Objective:To investigate the clinical significance and inducing factors of abnormal intraoperative neurophysiological monitoring (IONM) signals during surgery for cervical degenerative diseases.Methods:A retrospective analysis was performed on 586 patients who underwent cervical degenerative disease surgery with IONM at the Department of Orthopedics, The First Affiliated Hospital of Soochow University, from April 2015 to April 2024. Surgical approaches included 380 anterior spinal canal decompression and fusion procedures, 154 posterior spinal canal decompression and fusion procedures (including single-door laminoplasty, total laminectomy, and hemilaminectomy), and 52 combined anterior-posterior surgeries. The multimodal IONM protocol employed transcranial electrical stimulation motor evoked potentials (TES-MEP) and cortical somatosensory evoked potentials (CSEP), combined with electromyography (EMG). Bilateral deltoid muscles, thenar/hypothenar muscles and abductor hallucis muscles were monitored in all patients. Intraoperative MEP, SEP, and EMG results were recorded to analyze the causes of abnormal signals, intraoperative response strategies, and postoperative neurological function and outcomes. Fourfold table chi-square tests were used to analyze factors possibly associated with IONM alerts.Results:Among the 586 cervical surgeries, 17 cases (2.9%) exhibited abnormal IONM signals. These included 4 cases of anterior cervical discectomy and fusion (ACDF), 4 cases of anterior cervical corpectomy and fusion (ACCF), and 2 cases of combined anterior-posterior surgeries for cervical spondylotic myelopathy; and 5 posterior surgeries and 2 anterior ACCF procedures for ossification of the posterior longitudinal ligament (OPLL). The rate of abnormal IONM signals was significantly higher in patients with maximum spinal cord compression (MSCC)>60% (5.8%, 12/208) than in those with MSCC≤60% (χ 2=9.417, P=0.002); in patients with intraoperative hypotension during posterior surgery (mean arterial pressure reduction>20% from baseline, cumulative duration>20 min), the abnormal IONM rate was 22.2% (6/27), which was significantly higher than that in patients without intraoperative hypotension (χ 2=33.542, P<0.001); in patients who underwent calcified tissue removal during anterior surgery, the abnormal IONM rate was 9.3% (5/54), which was significantly higher than that in patients without calcified tissue removal (χ 2=13.162, P=0.003). Thus, MSCC>60%, intraoperative hypotension during posterior surgery, and calcified tissue removal during anterior surgery may be inducing factors for abnormal IONM signals. Among the 17 patients with monitoring abnormalities, 8 cases showed no significant improvement after corresponding intraoperative treatments, and 7 of these 8 cases experienced varying degrees of muscle strength decline and sensory numbness immediately after surgery; 9 cases showed partial or complete recovery of signals, among which 8 cases had no new-onset neurological impairment after surgery, and 1 case developed unilateral upper limb grip strength decline. IONM demonstrated a sensitivity of 0.8750 and specificity of 0.8889. Conclusions:Multimodal IONM can detect electrophysiological abnormalities of spinal cord nerve function during cervical degenerative disease surgery, providing real-time warning of potential nerve damage during the operation. The proportion of abnormal IONM signals is relatively high in cases with MSCC>60%, intraoperative hypotension during posterior cervical surgery, or calcified tissue removal during anterior cervical surgery.

Objective:To investigate the clinical features, pathologic characteristics, treatment and prognosis of primary mediastinal large B-cell lymphoma (PMBCL) in children.Methods:Clinical data including clinical manifestations, treatment, clinical efficacy of 8 cases of childhood PMBCL treated in Beijing Children′s Hospital, Capital Medical University from March 2017 to February 2024 were collected retrospectively, the clinical characteristics and prognosis of them were summarized.Results:Among the 8 children, there were 5 males and 3 females. The age at the time of initial diagnosis was 11.0 (10.3, 13.5) years. The first clinical symptoms were cough (8 cases) and stridor (6 cases). The lesions most often involved the mediastinum (8 cases), lungs (5 cases, hilum more often), pericardium (5 cases), and pleura (4 cases). Extra thoracic invasion was present in 4 cases, 7 cases had huge tumor lesions and 7 cases were phase Ⅲ clinical stage. Except for 1 case who underwent surgical resection of the tumor, the remaining 7 cases were treated with DA-EPOCH+R (dose adjusted-etoposide+prednisone+vincristine+cyclo-phosphamide+doxorubicin+rituximab) chemotherapy. The follow-up time was 25.0 (10.5, 43.3) months, with 7 cases in complete and partial metabolism response, 1 case had disease progression. All 8 cases survived.Conclusions:PMBCL is most common in school-age boys and most of them present with huge mediastinal tumor focus. PMBCL expresses B-cell spectrum antigens and weakly expresses CD30.The application of DA-EPOCH+R is effective in the treatment of PMBCL in children.

