idiopathic nonsclerotic portal hypertension syndrome
Introduction
Introduction to idiopathic non-sclerosing portal hypertension syndrome Idiophothic Nonirrhotic Portal Hypertension (Syndrome), also known as intrahepatic anterior sinus obstructive portal hypertension. With clinical manifestations of portal hypertension, it can repeatedly vomit blood and black feces, have good tolerance to gastrointestinal bleeding without ascites, jaundice, coma and other decompensation performance; laboratory liver function can be normal. Portal vein thrombosis, portal vein stenosis, portal hemangioma and intrahepatic venous artery spasm can cause partial portal vein, complete obstruction or increased portal blood flow, which is an important cause of non-cirrhotic portal hypertension. basic knowledge The proportion of illness: 0.001% Susceptible people: no specific population Mode of infection: non-infectious Complications: portal vein thrombosis
Cause
The etiology of idiopathic non-sclerosing portal hypertension syndrome
Portal vein thrombosis. Portal thrombosis (PT) can occur in any part of the portal vein and refers to thrombosis in the portal vein, superior mesenteric vein, inferior mesenteric vein, or splenic vein. Portal vein thrombosis can cause obstruction of the portal vein, causing increased portal pressure and intestinal congestion, which is the main cause of extrahepatic portal hypertension.
Portal vein stenosis, portal hemangioma, and intrahepatic venous artery spasm can cause partial or complete obstruction of the portal vein or increased portal blood flow, which is an important cause of non-cirrhotic portal hypertension.
Prevention
Idiopathic non-sclerosing portal hypertension prevention
Care should be taken to avoid the application of drugs that damage the liver. Patients who have been found should be given appropriate protective measures, such as appropriate reduction of labor intensity, regular follow-up of health checkups, early detection and early treatment, active treatment of primary diseases, prevention of complications, and maintenance of health. And extend life.
Complication
Idiopathic non-sclerosing portal hypertension syndrome Complications portal vein thrombosis
Can be complicated by portal vein thrombosis, portal vein stenosis, portal hemangioma and intrahepatic venous artery spasm and other diseases.
1. Portal thrombosis (PT) can occur in any part of the portal vein, which refers to thrombosis in the portal vein, superior mesenteric vein, inferior mesenteric vein or splenic vein.
2. Hemangioma (haemangioma) is a congenital benign tumor or vascular malformation, which is more common in the birth of a baby or shortly after birth. It originates from residual embryonic hemangioblasts, and active endothelium-like germs invade adjacent tissues to form endothelial splines. Suspension, after the tube is connected with the remaining blood vessels to form a hemangioma, the intratumoral blood vessels are self-contained and are not connected to the surrounding blood vessels.
Symptom
Symptoms of idiopathic non-sclerosing portal hypertension syndrome Common symptoms Upper gastrointestinal bleeding, jaundice, coma, splenomegaly, repeated vomiting, portal hypertension, ascites
With clinical manifestations of portal hypertension, it can repeatedly vomit blood and black feces, have good tolerance to gastrointestinal bleeding without ascites, jaundice, coma and other decompensation performance; laboratory liver function can be normal.
1. Clinical and radiological examination has a positive portal hypertension, including obvious splenomegaly, esophageal varices, repeated upper gastrointestinal bleeding, and normal or normal liver function.
2. Splenic portal vein angiography showed no obstruction and stenosis of the extrahepatic portal vein.
3. According to liver biopsy, the characteristic changes of pathological examination can be diagnosed.
Examine
Examination of idiopathic non-sclerosing portal hypertension syndrome
Idiopathic non-sclerosing portal hypertension syndrome examination project:
Biochemical examination of vitamin A, abdominal plain film, liver function test, ultrasound diagnosis of liver disease, liver, gallbladder, pancreas, spleen MRI examination.
1. Radiological examination. The development of radiodiagnosis is very rapid and has evolved from a single X-ray diagnosis to a comprehensive diagnosis of medical imaging.
2. splenic portal vein angiography.
3. Liver biopsy.
1 irregular liver capsule thickening.
2 The portal vein area has fibrous tissue hyperplasia, and there are fibrous tendons extending into the lobules. The fibrous tendon is very fibrous and can be found by reticular staining.
3 The lobular structure is slightly deformed, and the central vein is squeezed or irregular.
Diagnosis
Diagnosis and differentiation of idiopathic non-sclerosing portal hypertension syndrome
diagnosis
According to liver biopsy, characteristic changes in pathological examination can be diagnosed.
Differential diagnosis
1. Other causes of anterior sinus obstruction such as congenital or recurrent hepatic fibrosis, multiple children with polycystic kidney disease before the age of 10, liver biopsy see massive fibrous tissue hyperplasia in the portal area, in the form of a cord Stretching into the lobes is the main basis.
2. Portal hypertension caused by post-sinus obstruction, such as hepatic vein occlusion, hepatic vein branch obstruction, sudden hepatic pain, liver progressive liver and refractory ascites.
3. Portal hypertension caused by sinus or intrahepatic sinus obstruction Long-term use of azathioprine, 6-MP, a large amount of vitamin A caused by abnormal increase of collagen fibers in the gap of Disse, portal vein caused by fibrosis around the sinusoid High pressure, should be carefully asked about the medical history to identify.
4. Extrahepatic portal vein thrombosis or stenosis, often accompanied by abdominal distension, abdominal pain, blood in the stool and ascites, etc., can be confirmed by splenic portal vein angiography.
