Objective To analyze the clinical characteristics of IgG4-related disease (IgG4-RD),guide the selection of therapeutic drugs,and to explore the significance of potential tumor identification for IgG4-RD.Methods A total of 92 patients diagnosed with IgG4-RD and admitted to this hospital from January 1,2017 to December 31,2021were selected as the research subjects by using the Yidu Cloud system.The clinical data conducted the summary analysis. The clinical characteristics of IgG4-RD were summarized.Results The mean age of IgG4-RD was definitely diagnosed in the 92 patients was (58.1±11.3)years old,with 65 male ca-ses (70.7％) and 27 female cases (29.3％).The most commonly affected organ tissues were lymph nodes (37 cases,40.2％),pancreas (33 cases,35.9％) and salivary glands (31 cases,33.7％).In the patients woth the 92 patients,28 cases (30.4％) had involvement of a single organ tissue,while 32 cases (34.8％) had involvement of two or more organs.In the 92 patients,89 cases received steroid therapy,and 71 cases received immunosup-pressive therapy,in which 45 cases (63.4％) used cyclophosphamide.The initial treatment effective rate (72.7％ vs. 55.6％) and one-year non-recurrence rate (38.2％ vs. 20.0％) of the steroid combined immuno-suppressive therapy group were better than those of the single steroid group,but the differences were not sta-tistically significant (P>0.05).The proportion of the patients with tumor comorbidity and IgG4 level>40 g/L (18.2％) was significantly higher than that of the non-tumor comorbidity (1.2％),and the difference was statistically significant (P<0.05).However,there was no statistically significant difference in the proportion of patients with tumor comorbidity compared to the non-tumor comorbidity in other IgG4 level groups (P>0.05).Conclusion IgG4-RD is more common in middle-aged and elderly men,lymph nodes,pancreas and sal-ivary glands are commonly involved,and most patients have the double organs and multiple organs involve-ment. The combination use of hormone and immunosuppressant in treatment is recommended .The IgG4 lev-el>40 g/L in the patients with IgG4-RD may has the suggestive significance for complicating tumor.

Objective:To summarize the best evidence for the prevention and management of radiation esophagitis in patients with chest tumors, so as to provide basis for the prevention and management of radiation esophagitis in patients.Methods:The research question was proposed based on the population, intervention, professional, outcome, setting and type of evidence (PIPOST) model, and guidelines, expert consensus, clinical decision-making, evidence summary, systematic review, and randomized controlled trials on the prevention and management of radiation esophagitis in chest tumor patients were systematically searched on UpToDate, BMJ Best Practice, Cochrane Library, PubMed, American Society of Clinical Oncology, China National Knowledge Infrastructure, WanFang Data, and other databases and websites based on the "6S" model. The search period was from database establishment to August 1, 2023. Two researchers independently evaluated the quality of the literature and extracted and integrated evidence from the literature that met the quality standards.Results:A total of ten articles were included, including three guidelines, one expert consensus, one systematic review, two systematic evaluations, and three randomized controlled trials. Twenty-three pieces of evidence were summarized from six dimensions of building multidisciplinary teams, screening and evaluation, nutritional support, drug and non-drug prevention, integrated traditional Chinese and western medicine treatment, and health education.Conclusions:The included evidence can provide a theoretical basis for the prevention and management of radiation esophagitis in patients undergoing chest tumor radiotherapy. Clinical medical and nursing personnel can provide personalized interventions to patients based on clinical practice.

