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Diagnosis of diseases of a liver in all age groups is based on the following principles: 1) a careful assessment of symptoms on the basis of the anamnesis and physical inspection; 2) strict selection of necessary functional trials; 3) reasonable use of a method of a biopsy of a liver and the correct interpretation of the obtained data; 4) careful selection of methods of obtaining images and correct interpretation of their results.
Symptomatology. The family anamnesis is especially important for diagnosis of the diseases of a liver which are shown at early chest age. Hereditary disorders and anomalies of a structure of a liver and bilious ways prove in several days or weeks after the birth; they lead to development of a chronic or lethal disease of a liver if it is not possible to diagnose timely them and to begin to treat.
Jaundice — the earliest and often only sign of the majority of diseases of a liver at early chest age. The reason of jaundice needs to be established as soon as possible as it can be caused by the toxic not conjugated bilirubin, to serve as display of sepsis, to be a sign of such metabolic frustration as a galactosemia which is giving in to treatment, or the congenital anomaly of bilious ways demanding kirurgichesky treatment. Indications of a pas jaundice at mother to or during pregnancy allow to assume transfer of hepatitis B from mother to the child. At children of more advanced age jaundice can indicate with big degree of probability hepatitis A. Other diseases (chronic active hepatitis, Wilson's illness — Konovalova or insufficiency of a1-antitrypsin) should be meant when jaundice has persistent character or other signs allowing to suspect their (tab. 12-14) are observed.
Other very frequent symptom of diseases of a liver — a hepatomegalia. At inspection it is necessary to define borders, a form and a consistence of a liver. The bottom edge of a liver is palpated 2 — 3 cm below than the right costal arch at babies and on 1 — 2 cm — at children of advanced age. Liver shift other bodies or a tumor can make wrong impression about its increase. It is perkutorno necessary to define the upper edge of a liver, and then to measure distance between it and bottom edge on the right sredinoklyuchichno-mamillar line. Though this size changes depending on age and the body weight of the child, the upper edge is localized within 1 cm from the fifth intercostal space. If in such a way it is not possible to determine the liver sizes, it is necessary to resort to radio isotope scanning (ultrasonic scanning in this respect yields less exact results). Physical research of a liver includes a palpatorny assessment of its morbidity, auscultation with the purpose to find a friction murmur and comes to an end with a careful palpation of an abdominal cavity to reveal increase in a spleen, the tumoral weight or sites of morbidity. Extrahepatic physical symptoms of diseases of a liver are listed in tab. 12-15.
Functional indicators of diseases of a liver. A liver — multifunctional body therefore the set of laboratory tests is developed and big efforts are made to make them specific, sensitive and reliable. And still only in rare instances additional researches significantly change the diagnosis made by means of routine functional trials which short list includes definition in serum of the general and conjugated (direct) bilirubin, aspartate aminotransferase and alaninaminotranspherase, an alkaline phosphatase, albumine, fractions of gamma-globulins, and also a prothrombin time. At jaundice and a hepatomegalia at children of any age define a1-antitrypsin, and for diagnosis of a syndrome of Ray, it is important to hepatocellular insufficiency and hepatic encephalopathy to know ammonia level in blood.
A. The liver diseases proceeding with jaundice
Newborns and children of chest age
Infections (inborn and acquired)
Bacterial — the sepsis (caused E. coli, etc.)