Objective:To evaluate the clinical characteristics, pathology, treatment and prognosis of blastic plasmacytoid dendritic cell neoplasm (BPDCN) in children.Methods:Clinical data (including gender, age of disease onset, affected sites, treatment, timing of allogeneic hematopoietic stem cell transplantation (allo-HSCT), etc.) of 7 children with BPDCN who were admitted to Beijing Children′s Hospital, Capital Medical University from December 2018 to December 2023 were analyzed retrospectively. Clinical outcomes were also assessed, with patients followed up until December 2024.Results:Among 7 patients, there were 3 males and 4 females. Age at disease onset ranged from 3.2 to 12.9 years. Initial presentations included subcutaneous nodules in 5 cases, rash in 1 case, and ankle pain in 1 case. Extra-cutaneous involvement was seen in the bone marrow, lymph nodes, and central nervous system. Six patients received induction chemotherapy using a modified lymphoblastic lymphoma regimen, 1 patient received the high-risk protocol for pediatric lymphoblastic lymphoma/leukemia and salvage therapy regimens. Allo-HSCT was performed soon after chemotherapy remission. The time to bridge allo-HSCT was 3.5 to 6.5 months. The follow-up time was 1.6 to 6.0 years. Six patients were in disease-free survival, while 1 patient survived with disease after recurrence following transplantation.Conclusions:BPDCN is rare in children and presents diverse clinical manifestations, with skin involvement being the predominant feature. Early allo-HSCT following complete remission with chemotherapy can improve prognosis.

Objective:To investigate the clinical significance and inducing factors of abnormal intraoperative neurophysiological monitoring (IONM) signals during surgery for cervical degenerative diseases.Methods:A retrospective analysis was performed on 586 patients who underwent cervical degenerative disease surgery with IONM at the Department of Orthopedics, The First Affiliated Hospital of Soochow University, from April 2015 to April 2024. Surgical approaches included 380 anterior spinal canal decompression and fusion procedures, 154 posterior spinal canal decompression and fusion procedures (including single-door laminoplasty, total laminectomy, and hemilaminectomy), and 52 combined anterior-posterior surgeries. The multimodal IONM protocol employed transcranial electrical stimulation motor evoked potentials (TES-MEP) and cortical somatosensory evoked potentials (CSEP), combined with electromyography (EMG). Bilateral deltoid muscles, thenar/hypothenar muscles and abductor hallucis muscles were monitored in all patients. Intraoperative MEP, SEP, and EMG results were recorded to analyze the causes of abnormal signals, intraoperative response strategies, and postoperative neurological function and outcomes. Fourfold table chi-square tests were used to analyze factors possibly associated with IONM alerts.Results:Among the 586 cervical surgeries, 17 cases (2.9%) exhibited abnormal IONM signals. These included 4 cases of anterior cervical discectomy and fusion (ACDF), 4 cases of anterior cervical corpectomy and fusion (ACCF), and 2 cases of combined anterior-posterior surgeries for cervical spondylotic myelopathy; and 5 posterior surgeries and 2 anterior ACCF procedures for ossification of the posterior longitudinal ligament (OPLL). The rate of abnormal IONM signals was significantly higher in patients with maximum spinal cord compression (MSCC)>60% (5.8%, 12/208) than in those with MSCC≤60% (χ 2=9.417, P=0.002); in patients with intraoperative hypotension during posterior surgery (mean arterial pressure reduction>20% from baseline, cumulative duration>20 min), the abnormal IONM rate was 22.2% (6/27), which was significantly higher than that in patients without intraoperative hypotension (χ 2=33.542, P<0.001); in patients who underwent calcified tissue removal during anterior surgery, the abnormal IONM rate was 9.3% (5/54), which was significantly higher than that in patients without calcified tissue removal (χ 2=13.162, P=0.003). Thus, MSCC>60%, intraoperative hypotension during posterior surgery, and calcified tissue removal during anterior surgery may be inducing factors for abnormal IONM signals. Among the 17 patients with monitoring abnormalities, 8 cases showed no significant improvement after corresponding intraoperative treatments, and 7 of these 8 cases experienced varying degrees of muscle strength decline and sensory numbness immediately after surgery; 9 cases showed partial or complete recovery of signals, among which 8 cases had no new-onset neurological impairment after surgery, and 1 case developed unilateral upper limb grip strength decline. IONM demonstrated a sensitivity of 0.8750 and specificity of 0.8889. Conclusions:Multimodal IONM can detect electrophysiological abnormalities of spinal cord nerve function during cervical degenerative disease surgery, providing real-time warning of potential nerve damage during the operation. The proportion of abnormal IONM signals is relatively high in cases with MSCC>60%, intraoperative hypotension during posterior cervical surgery, or calcified tissue removal during anterior cervical surgery.