Objective: To examine the feasibility and surgical approach of removing type D trigeminal schwannoma through nasal cavity and nasal sinus under endoscope. Methods: Eleven patients with trigeminal schwannoma who were treated in the Department of Otorhinolaryngology, Qilu Hospital of Shandong University from December 2014 to August 2021 were analyzed retrospectively in this study. There were 7 males and 4 females, aged (47.5±13.5) years (range: 12 to 64 years). The neoplasm involved the pterygopalatine fossa, infratemporal fossa, ethmoidal sinus, sphenoid sinus, cavernous sinus, and middle cranial fossa. The size of tumors were between 1.6 cm×2.0 cm×2.0 cm and 5.7 cm×6.0 cm×6.0 cm. Under general anesthesia, the tumors were resected through the transpterygoid approach in 4 cases, through the prelacrimal recess approach in 4 cases, through the extended prelacrimal recess approach in 2 cases, and through the endoscopic medial maxillectomy approach in 1 case. The nasal endoscopy and imaging examination were conducted to detect whether neoplasm recurred or not, and the main clinical symptoms during follow-up. Results: All the surgical procedures were performed under endonasal endoscope, including Gross total resection in 10 patients. The tumor of a 12-year-old patient was not resected completely due to huge tumor size and limited operation space. One patient was accompanied by two other schwannomas located in the occipital region and the ipsilateral parotid gland region originating from the zygomatic branch of the facial nerve, both of which were removed concurrently. After tumor resection, the dura mater of middle cranial fossa was directly exposed in the nasal sinus in 2 cases, including 1 case accompanied by cerebrospinal fluid leakage which was reconstructed by a free mucosal flap obtained from the middle turbinate, the other case was packed by the autologous fat to protect the dura mater. The operation time was (M(IQR)) 180 (160) minutes (range: 120 to 485 minutes). No complications and deaths were observed. No recurrence was observed in the 10 patients with total tumor resection during a 58 (68) months' (range: 10 to 90 months) follow-up. No obvious change was observed in the facial appearance of all patients during the follow-up. Conclusion: Type D trigeminal schwannoma involving pterygopalatine fossa and infratemporal fossa can be removed safely through purely endoscopic endonasal approach by selecting the appropriate approach according to the size and involvement of the tumor.
Male ; Female ; Humans ; Child ; Retrospective Studies ; Endoscopy/methods* ; Nasal Cavity/surgery* ; Neurilemmoma/surgery* ; Cranial Nerve Neoplasms/surgery*

Objective: This cross-sectional investigation aimed to determine the incidence, clinical characteristics, prognosis, and related risk factors of olfactory and gustatory dysfunctions related to infection with the SARS-CoV-2 Omicron strain in mainland China. Methods: Data of patients with SARS-CoV-2 from December 28, 2022, to February 21, 2023, were collected through online and offline questionnaires from 45 tertiary hospitals and one center for disease control and prevention in mainland China. The questionnaire included demographic information, previous health history, smoking and alcohol drinking, SARS-CoV-2 vaccination, olfactory and gustatory function before and after infection, other symptoms after infection, as well as the duration and improvement of olfactory and gustatory dysfunction. The self-reported olfactory and gustatory functions of patients were evaluated using the Olfactory VAS scale and Gustatory VAS scale. Results: A total of 35 566 valid questionnaires were obtained, revealing a high incidence of olfactory and taste dysfunctions related to infection with the SARS-CoV-2 Omicron strain (67.75%). Females(χ2=367.013, P<0.001) and young people(χ2=120.210, P<0.001) were more likely to develop these dysfunctions. Gender(OR=1.564, 95%CI: 1.487-1.645), SARS-CoV-2 vaccination status (OR=1.334, 95%CI: 1.164-1.530), oral health status (OR=0.881, 95%CI: 0.839-0.926), smoking history (OR=1.152, 95%CI=1.080-1.229), and drinking history (OR=0.854, 95%CI: 0.785-0.928) were correlated with the occurrence of olfactory and taste dysfunctions related to SARS-CoV-2(above P<0.001). 