Objective:To investigate the short-term efficacy and safety of rituximab (RTX) in children with calcineurin inhibitor (CNI) resistant steroid resistant nephrotic syndrome (SRNS).Methods:A retrospective case analysis was conducted. Thirteen children with CNI resistant SRNS who were regularly treated with RTX (375 mg/m 2 per dose (maximum dose 500 mg), 1 dose per week, a total of 4 doses) in Department of Nephrology, Children′s Hospital of Fudan University from January 2016 to December 2023 were enrolled. The general data, disease related information, urinary protein/creatinine, serum albumin, blood creatinine before RTX treatment, immunosuppressants, adverse events, and monthly urinary protein/creatinine, serum albumin, and blood creatinine indexes within 6 months after RTX treatment were collected. The changes of urinary protein/creatinine, serum albumin and estimated glomerular filtration rate (eGFR) before and after RTX at 3 and 6 months were analyzed by using paired sample t test and Wilcoxon signed-rank test. Results:Among the 13 patients, 8 were male and 5 were female. The age of disease onset was 4.0 (2.9, 6.8) years and the age of RTX treatment was 9.8 (5.9, 13.6) years. There were 8 cases of focal segmental glomerulosclerosis, 3 cases of minimal change disease and 2 cases of mesangial proliferative glomerulonephritis. No clinically significant gene variation was detected in 12 cases and the other one did not receive gene test. Before RTX treatment, 11 cases were in chronic kidney disease stage G1, and 1 case each was in stage G2 and stage G3. Ten children completed 4 doses of RTX treatment, 1 patient completed 3 doses, and 2 patients completed 2 doses. Urinary protein/creatinine in 13 children at 3 and 6 months after RTX treatment was significantly lower than baseline (0.60 (0.13, 2.04), 0.49 (0.28, 1.10) vs. 1.44 (0.76, 4.11) mg/mg, Z=-2.34, -2.34, both P<0.05), and serum albumin was significantly higher than baseline ((35±8), (34±7) vs. (30±6) g/L, t=2.30, 2.60, both P<0.05). The eGFR at 6 months after RTX treatment was not significantly different from the baseline ((110±32) vs. (113±35) ml/(min·1.73 m 2), t=-0.76, P>0.05)). No serious adverse reactions occurred in this study. Conclusion:RTX could reduce urinary protein and increase serum albumin in short-term treatment in children with CNI resistant SRNS without significant side effects.

Objective:To evaluate the efficacy of corticosteroids in male children with Duchenne muscular dystrophy (DMD), and provide evidence for the rational clinical use of medication.Methods:This was a multicenter medical record series study which conducted from January 15 th to March 14 th, 2025. A total of 53 male children with DMD admitted to the Department of Rehabilitation of Children′s Hospital, Zhejiang University School of Medicine, Children′s Hospital of Chongqing Medical University and Affiliated Children′s Hospital of Soochow University from 2020 to 2024 were enrolled. Clinical data, corticosteroid usage, and the follow-up data were collected. The North star ambulatory assessment (NSAA) was used as the primary efficacy indicator. Generalized estimating equations (GEE) exchangeable working matrices were used for longitudinal analysis, and the least squares mean were used to compare the change trend of the efficacy evaluation index across different medication durations. Results:The age at the initiation of corticosteroid treatment was (6.3±1.9) years. The follow-up duration was 1.2 (0.9, 2.2) years. After treatment, the raw scores and linear scores of NSAA were both significantly higher than those before treatment ((22±7) vs. (19±5) points, (60±16) vs. (53±8) points; t=3.98, 3.69; both P<0.001). The 10 meter running time and time rising from floor were both shorter than those before treatment (6 (4, 8) vs. 7 (6, 9) s, 5 (3, 6) vs. 6 (5, 9) s; Z=2.62, 3.47; both P<0.01). GEE model analysis revealed all nonlinear correlation between motor function (NSAA linear score, 10-meter running velocity, and rising from floor velocity) and the duration of corticosteroid treatment (all P<0.05). Least squares mean comparison all showed that the medication effect first increased and then decreased with duration, reaching the peak at 1.1-2.0 years after treatment (all P<0.05). Conclusions:Corticosteroids can improve the motor function in male children with DMD, with the maximum treatment effect occurring 1 to 2 years after the initiation of treatment. It is necessary to comprehensively leverage time-varying efficacy of corticosteroids to optimize individualized treatment regimens for maximal motor function benefits in children with DMD.