Objective:To investigate the clinical features, pathologic characteristics, treatment and prognosis of primary mediastinal large B-cell lymphoma (PMBCL) in children.Methods:Clinical data including clinical manifestations, treatment, clinical efficacy of 8 cases of childhood PMBCL treated in Beijing Children′s Hospital, Capital Medical University from March 2017 to February 2024 were collected retrospectively, the clinical characteristics and prognosis of them were summarized.Results:Among the 8 children, there were 5 males and 3 females. The age at the time of initial diagnosis was 11.0 (10.3, 13.5) years. The first clinical symptoms were cough (8 cases) and stridor (6 cases). The lesions most often involved the mediastinum (8 cases), lungs (5 cases, hilum more often), pericardium (5 cases), and pleura (4 cases). Extra thoracic invasion was present in 4 cases, 7 cases had huge tumor lesions and 7 cases were phase Ⅲ clinical stage. Except for 1 case who underwent surgical resection of the tumor, the remaining 7 cases were treated with DA-EPOCH+R (dose adjusted-etoposide+prednisone+vincristine+cyclo-phosphamide+doxorubicin+rituximab) chemotherapy. The follow-up time was 25.0 (10.5, 43.3) months, with 7 cases in complete and partial metabolism response, 1 case had disease progression. All 8 cases survived.Conclusions:PMBCL is most common in school-age boys and most of them present with huge mediastinal tumor focus. PMBCL expresses B-cell spectrum antigens and weakly expresses CD30.The application of DA-EPOCH+R is effective in the treatment of PMBCL in children.

Objective:To evaluate the clinical characteristics, pathology, treatment and prognosis of blastic plasmacytoid dendritic cell neoplasm (BPDCN) in children.Methods:Clinical data (including gender, age of disease onset, affected sites, treatment, timing of allogeneic hematopoietic stem cell transplantation (allo-HSCT), etc.) of 7 children with BPDCN who were admitted to Beijing Children′s Hospital, Capital Medical University from December 2018 to December 2023 were analyzed retrospectively. Clinical outcomes were also assessed, with patients followed up until December 2024.Results:Among 7 patients, there were 3 males and 4 females. Age at disease onset ranged from 3.2 to 12.9 years. Initial presentations included subcutaneous nodules in 5 cases, rash in 1 case, and ankle pain in 1 case. Extra-cutaneous involvement was seen in the bone marrow, lymph nodes, and central nervous system. Six patients received induction chemotherapy using a modified lymphoblastic lymphoma regimen, 1 patient received the high-risk protocol for pediatric lymphoblastic lymphoma/leukemia and salvage therapy regimens. Allo-HSCT was performed soon after chemotherapy remission. The time to bridge allo-HSCT was 3.5 to 6.5 months. The follow-up time was 1.6 to 6.0 years. Six patients were in disease-free survival, while 1 patient survived with disease after recurrence following transplantation.Conclusions:BPDCN is rare in children and presents diverse clinical manifestations, with skin involvement being the predominant feature. Early allo-HSCT following complete remission with chemotherapy can improve prognosis.

Clinical data of 15 primary central nervous system lymphoma (PCNSL) children aged ≤18 years admitted to our hospital between May 2013 to May 2023 were retrospectively analyzed. Our goal was to summarize the clinical features of children and investigate the therapeutic effect of a high-dose methotrexate (HD-MTX) based chemotherapy regimen on this disease. The male-to-female ratio was 2.7∶1, and the median age was 7.2 (2.3-16.4) years at diagnosis. The initial clinical symptoms were primarily cranial hypertension, with imaging findings revealing multiple lesions. Pediatric PCNSL with normal immune function has a favorable prognosis with HD-MTX-based chemotherapy. Patients with a stable disease can be treated with minimal or no maintenance. HD-MTX-based chemotherapy remains effective when the disease progresses or recurs after an initial course of non-HD-MTX-based chemotherapy.

