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The congenital (teratogenic) and acquired anomalies of a structure of bilious ways in many cases are indiscernible. It is possible to think of teratogenic defect if it is combined with anomalies of a structure of kidneys (as, for example, at inborn fibrosis of a liver, a polycystosis of a liver and kidneys). The nature of such often found anomalies as an atresia inside - and extrahepatic bilious ways, a hypoplasia and expansion of the bilious courses, remains not clear. Such anomalies usually inborn, i.e. are already available at the birth, but it does not explain their origin. The inflammatory or toxic processes which arose on the 4th week of pre-natal development can cause secondary deformations, influencing forming of bodies of a fruit. Delay or blockade of a zhelchevydeleniye owing to an underdevelopment or deformation of bilious channels cause cholestatic jaundice, formation of stones in a gall bladder, an itch, a cholangitis, hepatocellular insufficiency and cirrhosis. Nevertheless it is necessary to aim to differentiate the teratogenic and acquired defects as it has important clinical value. Surgical correction of congenital anomalies can save the child's life while at the anomalies caused by the progressing inflammatory, infectious or exchange processes, surgical intervention is inefficient and it is even contraindicated.
Below anomalies of bilious ways which knowledge will form a basis for the correct diagnosis and treatment are described. Except for inborn fibrosis of a liver and a polycystosis of a liver and kidneys, anomalies of a structure of bilious ways complicate bile current that leads to developing of mechanical jaundice.
ATRESIAS AND HYPOPLASIAS
Atresias of extrahepatic and large intra hepatic channels belong to the most often found anomalies of bilious ways. Frequency of an atresia of extrahepatic channels makes 5-10 cases on 100 000 live-born or 3 — 15 cases on 100 000 hospitalized children of chest age. For comparison it is possible to tell that choledochal cysts, anomaly, the second for frequency, meet in 0,5 cases on 100 000 hospitalization.
Atresias of extrahepatic bilious channels can be divided into 2 groups empirically: the 1st group — giving in to surgical correction (choledochojejunostomy); the 2nd group — the options which in the past were considered inoperable, and which now are subject to a radical portoenterostomiya (creation of a direct anastomosis between the decapsulated opened liver surface in the field of gate and intestines, or the operation Kasai). The reason of atresias of extrahepatic bilious channels remains obscure. Release of the meconium painted by bile at newborns with an extrahepatic atresia, detection of normally created bilious ways "hammered" with inflammatory cells (fig. 12-26, a) and existence of the sclerosed remains of channels in zones where there is no inflammation any more (fig. 12-26, b) — all this indicates a possibility of the acquired defeat of normally developed bilious ways.
Characteristic histologic signs of an extrahepatic atresia — periportal fibrosis and proliferation of interlobular bilious ductules (fig. 12-27). These changes confirmed with quantitative data on blockade of current of bile (allocation with a stake during 3 days less than 5% of the entered dose of the Bengalese pink, marked 1311), indicate carrying out an operational holangiografiya the need to specify atresia type.
Without treatment (choledochojejunostomy) average life expectancy of the child with an atresia of extrahepatic channels makes 1,5 — 2,5 years. At such children portal hypertensia, ascites and gastrointestinal bleedings develop. Treatment of these complications and maintenance of adequate food are covered in section 12.101. About 40 — 50% of the patients who underwent radical operation survive during 5 flyings and more, but at many of them the chronic cholangitis per se or in combination with heavy portal hypertensia develops. The success of operation directly depends on diameter of bilious channels in the place of a section in portal fissures.
Fig. 12-26. and. The large branch of a hepatic channel passing through fibrous fabric in portal fissures. The gleam is filled with the cells characteristic of an acute and chronic inflammation which, apparently, penetrate a wall of a channel and infest a surrounding parenchyma (shooter). Material is received at portoenterostomip at the 8-week child. X 100. The Sklerozirovapny branch of a hepatic channel in the field of a hepatoenteric anastomosis in portal fissures. Lack of inflammatory elements pays attention. Material is received at autopsy of the same child in 8 hours
Fig. 12-27. A liver at an atresia of extrahepatic bilious channels. The portal zone is expanded due to growth of the fibrous fabric containing the proliferating bilious channels (shooters). X 100.