Purpose To identify the distinct MRI findings of gastric-type endocervical adenocarcinoma(GEA)that can help differentiate it from usual-type endocervical adenocarcinoma(UEA)and reveal the radiologic-pathologic correlation.Materials and Methods All consecutive patients with cervical GEA(27 cases)and UEA(45 cases)treated at Shanghai First Maternity and Infant Hospital from January 2015 to August 2023 were included retrospectively.All patients underwent enhanced pelvic MRI examination.The clinical characteristics,tumor location,tumor shape,presence and size of cysts,and presence of hydrometra were evaluated between the two groups.Results The clinical characteristics included vaginal discharge in 12 cases of GEA and one case of UEA,vaginal bleeding in seven cases of GEA and 28 cases of UEA(P=0.048).Additionally,there were two cases of Peutz-Jeghers syndrome in GEA.Among the 27 GEA cases,the lesion was in the upper cervix in nine cases and involved the entire cervix in 16 cases;among the 45 UEA cases,the lesions were in the upper,lower and entire cervix in 12,29 and four cases,respectively(P=0.023).Regarding the tumor growth pattern,in the GEA group,there were nine cases of mass-forming growth pattern and 18 cases of diffuse infiltrative pattern;in the UEA group,there were 35 cases of mass-forming growth pattern and ten cases of diffuse infiltrative pattern,with a statistically significant difference(χ2=10.718,P=0.014).In 27 GEA cases,14 cases had observed intrauterine fluid,while in 45 UEA cases,eight cases had observed intrauterine fluid(χ2=12.657,P=0.002).Among the GEA cases,five had no cysts,12 had microcysts and ten had macrocysts;among the UEA cases,14 had no cysts,31 had microcysts and macrocysts were not observed(P=0.001).The maximum diameter of the GEA masses was(4.030±0.375)cm,and that of UEA masses was(2.315±0.769)cm,with a statistically significant difference(t=7.134,P<0.001).Conclusion By integrating multiple MRI features of the diffuse infiltrative growth pattern(such as the predominant location in the upper or entire cervix,frequent association with uterine cavity effusion or larger cysts,and a maximum mass diameter≥4 cm),it aids in distinguishing GEA from UEA.

Objective To investigate the nebulization characteristics of human interferon α1b for injection.To characterize and compare the delivery rate,total drug substance and the aerodynamic characteristics between the two types of nebulizer.Methods Connect two types of nebulizers with a breathing simulator,respectively,and simulate the breathing patterns of an infant and child.Measure the delivery rate,total delivered dose,and delivery uniformity.The aerodynamic properties of human interferon α1b for injection were evaluated by the next generation impactor(NGI).The content of human interferon α1b was quantified by double antibody sandwich ELISA.Results In the three batches of samples in infant mode,the delivery rate and total delivered dose determind by A nebulizer were 0.45,0.49,0.44 μg·min-1,3.06,3.21,3.81 μg,respectcively;and 0.12,0.14,0.16 μg·min-1,0.73,0.73,0.75 μg,respectcively by B nebulizer.In child mode,the delivery rate and total delivered doses determined by A nebulizer were 1.36,1.49,1.20 μg·min-1,7.44,7.17,and 6.54 μg,respectcively;and 0.37,0.36,0.43 μg·min-1,1.66,1.59,and 1.41 μg,respectcively by B nebulizer.In child and infant mode,the ten results of the total drug substance delivered determined by nebulizer A were both between 65%to 135%of the average.The FPD,FPF,MMAD,and GSD determined by A neublizer of three batch samples were 2.48,2.92,2.35 μg,59.0%,57.4%,59.1%,4.18,4.34,4.15 μm,1.94,1.98,2.01,respectively.The FPD,FPF,MMAD and GSD determined by B neublizer of three batches samples were 2.70,3.38,3.06 μg,67.6%,66.4%,66.3%,3.55,3.65,3.68 μm,2.03,2.04,2.06,respectively.Conclusions The data obtained in this research characterized the in vitro nebulization characteristics of human interferon α1b for injection and provided a theoretical basis and reference for in vitro study and clinical practice.The influence of different types of nebulizers on nebulization characteristics was evaluated as well.It is suggested that the quality standard of nebulizers be strictly formulated and the use of nebulizers be standardized.

