GEPATO-LIENALNYY SYNDROME (grech, hepar, hepat[os] liver + lat. lien a spleen), or a pechenochnoselezenochny syndrome — the combined damage of a liver and spleen with increase in both bodies. G - hp is not a nosological form therefore the term cannot be used as the main diagnosis.
G. - hp develops as a complication various acute and hron, diffusion damages of a liver, is observed at general diseases of blood, the inborn and acquired defects of portal venous system, including at a tromboflebitichesky splenomegaly; at hron, infectious and parasitic diseases, at some disturbances of exchange (an amyloidosis, a xanthomatosis, a porphyria, hemochromatosis). G. is the most frequent - hp is observed at patients with cirrhosis (preferential portal and postnecrotic type), at hron, aggressive hepatitis, a splenomegalichesky form of an acute viral hepatitis, cancer of a liver, acute and hron, leukoses. It is observed also at malaria, a visceral leushmaniosis, an alveolar echinococcosis, an infectious mononucleosis, a long septic endocarditis, syphilis, a brucellosis, visceral tuberculosis.
The Sochetannost of damage of a liver and spleen is explained by close connection of both bodies with system of a portal vein, richness of their parenchyma. elements of retikulo-histiocytic system, and also community of their innervation and ways of a lymph drainage.
The pathological anatomy and a pathogeny
Patol, anatomy and G.'s pathogeny - hp in each separate case are defined by a basic disease, about the Crimea also the main are connected a wedge, manifestations. Degree gepato-and splenomegalies fluctuates depending on character and a stage of the main disease and not always reflects weight patol, process.
G - hp at diseases of a liver is combined with signs of its insufficiency (symptoms of «vascular asterisks», «hepatic palms», a skin itch, jaundice, a disproteinemia, etc.) or with symptoms of portal hypertensia which are characteristic also, especially at a prematurity, for G. - hp at damages of a portal vein and its branches.
For G. - hp at a suprahepatic form portal hypertensia (see) in connection with an endophlebitis of hepatic veins (see. Kiari disease ) persistent ascites, a considerable hepatomegalia at rather small increase in a spleen are characteristic. At a subhepatic form of portal hypertensia (a tromboflebitichesky splenomegaly, a stenosis of portal and splenic veins) of G. - hp is characterized by a considerable splenomegaly at small increase in a liver, lack of signs of its insufficiency, jaundice, but sometimes it proceeds with fever, pains in a spleen, signs of an episplenitis and is frequent with the phenomena of a hypersplenism. Often at the same time the varicosity of a gullet and stomach, esophageal and gastric bleedings are observed.
The clinical picture
At diseases of blood G. - hp is combined with complaints of patients to the general weakness, ostealgias; pallor of skin, a hemorrhage, fever (are noted at acute leukoses, a lymphogranulomatosis), system or regional increase limf, nodes. Increase in a liver usually moderate (at an osteomyelosclerosis — considerable), jaundice happens seldom, preferential hemolitic. The spleen is a little increased at acute leukoses, but is huge at hron, a myeloleukemia when the splenomegaly precedes increase in a liver, and at an osteomyelosclerosis. Earlier increase in a liver is characteristic for hron, a lymphoid leukosis. Changes haemo - and miyelogramm have crucial diagnostic importance.
G.'s Recognition - hp comes down to identification of the increased livers and a spleen; more difficult, but main task in the presence of G. - hp is the differential diagnosis of the main disease. Its basis at the first stages is made by studying of the anamnesis and physical inspection of the patient which data define character and the sequence of additional researches. Anyway carry out gematol. inspection also specify a functional condition of a liver by laboratory methods. If necessary carry out rentgenol. the research of a gullet, stomach and duodenum with a survey X-ray analysis (sometimes a X-ray tomography) an upper half of an abdominal cavity, is conducted immunol, by researches (definition of HB antigen, alpha-fetoprotein, latex test etc.). At suspicion on a hemoblastosis or hemolitic anemia investigate punctate of marrow or limf, a node, apply immunol, tests (see. Hemolitic anemia ). Infectious and parasitic diseases apply all arsenal to diagnosis special, including serol. researches. In the plan of inspection at G. - hp can be included isotope methods of a research of a liver and spleen (see. Liver , methods of a research; Spleen , methods of a research), but if their results are not evidential, the question of a biopsy of a liver and spleen — puncture is considered or at a laparoscopy (see. Peritoneoskopiya ). Tsitol., including cytochemical, the research of punctates gains crucial importance in diagnosis amyloidosis (see), to Gosha of a disease (see), Nimanna — Peak of a disease (see), hemochromatosis (see) and other exchange diseases. In differential diagnosis of various forms of portal hypertensia are more and more widely applied splenoportografiya (see) and a splenoportometriya, the selection gepatovenografiya, and also (especially at suspicion on a new growth of a liver) tseliakografiya (see).
