HEPATOSES

From Big Medical Encyclopedia

HEPATOSES (hepatosis, singular; Greek hepar, hepat[os] a liver + - osis) — the general name of a number of the diseases of a liver which are characterized by primary disbolism of hepatocytes and morphologically shown their dystrophy without essential mesenchymal and cellular reaction. The term «hepatoses» is offered R. Ressle in 1930. In group hron, hepatoses of E. M. Tareev includes exchange damages of a liver; A. D. Myasnikov designated this term dystrophy of a liver.

Depending on the nature of disbolism distinguish the following main types G. — fatty (a synonym: obesity of a liver, steatosis, fatty dystrophy of a liver), cholestatic, pigmental. Carry Gilbert's syndromes to the last (a synonym the juvenile alternating Meylengrakht's jaundice), Kriglera — Nayara (a synonym not hemolitic kernicterus), Dubina — Johnson (a synonym not hemolitic form hron, jaundices) and the Rotor.

The etiology

Acute fatty G. most often develops at poisoning with phosphorus, arsenic, a dichloroethane, alcohol, at influence of some medicines (tetracyclines in high doses), can be observed in recent weeks pregnancies that explain with the phenomena of proteinaceous starvation (owing to preferential intake of nutrients in a fruit), and also at the vomiting connected with toxicosis.

Etiol. the factors leading to development hron of fatty G., are various, among them alcohol is on the first place. Besides, such diseases as a diabetes mellitus (is more often at elderly), the general obesity, a proteinaceous and vitamin deficiency (at a hyponutrient or owing to hron, diseases of the digestive system), and also hron matter, intoxication perchloromethane, organophosphorous connections, bacterial toxins.

Reception of medicines — derivatives of a fenotiazin (aminazine) and hormonal steroid drugs (analogs of testosterone, are oestrogenic, gestagena), anticoagulants, antibiotics is the frequent reason of development of cholestatic G. Cholestatic G. can develop in time of pregnancy (recurrent jaundice of pregnant women); sometimes the etiology remains obscure.

Pigmental G. develop owing to genetically determined enzymopathies. At Gilbert's syndrome assume the autosomal and dominant nature of inheritance, at Krigler's syndrome — Nayara — autosomal and recessive, at syndromes the Cudgel — Johnson and the Rotor — autosomal and dominant.

The pathogeny

is Fatty G. a consequence of disturbance of lipid metabolism. At acute fatty G. obesity of a liver results from decomposition of the fat which is contained in a liver and is normal. The pathogeny hron, fatty G. comes down to an imbalance between receipt of lipids in a liver and their metabolism in a liver, education and an exit of lipoproteids from a liver. Cholestatic G. is connected with development of an intra hepatic cholestasia owing to disturbance of exchange of cholesterol and bilious to - t, disturbances of education in a hepatic cell of bilious micelles and difficulties of outflow of bile.

At Gilbert's syndrome pairing function of a liver decreases (in connection with insufficiency of a glyukuroniltransferaza), and also dysfunction of capture of bilirubin, at Krigler's syndrome — Nayara is found full or almost total absence of a glyukuroniltransferaza in a liver. At syndromes the Cudgel — Johnson and the Rotor is broken excretory function of a liver.

A histologic picture

At acute fatty G. find the sharp obesity of hepatic cells which was more expressed in the center of segments and in an intermediate zone without necrobiosis of hepatocytes and inflammatory reaction. At hron, fatty G. in hepatocytes reveal the increased amount of fat (triglycerides). At zone obesity lipids find in separate hepatocytes, at diffusion — in all parenchyma.

Hron, fatty G. can pass in hron, hepatitis at an alcohol abuse, against the background of infections, etc.: proteinaceous dystrophy and necroses of hepatocytes develop, mesenchymal and cellular reaction joins, cirrhosis can develop further.

Characteristic morfol, cholestatic G.'s differences are existence of a bilious pigment in hepatocytes and star-shaped retikuloendoteliotsita (a large number it in the center of segments). In the course of a disease the pigment collects also in intra lobular bilious tubules, develops catarral and occlusive cholangitis (see). Usually proteinaceous dystrophy and a necrobiosis of hepatocytes, and also inflammatory reaction joins these phenomena, i.e. the hepatosis turns into hepatitis.

At Gilbert's syndrome most often find the increased amount of lipofuscin and fat in hepatocytes, a glycogenosis of their kernels, activation of star-shaped retikuloendoteliotsit. At electronic microscopic examination damage of a sinusoidal membrane of a hepatocyte and a hyperplasia of a smooth endoplasmic reticulum to it comes to light.

