Objective: To explore the impact of nuclear factor-kappa B (NF-κB) in patients with left-to-right shunt congenital heart disease (CHD) combining pulmonary arterial hypertension (PAH) and its clinical signiifcance. Methods: A total of 78 relevant patients were enrolled in this study. According to mean pulmonary artery pressure (mPAP) measured during operation, the patients were divided into 4 groups: Non-PAH group, the patients with mPAP≤25 mmHg,n=20, Mild PAH group, 25 mmHg mPAP≤35 mmHg,n=21, Moderate PAH group, 35 mmHg45 mmHg,n=23. 2 mL pulmonary artery blood was taken from each patient and plasma level of NF-κB was measured by ELISA to study the relationship between NF-κB and mPAP. Results: Pulmonary arterial plasma levels of NF-κB were increased accordingly as in Non-PAH group was (180.59 ± 10.16) ng/L, in Mild PAH group was (572.83 ± 34.80) ng/L, in Moderate PAH group was (980.85 ± 24.95) ng/L and in Severe PAH group was (1 253.4 ±130.8) ng/L, allP<0.01; NF-κB level was positively related to mPAP (r=0.856,P<0.01). Conclusion: Plasma level of NF-κB was involved in the pathological physiology process of left-to-right shunt CHD combining PAH, it couldbe used as one of the references for distinguishing the severity of PAH and for monitoring the dynamic changes of PAH in relevant patients.

Objective To describe the clinical, neuroimaging and genetic profiles of amyotrophic lateral sclerosis with frontotemporal lobe degeneration ( ALS-FTLD).Methods From August 2011 to May 2015, patients with FTLD or other types of neurodegenerative dementia were physically examined in detail and electromyography was performed to those with suspected dysarthria, limb atrophy or weakness.Cognitive and behavioral screenings were performed to all ALS patients.Patients with ALS-FTLD entered further analysis of neuroimaging and genetics.Results Among the 8 patients diagnosed as ALS-FTLD, 4 patients began with personality change or amnesia, while diseases in the remaining 4 cases began with limb weakness or dysarthria.Dementia type of 7 cases was behavioral variant FTLD ( bvFTD) and 1 case was diagnosed as semantic dementia.Electromyography of all the 8 patients showed diffuse neurogenic changes.Constructional neuroimaging of 6 patients showed cerebral atrophy predominantly in frontal and temporal lobes.Fluorodeoxyglucose-positron emission tomography was conducted in 5 patients, indicating hypometabolism mainly in frontal and ( or) temporal lobes.NeuroQ analysis revealed that bilateral frontal lobes were the most hypometabolic areas for ALS-FTLD.Among 4 patients who underwent genetic screening, 1 patient was C9ORF72 mutation carrier.Conclusions bvFTD is the major type of dementia in the context of ALS.Metabolic neuroimaging could assist accurate diagnosis, and it reveals that bilateral frontal lobes are the most hypometabolic areas for ALS-FTLD.C9ORF72 gene mutation is an important pathogenic mutation for ALS-FTLD, although it is rare in Chinese population.