44.62% (4 391/9 840) of the patients who had not recovered their sense of smell and taste also suffered from nasal congestion, runny nose, and 32.62% (3 210/9 840) suffered from dry mouth and sore throat. The improvement of olfactory and taste functions was correlated with the persistence of accompanying symptoms(χ2=10.873, P=0.001). The average score of olfactory and taste VAS scale was 8.41 and 8.51 respectively before SARS-CoV-2 infection, but decreased to3.69 and 4.29 respectively after SARS-CoV-2 infection, and recovered to 5.83and 6.55 respectively at the time of the survey. The median duration of olfactory and gustatory dysfunctions was 15 days and 12 days, respectively, with 0.5% (121/24 096) of patients experiencing these dysfunctions for more than 28 days. The overall self-reported improvement rate of smell and taste dysfunctions was 59.16% (14 256/24 096). Gender(OR=0.893, 95%CI: 0.839-0.951), SARS-CoV-2 vaccination status (OR=1.334, 95%CI: 1.164-1.530), history of head and facial trauma(OR=1.180, 95%CI: 1.036-1.344, P=0.013), nose (OR=1.104, 95%CI: 1.042-1.171, P=0.001) and oral (OR=1.162, 95%CI: 1.096-1.233) health status, smoking history(OR=0.765, 95%CI: 0.709-0.825), and the persistence of accompanying symptoms (OR=0.359, 95%CI: 0.332-0.388) were correlated with the recovery of olfactory and taste dysfunctions related to SARS-CoV-2 (above P<0.001 except for the indicated values). Conclusion: The incidence of olfactory and taste dysfunctions related to infection with the SARS-CoV-2 Omicron strain is high in mainland China, with females and young people more likely to develop these dysfunctions. Active and effective intervention measures may be required for cases that persist for a long time. The recovery of olfactory and taste functions is influenced by several factors, including gender, SARS-CoV-2 vaccination status, history of head and facial trauma, nasal and oral health status, smoking history, and persistence of accompanying symptoms.
Female ; Humans ; Adolescent ; SARS-CoV-2 ; Smell ; COVID-19/complications* ; Cross-Sectional Studies ; COVID-19 Vaccines ; Incidence ; Olfaction Disorders/etiology* ; Taste Disorders/etiology* ; Prognosis

Objective: To investigate the relationship between the expression levels of Plakoglobin protein in residual lesions after neoadjuvant chemotherapy (NAC) and the prognosis of breast cancer patients. Methods: Clinical and pathological data from 174 breast cancer patients who underwent surgery after receiving NAC at the Cancer Hospital of Chinese Academy of Medical Sciences from January 2009 to December 2017 were collected. The expression level of Plakoglobin in residual cancer lesions was evaluated by immunohistochemistry. The correlation between Plakoglobin expression level and clinicopathological features was analyzed. Survival analysis was performed using the Kaplan-Meier method, and Cox proportional hazard regression models were used for factor analysis. Results: Among the 174 patients, 140 had low expression of Plakoglobin, and 34 had high expression. The median disease-free survival (DFS) and overall survival (OS) in the Plakoglobin low expression group were 59.46 and 71.68 months, respectively, both of which were higher than those in the high expression group (36.58 and 47.26 months, respectively, both P<0.05). Univariate analysis showed that Plakoglobin expression, pathological N stage, lymphovascular invasion status, histological grade, Ki-67, and molecular subtypes were associated with OS (all P<0.05), while pathological N stage, histological grade, and Ki-67 were associated with DFS (all P<0.05). Multivariate analysis revealed that Plakoglobin expression (HR=2.438, 95% CI: 1.256-4.735, P=0.008) was an independent predictor for OS, and Ki-67 (HR=2.228, 95% CI: 1.316-3.773, P=0.003) was an independent predictor for DFS. Conclusion: In breast cancer patients with residual lesions after NAC, those with low Plakoglobin expression have relatively longer OS and Plakoglobin is an independent prognostic factor for OS.
