Secondary biliarny cirrhosis
Secondary biliarny cirrhosis – the diffusion progressing damage of a liver with the expressed fibrous and nodal reorganization of a parenchyma which cornerstone long obstruction of extra hepatic bilious ways is. Secondary biliarny cirrhosis is characterized by jaundice, a skin itch, a hyperthermia, pains in the right podreberye. For diagnosis of pathology the research of biochemical indicators of blood, ultrasonography, a chreskozhny chrespechenochny holangiografiya or an endoscopic retrograde holangiopankreatografiya, a liver biopsy is conducted. At secondary biliarny cirrhosis surgical elimination of obstruction of the general bilious channel is shown.
Secondary biliarny cirrhosis
In hepatology and gastroenterology primary and secondary biliarny cirrhoses make 5-10% among cirrhoses of a liver of various etiology. Men get sick with secondary biliarny cirrhosis twice more often than women. Usually the disease develops at persons at the age of 25-50 years; sometimes occurs at children, mainly in combination with nonspecific ulcer colitis. Secondary biliarny cirrhosis can be also combined with an ileitis, tireoidity, diabetes of the I type.
Development of secondary biliarny cirrhosis is caused by long partial or full violation of outflow of bile from a liver owing to obstruction of a holedokh (the general bilious channel) or one of its large branches.
As immediate causes of obstruction of a holedokh at adults postoperative striktura or stones in bilious channels which are followed holangity most often act. Thus, pathogenetic secondary biliarny cirrhosis is closely connected with cholelithiasis, holelitiazy and operations on the bilious ways (most often a holetsistektomiya). Sometimes bilious channels leads the long course of chronic pancreatitis to education.
Patients with a pancreatic cancer or bilious ways, as a rule, do not live up to secondary biliarny cirrhosis. However in rare instances at slow development of cancer of a head of a pancreas, a faterov of a nipple or the bilious courses there can be a mechanical obstruction of extra hepatic bilious channels. Also carry benign tumors and a cyst of a holedokh to the rare reasons of secondary biliarny cirrhosis.
Long extra hepatic , caused by one of the reasons designated above, leads to education centrolobular, and, further, and periportalny necroses, to growth and expansion of bilious tubules and mezhdolkovy bilious channels. Further the phenomena of an aseptic or bacterial holangit and a perikholangit develop that leads to the progressing expansion of portal paths in view of their hypostasis and fibrous changes.
Bile from the damaged intra hepatic channels accumulates in sites of periportalny necroses, forming, the so-called, bilious "lakes" surrounded with the ksantomny cages rich with cholesterol. As well as at any damage of a parenchyma of a liver, the processes of regeneration of hepatocytes which are followed by development of melkouzelkovy cirrhosis begin. Depending on extent of obstruction secondary biliarny cirrhosis can develop in terms from 3-12 months up to 5 years.
In the beginning diseases the symptomatology of primary pathology which caused obstruction of bilious channels prevails. Further development of a disease is similar to primary biliarny cirrhosis.
At secondary biliarny cirrhosis the phenomena of jaundice and a skin itch are most expressed. At increase of jaundice emergence of dark urine and decoloured a calla is observed. Nausea is often noted, subfebrilitt, incidental fever, pains in the right departments of a stomach connected by a recurrence of a holangit or bilious colic. The progressing decrease in body weight and bystry fatigue, , a splenomegaliya and a gepatomegaliya is characteristic.
Accession of portal hypertensia and liver failure is observed in a late stage of secondary biliarny cirrhosis. Development of hepatic abscesses and a pileflebit can serve as complications of secondary biliarny cirrhosis.
At recognition of secondary biliarny cirrhosis it is important to consider clinical laboratory signs of extra hepatic obstruction of bilious ways, data of the anamnesis (existence of ZhKB, operations on the bilious ways, a holangita), features of a current of the early period of a disease.
At survey of the patient with secondary biliarny cirrhosis typical yellowness of integuments, raschesa of skin, a ksantelazma and a ksantoma comes to light. The palpation of a stomach reveals a gepatosplenomegaliya, morbidity of a liver. In biochemical tests of blood increase in level of cholesterol, bilirubin, aminotransferases, alkaline phosphatase comes to light. At 40-50% of patients levels of γ-globulins and IgM increase. Data of the general blood test are characterized by anemia, leykotsitozy, acceleration of SOE; the general analysis of urine – intensive coloring of urine, existence of bilious pigments, a proteinuria.
By results of ultrasonography of an abdominal cavity (ultrasonography of a liver and bilious ways) the gepatosplenomegaliya, the reason of obstruction of biliary tract (a stone, a striktura, a tumor etc.) is defined. Finally the reason of obstruction manages to be found out when carrying out a chreskozhny chrespechenochny holangiografiya or retrograde holangiopankreatografiya.
In need of confirmation of the developed secondary biliarny cirrhosis carrying out a biopsy of a liver is shown.
Paramount task at secondary biliarny cirrhosis is the decompression of bilious ways and normalization of outflow of bile. Surgical tactics can include a holedokhotomiya, a holedokhostomiya, extraction of concrements from a holedokh and bilious channels (including at RHPG), an endoscopic buzhirovaniye extra hepatic bilious channels, endoscopic stenting of a holedokh, balloon dilatation of bilious channels, external drainage of bilious channels. Restoration of passability of bilious ways leads to noticeable improvement of a condition of patients and increase in life expectancy even at the begun cirrhosis.
At ineradicable obstruction of bilious ways for the purpose of the prevention of an infection or a recurrence of a holangit antibiotic treatment is carried out. Also at secondary biliarny cirrhosis reception of gepatoprotektor, vitamins of group B and vitamins-antioxidants (And, With, E), selenium is shown. For reduction of a skin itch antihistamines, tranquilizers are used.
In late stages of secondary biliarny cirrhosis transplantation of a liver is shown.
Forecast and prevention
In case obstruction of bilious ways remains not eliminated, the disease progresses. Esophageal, gastric or intestinal bleedings, thrombosis of a vorotny vein, ascites, a gepatotsellyulyarny carcinoma, interkurrentny infections can become complications of a late stage of secondary biliarny cirrhosis.
Life expectancy at secondary biliarny cirrhosis in many respects is defined by the reasons of obstruction of bilious channels and a possibility of their elimination. After transplantation of a liver development of a recurrence and cirrhosis is not excluded.
Prevention of secondary biliarny cirrhosis demands timely diagnosis and elimination of the diseases leading to obstruction of extra hepatic bilious ways.