Primary intrahepatic cirrhosis syndrome

　　Primary intrahepatic cirrhosis syndrome may be due to abnormal autoimmune response leading to non-suppurative destructive inflammation of the interlobular bile ducts and intralobular bile ducts, accompanied by bile duct hyperplasia; destruction and scarring; destruction of liver lobular structure and regeneration of nodules; and significant bile stasis in the histological changes of cirrhotic tissue. It can also be caused by long-term alcohol abuse, as well as hepatitis virus infection, leading to non-specific inflammation and fibrosis of intrahepatic bile ducts.

　　Primary intrahepatic cirrhosis syndrome can cause hepatic encephalopathy, upper gastrointestinal hemorrhage, hepatorenal syndrome, is prone to develop various infections, such as bronchitis, pneumonia, tuberculous peritonitis, primary peritonitis, biliary tract infection, and sepsis caused by Gram-negative bacilli, etc.

　　1. Upper gastrointestinal hemorrhage

　　Upper gastrointestinal bleeding in liver cirrhosis is mostly due to the rupture of esophageal and gastric fundus varices, but it should be considered whether there are complications such as peptic ulcers, acute hemorrhagic and erosive gastritis, or Mallory-Weiss syndrome. Bleeding from varices is often caused by rough, hard, or angular foods that cause trauma, erosion of the esophagus by gastric acid reflux, severe vomiting, etc. Vomiting blood and black stools may occur. If the amount of bleeding is not much, only black stools may occur. Large amounts of bleeding can cause shock. Under conditions of ischemia and hypoxia in the liver, liver function often deteriorates. The loss of plasma proteins due to bleeding can lead to the formation of ascites. After the blood in the intestines is decomposed by bacteria, ammonia is absorbed by the intestinal mucosa, which can induce hepatic encephalopathy and even lead to death. After bleeding, the enlarged spleen may shrink or cannot be palpated.

　　2. Infection

　　Due to the decline in immune function of the body, hyperfunction of the spleen, and the establishment of collateral circulation between the portal and systemic veins, there is an increased opportunity for pathogenic microorganisms to enter the systemic circulation, hence the tendency to develop various infections, such as bronchitis, pneumonia, tuberculous peritonitis, primary peritonitis, biliary tract infection, and sepsis caused by Gram-negative bacilli, etc. Primary peritonitis refers to acute peritoneal inflammation in the abdominal cavity of patients with liver cirrhosis without organ perforation, with an incidence rate of 3% to 10%. It often occurs in patients with a large amount of ascites, and is usually caused by Escherichia coli. The cause is that the phagocytic function of phagocytes is weakened during liver cirrhosis, bacteria in the intestines multiply abnormally, and enter the abdominal cavity through the intestinal wall. Additionally, due to changes in vascular structures inside and outside the liver, bacteria can also cause sepsis or introduce contaminated lymph fluid from beneath the liver capsule or from the portal lymph nodes into the abdominal cavity, leading to infection. Clinical manifestations include fever, abdominal pain, distension, tenderness and rebound pain on the abdominal wall, increased ascites, elevated blood leukocyte count, cloudy ascites, which is between exudative and transudative fluids. Ascites culture may show bacterial growth. A few patients may not have abdominal pain or fever, but instead present with hypotension or shock, refractory ascites, and progressive liver dysfunction.

　　18. 3, hepatorenal syndrome

　　When liver cirrhosis complicated with refractory ascites is not treated appropriately or the efficacy is poor, it is easy to develop hepatorenal syndrome. The characteristics are oliguria or anuria, low blood sodium and low urinary sodium. There are no organic changes in the kidneys, so it is also called functional renal failure. The pathogenesis is not yet fully understood.

　　Primary biliary cirrhosis is an autoimmune liver disease, a chronic intrahepatic cholestasis of unknown etiology. Clinically, it has an insidious onset and is more common in female patients. The clinical manifestations include fatigue, generalized pruritus, jaundice, hyperpigmentation, and (or) xanthomas. Abdominal pain, nausea, vomiting, edema, ascites, and variceal bleeding in the esophagus can also be the initial manifestations. Liver biopsy shows characteristic pathological changes, and the disease can be diagnosed by excluding extrahepatic obstructive jaundice and secondary biliary cirrhosis.