At Krigler's syndrome — Nayara the changes of tissue of liver found at light microscopy are insignificant. At a research under a supermicroscope find the same structural disturbances of hepatocytes, as at Gilbert's syndrome. Signs of damage of a nervous system, a myocardium and skeletal muscles are observed morfol.

At a syndrome the Cudgel — Johnson the dark color of a liver caused by accumulation in hepatocytes of a coarse-grained pigment is characteristic; at a syndrome of the Rotor of change of a liver in the form of a pigment in hepatocytes come to light only under a supermicroscope. Disturbance of ultrastructure of a hepatocyte is characteristic of both syndromes.

The clinical picture

Acute fatty G. develops without clear prodromal stage and is shown by violently progressing pechenochnokletochny insufficiency with a dispeptic syndrome, jaundice and hemorrhagic diathesis. The liver is usually not increased or increase it is insignificant. Expressed is characteristic hypoglycemia (see). In blood find a leukocytosis, a superactivity of an alkaline phosphatase; activity of aminotransferases and indicators of a thymol turbidity test are normal or slightly increased.

For hron, fatty G. increase in the sizes of a liver, sometimes considerable is characteristic. The liver has a smooth surface, stupid edge, the condensed consistence. Weakness, pains in right hypochondrium, the dispeptic phenomena are often observed. Indicators of functional trials of a liver are a little changed — the hepatic clearance during the conducting vofaverdinovy and bromsulfoftaleinovy tests is most often increased (see. Bromsulfoftaleinovaya test , Vofaverdinovaya test ), activity of aminotransferases in blood is increased slightly, there is not expressed disproteinemia.

Cholestatic G. is shown by a syndrome of an intra hepatic cholestasia: jaundice of various intensity, an itch of skin, light the kcal, dark urine, in blood serum hyperactivity of an alkaline phosphatase, leucineaminopeptidase, increased the maintenance of lipids (cholesterol, phospholipids and respectively (beta lipoproteids), bilious to - t. Hepatomegalia non-constant. The cytolytic syndrome can join.

Cholestatic G. — are more often an acute disease, but also the long current can be observed. Cholestatic G. of pregnant women, as a rule, passes right after childbirth, but quite often repeats at the subsequent pregnancies. Genetically determined returnable form of a high-quality intra hepatic cholestasia [Sammerskill, Walsh (W. Summerskill, V. Walshe), is described 1959; N. Tygstrup, 1960].

Gilbert's syndrome find more often in patients of youthful and young age, it is frequent in connection with infectious hepatitis and other acute infectious diseases. Men are ill more often. Main symptoms: easy, the changing intensity jaundice, weakness, the dispeptic phenomena, displays of vegetative dystonia, an abdominal pain, small increase in the sizes of a liver. Hyperbilirubinemia (see) it is caused by preferential increase in blood of free (indirect) bilirubin. Small increase in activity of alaninaminotranspherase, a sorbitdegidrogenaza is periodically observed. The course of a disease chronic with aggravations.

At Krigler's syndrome — Nayara jaundice is sharply expressed, it develops in the first days or hours after the birth, is followed heavy nevrol, the disturbances caused by toxic effect of free bilirubin. Patients lag behind in physical. and mental development. Changes of indicators of functional trials of a liver are periodically observed. The hyperbilirubinemia exceeds 20 mg of %, find only free bilirubin.

Syndromes the Cudgel — Johnson and the Rotor are shown at young age. The main symptom — the jaundice caused by preferential accumulation of the connected (direct) bilirubin in blood. Patients complain of dispeptic disturbances, increased fatigue, abdominal pains. Small increase in the sizes of a liver, periodically — minor change of indicators of functional trials of a liver is observed. The syndrome the Cudgel — Johnson occurs at men more often. During the conducting bromsulfoftaleinovy test at patients with a syndrome the Cudgel — Johnson the content of paint in blood repeatedly increases (after 45 min.), at patients with a syndrome of the Rotor removal of paint is slowed down. At a syndrome the Cudgel — Johnson usually both at peroral, and at intravenous cholecystographies (see) bilious ways and a gall bladder or are not contrasted at all, or are filled poorly and with delay, and at a syndrome of the Rotor — are not contrasted even at the intravenous cholecystography. Current of both syndromes chronic, wavy.

The diagnosis

Hron, can be suspected by fatty G. on the basis of anamnestic and a wedge. - biochemical, this. Authentically the diagnosis is established by a puncture biopsy of a liver, edges is the main method of differential diagnosis of fatty G. (see. Liver , methods of a research).

