Atresia of bilious ways
Contents:
Description:
The atresia of bilious ways (especially intra hepatic) is in most cases connected with the hepatitis postponed vnutriutrobno more often caused by one of reoviruses. At some children developing of this malformation is caused by the adverse factors operating on the 4-8th week of an antenatal life. Usually such children have also malformations of other bodies (more often than kidneys, heart, a backbone). Some children have an association with a polysplenia, trisomies on 13 and 18 couples of autosomes. Frequency: 1 patient on 10 000 — 30 000 newborns.
Allocate atresias of extrahepatic bilious ways (without or in combination with an atresia of a gall bladder), atresias of intra hepatic bilious channels (without or in combination with atresias of extrahepatic bilious ways), total.
Symptoms of the Atresia of bilious ways:
The clinic of atresias of bilious ways is characterized by emergence of jaundice or from the first days of life, or during 2–3 weeks of life. The chair is akholichny though the first days of life maybe it is painted. There is no stercobilin in a chair. Urine is painted intensively. Jaundice progressively accrues at the expense of direct bilirubin. The liver increases and becomes dense. By 2 months of life also the spleen significantly increases, portal hypertensia with ascites develops, the general state worsens. In the first month of life the general state can be not broken, and the child can even add body weight, it is quite good to suck. In a week after emergence of the decoloured chair vitamin the K-scarce hemorrhagic syndrome, and after emergence of portal hypertensia — a hematemesis can develop. By 5–6 months of life (without the surgical help) biliary cirrhosis, peripheral neuropathy develop. The atresia of intra hepatic bilious channels is often combined with existence at children of other anomalies of a structure: persons, hearts, backbone, vascular bed of a liver. In addition to jaundice and an acholia of a chair there can be also xanthomas on skin, an itch, a steatorrhea, a hemorrhagic syndrome, neuromuscular frustration (loss of tendon jerks, signs of disturbance of a craniocereberal innervation, paresis of the lower extremities — a consequence of deficit of vitamin E).
At biochemical researches find in blood serum the low level of factors of a prothrombin complex, a hypoproteinemia, a hypoalbuminemia, and high levels of direct bilirubin, activity of an alkaline phosphatase, leucineaminopeptidase, 5 nucleotidases.
Reasons of the Atresia of bilious ways:
The reasons of an atresia are precisely unknown. It develops at an early stage of an embryogenesis, any communication with the medicines or inoculations coming to mother's organism during pregnancy is not seen. In the USA it occurs at 1 child from about 10-20 thousand. Girls with an atresia are born a little more often than boys. Cases when with an atresia only one child from several in a family is born are usual (and even only one of twins).
Treatment of the Atresia of bilious ways:
Not later than over two-month children as the developing cirrhosis sharply reducing survival percent can destroy positive takes of operation is desirable to make operation. Now reconstructive operations (the Kasai procedure, the Japanese surgeon who by name offered it) are widely used at which timely performance the positive forecast is expected at 30-40% of children. In the absence of effect or impossibility of reconstructive operation (for example, disturbances of intra hepatic channels) performing transplantation of a liver is shown.