　　The prevention of primary intrahepatic cirrhosis syndrome is very important. Regular physical examinations should be conducted, generally once a year, including liver function tests and ultrasound, to achieve early detection and treatment. Specific preventive measures for primary intrahepatic cirrhosis syndrome include the following aspects:

　　10. For patients with elevated blood ammonia or extremely poor liver function, protein intake should be restricted to prevent liver coma. Those with ascites should follow a low-sodium or salt-free diet.

　　9. In terms of diet, provide sufficient nutrition, diversify food choices, supply high-value proteins containing amino acids, a variety of vitamins, low-fat, and low-fiber diets. It is necessary to prevent rough and fibrous foods from damaging the esophageal varices, causing massive bleeding.

　　8. Pay attention to changes in bleeding, purpura, fever, and symptoms of the central nervous system, and contact the doctor in a timely manner.

　　7. Measure abdominal circumference and urine output daily. Abdominal obesity is a method to differentiate fatty liver.

　　During laboratory tests, the serum bilirubin level increases, mainly due to the increase in direct bilirubin. Alkaline phosphatase, cholesterol, and IgM are also elevated. Mitochondrial antibodies, smooth muscle antibodies, and antinuclear antibodies are positive, and albumin is reduced while γ-globulin is elevated. If diagnosis is difficult and there is a high suspicion of the disease, liver biopsy can be performed for pathological examination. Characteristic pathological changes can be observed, and the disease can be diagnosed by excluding extrahepatic obstructive jaundice and secondary biliary cirrhosis.

　　Patients with primary intrahepatic cirrhosis syndrome should consume foods rich in folic acid, with mild taste and easy to digest, and limit the intake of foods containing high-quality protein. They should avoid eating foods containing alcohol or fried and indigestible foods. The specific dietary taboos for patients with primary intrahepatic cirrhosis syndrome include the following aspects:

　　1. Mainly eat rice and noodles as staple food

　　Satisfy the sugar intake of about 400g per day, aiming to protect and promote the regeneration of liver cells. However, due to the damaged liver function of patients with liver cirrhosis, excessive sugar conservation can easily lead to obesity, hyperlipidemia, atherosclerosis, and diabetes. Therefore, it is cautious to increase the sugar intake or eat sweet foods in addition to staple foods.

　　2. Protein foods should be less and refined

　　60g of high-efficiency protein per day in the diet can meet the needs. Fish, lean meat, eggs, milk, soy products, and other ideal high-quality proteins can be alternated for consumption. Due to the decrease in the liver's ability to process protein during liver cirrhosis, the content of protein in the diet of patients with advanced liver cirrhosis should be controlled according to the condition. When there is a tendency towards hepatic encephalopathy, it is not advisable to exceed 20g per day.

　　3. Eat more fresh vegetables and fruits

　　Fresh vegetables and fruits are rich in vitamins, minerals, and trace elements, which are the best food. The daily consumption is about 700-900g.

　　4. Limit the intake of fats and oils

　　Limit the intake of fats and oils to no more than 20g per day, as fats in the body need to be broken down and utilized by the liver through the secretion of bile. If liver cells with weakened liver function are unable to decompose and process the fats ingested, it will cause fatty infiltration of the liver. Especially animal fats can further increase the burden on the liver.

　　5. Limit water and salt intake

　　For patients with edema or swelling, it is necessary to control the intake of sodium and water. However, if salt restriction or consumption of unsalted soy sauce is long-term, it is easy to lead to hyponatremia and hyperkalemia. Therefore, the positive approach is to try to enhance the appetite and digestion of the patients, often use less salted sweet and sour pickles as side dishes, and at the same time ensure the absorption and utilization of protein to increase the colloid osmotic pressure in the plasma, thereby fundamentally eliminating edema.

　　The Western medical treatment for primary intrahepatic cirrhosis syndrome is immunosuppressive therapy with adrenal cortical hormones or azathioprine. Itchiness of the skin can be treated with antihistamines or cholestyramine, and aluminum hydroxide can also be tried. Other symptomatic treatment and prevention of complications such as upper gastrointestinal bleeding, hepatic encephalopathy, and ascites can be carried out. The principle of treatment is to protect the liver, maintain the disorder of the internal environment, avoid severe liver function abnormalities leading to senile encephalopathy and threaten life, so it is very important to monitor the level of blood ammonia.