Humans ; Female ; Prognosis ; Breast Neoplasms/surgery* ; Ki-67 Antigen/analysis* ; Neoadjuvant Therapy/methods* ; gamma Catenin ; Neoplasm, Residual ; Disease-Free Survival ; Retrospective Studies ; Antineoplastic Combined Chemotherapy Protocols/therapeutic use*

Objective: To investigate the relationship between the expression levels of Plakoglobin protein in residual lesions after neoadjuvant chemotherapy (NAC) and the prognosis of breast cancer patients. Methods: Clinical and pathological data from 174 breast cancer patients who underwent surgery after receiving NAC at the Cancer Hospital of Chinese Academy of Medical Sciences from January 2009 to December 2017 were collected. The expression level of Plakoglobin in residual cancer lesions was evaluated by immunohistochemistry. The correlation between Plakoglobin expression level and clinicopathological features was analyzed. Survival analysis was performed using the Kaplan-Meier method, and Cox proportional hazard regression models were used for factor analysis. Results: Among the 174 patients, 140 had low expression of Plakoglobin, and 34 had high expression. The median disease-free survival (DFS) and overall survival (OS) in the Plakoglobin low expression group were 59.46 and 71.68 months, respectively, both of which were higher than those in the high expression group (36.58 and 47.26 months, respectively, both P<0.05). Univariate analysis showed that Plakoglobin expression, pathological N stage, lymphovascular invasion status, histological grade, Ki-67, and molecular subtypes were associated with OS (all P<0.05), while pathological N stage, histological grade, and Ki-67 were associated with DFS (all P<0.05). Multivariate analysis revealed that Plakoglobin expression (HR=2.438, 95% CI: 1.256-4.735, P=0.008) was an independent predictor for OS, and Ki-67 (HR=2.228, 95% CI: 1.316-3.773, P=0.003) was an independent predictor for DFS. Conclusion: In breast cancer patients with residual lesions after NAC, those with low Plakoglobin expression have relatively longer OS and Plakoglobin is an independent prognostic factor for OS.
Humans ; Female ; Prognosis ; Breast Neoplasms/surgery* ; Ki-67 Antigen/analysis* ; Neoadjuvant Therapy/methods* ; gamma Catenin ; Neoplasm, Residual ; Disease-Free Survival ; Retrospective Studies ; Antineoplastic Combined Chemotherapy Protocols/therapeutic use*

Objective: To investigate the clinicopathological characteristics of H3K27-altered diffuse midline glioma (DMG), and to analyze DMG's prognostic factors, and subsequently, to study the possibility of using NTRK as a therapeutic target for DMG. Methods: A total of 232 DMG diagnosed at the Sanbo Brain Hospital, Capital Medical University, Beijing, China from July 2016 to March 2021 were collected. Their clinical, radiological and pathological features, the ratio of MGMT promoter methylation, expression of NTRK, and characteristics of NTRK gene fusion were analyzed. The prognostic values of different factors were also studied, including age, tumor location, histological grade, gene and protein expression of NTRK, and postoperative adjuvant therapy. Results: Among the 232 DMG cases, there were 8 patients with both primary and relapse tumors on the record. Thus, a total of 224 patients were analyzed, including 118 males and 106 females. There were 126 adults (>18 years of age) and 98 children (≤18 years of age). Notably, the most frequent location was thalamus (41/126, 32.5%) in adults, but brainstem (59/96, 60.2%) in children. The lesions showed T1 hypointensity or isointensity, and T2 hyperintensity. However, contrast enhancement patterns of the tumors varied, with many tumors lacking contrast-enhancing. The histological grades included grade 2 (9/224, 4.0%), grade 3 (41/224, 18.3%) and grade 4 (174/224, 77.7%). Two hundred and twenty-four DMGs were diffusely positive for H3K27M and negative for H3K27me3. The ratio of MGMT promoter methylation was low (1/45, 2.2%). One hundred and seventy-seven of the 224 cases (177/224, 79.0%) were positive for NTRK. Fifty cases were analyzed using fluorescence in situ hybridization. Among them, five DMGs (positive rate, 10.0%) were NTRK fusion positive. This study showed that there were no differences between adult and pediatric DMGs in histological grading, expression of NTRK, and NTRK gene fusion. One hundred and fifty-nine patients were included in the follow-up analysis (P>0.05). During the follow-up period, 109/159 patients (69.6%) died of the disease, with a median survival time of 12 months (range 1 to 55 months). Univariate log-rank analysis showed that age, location, surgical procedure and postoperative adjuvant therapy were associated with overall survivals of the DMG patients (P<0.05). Conclusions: The prognosis of DMG is poor overall. There are differences between adult and pediatric DMGs in anatomic location and prognosis, but not in other features. NTRK1 gene fusion is detected in 10.0% of the tumors. It suggests that TRK inhibitor might be a choice for treating DMG.