The number of positive takes of the operation Kasai sharply decreases if it is made at children 2 months are more senior. Need to do operation at the earliest age is connected with potential danger as it is difficult to distinguish a true atresia from an acute stage of neonatal hepatitis and other parenchymatous jaundices when neither the operational holangiografiya, nor sighting find the thin, fallen-down, but functioning channels. In these circumstances "hypoplastic" channels sometimes cross, and patients are exposed to an unnecessary portoenterostomiya. Moreover, at children 2 months results of the operation Kasai in general same, as after a holedkhoyeyunostomiya are more senior. The matter is that the operation Kasai is effective only in that case when it is carried out before full closing of bilious ways in portal fissures, i.e. at the hypoplasia of bilious ways preceding a full atresia. Remains to unknown whether the operation Kasai influences process of a sklerozirovaniye.
Atresia of intra hepatic bilious channels, and also the quantity or hypoplasia reduced pkh — a basic reason of the heavy chronic cholestasia beginning soon after the birth. At considerable part of patients this frustration is combined with anomalies of a structure of the person, heart, backbone, with a syndrome of an arteriopechenochny dysplasia (fig. 12-28). In the bioptata of a liver received in the first month after the birth often observe the inflammatory reaction taking interlobular bilious ductules. At the subsequent biopsies it is visible that inflammatory process abates, however it reduction of number of channels and reduction of their diameter (fig. 12-29) is a consequence. Thus, the hypoplasia of intra hepatic channels can be considered as an effect of the sclerosing cholangitis which is coming to an end at chest age.
The hypoplasia of intra hepatic bilious ways is shown by jaundice and a hepatomegalia on the first month of life. At heavy disease level serumal aminotraisferaz, an alkaline phosphatase (norms are 5 — 20 times higher) and lipids (cholesterol, phospholipids, triglycerides) quickly increases; corresponding changes at an atresia of extrahepatic bilious ways develop more slowly. At a hypoplasia of intra hepatic bilious ways xanthomas appear. Frequent symptoms: severe itch, steatorrhea and tendency to bleedings. At children 5 years are more senior the complex of neuromuscular frustration including lack of deep tendon jerks, defeat of third cranial nerves, paresis of the lower extremities, a pallesthesia is observed. These signs reflect changes in area of a front horn owing to deficit of vitamin E; they disappear if the content of vitamin E in blood is supported at the normal level.
Fig. 12-28. A face of the patient at Alagille-Watson syndrome (an arteriopechenochny dysplasia). Excessive development of a frontal bone, gipsrtelorizm, anti-Mongoloid section of eyes, flat ridge of the nose, small chin. In the right supraclavicular area raschesa are visible. Also the stenosis of a pulmonary artery and defects of front arches of vertebrae are found in the patient.
Treatment includes a diet with the low content of fats and enriched with proteins; at a heavy liver failure add triglycerides with average chains. Special attention should be paid on administration of fat-soluble vitamins as use of a holestiramin suppresses their absorption. A, D, E and K vitamins apply in the form of aqueous solutions. Use 25 hydroxyvitamins D does not give any advantages in comparison with administration of the vitamin D. A lack of vitamin E compensate by reception it inside in a dose of 1000 ME/days; however at some patients drug is almost not soaked up even at its introduction in the specified doses. In such cases the intramuscular injections of vitamin E allowing to maintain necessary concentration in blood are shown. It is important to begin replacement therapy with vitamin E timely as the above-stated neuromuscular frustration to the 6th 8-year age can become irreversible.
Holestiramin in a dose of 8 — 16 g/days (in mix with fruit juice) reduces an itch and a hyperbilirubinemia, normalizes other functional indicators of a liver. However this drug is badly transferred by many patients, it extends a prothrombin time and in rare instances can cause impassability of intestines. At some patients there comes improvement under the influence of phenobarbital in a dose of 3 — 5 mg/(kg-days) (treatment will be out under. control of content of drug in blood). It stimulates biliary secretion owing to what the itch and jaundice decrease, partially or completely xanthomas resolve. Sometimes at the patients who are not reacting on holestiramin or phenobarbital, is possible to achieve improvement at the combined treatment by these drugs.
The forecast improves if by means of a diet and medicamentous means it is possible to eliminate effects of a heavy cholestasia and malabsorption of fats. Patients live up to ten-year age, and some till 20 flyings. Gradually at most of patients biliary cirrhosis develops, at some process is stabilized without forming of the expressed fibrosis of a liver and disturbance of an intra hepatic blood-groove.