Objective:To investigate the clinical diagnosis and treatment of IgG4-related disease（IgG4-RD） primarily involving the nasal cavity and skull base. Methods:A retrospective analysis was conducted on the clinical data of 8 patients with IgG4-RD primarily involving the nasal cavity and skull base who visited the Nasal and Skull Base Surgery Department at Xiangya Hospital from October 2017 to January 2024. The cohort comprised 4 males and 4 females, aged 8 to 69 years. Clinical data, laboratory examination results, imaging findings, histopathological results, and treatment plans were collected. The clinical manifestations, diagnosis, treatment and follow-up results of IgG4-RD primarily involving nasal cavity and skull base were summarized and previous literature were also reviewed. Results:The initial symptoms in the 8 patients included nasal congestion, headache, sensory function decline, and facial deformities. Three patients also had parotid and pulmonary involvement. Among the 8 patients, 4 underwent partial surgical resection combined with glucocorticoid therapy; 1 underwent partial surgical resection combined with glucocorticoid and immunosuppressant therapy; 1 received glucocorticoid therapy alone; and 2 received glucocorticoid combined with immunosuppressant therapy. Follow-up was conducted one month after treatment, lasting from 5 to 79 months. During the follow-up period, recurrence was observed in 1 patient treated with glucocorticoid combined with immunosuppressants and in 1 patient treated with glucocorticoid alone, while the other 6 patients achieved significant remission. Conclusion:The diagnosis of nasal cavity and skull base IgG4-RD requires the combination of histopathology, laboratory tests, and imaging results. Treatment primarily includes glucocorticoids or combined immunosuppressants. For patients with significant compression symptoms, sensory function impairment, or facial deformities, surgical resection is an important treatment option. Given the high risk of recurrence, early intervention, active treatment, and long-term follow-up are crucial.
Humans ; Male ; Skull Base/pathology* ; Female ; Middle Aged ; Retrospective Studies ; Aged ; Nasal Cavity/pathology* ; Adult ; Immunoglobulin G4-Related Disease/therapy* ; Immunoglobulin G ; Child ; Young Adult ; Adolescent

Objective:To analysis the molecular characteristics of chronic rhinosinusitis with nasal polyps (CRSwNP), to unravel its pathophysiological mechanisms, and to develop a prognostic model capable of effectively predicting postoperative recurrence.Methods:The data from three datasets (GSE198950, GSE179265, and GSE136825) were integrated, comprising 39 control cases, 16 cases of chronic rhinosinusitis without nasal polyps, and 89 cases of CRSwNP. Differential expression genes (DEGs) were identified based on adjusted P<0.05 and Log2FC>1. KEGG and GO enrichment analyses, as well as STRING node scoring, were conducted. Variable selection was performed using random forest and least absolute shrinkage and selection operator regression (LASSO), with key nodes identified through intersection analysis. Mann-Whitney U test was applied, and variables with P<0.05 were included in the model. A prognostic model for CRSwNP was constructed using logistic regression, externally validated using RNA-seq data, and evaluated with receiver operating characteristic (ROC) curve analysis to calculate the area under the curve (AUC). Results:This research illustrated both upregulated and downregulated DEGs in CRSwNP, activating pathways like neuroactive ligand-receptor interaction and IL-17 signaling, while inhibiting calcium signaling and gap junctions. Key nodes identified through random forest and LASSO, including G protein subunit γ4 ( U=3.00 P=0.028), Cholecystokinin ( U=0.50, P=0.006), Epidermal growth factor ( U=1.00 P=0.008), and Neurexin-1 ( U=0.00, P=0.004), showing statistical significance in external validation. The prognostic model, visualized in a line graph, exhibited high reliability (C-index=0.875,AUC=0.866). The ROC curve in external validation indicated its effectiveness in predicting postoperative recurrence (AUC=0.859). Conclusions:This study integrates multiple datasets on CRSwNP to provide a comprehensive description of its molecular features. The prognostic model, built upon key nodes identified through random forest and LASSO analyses, demonstrates high accuracy in both internal and external validations, thus providing robust support for the development of personalized treatment strategies for CRSwNP.

