P.J.Grigoriev, E. P.Jakovenko RGMU, Moscow

Intrahepatic holestaz (VH) can develop at level gepatotsitov or vnutripechenochnyh bilious channels. According to it allocate VH, obuslovlennyj with defeat gepatotsitov and kanalikul, and VH, connected with defeat protokov.

the Aetiology and patogenez VH are diverse. Gepatotselljuljarnyj and kanalikuljarnyj holestazy can be caused virus, alcoholic, medicinal, toksicheskim defeat of a liver, stagnant warm insufficiency, endogennymi metabolicheskimi infringements (holestaz pregnant women, at mukovistsidoze, at alfa-1-antitripsinovoj insufficiency, etc.) . ekstralobuljarnyj (duktuljarnyj) holestaz it is characteristic for primary biliarnogo a cirrhosis and primary sklerozirujushchego holangita which aetiology is unknown, and also for some drugih diseases with the known reasons, for example, for secondary sklerozirujushchego holangita.

At gepatotselljuljarnom and kanalikuljarnom holestazah are mainly amazed transportnye systems of membranes, and for ekstralobuljarnogo holestaza it is characteristic povrezhdenie epitelija bilious channels. For VH receipt in blood, and sledovatelno, and in a fabric, various components of bile, mainly bilious kislot, and their deficiency or absence in a gleam of a duodenal gut and others otdelah intestines is characteristic. At holestaze superfluous concentration of components of bile in pecheni and organism fabrics calls hepatic and system pathological protsessy, causing corresponding clinical and laboratory displays bolezni.

At the heart of formation of clinical symptoms three lay faktora: - superfluous receipt of bile in blood and fabrics; - reduction of quantity or absence of bile in intestines; - influence of components of bile and its toxic metabolitov on hepatic kletki and kanaltsy.

the Clinical picture

Characteristic symptoms holestaza, including VH, are: a skin itch, zheltuha, ksantomy, ksantelazmy, aholichnyj a chair, urine of dark brown colour. Vyrazhennost symptoms VH depends on the basic disease, infringement ekskretornoj functions gepatotsitov and pechenochno-cellular insufficiency.

the Skin itch usually precedes jaundice occurrence.

At VH the symptoms connected with a lack of bile in a gleam kishechnika and infringement vsasyvanija of fats (steatoreja, growing thin, deficiency zhirorastvorimyh vitamins) join.

At it is long existing holestaze naturally come to light: Deficiency of vitamin D (ossalgii, proksimalnaja miopatija, an osteoporosis, sometimes osteomaljatsija), vitamin E (muscular weakness, mozzhechkovaja ataksija), vitamin K (gemorragichesky a syndrome, gipoprotrombinemija), vitamin A ("night blindness", giperkeratoz skin, kseroftalmija, keratomaljatsija). At formation biliarnogo a cirrhosis prisoedinjajutsja signs biliarnoj hypertensia (persistent meteorizm, astsit, splenomegalija) and pechenochno-cellular insufficiency (loss of weight of a body, an atrophy myshts, gipoalbuminemija, etc.). To characteristic laboratory signs VH carry: uvelichenie in blood alkaline fosfatazy (SHCHF) and scale-gljutamintranspeptidazy (GGTP) do three and more norms, and also increase in cholesterol blood (over ones and a half norm), bilious acids (to one and a half norms and above) and some other markers (lejtsinaminopeptidazy i other) Having certain diagnostic value.

Level transaminaz is usually raised, but, as a rule, it below levels fermentov holestaza and only at quickly progressing holestaze level AsAT and AlAt sharply increases, especially at vnepechenochnom an obstructive syndrome.

, but unessential laboratory sign VH level increase konjugirovannogo bilirubin, cholesterol and its derivatives is Important.

To the symptoms connected with a delay lipidov in an organism, carry ksantomy on kozhe, in internal bodies, including on covers of nervous trunks with projavlenijami polinejropatii.

