Objective: Compare the clinical and pathological features of patients with porto-sinusoidal vascular disease (PSVD) and liver cirrhosis (LC) to reduce the rate of misdiagnosis and underdiagnosis.
Methods: A total of 45 PSVD and 48 LC inpatients were included in the study as the study group and control group respectively. Clinical, imaging and liver pathology data were collected for each patient.
Results: The ratio of male to female in the two groups was 1:1.25 and 0.78:1, respectively, and the average age of PSVD patients was lower.Compared to patients with cirrhosis, PSVD patients had better overall liver function. Compared to LC patients, PSVD patients had better overall liver function. Patients with PSVD had better overall liver function, and although most patients in both groups had a Child-Pugh score of B, more than two times fewer patients in the PSVD group had a Child-Pugh score of C and/or a model for end-stage liver disease(MELD) ≥ 10 than in the LC group. The initial diagnosis rate of PSVD patients was 6.67%, and nearly half of PSVD patients were misdiagnosed as LC, while the initial diagnosis rate of liver cirrhosis was 95.83%, and the diagnostic rate was higher (P<0.001). Both groups had obvious manifestations of portal hypertension on imaging, and there were significant differences in portal vein flow velocity, extrahepatic bile duct diameter and splenomegaly (P<0.05). The main specific manifestations of liver histopathology in PSVD patients were portal occlusion, nodular regenerative hyperplasia, incomplete septal cirrhosis/fibrosis, etc. The non-specific manifestations were fine bile duct reaction fine bile duct reaction and liver tissue inflammatory activity grading and liver fibrosis stage (GS) ≤ G2S2 (33,73.33%).
Conclusion: There are certain differences in disease characteristics and similarities between PSVD and LC, and the diagnosis still needs to be made in conjunction with liver pathology on the basis of routine investigations.
Immune-mediated liver injury is associated with excessive activation of invariant natural killer T (iNKT) cells, and the role and regulatory mechanisms of UDP-glucosylceramide glucosyltransferase (UGCG) in iNKT cell-mediated liver injury remain poorly defined. Here, we report UGCG induced endoplasmic reticulum (ER) stress, which is beneficial for hepatocytes to resist injury caused by iNKT cells, thereby improving immune-mediated liver injury outcomes in mice. UGCG levels were elevated in mice treated with concanavalin A, with excessive activation of iNKT cells. However, after UGCG inhibition, liver injury were alleviated along with reduced activation of iNKT cells. Interestingly, hepatocytes showed marked autophagy upon inhibition of UGCG. Mechanistically, inhibition of UGCG caused an increase in glucose-regulated protein 78, LC3B-II/LC3B-I ratio and phosphorylated NF-κB p65, thereby triggering ER stress-mediated autophagy. These findings showed ER stress links UGCG with hepatic autophagy.
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