Objective:To investigate the short-term efficacy and safety of rituximab (RTX) in children with calcineurin inhibitor (CNI) resistant steroid resistant nephrotic syndrome (SRNS).Methods:A retrospective case analysis was conducted. Thirteen children with CNI resistant SRNS who were regularly treated with RTX (375 mg/m 2 per dose (maximum dose 500 mg), 1 dose per week, a total of 4 doses) in Department of Nephrology, Children′s Hospital of Fudan University from January 2016 to December 2023 were enrolled. The general data, disease related information, urinary protein/creatinine, serum albumin, blood creatinine before RTX treatment, immunosuppressants, adverse events, and monthly urinary protein/creatinine, serum albumin, and blood creatinine indexes within 6 months after RTX treatment were collected. The changes of urinary protein/creatinine, serum albumin and estimated glomerular filtration rate (eGFR) before and after RTX at 3 and 6 months were analyzed by using paired sample t test and Wilcoxon signed-rank test. Results:Among the 13 patients, 8 were male and 5 were female. The age of disease onset was 4.0 (2.9, 6.8) years and the age of RTX treatment was 9.8 (5.9, 13.6) years. There were 8 cases of focal segmental glomerulosclerosis, 3 cases of minimal change disease and 2 cases of mesangial proliferative glomerulonephritis. No clinically significant gene variation was detected in 12 cases and the other one did not receive gene test. Before RTX treatment, 11 cases were in chronic kidney disease stage G1, and 1 case each was in stage G2 and stage G3. Ten children completed 4 doses of RTX treatment, 1 patient completed 3 doses, and 2 patients completed 2 doses. Urinary protein/creatinine in 13 children at 3 and 6 months after RTX treatment was significantly lower than baseline (0.60 (0.13, 2.04), 0.49 (0.28, 1.10) vs. 1.44 (0.76, 4.11) mg/mg, Z=-2.34, -2.34, both P<0.05), and serum albumin was significantly higher than baseline ((35±8), (34±7) vs. (30±6) g/L, t=2.30, 2.60, both P<0.05). The eGFR at 6 months after RTX treatment was not significantly different from the baseline ((110±32) vs. (113±35) ml/(min·1.73 m 2), t=-0.76, P>0.05)). No serious adverse reactions occurred in this study. Conclusion:RTX could reduce urinary protein and increase serum albumin in short-term treatment in children with CNI resistant SRNS without significant side effects.

Objective:To evaluate the efficacy of corticosteroids in male children with Duchenne muscular dystrophy (DMD), and provide evidence for the rational clinical use of medication.Methods:This was a multicenter medical record series study which conducted from January 15 th to March 14 th, 2025. A total of 53 male children with DMD admitted to the Department of Rehabilitation of Children′s Hospital, Zhejiang University School of Medicine, Children′s Hospital of Chongqing Medical University and Affiliated Children′s Hospital of Soochow University from 2020 to 2024 were enrolled. Clinical data, corticosteroid usage, and the follow-up data were collected. The North star ambulatory assessment (NSAA) was used as the primary efficacy indicator. Generalized estimating equations (GEE) exchangeable working matrices were used for longitudinal analysis, and the least squares mean were used to compare the change trend of the efficacy evaluation index across different medication durations. Results:The age at the initiation of corticosteroid treatment was (6.3±1.9) years. The follow-up duration was 1.2 (0.9, 2.2) years. After treatment, the raw scores and linear scores of NSAA were both significantly higher than those before treatment ((22±7) vs. (19±5) points, (60±16) vs. (53±8) points; t=3.98, 3.69; both P<0.001). The 10 meter running time and time rising from floor were both shorter than those before treatment (6 (4, 8) vs. 7 (6, 9) s, 5 (3, 6) vs. 6 (5, 9) s; Z=2.62, 3.47; both P<0.01). GEE model analysis revealed all nonlinear correlation between motor function (NSAA linear score, 10-meter running velocity, and rising from floor velocity) and the duration of corticosteroid treatment (all P<0.05). Least squares mean comparison all showed that the medication effect first increased and then decreased with duration, reaching the peak at 1.1-2.0 years after treatment (all P<0.05). Conclusions:Corticosteroids can improve the motor function in male children with DMD, with the maximum treatment effect occurring 1 to 2 years after the initiation of treatment. It is necessary to comprehensively leverage time-varying efficacy of corticosteroids to optimize individualized treatment regimens for maximal motor function benefits in children with DMD.

Objective:To investigate the efficacy and safety of allopurinol in stable coronary heart disease patients with asymptomatic hyperuricemia (AH).Methods:Sixty stable coronary heart disease with AH patients admitted to the Changsha Third Hospital from January 2022 to December 2023 were selected. Patients were randomly divided into an observation group (allopurinol treatment group) and a control group (placebo group). The results of tablet exercise tests, treatment efficacy, blood uric acid levels, liver and kidney function indicators, and incidence of adverse events were compare and analyzed between two groups of patients.Results:The observation group had better exercise termination time, maximum ST descent time, and ST recovery time than the control group (all P<0.05). The total effective rate of the observation group after 6 weeks of treatment was significantly higher than that of the control group, and the difference was statistically significant (χ 2=5.455, P=0.02). There was no statistically significant difference in blood uric acid levels and liver and kidney function indicators between the two groups before treatment (all P>0.05). After treatment, both groups showed significant improvement in blood uric acid levels and liver and kidney function indicators compared to before treatment (all P<0.05). The levels of blood uric acid and liver and kidney function indicators in the observation group were significantly better than those in the control group after treatment (all P<0.05). The incidence of adverse events in the observation group during treatment was lower than that in the control group (χ 2=5.192, P=0.023). Conclusions:Allopurinol has a certain therapeutic effect on stable coronary heart disease with asymptomatic hyperuricemia, which helps patients enhance their physical activity and reduce the incidence of cardiovascular events.

