| Objective:The clinical data of 217 patients with non-cirrhotic portal hypertension were retrospectively analyzed to better guide the clinician's diagnosis and treatment,and to reduce the rate of misdiagnosis and missed diagnosis.Methods:The general data,laboratory examination and imaging examination of 217 patients with non-cirrhotic portal hypertension in the First Hospital of Jilin University from January 2012 to December 2018 were retrospectively analyzed.217 patients with non-cirrhotic portal hypertension and 500 patients with cirrhotic portal hypertension at the same time were used as study group and control group,respectively,comparing general data,laboratory examination,imaging examination and complications.217 patients were divided into pancreatic and non-pancreatic groups according to whether they were pancreatic diseases,and general data,laboratory tests,imaging examinations and complications were compared.Results:(1)General information and complications:Distribution of 217 primary diseases: 140 cases of pancreatic portalhypertension(64.52%),19 cases of primary portal vein thrombosis(8.76%),17 cases of cavernous transformation of portal vein(7.83%),12 cases of primary myelofibrosis(5.53%),10 cases of idiopathic non-cirrhotic portal hypertension(4.61%),9 cases of hepatic sinusoidal obstruction syndrome(4.15%),6 cases of congenital hepatic fibrosis(2.76%),other types 4 cases(1.84%)(including 3 cases of Gaucher disease,1 case of Sea-blue histiocytosis).In the etiology of pancreatic portal hypertension,92 cases of pancreatic tumors(65.71%),26 cases of pancreatic pseudocysts(18.57%),19 cases of pancreatitis(13.57%),3 cases of autoimmune pancreatitis(2.15%).Abdominal pain,bloating,splenomegaly,ascites,and gastrointestinal bleeding are the most common clinical manifestations.There were 101 cases(72.14%)of abdominal pain and 48 cases(34.29%)of splenomegaly in patiens with pancreatic portal hypertension.There were 14 cases(73.68%)of abdominal pain and 14 cases(73.68%)of splenomegaly in patiens with primary portal vein thrombosis.There were 16 cases(94.12%)of splenomegaly and 12cases(70.59%)of gastrointestinal bleeding in patiens with primary cavernous transformation of portal vein.There were 12 cases(100%)of splenomegaly in patients with primary myelofibrosis.There were 8 cases(80%)of splenomegaly,6 cases(60%)with gastrointestinal hemorrhage,6 cases(60%)with hypersplenism in patients with idiopathic non-cirrhotic portal hypertension.There were 8 cases(88.89%)of abdominal distension,9 cases(100%)of ascites in patients with hepatic sinusoidal obstruction syndrome.There were 6 cases(100%)of splenomegaly,6 cases(100%)of hypersplenism in patients with congenital liver fibrosis.3 cases Patients with Gaucher disease had splenomegaly,and 2 patients had ascites;1 patient with sea-blue histiocytosis was young women with abdominal pain,jaundice,splenomegaly and hypersplenism,her Child grade was grade A.No hepatic encephalopathy occurred in all patients.The median hemoglobin of primary cavernous transformation of portal vein,primary myelofibrosis and congenital hepatic fibrosis were 79g/L,75.5g/L and 77.5g/L,respectively.The median platelet count in patients with idiopathic non-cirrhotic portal hypertension and congenital hepatic fibrosis was 67×109/L and 66×109/L,respectively.The median AST was 98(40.4,659)U/L and median ALT was 116.2(25.5,577.7)U/L in patients with hepatic sinusoidal obstruction syndrome,while transaminase was normal or only mildly elevated in patients with other diseases.The median cholinesterase level in patients with congenital hepatic fibrosis was 2570U/L.Among the 3patients with gaucher's disease,1 patient presented moderate anemia(79g/L),and the other 2 patients presented mild anemia.Transaminase was slightly elevated in all 3 gaucher's disease patients.Cholinesterase decreased significantly in 3 gaucher's disease patients,and in 1 patient,the levels ofcholinesterase and albumin decreased significantly.Platelet and leukocyte count decreased significantly in 1 patient with sea-blue histiocytosis,this petients' s liver function and blood coagulation were changed slightly.(2)Analysis of non-cirrhotic portal hypertension group and cirrhotic portal hypertension group:Patients in the non-cirrhotic group were younger than those in the cirrhotic group(P<0.05).In the non-cirrhotic group,fatigue,abdominal distension,edema,splenomegaly,hypersplenism,ascites were less common,and abdominal pain was more common(P<0.05).In the non-cirrhotic group,the degree of blood routine,blood coagulation routine and liver function abnormality was small(P<0.05).Grade C of child-pugh was less common in the non-cirrhotic group(P<0.05).Spontaneous peritonitis,urinary tract infection,hepatic encephalopathy and other complications were less common in the non-cirrhotic group(P<0.05).(3)Analysis of pancreatic portal hypertension group and non-pancreatic portal hypertension group:Pancreatic group was older,and diabetes and hypertension were more common(P<0.05).Weakness,edema,gastrointestinal bleeding,splenomegaly and hypersplenism were more common in the non-pancreatic group,and abdominal pain was more common in the pancreatic group(P<0.05).In the non-pancreatic group,the decrease of hemoglobin and platelets was moreobvious,the coagulation function was worse,and the liver function changed more(P<0.05).Conclusions:(1)In non-cirrhotic portal hypertension,pancreatic portal hypertension is relatively common,and other diseases are rare such as congenital hepatic fibrosis and Gaucher disease.(2)Non-cirrhotic portal hypertensive liver injury is mild,hepatic encephalopathy,spontaneous peritonitis,urinary tract infection and other complications are rare.However,hepatic sinusoidal obstruction syndrome is relatively severe.(3)Pancreatic portal hypertension is more common in men than in women.Pancreatic portal hypertension is often caused by pancreatic tumors,pancreatic pseudocysts,and swollen pancreatitis. |
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