Onset of the disease is characterized by developing hepatocellular insufficiency, which then develops to portal hypertension. Prediction of disease may be advantageous if eliminate the cause of bile stagnation. If this is not possible due to a lack of qualified doctors or by individual the characteristics of each person - to develop a strong liver failure in violation of the majority its functions. Result - the inevitable death.
What it is?
Biliary cirrhosis (BCP) - a disease in which for various reasons, broken cross biliary ducts, because of which the flow of bile in the intestine is reduced or terminated. By etiology isolated primary and secondary forms of the disease.
Causes of
Set the specific cause of the formation of biliary cirrhosis has not yet been able to. We look at some of the theory of its formation:
- a genetic predisposition to the disease;
- previous infections of the liver - viral, bacterial, parasitic;
- toxic liver damage;
- autoimmune inflammation.
Confirm a direct link between these states and the formation of cirrhosis is not currently possible.
First, under the influence of certain reasons lymphocytes begin to destroy bile duct cells - activated inflammation therein. Because inflammation is broken and develops ductal patency bile stasis. In these areas there is hepatocyte damage and inflammation develops again. Mass death of cells can lead to the formation of cirrhosis.
Classification
Primary BCP - autoimmune disease that manifests itself as chronic nonsuppurative destructive inflammation of the bile ducts (cholangitis). In the later stages it causes bile stasis in the ducts (cholestasis) and eventually causes the development of cirrhosis. Most often, women suffer from the disease of forty to sixty years.
- In stage I is limited to bile duct inflammation.
- In process stage II applies to the liver tissue.
- Step III. Hepatocytes - liver cells - begin to be transformed into connective tissue, forming adhesions, scars that "pull together" among themselves bile ducts.
- IV stage - typical cirrhosis.
Secondary biliary cirrhosis occurs against a background of prolonged impaired outflow of intrahepatic bile ducts due to their narrowing or blockage caused by other diseases. It is more common in men between thirty and fifty years. Without treatment, both forms of the disease eventually lead to liver failure, worsening the quality of life and reducing its duration.
Symptoms biliary cirrhosis
In the case of biliary cirrhosis symptoms it is advisable to group of primary and secondary form of the disease.
Thus, primary biliary cirrhosis is characterized by:
- staining of skin in a dark brown color, especially in the area of the blades, large joints, and eventually the whole body;
- Non-permanent skin itch that often occurs during the night's rest, while other irritants (eg, after contact with woolen goods or after taking a bath). Itching may last for many years;
- Increase in volume of the spleen is a common symptom of the disease;
- The emergence of the flat formation on the eyelids, which has the form of plaque. They are often only a few, xanthelasma may appear on the chest, hands, buttocks, elbows;
- People can begin to disturb pains in the right hypochondrium, in the muscles of the mouth often appears a bitter taste, slightly increased body temperature.
With the progression of the disease all the symptoms are worse, loss of appetite, itching becomes unbearable. Grubeyut areas of pigmentation, the skin swells, end phalanges thicken. The pain intensified, there is varicose veins of the esophagus and stomach can develop internal bleeding. Absorption of vitamins and nutrients is difficult, align hypovitaminosis symptoms. Lymph nodes are increased, there are disturbances in the digestive system.
The secondary form of the disease has similar symptoms, including:
- severe pain in the affected area of the liver;
- intense itching of the skin, worse at night;
- hepatic tenderness to palpation and increase of its dimensions;
- early appearance of jaundice;
- splenomegaly;
- increase in body temperature to febrile figures on a background of a developing infection.
Quickly enough, this form of the disease leads to cirrhosis and subsequent liver failure, the symptoms of which threaten the patient's life. In particular, the development of symptoms in a human liver failure are:
- nausea and vomiting, intestinal contents;
- dyspeptic disorders;
- discoloration of feces and urine color of dark beer;
- hepatic encephalopathy (dementia).
The condition can cause severe complications such as ascites, internal stomach and intestinal bleeding, coma and death.
Diagnostics
Diagnostic measures to detect primary biliary cirrhosis may have several stages:
- First of all patients with suspected cirrhosis of the liver, it is necessary to consult with several doctors - hepatologist, surgeon, gastroenterologist. Only they can reveal the disease, determine its extent, assign the following diagnostic measures and possible treatment.
- After the medical consultation, a patient with suspected cirrhosis necessarily directed to delivery of laboratory tests. Studies may include a detailed analysis of blood and urine, as well as a biopsy.
The third stage - instrumental diagnostics. It includes an examination of the spleen, kidney, liver, biliary tract, by performing ultrasound. Additionally conducted inspection of the internal organs using an endoscope, the introduction of the blood and gastric tract special substances which show real performance and functioning of the liver and biliary ducts.
Treatment of biliary cirrhosis
When diagnosing biliary cirrhosis therapies are based on reducing the intensity of its symptomatic manifestations, slowing further development, treatment and prevention of complications have joined them occurrence.
The course of treatment and selection of preparations is chosen individually by your doctor. Basically appoint:
Hepatic:
- Ursodeoxycholic acid (urosan, ursofalk) on overnight 3 capsules daily.
Immunosuppressants (only in primary biliary cirrhosis)
- Methotrexate 15mg a week or cyclosporin therapeutic dosage of 3 mg per 1 kg of body weight per day divided in 2 doses (morning and evening).
Anti-inflammatory therapy:
- Prednisolone 30 mg 1 time per day in the morning on an empty stomach after 8 weeks, the dose of the drug is reduced to 10 mg 1 time per day in the morning on an empty stomach.
Treatment of metabolic disorders of vitamins and minerals:
- kuprenil (D-penicillamine) 250 mg dissolved in one glass of water 3 times a day for 1.5 hours before mealtime;
- multivitamins (tsitrum, multitabs) 1 capsule 1 once a day;
- Stimol 1 sachet 2 times per day.
Treatment of pruritus:
- cholestyramine (questran) 4 mg 1.5 hours before meal 2 - 3 times per day;
- rifampin (Rimactane, benemitsin, tibitsin) po150 mg 2 times a day;
- antihistamines (Atarax, Suprastinum) 1 - 2 tablets 2 - 3 times a day.
In the case of secondary biliary cirrhosis is important to restore the normal flow of bile. To this end appointed endoscopy or surgery. If for some reason the data manipulation impossible, antibiotics to prevent cirrhosis of the transition to the thermal stage.
such manipulations can distinguish among types of surgery:
- choledochotomy;
- Holedohostomiya;
- Removal of stones from the biliary tract;
- Endoscopic probing;
- Endoscopic stenting;
- Expansion of the bile ducts;
- External drainage of the bile ducts.
Patients were assigned a diet №5. To use permitted vegetables, fruits, juices, fruit drinks, jelly, vegetable soups, low-fat meats, cereal.