Adult ; Male ; Female ; Humans ; Child ; Aged, 80 and over ; In Situ Hybridization, Fluorescence ; Glioma/pathology* ; Prognosis ; Gene Fusion ; Promoter Regions, Genetic

Objective: To explore the clinical significance of hepatitis B pregenomic RNA (pgRNA) for deciding antiviral therapy discontinuation in patients with chronic hepatitis B (CHB). Methods: Data of patients with CHB who were treated with long-term antiviral therapy in the Center for Infectious Diseases, West China Hospital of Sichuan University from January 2019 to December 2019 were collected. Drug discontinuity after evaluation of high-sensitivity HBV DNA and HBV pgRNA (HBV DNA ≤20 IU/ml and HBV pgRNA<150 copies/ml) was observed. The prospective observational study on 91 patients with HBeAg-negative CHB was conducted. The clinical conditions were followed up 3, 6 and 12 months after the drug discontinuation. The relationship between HBV pgRNA and relapse after drug discontinuation was analyzed. Results: From observation to 12 months after drug discontinuation, a total of 34 patients (37.4%) had developed recurrence and resumed antiviral therapy, and the cumulative recurrence rate within 12 months of drug discontinuation was 46.8%. Among the relapsed patients, 14 (41.2%) had biochemical breakthroughs, and all achieved good biochemical and virological responses after the resumption of antiviral therapy. The Cox multivariate proportional hazards regression analysis showed that the level of HBV pgRNA before drug discontinuation and the type of antiviral drugs taken were associated with recurrence after drug discontinuation. The risk of recurrence after drug withdrawal in the HBV pgRNA ≤50 copies/ml group was 2.316 times higher than that in the HBV pgRNA negative group (HR=2.316, 95%CI: 1.047-5.126, P=0.038). The risk of recurrence after drug withdrawal in the HBV pgRNA >50 copies/ml group was 3.45 times higher than that in the HBV pgRNA negative group (HR=3.450, 95%CI: 1.338-8.892, P=0.010). Conclusion: HBV pgRNA can be used to predict the risk of recurrence after antiviral therapy discontinuation in patients with CHB. Patients with negative serum HBV pgRNA before drug discontinuation have a relatively low risk of relapse after drug discontinuation, and drug discontinuation is not recommended for patients with HBV pgRNA >50 copies/ml.