Objective To investigate the effects and mechanism of modified Ditan Decoction in regulating miR-149 on liver injury in rats with chronic intermittent hypoxia.Methods Totally 18 male SD rats were randomly divided into control group,model group,and modified Ditan Decoction,with 6 rats in each group.The model group and the modified Ditan Decoction group were modeled in a low oxygen chamber.During the modeling period,the modified Ditan Decoction group was given gavage of the modified Ditan Decoction for 12 consecutive weeks.HE staining was used to observe the pathological changes of liver tissue;the ultrastructural changes of liver tissue were observed by transmission electron microscopy,TUNEL staining was used to observe hepatocyte apoptosis,the contents of ALT,AST,SOD and MDA in liver tissue were detected,the mRNA and protein expression of miR-149,ATF6,GRP78 and CHOP in liver tissue were detected by RT-qPCR and Western blot.The correlation between the mRNA transcription levels of miR-149 and ATF6 was analyzed.Results Compared with the control group,a small amount of inflammatory cell infiltration in liver tissue of the model group,with irregular arrangement of liver cells,varying degrees of edema,expansion of endoplasmic reticulum,ribosome shedding,mitochondrial membrane rupture,and apoptosis rate of liver cells increased(P<0.05),the contents of ALT,AST and MDA significantly increased(P<0.05),while the content of SOD significantly decreased(P<0.05),the expression of miR-149 mRNA in liver tissue decreased(P<0.05),while the mRNA and protein expression of ATF6,GRP78 and CHOP increased(P<0.05).Compared with the model group,no obvious edema or inflammatory cell infiltration was observed in liver tissue of rats in the modified Ditan Decoction group,the liver cells were arranged in a regular manner,with slightly expanded endoplasmic reticulum and more uniform distribution of ribosomes,the mitochondrial membrane was relatively intact,and the apoptosis rate of liver cells decreased(P<0.05),the contents of ALT,AST and MDA decreased(P<0.05),while the content of SOD increased(P<0.05),the expression of miR-149 mRNA in liver tissue significantly increased(P<0.05),while the mRNA and protein expression of ATF6,GRP78 and CHOP significantly decreased(P<0.05).The level of miR-149 mRNA was significantly negatively correlated with that of ATF6 mRNA(r=-0.766).Conclusion Modified Ditan Decoction may inhibit liver oxidative stress response in chronic intermittent hypoxia rats and improve liver injury by regulating the miR-149/ATF6 pathway.

Objective:To evaluate the efficacy and safety of CD30 antibody-drug conjugates (ADC) brentuximab vedotin (BV) combined with chemotherapy in children with refractory or relapsed classic Hodgkin′s lymphoma (R/R cHL).Methods:Clinical data (including age, gender, B symptoms, clinical stage, previous treatment, etc.) of the 10 R/R cHL children diagnosed and treated at Beijing Children′s Hospital Affiliated to Capital Medical University from October 2021 to August 2023 were analyzed retrospectively. According to the different intensity of chemotherapy drugs, the dose of BV applied in the same course of treatment was 1.8 mg/kg for BV applied once every 3 weeks, and 1.2 mg/kg for BV applied once every 2 weeks. All 10 patients received at least 2 cycles of BV combined with chemotherapy and were evaluated every 2 cycles. The patients were followed up until May 31, 2024. The infusion reactions and adverse reactions after treatment were recorded.Results:In all 10 patients, there were 7 males and 3 females, the age ranged from 5.3-16.9 years, and there were 6 cases of refractory and 4 cases of relapsed. There were 6 cases of nodular sclerosis type, 2 cases of mixed cell type, 1 case of lymphocyte-rich type, and 1 case of lymphodepletion type. There were 5 cases of stage Ⅳ and 5 cases of stage Ⅲ. Previous treatment was mainly chemotherapy, 4 cases received radiotherapy and 1 case received programmed cell death protein 1 (PD-1) antibody therapy. The follow-up time ranged from 9 to 27 months. A total of 43 courses with 49 doses of BV alone or combined with chemotherapy were recorded, and the number of courses was 2 to 10 times. All 10 children responded to the treatment, and 9 achieved complete remission. BV infusion was successfully completed in all cases. A total of 28 cases of grade 3 or above adverse events were recorded, mainly myelosuppression, all of which were related to chemotherapy and did not affect sequential treatment.Conclusion:Brentuximab vedotin has demonstrated efficacy and a tolerable safety profile in the treatment of refractory and relapsed CD30-positive Hodgkin′s lymphoma in children.