Radiodiagnosis plays an important role in clarification of the nature of G. - hp. The plan of a research and the sequence of use of separate techniques the attending physician and the radiologist, proceeding from the anamnesis define jointly, a wedge, pictures and results of laboratory and radio isotope tests. In all cases raying and roentgenograms allowing to establish situation, the size and a shape of a liver and spleen are obligatory. Pictures allow to confirm that the hypochondrium probed in left a body really is a spleen; in difficult cases for allocation of a shadow of a spleen inflate air a large intestine. Contrasting of upper parts of a digestive tract helps to specify also the sizes of a liver and spleen and to exclude a varicosity of a gullet, stomach and duodenum.
For the differentiation of cirrhosis and other diseases which are followed by G. - hp, including reticuloses, the selection abdominal angiography is of great importance. With its help define character and extent of reorganization of vascular network and its function, prove existence or lack of the changes typical for cirrhoses find out passability of a spleno-portal trunk, localization out of - and intra hepatic shunts. In addition to diagnosis, these data are necessary for establishment to lay down. tactics and solutions of a question of a splenectomy. The angiography is very important for detection of some other the defeats connected with G. - hp, in particular tumors of a liver since it allows to find shifts and erosion of vessels, their breaks, emergence of neogenic vessels, disturbances of blood circulation in a zone of a tumor.
If to the patient make a paracentesis abdominis with deflation of ascitic liquid, then in exchange it is necessary to enter 1,5 — 2 l of oxygen or air (or inert gas). In the conditions of arising at the same time pneumoperitoneum (see) it is possible to study in detail situation, size, a configuration, a surface condition of a liver and spleen and existence of unions around them. At syphilitic damages of a liver and spleen it is possible to reveal a perihepatitis and sclero-gummous changes in a liver. Detection of specific helps to diagnose syphilis aortitis (see) and specific damages of bones. Especially valuably rentgenol, a research of bones at the diseases of blood and reticuloendothelial system causing G. - hp E.g., at osteomyelodisplasias, a number of inborn hemolitic anemias, at a disease to Gosha, at a xanthomatosis on roentgenograms of a skeleton come to light typical changes of bone structure and periostoses.
At suspicion of thrombosis of splenic and portal veins or anomaly of development of these vessels decisive diagnostic data receive by means of a splenoportografiya.
Results rentgenol, researches it is necessary to estimate in light a wedge, yielded and results of the radio isotope tests giving an idea of the central hemodynamics, a hepatic blood-groove, function of hepatocytes and reticuloendothelial elements of a liver and spleen.
Treatment of patients with G. - hp is directed to a basic disease.
The hepatolienal syndrome at children
the Hepatolienal syndrome at children is observed considerably more often than at adults. It is caused as anatomo-fiziol, features of the growing organism (the liver and a spleen at children are rather big, plentifully vaskulyarizirovana, have an insufficient differentiation of a parenchyma and poor development of connecting fabric), and an originality of reaction of reticuloendothelial system to disturbing factors. Functional lability of reticuloendothelial system and a possibility of easy return to embryonal type of a blood formation, emergence of a myeloid and lymphoid metaplasia is observed at children not only at a disease of system of blood as at adults, but often occurs under the influence of various intoxications and infections. Feature of hepatolienal system at children is also bigger content in fabrics of the growing organism of undifferentiated mezenkhimny cells. Poor development of connecting fabric in a liver and a spleen is a propitious moment since to a certain extent protects these bodies from considerable intersticial defeats therefore at chest age typical cirrhotic changes are observed extremely seldom. M. S. Maslov considered that for G.'s development - hp internal causes, inborn weakness and constitutional predisposition play a large role and are manifestation of an originality of connecting fabric. In G.'s development - hp immunopato l. the mechanism plays, apparently, a significant role. At children with G. - hp of an autoantibody to antigen from a spleen come to light considerably more often than at other diseases.
At differential diagnosis long and hron, gepatosplenomegaliya at children it must be kept in mind infectious diseases (a pre-natal infection of a fruit, sepsis, tuberculosis, malaria, an infectious mononucleosis, etc.), big group of diseases of system of blood (a hemolitic disease of newborns, inborn hemolitic anemias, hemoglobinopathies, an acute leukosis, a lymphogranulomatosis, etc.), inborn disbolism (carbohydrate, fatty, proteinaceous), a vein thrombosis of a spleen and portal vein, an obliterating endophlebitis of vessels of a liver, anomaly of a structure of venous network in portal system.
S. D. Podymova, R. P. Zolotnitskaya; L. D. Lindenbraten (rents.), A. V. Papayan, R. 3. Schwartz (ped.).