • Sex: Biliary tract stenosis is more common in women than men; age: Biliary tract stenosis is a disease that only appears in infants.

    Prevention of Congenital bile stenosis's disease

    congenital bile stenosis is a disease that appears at the time of birth, but the disease is not inherited and does not spread from the sick to a healthy person. Currently, there is no preventive measure for couples who have children without congenital biliary stenosis. The early detection and treatment of the disease play an important role in improving the child's bile system and avoiding the risk of death.

    Diagnostic measures for Congenital bile stenosis's disease

    There are many liver diseases that cause symptoms similar to biliary stasis so many tests should be performed before the diagnosis is biliary stasis. Especially, trying to screen the cause of jaundice in children may be confused with cholestasis. 

    Tests needed for determining and distinguishing diagnosis include: blood tests, urine tests, liver function tests, blood cell count, blood, marrow and functional tests Blood coagulation. Image diagnostic methods play an important role in determining biliary stenosis. In which ultrasound is a first indication in cases where there is a suspected congenital biliary tract narrow. It can be used to assess the liver system and can eliminate other surgical abnormalities. High frequency ultrasound has been shown to help improve sensitivity, specificity and accuracy in diagnosing biliary inflammation. In addition to the congenital biliary ultrasound other methods that are also often used are abdominal cutting, helping to identify real abnormalities. Liver biopsy, is a test to take a very small needle tissue sample, identifying liver cell abnormalities.

    Congenital bile stenosis's disease treatments

    Surgery is the main treatment for congenital bile stenosis. The goal of treatment is to help the flow of bile fluid are cleverly used to avoid liver failure. Kasai surgery, also known as the liver umbilical connection method, is a common method that is commonly applied, the method is named after the Japanese doctor creating this method. The narrowed bile ducts are removed and replaced by a new bile duct system made from a piece of small intestine, allowing the bile from the liver to flow into the intestine through this new pipe. This can be partially reduced or completely reduced the condition of jaundice but not guaranteed to recover liver lesions that have appeared earlier.

    The most effective Kasai method when implemented early is usually when children under 3 months old, the success rate is up to 80%. Cases of good reactions, the symptoms of jaundice and other symptoms often disappear after a few weeks. 

    If Kasai surgery fails to circulate the flow of bile fluid, children will experience problems: Vitamin deficiency, slow development, digestive bleeding, infection. The treatment after surgery should pay attention to monitor these signs.

    If the kasai method is unsuccessful, there is only a liver transplant. The success rate of the current liver transplant cases is about 75%. However, the subject of liver donation at this age is extremely rare, so finding the appropriate donation is extremely difficult. Unable to find appropriate and timely donation liver, the patient will die.

    Biliary stenosis is a congenital disease that is now identified as a non -genetic, non -transmission disease, but there is no measure to prevent it. Kasai surgery is applied as the first step in treatment until the child grows and develops, then the child should perform liver transplant, to achieve long -term healing effect.

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