Objective:To investigate the efficacy and safety of posaconazole in the prevention of invasive pulmonary aspergillosis (IPA) in patients with liver failure treated with glucocorticoids (GC).Methods:The study was an observational study. Patients with early and middle stages of liver failure hospitalized in the Department of Infectious Diseases of Hebei Medical University Third Hospital, who received GC treatment between February 2016 and February 2022 were included. The patients were divided into trial group (with posaconazole suspension (200 mg each time, three times daily)) and control group (without posaconazole) according to whether posaconazole was used during treatment. Two groups of patients were matched of 1∶2 ratio according to age, gender and baseline model for end-stage liver disease (MELD) score. The basic information, laboratory examination results, adverse reactions of posaconazole, incidence of invasive Aspergillus infection and therapeutic effect of patients were collected. Statistical analysis was performed using the chi-square test, logistic regression analysis was used to screen risk factors for IPA, the receiver operator characteristic (ROC) curve was used to evaluate the predictive ability of the risk factors, Kaplan-Meier survival curves was used to analyze patient′s survival, and Log-rank test was used to compare the survival rates between the trial group and control group. Results:A total of 108 patients (36 in trial group and 72 in control group) were enrolled. There were no statistical differences between the two groups in terms of the etiology of liver diseases, baseline laboratory findings and risk factors for invasive Aspergillus infection (all P>0.05). There were 21 cases of IPA during hospitalization, with a total infection rate of 19.4%(21/108), including 5.6%(2/36) in the trial group and 26.4%(19/72) in the control group. The difference of IPA incidences between the two groups was statistically significant ( χ2=6.65, P=0.010). Logistic regression analysis suggested that elevated C-reactive protein, GC application more than seven days and cumulative dose of GC were independent risk factors for IPA in patients with liver failure treated with GC (odds ratio ( OR)=1.080, 15.266, 1.004, respectively, all P<0.05). The ROC curve showed that the cut-off value of C-reactive protein was 6.00 mg/L, and cumulative dose of GC was 490 mg. There were no statistical differences between the two groups in terms of adverse effects such as neutropenia, thrombocytopenia, gastrointestinal bleeding, nausea and vomiting rates (all P>0.05), and there were no patients with visual disturbances or discontinuation of medication. Cumulative deaths were 20(18.5%), and 88(81.5%) patients survived in this study. There were 11(52.4%) deaths among 21 patients with IPA and nine (10.3%) deaths among 87 patients without IPA. The difference of survival rates between patients who developed and did not develop IPA was statistically significant ( χ2=21.31, P<0.001). Conclusions:Posaconazole may be helpful in reducing the incidence of concurrent IPA morbidity in patients with liver failure treated with GC, thereby improving survival rates with few adverse effects.

Surgery is an accepted standard in the treatment of adenocarcinoma of esophagogastric junction (AEG), but the efficacy of surgery alone for locally advanced AEG is limited. In-depth studies concerning combined therapy for AEG have been carried out worldwide, including neoadjuvant chemotherapy (nCT), neoadjuvant chemoradiotherapy (nCRT), perioperative chemotherapy (pCT), postoperative chemoradiotherapy, etc. Significantly, the contribution of nCRT and pCT to improving the prognosis of locally advanced AEG patients has been shed light on. Compared with that, multimodality treatment for AEG patients is not well established in China. An attempt was thus made to take an overview of the evidence-based research advance regarding integrated therapy of AEG.

Objective: To evaluate the safety and efficacy of ombitasvir/paritaprevir/ritonavir (OBV/PTV/r) 25/150/100 mg once daily combined with dasabuvir 250mg, twice daily in non-cirrhotic Chinese adult patients with newly diagnosed and treated chronic HCV genotype 1b infection. Methods: A randomized, double-blind, placebo-controlled, multicenter phase 3 clinical trial was conducted in mainland China, Korea, and Taiwan.Safety and efficacy of OBV/PTV/r plus DSV administered for 12 weeks were evaluated in a newly diagnosed and treated (interferon alpha /pegylated interferon alpha) and ribavirin non-cirrhotic adults with chronic HCVgenotype 1b infection. Patients randomly received OBV/PTV/r plus DSV for 12 weeks (Group A), or placebo for 12 weeks (Group B) followed by an open-label phase of OBV/PTV/r plus DSV for 12 weeks. Sustained response (SVR12) rate obtained at 12 weeks and (SVR24) 24 weeks after discontinuation of treatment, and the incidence of adverse events and laboratory abnormalities after double-blind and open-label phase treatment were assessed. Results: A total of 410 cases of Chinese patients were included and randomly assigned to group A and B (with 205 cases in each group) in a 1:1 ratio. The rates of SVR12 and SVR24 were 99% (95% CI: 94.8% - 99.8%) in the newly diagnosed patients in group A (205 patients) and the rates of SVR12 and SVR24 were 100% in treated patients (95% CI: 96.3% - 100%). Different baseline characteristics had no effect on SVR12 and SVR24 rates. Most of the adverse events occurred were mild, asymptomatic, and≥ 3 laboratory abnormalities during treatment were rare, including elevation of alanine aminotransferase (2 cases in double-blind stage A group), aspartate aminotransferase (Double-blind stage A (3 cases) and total bilirubin (1 case in open-label phase B group); however, those mild adverse events could be recovered after drug withdrawal or discontinuation. only1 person discontinued drugs due to adverse events (Group B, open-label phase). Conclusion The 12 weeks treatment course of OBV/PTV/r combined with DSV produced 99% ~ 100% rates of SVR12 and SVR24 in non-cirrhotic Asian adult patients with newly diagnosed and treated chronic HCV genotype 1b infection, and the tolerance and safety were good.