Holestaz – the clinical laboratory syndrome which is characterized by increase in blood of content of substances, ekskretiruyemy with bile, owing to violation of production of bile or its outflow. Symptoms include a skin itch, yellowness, locks, smack of bitterness in a mouth, morbidity in the right podreberye, dark color of urine and decolouration a calla. Diagnostics of a holestaz consists in determination of level of bilirubin, ShchF, cholesterol, bilious acids. From tool methods use ultrasonography, a X-ray analysis, gastroscopy, a duodenoskopiya, a holegrafiya, KT and others. Treatment is complex, gepatoprotektor, antibacterial medicines, cytostatics and medicines of ursodezoksikholevy acid are appointed.
Holestaz - the delay or the termination of release of bile caused by violation of its synthesis by hepatic cells or interruption of transport of bile on bilious channels. Prevalence of a syndrome has average values - about 10 cases on 100 thousand of the population a year. This pathology comes to light at males after 40 years more often. A separate form of a syndrome – pregnant women which frequency among total number of the registered cases makes about 2%. Relevance of a problem is caused by difficulties of diagnostics of this pathological syndrome, identification of primary link of pathogenesis and selection of the further rational scheme of therapy. Gastroenterologists, and in need of performing surgery - surgeons are engaged in conservative treatment of a syndrome of a holestaz.
Reasons and classification of a holestaz
The etiology and pathogenesis of a holestaz are defined by a set of factors. Depending on the reasons allocate two main forms: extra hepatic and intra hepatic . Extra hepatic it is formed at mechanical obstruction of channels, stones of biliary tract act as the most widespread etiologichesky factor. Intra hepatic develops at diseases of gepatotsellyulyarny system, as a result of damage of intra hepatic channels or combines both links. At this form there are no obstruction and mechanical damages of a biliarny path. Thereof the intra hepatic form is in addition subdivided into the following subspecies: gepatotsellyulyarny at which there is a damage of hepatocytes; kanalikulyarny, proceeding with defeat of transport systems of membranes; ekstralobulyarny, connected with violation of structure of an epithelium of channels; mixed .
One or several mechanisms are the cornerstone of manifestations of a syndrome of a holestaz: receipt of components of bile to the blood course in excess volume, reduction or absence it in intestines, action of elements of bile on tubules and cells of a liver. As a result bile gets into blood, causing emergence of symptoms and defeat of other bodies and systems.
Depending on character of a current divide on sharp and chronic. Also this syndrome can proceed in a bezzheltushny and icteric form. In addition allocate several types: partial – is followed by decrease in secretion of bile, dissotsianny – it is characterized by a delay of separate components of bile, total – proceeds with violation of intake of bile in a duodenum.
According to modern gastroenterology, in emergence of a holestaz the leading value has injury of a liver of virus, toxic, alcoholic and medicinal character. Also in formation of pathological changes the essential part is assigned to heart failure, metabolism violations ( pregnant women, and others) and to damage of mezhdolkovy intra hepatic bilious channels (primary biliarny cirrhosis and primary skleroziruyushchiya holangit).
Symptoms of a holestaz
At this pathological syndrome of manifestation and pathological changes are caused by excess amount of bile in hepatocytes and tubules. Degree of expressiveness of symptoms depends on the reason which caused , weights of the toxic defeat of cells of a liver and tubules caused by violation of transport of bile.
A number of the general symptoms is characteristic of any form of a holestaz: increase in the sizes of a liver, pain and feeling of discomfort in a zone of the right podreberye, a skin itch, akholichny (decoloured) kcal, dark color of urine, violation of processes of digestion. Characteristic feature of an itch is its strengthening in the evening and after contact with warm water. This symptom influences psychological comfort of patients, causing irritability and sleeplessness. When strengthening expressiveness of pathological process and level of obstruction of kcal loses coloring before full decolouration. The chair at the same time becomes frequent, becoming liquid and fetid.
Owing to deficiency in intestines of bilious acids which are used for absorption of fat-soluble vitamins (And, E, K, D), in Calais raises the level of fatty acids and neutral fat. Due to violation of absorption of vitamin K at the long course of a disease at patients fibrillation time increases that is shown by the raised bleeding. The lack of vitamin D provokes decrease in density of a bone tissue therefore patients are disturbed by extremity, backbone pains, spontaneous changes. At long insufficient absorption of vitamin A visual acuity decreases and there is a gemeralopiya which is shown deterioration in adaptation of eyes in the dark.
