ObjectivePrimary biliary cholangitis (PBC) is an autoimmune disease with significant gender difference. X chromosome inactivation (XCI) plays important roles in susceptibility to diseases between genders. This work focuses on the differences of LncRNA XIST in several defined immune cells populations as well as its effects on naive CD4+ T cells proliferation and differentiation in patients with PBC.MethodsNKs, B cells, CD4+ T, and CD8+ T cells were separated by MicroBeads from peripheral blood mononuclear cells (PBMCs) of PBC patients and healthy control (HC). The expression levels of LncRNA XIST in these immune cells were quantified by qRT-PCR and their subcellular localized analyzed by FISH. Lentivirus were used to interfere the expression of LncRNA XIST, and CCK8 was used to detect the proliferation of naive CD4+ T cells in PBC patients. Finally, naive CD4+ T cells were co-cultured with the bile duct epithelial cells (BECs), and the effects of LncRNA XIST on the typing of naive CD4+ T cells and related cytokines were determined by qRT-PCR and ELISA.ResultsThe expression levels of LncRNA XIST in NKs and CD4+ T cells in PBC patients were significantly higher than those in HC, and were primarily located at the nucleus. LncRNA XIST could promote the proliferation of naive CD4+ T cells. When naive CD4+ T cells were co-cultured with BECs, the expressions of IFN-γ, IL-17, T-bet and RORγt in naive CD4+ T cells were decreased.ConclusionLncRNA XIST was associated with lymphocyte abnormalities in patients with PBC. The high expression of LncRNA XIST could stimulate proliferation and differentiation of naive CD4+ T cells, which might account for the high occurrence of PBC in female.

Objective: It is very important to accurately assess the transannular patch (TAP) in the surgical treatment of tetralogy of Fallot (TOF). The pulmonary annulus index (PAI; the actual pulmonary annulus diameter divided by the expected pulmonary annulus diameter), GA ratio (the ratio of pulmonary annulus and aortic annulus), PAAI (the ratio of pulmonary annulus cross-section and aortic annulus crosssection), and pulmonary annulus Z score (PA Z score) were compared. This study aimed to analyze and explore the application value of PAI in predicting the need for TAP in children undergoing TOF repair.Methods: In total, 130 patients who were diagnosed with TOF and underwent TOF repair in Beijing Anzhen Hospital affiliated to Capital Medical University from December 2018 to December 2019 were retrospectively studied. In total, 112 cases were included in this study and 18 cases were excluded, of which 16 cases were aged above 18 years and 2 cases suffered from Down syndrome. They were divided into TAP group and no TAP group; the values of the pulmonary annulus and aortic annulus were measured. GA ratio, PAI, PAAI, the pulmonary annulus Z score, and main pulmonary artery (MPA) Z score were calculated to perform statistical analysis.Results: A total of 112 patients were included in the study, aged 4-177 months, with an average of 22.87 ± 30.21 months; 66 males and 46 females; weighing 5.3-29 kg, with an average of 9.94 ± 4.08 kg; three cases died, one case died of sepsis caused by pulmonary infection, one case died of low cardiac output syndrome, and one case died of multiple organ failure. In total, 62 cases (55.8%) did not undergo TAP and 50 cases (44.2%) underwent TAP. The pulmonary annulus Z score, main pulmonary artery Z score, and PAI in the TAP group were smaller than those in the no TAP group (p < .05). Receiver operating curve (ROC) analysis showed that when the cut-off value of pulmonary annulus was −1.98, the area under the curve (AUC) was 0.88, the sensitivity was 80%, and the specificity was 71%; when the cutoff value of PAI was 0.53, AUC was 0.85, the sensitivity was 75%, and the specificity was 80%; when the cut-off value of GA ratio was 0.55, AUC was 0.85, the sensitivity was 76%, and the specificity was 80%. The area under the PAAI curve was 0.85, the sensitivity was 76%, and the specificity was 79%. When the pulmonary valve Z score, PAI, GA ratio, PAAI, and MPA Z score were all greater than the dividing value, TAP was avoided in more than 90% of children with TOF. When the pulmonary valve Z score, PAI, GA ratio, PAAI, and the main pulmonary artery Z score were all below the dividing value, more than 90% of children with TOF needed TAP. Conclusion:The predictive effect of PAI as a simple and effective predictor of TAP in TOF radical operation is the same as that of pulmonary annulus Z score, and combining it with the main pulmonary artery Z score was the most accurate method of prediction.main pulmonary artery Z score, pulmonary annulus index, pulmonary annulus Z score, tetralogy of Fallot, transannular pat...

Background Wilson’s disease (WD) is a rare autosomal recessive disease associated with defective biliary excretion of copper. The simultaneous occurrence of WD and systemic lupus erythematosus (SLE) has seldom been reported. Therefore, this study aimed to report the co-occurrence of SLE and WD with hepatic involvement in a patient so as to improve the understanding of the coexistence of these two conditions. Case presentation A 35-year-old woman with SLE was found to have liver fibrosis during a routinely abdominal ultrasound examination. Her laboratory evaluation showed low serum ceruloplasmin and high 24 h urine copper levels. The slit-lamp examination revealed the presence of Kayseri–Fleischer ring in her cornea. Liver biopsy demonstrated the enlargement of the portal area with hyperplasia of the fibrous tissue, infiltration of lymphoid plasma cells, swelling of hepatocytes, and steatosis, demonstrating liver fibrosis. Ensuing genetic testing confirmed the diagnosis of WD. Conclusions Clinicians should bear in mind that unexplained liver fibrosis in patients with SLE may be related to WD, so as to avoid a missed or delayed diagnosis.

Objective: It is very important to accurately assess the transannular patch (TAP) in the surgical treatment of tetralogy of Fallot（TOF）. Methods: 130 patients who were diagnosed with TOF and underwent TOF repair. 112 cases were included in this study. They were divided into TAP group and no TAP group; the values of pulmonary annulus and aortic annulus were measured. GA ratio, PAI, PAAI, the pulmonary annulus Z-score and main pulmonary artery (MPA) Z-score were calculated to do statistically analyze. Results: A total of 112 patients were included in the study.62 cases (55.8%) did not transannular patch, 50 cases (44.2%) undergoing transannular patch. The pulmonary annulus Z-score, main pulmonary artery Z-score and PAI in TAP group were smaller than those in no TAP group (P < 0.05). ROC analysis showed that when the cutoff value of pulmonary annulus at -1.98 ,the area under curve (AUC) was 0.88, the sensitivity was 80%, the specificity was 71%; when the cut-off value of PAI at 0.53 ,AUC was 0.85, the sensitivity was 75%, the specificity was 80%; when the cutoff value of GA ratio at 0.55 ,AUC was 0.85, the sensitivity was 76%, and the specificity was 80%. The area under the PAAI curve (AUC) was 0.85, the sensitivity was 76%, and the specificity was 79%. Conclusion: The predictive effect of pulmonary annulus index as a simple and effective predictor of TAP in TOF radical operation is the same as that of pulmonary annulus Z-score.