Skleroziruyushchy holangit – the rare chronic disease of bilious channels causing bile outflow violation, its stagnation and damage of cells of a liver with further development of a liver failure. Pathology it is long proceeds without clinical manifestations. Main symptoms: pain in a stomach, jaundice, an itch, weakness, weight loss. Diagnostic actions consist in performing blood test and a calla, ultrasonography of abdominal organs, MRPHG, ERPHG, liver biopsies. Treatment is directed to knocking over of symptoms, delay of pathological process and restoration of functions. Transplantation of a liver gives the greatest effect.
Skleroziruyushchy holangit - it is the inflammatory disease of intra hepatic and extra hepatic bilious channels which is characterized by their sklerozirovaniye (scarring) and violation of outflow of bile. Is followed by formation of cirrhosis, portal hypertensia and liver failure. Skleroziruyushchy holangit meets frequency of 1-4:100 000 though it is rather difficult to judge prevalence of pathology as it it is long proceeds asymptomatically. Besides, in most cases (to 80%) the disease is accompanied by chronic pathology of digestive tract (ulcer colitis, a disease Krone, etc.) which symptoms act into the forefront. It leads to progressing of pathological process, increase of adverse symptoms and complications. Statistically, incidences of a skleroziruyushchy holangit are subject men aged from 25 up to 40 years and the woman of more advanced age. Developing of a disease at children is not excluded though in pediatrics it is registered extremely seldom.
Reasons of a skleroziruyushchy holangit
Allocate primary and secondary holangit of skleroziruyushchiya. The causes of primary skleroziruyushchy holangit are still unknown. Scientists constantly make various hypotheses, but any of them is not confirmed for 100% yet. An important role in development of pathology is played by hereditary predisposition in combination with immunity work violation. This theory is based on statistical data which registered family cases of a skleroziruyushchy holangit and autoimmune diseases. Also the theory about negative impact of toxins, bacteria and viruses on bilious channels moves forward.
The disease can develop at any person, but there is a number of the contributing factors increasing risk of emergence of pathology: age (people most often are ill 30 years are more senior), a floor (men more suffer from a disease), presence of a disease at relatives.
Secondary skleroziruyushchiya holangit it is well studied, toxic damage of channels, thrombosis and violation of work of a hepatic artery, post-operational complications, stones to bilious ways, anomalies of development, a cytomegalovirus and at AIDS, some medicines, a holangiokartsinoma can be the reasons of its formation.
Irrespective of the causes, pathology causes violation of work of bilious channels and a liver failure. The inflammation of bilious channels leads to the fact that they are replaced with cicatricial fabric and are fallen down, it leads to bile outflow violation, its stagnation and the return absorption to the blood course. Thus there is jaundice. Stagnation of bile and increase in pressure in bilious ways because of violation of its outflow provoke irreversible changes in hepatic cells and their death that is shown by a liver failure.
Symptoms of a skleroziruyushchy holangit
The long time of skleroziruyushchiya holangit proceeds asymptomatically, at early stages the disease can be suspected on violations of biochemical blood test (change of activity of enzymes is defined). At development of dysfunction of a liver and scarring of bilious ways the following signs appear: pain in a stomach, an itch, yellowing of mucous membranes, skin and skler of eyes, temperature increase of a body, weakness and fatigue, weight loss.
Chronic violation of outflow of bile causes emergence of a steatorea (increase in a share of fat in kalovy masses), a lack of fat-soluble vitamins (A, D, E and K), osteoporosis. Long stagnation of bile leads to development of secondary cirrhosis which is the reason of portal hypertensia, ascites and bleedings. Formation of stones in bilious channels and them is not excluded, it is even more aggravating a situation. During external survey the gastroenterologist finds yellowness of integuments and mucous, increase in the sizes of a liver and spleen, excessive pigmentation of skin.
Diagnostics of a skleroziruyushchy holangit
For confirmation of the diagnosis of a skleroziruyushchy holangit the gastroenterologist carries out collecting the anamnesis and complaints, external survey and additional methods of a research: blood test, calla, ERPHG, MRPHG, ultrasonography of abdominal organs, liver biopsy.
Blood test allows to reveal hyperactivity of hepatic enzymes (nuclear heating plant, ALT). During the analysis of excrements change of characteristics a calla is defined: it can be light, tar-like (because of bleedings), sticky and fat.
Also the endoscopic retrograde pankreatokholangiografiya is carried out: the method is based on introduction of X-ray contrast substance in in the place of falling of a bilious channel into a 12-perstny gut and a further X-ray analysis on which segments of narrowing of channels are visible. A magnetic and resonant pankreatokholangiografiya (MRPHG) - more sparing research (unlike the first way), but at the same time rather informative. It allows to receive the accurate image of a liver and bilious channels and the developing deviations. Ultrasonography - less exact method of diagnostics, a research gives the chance to determine the liver sizes, and also to reveal the damaged sites of a liver and change in bilious channels. The biopsy of a liver helps to estimate completely degree of expressiveness of pathology (scarring processes).
Treatment of a skleroziruyushchy holangit
Treatment of a skleroziruyushchy holangit is directed to delay of progressing of pathology, elimination of adverse symptoms and complications. It is possible to liquidate clinical signs by purpose of a diet (restriction of fats, increase in concentration of vitamins and calcium); at development of hypovitaminoses intravenous administration of vitamin medicines is shown. It is possible to lower a skin itch by means of ursodezoksikholevy acid and antihistamines (, , etc.).
At development of a bacterial holangit antibacterial medicines are appointed (ampicillin, , tsefotaksy, etc.). Zhelchesvyazyvayushchy means can facilitate a condition of patients ().
Very often resort to surgical manipulations which help to slow down progressing of a skleroziruyushchy holangit and to improve a condition of patients. At detection bilious channels carry out balloon dilatation for the purpose of expansion of bilious ways; if it is impossible, then prosthetics of channels or excision of affected areas is shown. Stones in bilious ways delete at endoscopic operations. But efficiency of these manipulations is estimated ambiguously as they can only slow down progressing of a disease, and also quite often cause complications. The greatest possible results are yielded by transplantation of a liver (especially at cirrhosis and a liver failure). In 15-20% after liver transplantation perhaps repeated development of a skleroziruyushchy holangit.
Forecast and prevention of a skleroziruyushchy holangit
Forecast of a skleroziruyushchy holangit adverse. Longevity of patients from the moment of emergence of the first symptoms of pathology makes about 10-12 years. Transplantation of a liver – the only method of treatment allowing to return patients to normal life. Statistically, survival of patients after the change which is carried out on the 1st year after diagnosis makes 92,2%, on the 2nd year – 90%, on the 5th - 86,4%, in 10 years - 69,8%.
There is no specific prevention of pathology. But it is possible to reduce risk of its emergence if in due time to treat diseases of bilious ways, cholelithiasis, inflammatory processes of a pancreas, and also to have periodic medical examinations in office of therapy or gastroenterology.