Objective:To evaluate the clinical efficacy of nasal pedicle tissue flap based on nasal blood supply in the reconstruction of nasal skull base defects.Methods:A retrospective analysis was conducted on 138 clinical cases of skull base tumors and cerebrospinal fluid rhinorrhea treated at the Department of Otolaryngology, Head and Neck Surgery at Xiangya Hospital of Central South University from March 2017 to March 2023. A total of 79 males and 59 females were enrolled, aged from 8 to 82 years, with a median age of 51 years, including 108 patients (78.3%) with skull base tumors and 30 patients (21.7%) with cerebrospinal fluid rhinorrhea (and/or meningoencephalocele). During the surgery, 88 cases (63.8%) were repaired with nasal septal mucosal flaps pedicled with the posterior nasal septal artery, 14 cases (10.1%) with mucosal flaps pedicled with the anterior ethmoidal artery on the lateral wall of the nasal cavity, 6 cases (4.3%) with mucosal flaps pedicled with the posterior lateral nasal artery on the lateral wall and nasal floor, 12 cases (8.7%) with mucosal flaps pedicled with the anterior ethmoidal artery and posterior ethmoidal artery, and 18 cases (13.0%) with nasal septal mucosal extension flaps pedicled with the sphenopalatine artery or internal maxillary artery. Patients were followed up for 12 to 72 months postoperatively. Endoscopic examination or skull base enhanced MRI was performed to assess the growth and tumor recurrence in the skull base repair area. The t-test was used for statistical analysis.Results:Among 138 patients, primary repair was successful in 133 patients (96.4%), while 5 patients (3.6%) experienced postoperative cerebrospinal fluid rhinorrhea. These 5 patients all underwent nasal septal mucosal flap repair with the posterior nasal septal artery as the pedicle. Complications included 1 case of mucosal flap necrosis, 1 case of mucosal flap central perforation, and 3 cases of mucosal flap survival peripheral leakage, of which were all successfully treated with a second repair.Conclusion:The use of nasal pedicle mucosal flap based on nasal blood supply is a reliable, safe, and effective technique for repairing skull base defects.

Objective:To evaluate the efficacy and safety of standardized dust mite allergen subcutaneous immunotherapy (SCIT) in children with allergic rhinitis (AR) during treatment.Methods:A total of 283 children with AR diagnosed with definite dust mite allergy and completed 2 to 3 years of SCIT who attended the Department of Otorhinolaryngology Head and Neck Surgery, Xiangya Hospital, Central South University, from August 2019 to October 2021 were included, including 205 males and 78 females, with a mean age of 10.8 years. The total nasal symptoms score (TNSS), symptom medication score (SMS), rhinoconjunctivitis quality of life questionnaire (RQLQ) and visual analogue scale (VAS) before and after 2 to 3 years′ treatment were recorded, and the differences before and after treatment were compared. Adverse reactions during SCIT were recorded to evaluate its safety. SPSS 22.0 software was used for statistical analysis.Results:The overall effectiveness rate during SCIT in 283 children with AR was 89.4% (253/283). Compared with baseline, all symptom scores, medication scores and quality of life scores were significantly lower after 2 to 3 years of SCIT (all P<0.05). Further group comparisons showed positive efficacy in patients with different clinical characteristics, including age, gender, smoking status, family history of AR, symptom severity, mono-or poly-allergy, and second immunization, with no statistically significant differences between groups (all P>0.05). A total of 12 735 injections were administered during the SCIT, and a total of 213 (1.67%) injections of local adverse reactions occurred, mainly in the initial treatment phase, and the diameter of the local air mass was mostly 5 to 20 mm; 71 (0.56%) injections of systemic adverse reactions occurred, mainly in the initial treatment phase, and most of them were grade 1 reactions with no serious systemic adverse reaction such as shock. Conclusion:Standardized dust mite SCIT has a good safety profile and definite efficacy in treating AR children with different clinical characteristics. It can significantly improve all symptoms, reduce the use of symptomatic drugs and improve their quality of life.

