Page 55 of 73
Features of diseases of a liver at newborns and babies
Liver diseases at babies differ on a clinical current, a histopathology and the forecast from liver diseases at more senior children. The heredity, factors operating during pre-natal development and after the delivery (diseases of mother, drug intake, a hypoxia, a hypoglycemia, the endocrine status), - and also a stage of pre-natal development define effects of influence of the teratogenic and damaging agents. The microorganisms and products of a metabolism almost harmless to a liver of children and adults can cause a lethal injury of a liver at newborns. The rubella, syphilis, toksoilazmoz and a herpes simplex, and also disturbances of a metabolism (deficit of a1-antitrypsin, a galactosemia and hereditary intolerance of fructose) cause a heavy cholestasia only in the first months of life. To the contrary, a basic reason of acute defeat of a parenchyma of a liver at children of more advanced age, hepatitis A, does not occur at newborns.
Formation of colossal cells is characteristic of an unripe liver. These big syncytial structures arise in all cases of a cholestasia at newborns and can be along with other factors the cause of an excretory hepatic unsufficiency in the first 4 months of life. Other feature consists that proliferative processes, such as fast development of connecting fabric, formation of pseudo-channels and pseudo-segments, often complicate interpretation of results of inspection. It is difficult to describe clinical and pathomorphologic characteristics of diseases of a liver at children of chest age terms which are used for designation of pathology of completely differentiated cellular structures. So, "neonatal hepatitis" — the term which is often confused to a viral hepatitis of children of advanced age actually means idiopathic defeat of a parenchyma (unlike pathology of bilious ways) which virus origin is not proved. Some authors use the term "cholestatic syndromes at children of chest age" for designation of all gepatobiliarny frustration of an unknown etiology.
Cirrhosis — an incurable disease which pathomorphologic picture consists of 3 components, i.e. a necrosis, fibrosis and regeneration. In segments of a liver of children of chest age there are zones aktivnodelyashchikhsya hepatocytes; these zones can be taken for the regeneration centers mistakenly; connecting fabric grows quickly, destroying very tectonics of a segment. Such picture reminds cirrhosis at adults, however the unripe liver possesses remarkable property to rassasyvat connecting fabric and to recover a structure of portal system. For example, at a galactosemia and a biliary atresia the morphological changes reminding cirrhosis at adults after treatment can disappear completely. Thus, the changes described by the term "cirrhosis" at adults can not give serious consequences at children of chest age.
The differential diagnosis and treatment of cholestatic states at children of chest and early age
The diagnosis of diseases of a liver and bilious ways demands system approach (fig. 12-22) from children of chest age. Stagnation of bile in a parenchyma and stagnation in bilious ways cannot be distinguished from each other clinically, according to standard biochemical tests. The transhepatic holangiografiya and an endoscopic retrograde holangiografiya at babies are practically not applied. Ultrasonic scanning perfectly reveals cysts, but does not allow to see minute structures in portal fissures. Isotope scanning gives the chance to judge expressiveness of a cholestasia, however to define, in or out of a liver bile current is blocked, by means of this method it is impossible.
The cholestasia of a metabolic origin can be found, investigating the content of a1-antitrypsin in serum, chlorides — in a secret of sweat glands, amino acids and galactoses — in urine. Infections can be diagnosed by means of cultivation of viruses, definition in blood of antiserum capacities, NBsAg, and also by means of reaction of VDBL. However at least 80% of cases of cholestatic jaundice at babies belong to cryptogenic defeats.
Fig. 12-22. System (algorithmic) approach to the differential diagnosis and treatment of cholestatic states at babies. (Thaler M. M.: JAMA 237:60, 1977. It is printed with the permission of the American medical association.)
At a cryptogenic cholestasia research is originally directed to distinguishing an intra hepatic form from extrahepatic. Existence of a bilious pigment in Calais indicates an intra hepatic origin of jaundice as intake of bile in intestines does not stop. In this case ultrasonic research (for detection of cysts of a liver and the general bilious channel) and a transdermal biopsy of a liver is shown. Studying of bioptat allows to judge weight of pathological process and existence of a cholangitis. Besides, the biopsy gives the chance to establish whether intensive formation of colossal cells ("hepatitis of newborns"), a hypoplasia of intra lobular channels or proliferative reaction in terminal channels in response to blockade of distal bilious ways takes place. It is possible to judge deficit of a1-antitrypsin on a cash! ichiya of PAS positive granules (fig. 12-23); the tsitomegalovirusny infection is identified on characteristic cytoplasmic inclusions (fig. 12-24), and the mucoviscidosis differs in existence of mucic traffic jams and fibrosis of fabrics in portal zones. Cultivation of tissue of the liver received at a biopsy sometimes helps to find out the nature of infectious process.
Akholichny of kcal can be manifestation of an atresia of extrahepatic channels. It is in this situation important to establish whether really bile comes to intestines. Measurement of excretion — the most sensitive and exact way of an assessment of current of bile. Kal and urine is collected separately during 72 h after intravenous administration Bengalese pink, marked 131I, in a dose 3 mkki/kg. Measure radioactivity a calla and express it as a percentage to the entered radioactivity. If with a stake during 3 days less than 5% of the entered dose are allocated, then it speaks about a total block of a zhelchevydeleniye. In parallel carry out scanning of an abdominal cavity in 24 and 48 h after an injection of radioactive paint. If radioactivity is found over area of intestines, then collecting a calla is stopped.
Simpler, though not such sensitive, a bile current assessment method — aspiration of intestinal contents through the probe entered into a duodenum. Existence in aspirate of bilious pigments after stimulation of reductions of a gall bladder excludes an atresia; the negative take testifies to a heavy cholestasia, but can be not only at an atresia.
Other methods of an assessment of extrahepatic current of bile are based on scanning of a liver and an abdominal cavity after an injection of one of new short-lived radionuclides. The diagnostic importance of an assessment of current of bile by means of these radionuclides (for example, 99tts) can be increased by preliminary strengthening of biliary secretion by means of phenobarbital. Sensitivity and specificity of these methods need to be specified. Indirect methods of an assessment of excretion of bile include definition of peroxidase of erythrocytes, lipoproteina-H before treatment holestiraminy, and also the level of serumal IgA. It is necessary to emphasize that the specified methods reflect cholestasia degree" but do not give an idea of its localization.
Fig. 12-23. A liver at insufficiency of a1-antitrypsin. The cytoplasmatic granules, characteristic PAS positive resistant to a diastase, surrounded with an aura are visible. X 400.
Fig. 12-24. A liver at a tsitomegalovirusny infection. Shooters point to the hepatocytes located around the central vein which size is increased owing to cytoplasmic inclusions (cytomegaly). X 100.
If the biopsy of a liver and radioactive research indicate extrahepatic blockade of bilious ways, then the operational holangiografiya through a gall bladder is shown. Detection of communication of the intra hepatic courses with a duodenum excludes an atresia. When the gall bladder is absent or is incorrectly created, carefully investigate portal fissures. Between kistozno expanded general bilious channel or the remains of the right and left hepatic channels and a small bowel, mainly lean, create an anastomosis. If biliary structures are impassable, then make a hepatic portoenterostomiya (operation Kasai).