Pediatric Budd-Chiari syndrome

　　1. Etiology

　　Compression, thrombosis, obstruction or wall membrane or reticular material of the hepatic segment of the hepatic vein or its main trunk and/or inferior vena cava. Various lesions adjacent to the main trunk of the hepatic vein and inferior vena cava, such as malignant tumors, hemangiomas, hematomas, amebic abscesses, cysts (such as hydatid cysts), gumma of syphilis, etc., can compress the hepatic vein. Blood diseases such as polycythemia (primary or secondary), sickle cell anemia, leukemia, hemolytic anemia, long-term use of oral contraceptives, abdominal trauma, chronic inflammatory bowel disease, acute alcoholic hepatitis, cholangitis, pancreatitis, pelvic infection, venous occlusive disease, and pregnancy can lead to thrombosis due to abnormal blood coagulation mechanisms or venous endothelial injury, resulting in the disease. In addition, 30% of cases have unknown etiology.

　　Second, pathogenesis.

　　The hepatic vein belongs to the portal venous system. In normal people, 70% of the splenic venous blood enters the portal vein. There are three aspects of pathophysiology that can form portal hypertension:

　　1. Phlebitis or thrombosis.

　　2. Liver cirrhosis.

　　3. Obstruction of the hepatic vein or its main trunk and/or the hepatic segment of the inferior vena cava. The main lesions in children with this syndrome are congenital hepatic vein stenosis and hepatic vein endometritis. It can lead to splenic dysfunction, often with pancytopenia, skin and mucosal bleeding, bone marrow hyperplasia, and other manifestations of splenic dysfunction. The common characteristics of both types of portal hypertension are hematemesis (upper gastrointestinal bleeding), splenomegaly, and ascites.

　　This disease may be complicated with hepatic vein, inferior vena cava thrombosis, congenital venous malformation, occlusive phlebitis: due to the obstruction of the hepatic veins,

　　1. Occlusive phlebitis:After the hepatic vein is blocked, it can cause an increase in portal vein pressure, obstruction of collateral circulation, leading to the formation of ascites and esophageal-gastric fundus varices, and umbilical umbilical cord-like venous radiating changes.

　　2. Inferior vena cava thrombosis:Due to the obstruction of the return of the inferior vena cava, the branches of the inferior vena cava can become dilated, and at the same time, edema can occur in the lower extremities.

　　1. Children from infancy to the elderly can suffer from this syndrome, and the incidence rate is roughly equal between males and females.

　　1. Acute BCS suddenly develops abdominal pain, rapid abdominal distension, physical examination may find jaundice, liver enlargement with tenderness, large amount of ascites, but negative hepatic jugular reflux sign, low blood pressure, less urine, blood bromosulfophthalein (BSP) retention, alanine aminotransferase (ALT) level rises, during surgery, the liver is enlarged, showing purple-blue, most patients die of circulatory disorders, liver function failure, or gastrointestinal bleeding. In chronic BCS, symptoms occur more slowly, and jaundice, splenomegaly, and spider nevi may occur.

　　Prevention: Preventing the formation of thrombosis is a disease prevention measure for this disease. In addition to general examinations before surgery, patients also need to undergo upper gastrointestinal barium meal or endoscopic examination to observe the degree of varices; ultrasound, CT, and MR2 to observe whether the hepatic veins and portal veins are unobstructed, and their spatial relationship; Doppler ultrasound to measure portal vein flow rate; on the day before surgery, perform selective superior mesenteric artery or splenic artery catheterization, perform selective portal vein angiography, and then send a metal wire through the femoral vein into the hepatic veins and take anterior and lateral views to understand the relationship between the hepatic veins and portal veins.

　　1、主要表现为BSP异常潴留，碱性磷酸酶和转氨酶轻度升高，血浆白蛋白可下降，但蛋白电泳γ球蛋白不升高，下腔静脉膜性闭塞者常有蛋白尿。

　　1. The main manifestation is abnormal retention of BSP, mild elevation of alkaline phosphatase and transaminase, plasma albumin may decrease, but the γ-globulin in protein electrophoresis does not increase, and patients with membranous occlusion of the inferior vena cava often have proteinuria.

　　Dietary taboos for children with Baudouin-Giannelli syndrome

　　1. There is no special diet for children with splenomegaly, sufficient calories, appropriate protein, rich and comprehensive vitamins, and appropriate fiber are enough. Fried dishes should be light.

　　2. Secondly, the diet structure should be reasonable. More vegetables and fruits should be eaten to supplement sufficient vitamins and fiber, which also helps to promote digestive function. When liver function is reduced, it often affects fat metabolism, so many chronic hepatitis patients may have post-hepatitis fatty liver. Therefore, the diet should be low in fat, low in sugar (excessive sugar entering the human body is easily converted into fat), and high in protein. Protein diet should include plant protein and animal protein, such as soy products, beef, chicken, fish, etc.

　　3. In addition, the amount of food should be appropriate. During liver disease, digestive function is weakened, and overeating often leads to indigestion and also increases the burden on the liver. Therefore, it is proposed that eating to 80% of fullness is the best. Overeating and feasting are harmful to the liver and gastrointestinal function.

　　1. Treatment

　　Radical and non-radical surgical procedures such as direct peritoneal rupture by finger or catheter, and various portosystemic shunts or occlusion procedures can be adopted. For those who cannot undergo surgery, efforts should be made to reduce ascites, which can be achieved by using diuretics or performing abdominal ultrafiltration and reinfusion, but the effect is temporary. There were reports of successful liver transplantation treatment in 1976.

　　2. Prognosis

　　Acute cases may quickly die from shock or hepatic encephalopathy after a few days of acute abdominal symptoms; the prognosis of chronic cases depends on the cause, the location and degree of obstruction, and whether it can be corrected by surgery, generally surviving for several months to several years.