At the chronic course of process there is a violation of exchange of copper which collects in bile. It can provoke formation of fibrous fabric in bodies, including in a liver. Due to increase in level of lipids formation by a ksant and , caused by adjournment of cholesterol under skin begins. Ksantoma have a characteristic arrangement on skin a century, under mammary glands, in a neck and a back, on a palmar surface of brushes. These educations arise at permanent increase in level of cholesterol within three and more months, at normalization of its level their independent disappearance is possible.
In certain cases symptoms are expressed slightly that complicates diagnostics of a syndrome of a holestaz and promotes a long current of a pathological state – from several months to several years. A certain part of patients for treatment of a skin itch the dermatologist, ignoring other symptoms.
Holestaz is capable to cause serious complications. Lasting jaundice more than three years is in most cases formed a liver failure. At a long and noncompensated current there is hepatic encephalopathy. At a small amount of patients in the absence of timely rational therapy development of sepsis is possible.
Diagnostics of a holestaz
Consultation of the gastroenterologist allows to reveal characteristic signs of a holestaz. When collecting the anamnesis determination of prescription of emergence of symptoms, and also degrees of their expressiveness and communication with other factors is important. At survey of the patient existence of yellowness of skin, mucous and a skler of various degree of expressiveness is defined. Also assessment of a condition of skin is carried out – having combed existence, a ksant and . By means of a palpation and percussion the expert often finds increase in a liver in sizes, its morbidity.
In results of the general blood test can be noted anemia, , the increased speed of subsidence of erythrocytes. In biochemical blood test the giperbilirubinemiya, a giperlipidemiya, excess of level of activity of enzymes comes to light (ALAT, ASAT and alkaline phosphatase). The analysis of urine allows to estimate existence in it of bilious pigments. An important point is definition of autoimmune nature of a disease by detection of markers of autoimmune damages of a liver: anti-mitochondrial, anti-nuclear antibodies and antibodies to gladkomyshechny cages.
Tool methods are directed to specification of a state and the sizes of a liver, gall bladder, visualization of channels and determination of their sizes, identification of an obturation or narrowing. Ultrasound examination of a liver allows to confirm increase in its sizes, change of structure of a gall bladder and defeat of channels. The endoscopic retrograde holangiopankreatografiya is effective for detection of stones and primary skleroziruyushchy holangit. The Chreskozhny chrespechenochny holangiografiya is used at impossibility of filling of bilious ways with contrast retrogradno; these methods in addition allow to carry out drainage of channels at obstruction.
The magnetic and resonant holangiopankreatografiya (MRPHG) has high sensitivity (96%) and specificity (94%); it is modern noninvasive replacement of ERHPG. In situations, difficult for diagnostics, the positron and issue tomography is applied. At ambiguity of results carrying out a biopsy of a liver is possible, but also the histologic method not always gives the chance to differentiate out of - and intra hepatic .
At differential diagnostics it is necessary to remember that the syndrome of a holestaz can meet at any pathological changes of a liver. Viral and medicinal hepatitises, belong to such processes, holangit and perikholangit. Separately it is worth allocating holangiokartsiny and tumors of a pancreas, intra hepatic tumors and their metastasises. There is a need of differential diagnostics with parasitic diseases, an atresia of bilious channels, primary skleroziruyushchy holangit less often.
Treatment of a holestaz
Conservative therapy begins with a diet with restriction of neutral fats and addition in a diet of fats of a phytogenesis. This results from the fact that absorption of such fats happens without use of bilious acids. Medicinal therapy includes purpose of medicines of ursodezoksikholevy acid, gepatoprotektor (ademetionin), tsitostatik (methotrexate). Symptomatic therapy is in addition applied: antihistaminic medicines, vitamin therapy, antioxidants.
As etiotropny treatment surgical methods are in most cases used. Operations of imposing of holetsistodigestivny and holedokhodigestivny anastomoz, external drainage of bilious channels, opening of a gall bladder and a holetsistektomiya concern to them. Separate category are the surgeries during the narrowings and stones of bilious channels directed to removal of concrements. In the rehabilitation period the physical therapy and physiotherapy exercises, massage and other methods of stimulation of natural protective mechanisms of an organism are applied.
Timely diagnostics, adequate medical actions and the supporting therapy allow to achieve from the most part of patients of recovery or permanent remission. At observance of preventive actions the forecast favorable. Prevention consists in observance of the diet excluding the use of spicy, fried food, animal fats, alcohol and also in timely treatment of the pathology causing stagnation of bile and injury of a liver.