Objective:To analyze the factors affecting delayed chemotherapy in children with Burkitt lymphoma (BL) and their influence on prognosis.Methods:Retrospective cohort study. Clinical data of 591 children aged ≤18 years with BL from May 2017 to December 2022 in China Net Childhood Lymphoma (CNCL) was collected. The patients were treated according to the protocol CNCL-BL-2017. According to the clinical characteristics, therapeutic regimen was divided into group A, group B and group C .Based on whether the total chemotherapy time was delayed, patients were divided into two groups: the delayed chemotherapy group and the non-delayed chemotherapy group. Based on the total delayed time of chemotherapy, patients in group C were divided into non-delayed chemotherapy group, 1-7 days delayed group and more than 7 days delayed group. Relationships between delayed chemotherapy and gender, age, tumor lysis syndrome before chemotherapy, bone marrow involvement, disease group (B/C group), serum lactate dehydrogenase (LDH) > 4 times than normal, grade Ⅲ-Ⅳ myelosuppression after chemotherapy, minimal residual disease in the interim assessment, and severe infection (including severe pneumonia, sepsis, meningitis, chickenpox, etc.) were analyzed. Logistic analysis was used to identify the relevant factors. Kaplan-Meier method was used to analyze the patients' survival information. Log-Rank was used for comparison between groups.Results:Among 591 patients, 504 were males and 87 were females, the follow-up time was 34.8 (18.6,50.1) months. The 3-year overall survival (OS) rate was (92.5±1.1)%,and the 3-year event-free survival (EFS) rate was (90.5±1.2)%. Seventy-three (12.4%) patients were in delayed chemotherapy group and 518 (87.6%) patients were in non-delayed chemotherapy group. The reasons for chemotherapy delay included 72 cases (98.6%) of severe infection, 65 cases (89.0%) of bone marrow suppression, 35 cases (47.9%) of organ dysfunction, 22 cases (30.1%) of tumor lysis syndrome,etc. There were 7 cases of chemotherapy delay in group B, which were seen in COPADM (vincristine+cyclophosphamide+prednisone+daunorubicin+methotrexate+intrathecal injection,4 cases) and CYM (methotrexate+cytarabine+intrathecal injection,3 cases) stages. There were 66 cases of chemotherapy delay in group C, which were common in COPADM (28 cases) and CYVE 1 (low dose cytarabine+high dose cytarabine+etoposide+methotrexate, 12 cases) stages. Multinomial Logistic regression analysis showed that the age over 10 years old ( OR=0.54,95% CI 0.30-0.93), tumor lysis syndrome before chemotherapy ( OR=0.48,95% CI 0.27-0.84) and grade Ⅲ-Ⅳ myelosuppression after chemotherapy ( OR=0.55,95% CI 0.33-0.91)were independent risk factors for chemotherapy delay.The 3-year OS rate and the 3-year EFS rate of children with Burkitt lymphoma in the delayed chemotherapy group were lower than those in the non-delayed chemotherapy group ((79.4±4.9)% vs. (94.2±1.1)%, (80.2±4.8)% vs. (92.0±1.2)%,both P<0.05). The 3-year OS rate of the group C with chemotherapy delay >7 days (42 cases) was lower than that of the group with chemotherapy delay of 1-7 days (22 cases) and the non-delay group (399 cases) ((76.7±6.9)% vs. (81.8±8.2)% vs. (92.7±1.3)%, P=0.002).The 3-year OS rate of the chemotherapy delay group (9 cases) in the COP (vincristine+cyclophosphamide+prednisone) phase was lower than that of the non-chemotherapy delay group (454 cases) ((66.7±15.7)% vs. (91.3±1.4)%, P=0.005). Similarly, the 3-year OS rate of the chemotherapy delay group (11 cases) in the COPADM1 phase was lower than that of the non-chemotherapy delay group (452 cases) ((63.6±14.5)% vs. (91.5±1.3)%, P=0.001). Conclusions:The delayed chemotherapy was related to the age over 10 years old, tumor lysis syndrome before chemotherapy and grade Ⅲ-Ⅳ myelosuppression after chemotherapy in pediatric BL. There is a significant relationship between delayed chemotherapy and prognosis of BL in children.