Humans ; Asthma/drug therapy* ; Biological Therapy

Objective:To analyze the epidemiological and clinical characteristics of patients with imported corona virus disease 2019 (COVID-19) in Beijing City.Methods:A case-control study was performed to retrospectively analyze 69 cases of imported COVID-19 from abroad and 147 cases of domestic confirmed COVID-19 from China as a control group from January 20 to March 20, 2020 admitted to Beijing Ditan Hospital, Capital Medical University.The epidemiological and clinical characteristics were compared.Statistical analysis were performed by t test, Mann-Whitney U test, chi-square test and Fisher exact test. Results:The main sources of the cases in the import group were from the United Kingdom, Italy, Spain and other European countries, with 44.9%(31/69) of the overseas students entering the country by air. The age of the imported group (27(21, 40) years) was lower than the domestic group (43 (32, 59)years), the difference between the two groups was statistically significant ( U=2 828.500, P<0.01). Compared with the domestic group, the proportion of cases with contact history of confirmed cases in the imported group was lower (30.4%(21/69) vs 68.0%(100/147)), the interval between onset and admission ≤seven days was higher (81.2%(56/69) vs 66.0%(97/147)), the proportion of cases with underlying diseases was lower (21.7%(15/69) vs 44.2%(65/147)). The differences between the two groups were all statistically significant ( χ2=26.935, 5.233 and 10.175, respectively, all P<0.05). The proportion of mild cases in the imported group was higher than that in the domestic group (42.0%(29/69) vs 10.9%(16/147)). Seventeen cases with olfactory abnormality and 12 cases with taste abnormality were found in the imported group, while no olfactory and taste abnormality was found in the domestic group. The proportions of fever, weakness, muscle soreness and dyspnea were all lower than those of the domestic group, the differences between the two groups were all statistically significant ( χ2=13.851, 8.118, 9.730 and 16.255, respectively, all P<0.01). The proportions of cases with decreased lymphocyte absolute numbers (37.7%(26/69) vs 67.3%(99/147)) and increased C reactive protein level (15.9%(11/69) vs 51.8%(72/139)) were both lower than the domestic group, and the differences between the two groups were both statistically significant ( χ2=18.015 and 24.722, respectively, both P<0.01). The proportions of cases with ground glass shadow and consolidation of chest computed tomography were lower than those of the domestic group and the differences between the two groups were all statistically significant ( χ2=11.961 and 5.099, respectively, all P<0.05). In terms of complications, the proportions of cases with acute respiratory distress syndrome and acute myocardial injury were lower (2.9%(2/69) vs 10.9%(16/147) and 4.3%(3/69) vs 14.0%(16/114), respectively), and there were statistically significant differences between the two groups ( χ2=4.017 and 4.335, respectively, both P<0.05). There were no cases received mechanical ventilation and extracorporeal membrane oxygenation in the imported group, and the proportions of patients received oxygen therapy and antibiotic treatment were significantly lower than those in the domestic group (13.0%(9/69) vs 26.5%(39/147) and 13.0%(9/69) vs 39.5%(58/147), respectively) and the differences between the two groups were statistically significant ( χ2=4.942 and 15.797, respectively, both P<0.05). Conclusions:The majority of imported COVID-19 cases are mainly from European countries, mostly young and middle-aged, and mostly mild and ordinary types.The symptoms of olfactory and taste abnormality are found for the first time.

Six patients with POEMS syndrome who received autologous peripheral blood stem cell transplantation (auto-PBSCT) were retrospectively analyzed.Conditioning regimen was high dose melphalan.Peripheral blood stem cells were collected after mobilization with cyclophosphamide (CTX) and growth factors.One patient presenting hydrothorax and ascites was treated with 3 cycles of lenalidomide and dexamethasone before mobilization.Auto-PBSCT was fairly tolerable.Hematopoietic reconstitution was successful in all patients without transplantation-related mortality.A decrease or normalization of serum vascular epithelial growth factor (VEGF) was observed in all patients at 3 months after transplantation.The neurological remission was seen in 5/6 patients.