Before to diagnose illnesses at which are shown VH, it is necessary iskljuchit vnepechenochnyj holestaz which also can is long to proceed under maskoj VH.

Vnepechenochnyj holestaz has following characteristic signs: nadstenoticheskoe rasshirenie the channels, revealed on ultrasonic, and blockade of channels (holedoholitiaz, striktura, etc.) Revealed at endoskopicheskoj retrogradnoj holangiografii (ERHG).

VH at level gepatotsitov, kanalikulov and even ekstralobuljarnyh bilious protokov, in essence, are clinically indiscernible, as intrahepatic biliarnyj the path represents continuous system and consequently any patologichesky process on liver level, including amazing gepatotsity, mozhet to be accompanied holestazom. Gepatotselljuljarnyj holestaz develops both at ostryh, and at chronic hepatitises virus, alcoholic, medicinal and toksicheskoj aetiologies, at a cirrhosis, and also at not inflammatory zabolevanijah a liver (amiloidoz, sarkoidoz, etc.) .

the Researches confirming VH

VH at primary biliarnom a cirrhosis (PBTS):

Primary sklerozirujushchy holangit:

Table 1. Pathogenetic approaches to medicamentous therapy VH

VH pregnant women:

Irrespective of an aetiology and nozologii VH, constant presence in superfluous kolichestve to bile in gepatotsitah and kanalikulah leads nekrozu gepatotsitov and razvitiju to pechenochno-cellular insufficiency. If holestaz it is observed in techenie three-five years the cirrhosis with development astsita is formed, hypostases and pechenochnoj encephalopathies. Weight and expressiveness of clinical symptoms and at VH chrezvychajno variabelny. But almost always process progresses, if not ustranjaetsja the reason which has caused its development, - for example, a medicine, alcohol, virus, bacteria etc.

Medicamentous treatment

In treatment any medical products influencing an aetiology and patogenez of illness if it is possible are used; Also attempts to render medikamentoznoe influence on separate symptoms are undertaken, for example, to eliminate deficiency vitaminov, to improve processes of digestion and vsasyvanija etc. However at everyone konkretnogo the patient should be applied only patogeneticheski proved lekarstvennuju therapy.

the Short characteristic of some preparations used at VH

1. Ademetionin-1,4-butandisul`fonat (S-ademetionin, geptal) - biologicheskoe the substance which is a part of fabrics and liquid environments of an organism and uchastvujushchee in reactions transmetilirovanija. Possesses antidepressive and gepatoprotektivnoj activity.

Within two-three weeks it is expedient to apply daily intravenously or vnutrimyshechno on 5-10 ml (400-800 mg), and further to accept inside on 400 mg (one tablet) two-four times a day on an empty stomach.

Course of treatment. At sharp VH ones and a half - two months, at chronic - with uchetom efficiency.

2. Ursodezoksiholevaja acid (UDHK) - possesses versatile pozitivnym action on gepatobiliarnuju system at VH and zhelchekamennoj illnesses. UDHK it is absorbed in a rectum. At regular intake (10 mg/kg in sutki) it joins in enterogepaticheskuju circulation, providing normalisation zhelcheobrazovatelnoj and secretory functions of a liver. The preparation ursofalk, appointed is more often applied at VH inside on 250 mg two-three times a day on long period. The combination with geptalom is possible.

3. Rifampicin (rifadin, rifaren, etc.) - at the heart of it pozitivnogo actions at VH lays an induction mikrosomalnyh liver enzymes. Preparat calls a number of by-effects (a nausea, vomiting, a diarrhoeia, a headache and dr.)

the Effect from application of the preparations used at VH, as a rule, by patients otsenivaetsja how they influence an itch (its expressiveness usually decreases cherez some days, but the itch only in one-two month from the beginning lechenija disappears. The effect is estimated by doctors, besides, on clinical and laboratory pokazateljam (levels SHCHF, GGTP, cholesterol, etc.).