Acute fatty G.'s recognition pregnant women is based on data a wedge, pictures and hl. obr. biochemical, the researches allowing to delimit it from a viral hepatitis (see. viral hepatitis ).

Cholestatic G.'s recognition is based on identification etiol, factors and a characteristic syndrome of a cholestasia: there is no preicteric period, the cytolytic syndrome is not expressed, in blood the Australian antigen is not found; data of a puncture biopsy help to differentiate it with a cholestatic form of hepatitis.

Differential diagnosis with subhepatic jaundices (see Jaundice) is based on lack of symptoms of the diseases causing these jaundices and on data of a laparoscopy and diagnostic laparotomy.

The diagnosis of a syndrome of Gilbert is based on identification of a hyperbilirubinemia at the expense of free bilirubin in the absence of signs of an increased hemolysis; data of a puncture biopsy of a liver exclude other forms of damage of a liver.

Recognition of a syndrome of Krigler — Nayara is based on clinical displays of a disease and its family character. It is necessary to differentiate it from jaundices at newborns and from the jaundices caused by increase in free bilirubin in blood (fiziol. jaundice, passing family hyperbilirubinemia, hemolitic jaundices of various genesis).

Syndromes the Cudgel — Johnson and the Rotor are distinguished on the basis of existence hron, jaundices with increase in level of the connected bilirubin in blood; for a syndrome the Cudgel — Johnson of a patognomonichna results of bromsulfoftaleinovy test and a puncture biopsy of a liver.

Treatment

At acute fatty R. are necessary early and vigorous fight against bleeding (hemotransfusions, introduction an epsilon - aminocaproic to - you, Haemophobinum, phthiocol, Rutinum), a hypoglycemia (transfusions of solution of glucose, administration of redoxons and group B, lipotropic means, glucocorticosteroids). Expediency of immediate delivery of pregnant women with acute fatty G. is disputable.

Treatment hron, fatty G. etiological. Appoint the diet containing complete proteins and vitamins and insignificant amount of fats in optimum quantities. Additional value has introduction of lipotropic means, anabolic hormones, B12 vitamin, a hydra of lysates and extracts of a liver.

For cholestatic G.'s treatment first of all it is necessary to reveal and cancel the medicines which were G.'s reason; appoint corticosteroids.

Sick with aggravations of a syndrome of Gilbert appoint sedatives.

At Krigler's syndrome — Nayara is recommended phototherapy (fluorescent lamps, quartz, a direct sunlight), exchange hemotransfusions.

Treatment of syndromes the Cudgel — Johnson and the Rotor is not developed.

The forecast

Hron, fatty G. is reversible if restructuring of segments of a liver did not begin. Acute fatty G.'s forecast of pregnant women is serious. Pigmental G.' forecast is favorable, except for Krigler's syndrome — Nayara, at Krom patients die at infantile or children's age more often.

Prevention

acute fatty G.'s Prevention — prohibition of tetracyclines in high doses, good nutrition during pregnancy. Prevention hron. Consists in elimination of the factors causing it. Repeated cholestatic G.' development prevents careful prescription of medicines, promoting development of a cholestasia.

Pigmental G.' prevention is not developed.


Bibliography: Blyuger A. F. E.Z's ikrup-nikova. A fatty hepatosis, in book: Usp. Hypatolum., under the editorship of E. M. Tareeva and A. F. Blyuger, page 199, Riga, 1966, bibliogr.; they, Hereditary pigmental hepatoses, L., 1975, bibliogr.; The multivolume guide to internal diseases, under the editorship of E. M. Tareeva, t. 5, page 237, 306, M., 1965, bibliogr.; A. L. butchers. Diseases of a liver and bilious ways, M., 1956, bibliogr.; Arias J. M and. lake of Chronic nonhemolytic un-conjujated hyperbilirubinemia with glucuronyl transferase deficiency, Amer. J. Med., v. 47, p. 395, 1969, bibliogr.; J o s k e R. A., McCully D. J. a. M a s t a g 1 i a F. L. Acute fatty liver of pregnancy, Gut, v. 9, p. 489, 1968; SchillingerH. Dubin — Johnson — Syndrom, Med. Klin., Bd 62, S. 161, 1967, Bibliogr.; it, Rotor — Syndrom, ibid., S. 201; Sherlock S. Drugs and the liver, in book: Alcoholic cirrhosis a. other toxic hepatopathias, ed. by A. Engel a. T. Larsson, p. 235, Stockholm, 1970, bibliogr.

A. F. Blyuger, AA. 3. Krupnikova.

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