Humans ; Hepatitis B virus/genetics* ; Hepatitis B e Antigens ; Hepatitis B, Chronic/drug therapy* ; DNA, Viral ; RNA ; Antiviral Agents/therapeutic use* ; Recurrence ; Hepatitis B Surface Antigens

Objective: To investigate the clinicopathological characteristics and differential diagnosis of pediatric SMARCB1/INI1-deficient poorly differentiated chordoma (PDC) of the skull base. Methods: Five cases of SMARCB1/INI1-deficient PDC were identified in 139 cases of chordoma diagnosed in Sanbo Brain Institute, Capital Medical University, Beijing, China from March 2017 to March 2021. The clinical and imaging data of the 5 PDCs were collected. H&E and immunohistochemical staining, and DNA methylation array were used, and the relevant literatures were reviewed. Results: All 5 PDCs were located at the clivus. The average age of the patients was 6.4 years, ranging from 3 to 16 years. Three patients were female and two were male. Morphologically, in contrast with classical chordomas, they presented as epithelioid or spindle tumor cells organized in sheets or nests, with necrosis, active mitoses, and infiltration into surrounding tissue. All cases showed positivity of CKpan, EMA, vimentin and brachyury (nuclear stain), and loss of nuclear SMARCB1/INI1 expression. S-100 protein expression was not frequent (2/5). Ki-67 proliferative index was high (20%-50%). All cases had over-expressed p53. It was necessary to differentiate SMARCB1/INI1-dificient PDC from SMARCB1/INI1-dificient tumors occurring at skull base of children or the tumors with epithelial and spindle cell morphological features. The 3 PDCs with DNA methylation testing showed the methylation profiles different from the pediatric atypical teratoid/rhabdoid tumors. They formed an independent methylation profile cluster. The clinical prognosis of the 5 patients was poor, and the overall survival time was 2-17 months. Conclusions: PDC is a special subtype of chordoma, which often affects children and occurs in the clivus. The PDC shares epithelioid or spindle cell morphologic features which are different from the classic chordoma. Besides the typical immunohistochemical profile of chordoma, PDC also has loss of nuclear SMARCB1/INI1 expression and distinct epigenetic characteristics.
Biomarkers, Tumor/genetics* ; Child ; Chordoma/genetics* ; Diagnosis, Differential ; Female ; Humans ; Male ; Prognosis ; Rhabdoid Tumor/diagnosis* ; SMARCB1 Protein/genetics* ; Skull Base

Objective:To compare the efficacy of transcatheter arterial embolization (TAE) with laparotomy in the treatment of severe liver injury.Methods:A retrospective cohort study was conducted to analyze the clinical data of 48 patients with severe liver injury admitted to 909th Hospital of Joint Logistics Support Force (Affiliated Dongnan Hospital of Xianmen University Medical College) from December 2013 to June 2020, including 28 males and 20 females; aged 16-75 years [(45.7±6.2)years]. There were 25 patients with grade III, 15 grade IV and 8 grade V according to the American Association for the Surgery of Trauma (AAST) classification. After general treatments such as infusion and hemostasis, TAE was performed in 26 patients (TAE group) and laparotomy in 22 patients (laparotomy group). The operation time and length of hospital stay were compared between the two groups. Erythrocyte, hemoglobin and serum creatinine were compared before operation and at postoperative 1 day. Alanine aminotransferase (ALT) and aspartate aminotransferase (AST) were analyzed before operation and at postoperative 1, 3, 7 days. Complications were observed.Results:All patients were followed up for 12-60 months [(17.1±9.1)months]. The operation time and length of hospital stay were (65.7±9.2)minutes and (21.6±6.6)days in TAE group, significantly shorter than (162.5±28.1)minutes and (31.5±7.4)days in laparotomy group ( P<0.05 or 0.01). There was no significant difference between the two groups referring to erythrocyte, hemoglobin and serum creatinine before operation and at postoperative 1 day (all P>0.05). There was no significant difference in ALT and AST between the two groups before operation (all P>0.05). TAE group showed ALT level of 1 154(884, 1 698)U/L, (975.3±400.9)U/L and (403.4±232.9)U/L at postoperative 1, 3, 7 days, significantly lower than 2 053(1 965, 2 132)U/L, (1 604.1±188.2)U/L and (915.3±160.5)U/L in laparotomy group (all P<0.05). TAE group showed AST level of (1 313.2±542.0)U/L, 525(302, 971)U/L and 174(84, 324)U/L at postoperative 1, 3, 7 days, significantly lower than (1 962.9±245.4)U/L, 1 478(1 089, 1 677)U/L and 837(674, 1 006)U/L in laparotomy group ( P<0.05 or 0.01). The complication rate was 26.9% (7/26) in TAE group, significantly lower than 59.1% (13/22) in laparotomy group ( P<0.05). Conclusion:For severe liver injury, TAE can significantly shorten operation time and length of hospital stay, accelerate the recovery of liver function and reduce the complication rate in comparison with laparotomy.