关键词:

Abstract:

Objective To summarize and analyze the clinical and pathological characteristics of patients with idiopathic non⁃cirrhotic portal hypertension（INCPH），and to provide basis for clinicians to identify the disease in the early stage. Methods The data of 115 patients with INCPH diagnosed in China⁃Japan Friendship Hospital from January 2016 to December 2021 were analyzed retrospectively，including demographic characteris⁃ tics，clinical manifestations，and laboratory，imaging and pathological manifestations. Results Among 115 patients with INCPH，there were 51 males（44.35%）and 64 females（55.65%），with a male⁃female ratio of about 1∶1.25. Patients aged from 12 to 79 years，with an average age of（47.89 ± 21.14）years. The majority Child⁃Pugh score of liver function was A（88.89%）. The onset clinical symptoms were splenomegaly（34/38，89.47%），hemocytopenia （38/45，84.44%）and upper gastrointestinal bleeding（25/41，60.97%）. The median values of WBC，NEUT，PLT and protein C activities were lower than the lower limit of normal，and the abnormal rate was more than 50%. The median values of ALT，AST，GGT，ALP，ALB，TBIL，CHE，TG and TC were all in the normal range，with low abnormal rate. Gastroscope found that the detection rate of esophageal and gastric fundus varices was as high as 77.42%；the detection rates of splenomegaly and portal hypertension in imaging were higher than 50%，and TE ⁃ LSM ≥ 7.3 kpa accounted for 19.0%. In addition to the main pathological changes of the liver，CD34（+）（25/27， 92.59%）of hepatic sinuses，dilatation of hepatic sinuses（33/91，36.26%），and irregular fibrous septum（23/91， 25.27%）were observed. No cirrhosis was found in all specimens. Conclusion INCPH patients has severe portal hypertension，mild liver function injury and good liver reserve function. The diagnosis of this disease requires closecombination of clinical and pathological findings.

Key words:

non?cirrhotic portal hypertension, hypersplenism, esophageal and gastric varices, portal obliterative venopathy, porto?sinusoidal vascular liver disease