Virus — caused by a cytomegalovirus, a virus of hepatitis B, a rubella, a koksaka, an echovirus, a herpes simplex virus
Parasitic — syphilis, toxoplasmosis Metabolic disturbances
Hereditary — deficit a1antitripsina, a galaktbzemiya, hereditary intolerance of fructose, a mucoviscidosis, a tyrosinosis, Nimann's illness — Peak
Acquired — a cholestasia and the damages of a liver connected with completely parenteral food, a heavy eritroblastoz Idiopathic frustration — neonatal hepatitis (giant-cell hepatitis), family cirrhosis, a hereditary lymphostasis with a cholestasia
Anomalies of a structure of bilious channels of the Atresia and hypoplasia — an atresia of extrahepatic bilious ways, a hypoplasia of intra hepatic bilious ways, a dysplasia of a hepatic artery
Cysts — a choledochal cyst, cystous expansion of the main intra hepatic channels (Karoli's illness), inborn fibrosis of a liver, a polycystosis of a liver and kidneys Children and teenagers
Chronic hepatitis — persistent hepatitis, chronic active hepatitis
Hereditary diseases — Wilson's illness — Konovalova (gepatotserebralny dystrophy), a mucoviscidosis, a porphyria Malignant diseases — leukoses, lymphoma, liver tumors (cholangioma)
Toxicoses — caused by gepatotoksichny substances, poisons (insecticides, hydrocarbons, organophosphorous connections, a hypervitaminosis And)
Parasitic invasions — shistosomoza, a hay fever, the visceral Larva migrans form
Idiopathic or secondary defeats — nonspecific ulcer colitis, a pseudorheumatism
B. The liver diseases proceeding with a hepatomegalia
Inborn fibrosis of a liver Inborn hepatic unsufficiency of the Illness of "accumulation"
Acute — a syndrome Flying (fats)
Chronic — a glycogenosis, mukopolisakharidoz, an illness to Gosha, Nimann's illness — Peak, gangliozidoz, a cholesterol accumulation illness, Uolman's illness
Food disturbances — completely parenteral food (surplus of calories), a kwasiorkor, diabetes Infiltrative processes — leukoses, lymphoma, gistotsitoz X, granulomas (a sarcoidosis, tuberculosis)
Primary — a hepatoblastoma, a hematoma, a gemangioepdotelioma Metastatic — a neuroblastoma, Uilms's tumor, tumors of gonads
Table 12-15. Physical symptoms of diseases of a liver
Early children's age
Nanocephalia — a congenital tsitomegalovirusny infection, a rubella, a toxoplasmosis
Characteristic features of the person — a dysplasia of arteries of a liver of Teleangievtaziya — a hereditary hemorrhagic teleangiectasia Aggalaktozemiya's Cataract
Pigmental Dystrophy of a retina and back embryotoxon — a dysplasia of arteries of a tsecheya
The abnormal auskultativny phenomena in lungs — a mucoviscidosis Neuromuscular frustration (a tremor, muscular weakness) — an illness of accumulation of fats
Itch — a chronic cholestasia of the Hemangioma — gemangiomatoz a liver the Carotenemia — a hypervitaminosis And
Kayser's rings — Fleischer — Wilson's illness — Konovalova Glossit — cirrhosis
Increase in kidneys — inborn fibrosis or a polycystosis of a liver Neuromuscular frustration — Wilson's illness — Konovalova Artrit and an uzlovatovy erythema — damage of a liver at nonspecific; ulcer colitis
Definition of fraction of serumal bilirubin allows to differentiate quickly hepatic and hemolitic jaundice, is rather sensitive way of identification even of insignificant hepatocellular insufficiency and dysfunction of zhelchevyvodyashchy system; this method is specific to liver diseases. Fast increase of level of the general serumal bilirubin at simultaneous reduction of amount of direct bilirubin can be an adverse predictive sign of heavy hepatocellular insufficiency (for example, at fulminant hepatitis).
Level of aminotransferases — a sensitive indicator even of insignificant injury of hepatocytes, and, therefore, its definition it is especially useful at anicteric forms of diseases of a liver, supervision over the patients receiving gepatotoksichny drugs, an assessment of dynamics of a disease and efficiency of treatment. Alaninaminotranspherase — almost exclusively "hepatic" enzyme, quantity it does not change during all childhood; aspartate aminotransferase is formed in several bodies, the quantity it decreases a little with age. Follows from told that test on alaninamino-transferase has some advantages, however in practice define both enzymes as their relative rise or decrease give valuable information at an acute hepatitis and fulminant hepatitis.
Increase of level of an alkaline phosphatase is characteristic of the obstructive or inflammatory processes proceeding in a biliary path. At children with a hypoplasia or sharp narrowing of intra hepatic channels activity of this enzyme is Considerably increased against very moderate increase in level of aminotransferases. Moderated (to 1,5 times) it is difficult to interpret exceeding of formal level of an alkaline phosphatase as at the growing child this enzyme can come to blood from a bone tissue. Simultaneous determination of activity of other enzymes (5 nucleotidases, gamma glutamiltransferazy, leucineaminopeptidases) allows correctly otsenyt increase of level of an alkaline phosphatase.
Level of a seralbumin and prothrombin time reflect the synthesizing function of hepatocytes. So, the quantitative assessment of content of albumine which elimination half-life makes 20 — 25 days can be useful to watch a liver course of a disease if necessary. For example, at chronic active hepatitis decrease in level of albumine and simultaneous increase of level of gamma-globulin indicate progressing of cirrhosis. The lengthening of a prothrombin time testimonial of suppression of development of a prothrombin can arise within several hours at such acute states as Ray's syndrome or an extensive necrosis of a liver caused by toxic drugs. Inefficiency of vitamin K in attempt to reduce a prothrombin time indicates extensive destruction of cells of a liver at the patients who are in a hepatic coma or a prekomatozny state. However unlike decrease in level of albumine lengthening of a prothrombin time is not specific only to liver diseases.