Objective To investigate the therapeutic effect of different doses of methylprednisolone (MP) in smoke inhalation-induced acute lung injury (SI-ALI).Methods Adult male Sprague-Dawley (SD) rats were divided into control group (group A,n = 6), smoke inhalation group (group B, smoke inhalation 30 minutes,n = 30) and smoke+MP 40, 4, 0.4 mg/kg intervention group (groups C, D, E; intraperitoneal injection of MP at 1 hour before smoke inhalation, n = 30) according to random number table method. The survival status of rats in each group was observed at 24 hours, and murine smoke inhalation induced trauma score (MSITS) according to the symptoms and signs of rats at 3 hours after smoke inhalation were scored. The blood of abdominal aorta of rats was collected. Then the rats were sacrificed to harvest bronchoalveolar lavage fluid (BALF) and lung tissue. The levels of interleukin (IL-6, IL-17a) in plasma and BALF were detected by enzyme linked immunosorbent assay (ELISA); the total number of white blood cells and the proportion of leukocytes or macrophages in BALF were calculated; the histopathological changes of lung were observed and the lung injury score was given; the expression of myeloperoxidase (MPO) and high mobility group protein B1 (HMGB1) in lung tissue were detected by Western Blot.Results The 24-hour survival rate of group B rats was 33.67％. The survivalrate of groups C, D and E (65.73％, 85.17％, 60.07％) were significantly higher than that of group B (allP < 0.05), and the survival rate of group D was significantly higher than that of groups C and E. Diffuse inflammatory cell infiltration, intra-alveolar hemorrhage and a large amount of edema fluid were seen in the lung tissue of group B; and the lung injury score was significantly higher than that of group A. Compared with group B, the lung injury in different doses of MP group were decreased to different degrees, while the lung injury scores in groups C and D were significantly decreased (3.31±1.37, 2.62±0.98 vs. 5.52±0.97, bothP < 0.01); correlation analysis showed that MSITS score was significantly and positively correlated with lung injury score (r = 0.862,P < 0.001). The levels of plasma inflammatory factors and BALF protein, inflammatory cells and inflammatory factors, and the expression of MPO, HMGB1 in group B were significantly higher than those in group A. Compared with group B, the levels of inflammatory factors in plasma, and protein content, inflammatory cells and inflammatory factors in BALF in different doses of MP group were decreased to different degrees, with significant differences in groups C and D [plasma: IL-17a (pg/L): 49.28±27.12, 36.57±16.52 vs. 191.79±88.21; IL-6 (ng/L): 206.47±109.96, 197.52±113.86 vs. 669.00±299.60; BALF: protein content (mg/L):892.0±164.5, 566.1±120.9 vs. 1838.0±145.8; white blood cell count (×109/L): 5.40±1.67, 2.81±1.20 vs. 9.02± 2.06; neutrophil ratio: 0.315±0.081, 0.273±0.080 vs. 0.590±0.096; IL-17a (ng/L): 22.63±8.62, 18.92±8.43 vs. 43.31±19.17; IL-6 (ng/L): 156.49±46.94, 123.66±64.91 vs. 253.43±80.03; allP< 0.01]; in addition, the expression of MPO and HMGB1 protein in lung tissues of MP groups with different doses were significantly decreased, the expression of MPO in group D was significantly lower than that in group E [MPO/β-actin (fold increase from group A):2.14±0.97 vs. 4.35±0.87,P < 0.01], the expression of HMGB1 in groups C and D were significantly lower than that in group E [HMGB1/β-actin (fold increase from group A): 1.77±0.73, 1.23±0.67 vs. 3.65±1.08, bothP < 0.05]. Conclusions MP can significantly improve the survival rate of SI-ALI rats and reduce the acute pulmonary and systemic inflammatory response. The MP effect of 4 mg/kg was better than 40 mg/kg and 0.4 mg/kg.

We report two rare cases of multiple myeloma (MM) with dural intracranial disease and TP53 deletion. The two patients presented with skull lytic lesion and dural involvement of myeloma. The association between intracranial involvement in MM and TP53 deletion has not been determined. The two patients received bortezomib-based intensive induction and got good response, just as that reported in literature. MM presenting with dural intracranial disease and TP53 deletion at diagnosis is associated with poor outcome. Multi-drug regime containing bortezomib followed by autologous or allogeneic stem cell transportation would improve the prognosis.
Antineoplastic Agents ; therapeutic use ; Antineoplastic Combined Chemotherapy Protocols ; Bortezomib ; therapeutic use ; Brain Neoplasms ; drug therapy ; genetics ; pathology ; Gene Deletion ; Humans ; Multiple Myeloma ; drug therapy ; genetics ; pathology ; Prognosis ; Treatment Outcome ; Tumor Suppressor Protein p53 ; genetics