Pediatric Budd-Chiari Syndrome

Introduction to Pediatric Bad-Giyari Syndrome Pediatric Bad-Giyari syndrome, also known as Budd-Chiarisyndrome. It refers to a group of diseases characterized by portal hypertension or portal and inferior vena cava hypertension caused by obstruction of the inferior vena cava above the hepatic vein or its opening. The most common are the inferior vena cava diaphragm and hepatic vein thrombosis above the hepatic vein opening. basic knowledge The proportion of sickness: 0.00001% Susceptible people: children Mode of infection: non-infectious Complications: cirrhosis, hypersplenism

The cause of pediatric Budd-Gihale syndrome

(1) Causes of the disease

The hepatic vein or its trunk and/or inferior vena cava liver segment is compressed, thrombosis, occlusion of new organisms or membrane or network on the wall, and the upper part of the posterior part of the liver adjacent to the hepatic vein trunk and inferior vena cava Such as malignant tumor, hemangioma, hematoma, amoebic abscess, cyst (such as hydatid cyst), syphilitic gum swelling, etc. can oppress the hepatic vein, polycythemia (primary or secondary), sickle cell anemia, leukemia , hemolytic anemia and other blood diseases, long-term use of contraceptives, abdominal blunt trauma, chronic inflammatory bowel disease, acute alcoholic hepatitis, cholangitis, pancreatitis, pelvic infections, venous occlusive disease and pregnancy, can be abnormal through blood coagulation mechanism, Or venous endothelium damage, resulting in thrombosis leading to the disease, in addition, 30% of cases are unknown.

In children, congenital hepatic vein stenosis and hepatic venous endarteritis are the main causes.

(two) pathogenesis

The hepatic vein belongs to the portal vein system. Normally, 70% of the splenic venous blood vessels enter the vein. There are three aspects of pathophysiology that can form portal hypertension: 1 phlebitis or thrombosis; 2 cirrhosis; 3 hepatic vein or its trunk and (or Inferior vena cava liver obstruction, the child with congenital hepatic vein stenosis, hepatic venous stenosis as the main lesion, secondary spleen hyperfunction, often with complete blood cell reduction, skin mucosal hemorrhage, bone marrow hyperplasia and other spleen function The performance of hyperthyroidism, the common features of intrahepatic and extrahepatic portal hypertension are hematemesis (upper gastrointestinal bleeding), splenomegaly and ascites.

Pediatric Bad-Gielli syndrome prevention

The causes of this disease are different, should pay attention to prevention and treatment of various infectious diseases, such as amoebic abscess, hydatid cyst, syphilitic gum, chronic inflammatory bowel disease, pelvic infection, etc., pay attention to regular diet and diet hygiene, prevent Alcoholic hepatitis, pancreatitis, cholangitis, etc.

Complications of pediatric Bad-Gielli syndrome Complications, liver cirrhosis, hypersplenism

Most patients with acute BCS die from circulatory disorders, liver failure or gastrointestinal bleeding. Chronic BCS can be complicated by cirrhosis and hypersplenism.

Pediatric Bard-Gihale syndrome symptoms Common symptoms Astragalus abdominal pain splenomegaly liver-jugular venous return positive ascites spider sputum liver failure intestinal bleeding

The disease can occur in infants to the elderly, and the incidence rate is roughly equal for men and women.

Acute BCS sudden abdominal pain, rapid expansion of the abdomen, physical examination can be found jaundice, liver with tenderness, a large amount of ascites, but negative signs of hepatic jugular venous return, low blood pressure, less urine, blood sulfonium bromide (BSP) retention, transaminase increased, When the operation is seen, the liver is large and purple-blue. Most of the patients die from circulatory disorders, liver failure or gastrointestinal bleeding. In chronic BCS, the symptoms occur slowly, and may have jaundice, splenomegaly, and spider mites.

Pediatric Bard-Gihale syndrome examination

Mainly manifested as abnormal retention of BSP, alkaline phosphatase and transaminase increased slightly, plasma albumin decreased, but protein gamma globulin did not increase, and inferior vena cava membranous occlusion often had proteinuria.

Can do laparoscopy, B-mode ultrasound, radionuclide scanning, angiography, such as hepatic venography, percutaneous hepatic venography, inferior vena cava angiography, selective celiac angiography.

Diagnostic identification of pediatric Budd-Gihale syndrome

The degree of liver function damage of this disease is relatively mild, combined with laboratory examination and auxiliary examination results.

The disease must be differentiated from cardiogenic hepatic congestion, cirrhosis and hepatic venous occlusive disease.

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