Transdermal biopsy of a liver. It is the most valuable diagnostic method of diseases of a liver at children. It gives information which is often extremely necessary for the characteristic of inborn defects of a metabolism. Histochemical methods allow to find in liver tissue enzymes (disturbance of an exchange of urea, a glycogenosis, hereditary intolerance of fructose), metals (for example, copper at Wilson's illness — Konovalova) and the accumulated metabolites (a glycogen, sphingomyelin, etc.) . The diagnosis and the choice of treatment at chronic active hepatitis, Ray's syndromes depends on histologic data and the Cudgel — Johnson, a hypoplasia of intra hepatic channels, inborn fibrosis of a liver, heavy portal hypertensia and an atresia of extrahepatic bilious ways. At last, by means of a biopsy it is possible to watch efficiency of treatment and emergence of complications at long reception of steroid hormones and antimetabolites (chronic active hepatitis, excess of iron at the R-thalassemia, hepatotoxic effects of chemotherapy, a recurrent cholangitis at patients after recovery operation on the bilious ways).
The transdermal biopsy of a liver is absolutely safe for the patient — there is no message on death either what - or the complications connected with this technique among thousands of children aged from 1 week till 15 flyings to which the biopsy was done by Mengini's needle. 2 premises are important for safe carrying out a biopsy: that it was done by the experienced specialist owning this Method and that patients were carefully selected. Contraindications: the prothrombin time of more than 16 with, falling of quantity of thrombocytes is lower 40 000, probability of localization of vascular or infectious defeat on the course of a tissue extractor and heavy ascites.
Methods of obtaining image.
Indications to visualization of a liver and bilious ways at children same, as well as at adults; use of radiological and radio isotope methods for inspection of babies has some features.
Ultrasonic scanning — the most useful method of detection of choledochal cysts, expanded bilious channels and stones of a gall bladder.
Radionuclide scanning is applied to exact size discrimination of a liver and detection of vascular anomalies. The dye which is widely applied when scanning Bengalese pink, marked 1311, is almost not used for inspection at children of early age with mechanical jaundice, and it can be replaced with other gepatofilny substance. At interpretation of data of radionuclide scanning often overdiagnose an atresia of extrahepatic bilious ways if marked substance does not appear in intestines during 24 h after an injection. However it can reflect existence of a heavy cholestasia of a parenchymatous or biliary origin. At an akholichny chair at babies radioactive drug does not come to intestines; in a recovery phase after heavy hepatitis of newborns the pigment can appear in Calais how it is possible to find radioactivity in intestines. The high-quality image received with the help 99tts-PIPIUK (paraizopropilimpnodiuksusny acid), in combination with strengthening of current of bile at children with a cholestasia (thanks to administration of phenobarbital in 7 days prior to research) can increase sensitivity and reliability of a stsintigrafiya at recognition of a cryptogenic cholestasia.
Computer Tomography (CT). To carry out this type of scanning at children of younger age technically difficult as the general anesthesia can be required to immobilize the child. This method allows to define tumor borders before surgical intervention. KT reveals cysts and expansions of bilious channels with a bigger accuracy, than ultrasonic research, but these advantages are resisted by complexity and high cost of research. Sometimes at inspection of the patient with the isolated hepatomegalia of KT allows to do without liver biopsy as with its help it is possible to distinguish fatty dystrophy from saturation of a liver a glycogen, on low radiological density in the first case and high density — in the second.
Transhepatic and endoscopic holangiografiya. Methods of obtaining the image at which a contrast agent is entered directly into the biliary highway at children of younger age for technical reasons do not apply. The transdermal transhepatic holangiografiya at which the special "skin" needle is entered into a liver parenchyma gives the chance to receive the image of expanded bilious channels, but at children of younger age this method is not applied as at them the intra hepatic courses have small diameter and besides at a cholestasia they are squeezed by surrounding cicatricial fabric. The endoscopic retrograde holangiopankreatografiya is successfully applied with the purpose to visualize all biliary path and pancreat ducts, but the equipment which is available now does not allow to use it for inspection of children more young than 3 years. Endoscopes of small diameter with the improved fiber optics will allow to use this method for inspection of the least patients.