Purpose To identify the distinct MRI findings of gastric-type endocervical adenocarcinoma(GEA)that can help differentiate it from usual-type endocervical adenocarcinoma(UEA)and reveal the radiologic-pathologic correlation.Materials and Methods All consecutive patients with cervical GEA(27 cases)and UEA(45 cases)treated at Shanghai First Maternity and Infant Hospital from January 2015 to August 2023 were included retrospectively.All patients underwent enhanced pelvic MRI examination.The clinical characteristics,tumor location,tumor shape,presence and size of cysts,and presence of hydrometra were evaluated between the two groups.Results The clinical characteristics included vaginal discharge in 12 cases of GEA and one case of UEA,vaginal bleeding in seven cases of GEA and 28 cases of UEA(P=0.048).Additionally,there were two cases of Peutz-Jeghers syndrome in GEA.Among the 27 GEA cases,the lesion was in the upper cervix in nine cases and involved the entire cervix in 16 cases;among the 45 UEA cases,the lesions were in the upper,lower and entire cervix in 12,29 and four cases,respectively(P=0.023).Regarding the tumor growth pattern,in the GEA group,there were nine cases of mass-forming growth pattern and 18 cases of diffuse infiltrative pattern;in the UEA group,there were 35 cases of mass-forming growth pattern and ten cases of diffuse infiltrative pattern,with a statistically significant difference(χ2=10.718,P=0.014).In 27 GEA cases,14 cases had observed intrauterine fluid,while in 45 UEA cases,eight cases had observed intrauterine fluid(χ2=12.657,P=0.002).Among the GEA cases,five had no cysts,12 had microcysts and ten had macrocysts;among the UEA cases,14 had no cysts,31 had microcysts and macrocysts were not observed(P=0.001).The maximum diameter of the GEA masses was(4.030±0.375)cm,and that of UEA masses was(2.315±0.769)cm,with a statistically significant difference(t=7.134,P<0.001).Conclusion By integrating multiple MRI features of the diffuse infiltrative growth pattern(such as the predominant location in the upper or entire cervix,frequent association with uterine cavity effusion or larger cysts,and a maximum mass diameter≥4 cm),it aids in distinguishing GEA from UEA.

Objective To investigate the nebulization characteristics of human interferon α1b for injection.To characterize and compare the delivery rate,total drug substance and the aerodynamic characteristics between the two types of nebulizer.Methods Connect two types of nebulizers with a breathing simulator,respectively,and simulate the breathing patterns of an infant and child.Measure the delivery rate,total delivered dose,and delivery uniformity.The aerodynamic properties of human interferon α1b for injection were evaluated by the next generation impactor(NGI).The content of human interferon α1b was quantified by double antibody sandwich ELISA.Results In the three batches of samples in infant mode,the delivery rate and total delivered dose determind by A nebulizer were 0.45,0.49,0.44 μg·min-1,3.06,3.21,3.81 μg,respectcively;and 0.12,0.14,0.16 μg·min-1,0.73,0.73,0.75 μg,respectcively by B nebulizer.In child mode,the delivery rate and total delivered doses determined by A nebulizer were 1.36,1.49,1.20 μg·min-1,7.44,7.17,and 6.54 μg,respectcively;and 0.37,0.36,0.43 μg·min-1,1.66,1.59,and 1.41 μg,respectcively by B nebulizer.In child and infant mode,the ten results of the total drug substance delivered determined by nebulizer A were both between 65%to 135%of the average.The FPD,FPF,MMAD,and GSD determined by A neublizer of three batch samples were 2.48,2.92,2.35 μg,59.0%,57.4%,59.1%,4.18,4.34,4.15 μm,1.94,1.98,2.01,respectively.The FPD,FPF,MMAD and GSD determined by B neublizer of three batches samples were 2.70,3.38,3.06 μg,67.6%,66.4%,66.3%,3.55,3.65,3.68 μm,2.03,2.04,2.06,respectively.Conclusions The data obtained in this research characterized the in vitro nebulization characteristics of human interferon α1b for injection and provided a theoretical basis and reference for in vitro study and clinical practice.The influence of different types of nebulizers on nebulization characteristics was evaluated as well.It is suggested that the quality standard of nebulizers be strictly formulated and the use of nebulizers be standardized.

Objective:To investigate the efficacy and safety of allopurinol in stable coronary heart disease patients with asymptomatic hyperuricemia (AH).Methods:Sixty stable coronary heart disease with AH patients admitted to the Changsha Third Hospital from January 2022 to December 2023 were selected. Patients were randomly divided into an observation group (allopurinol treatment group) and a control group (placebo group). The results of tablet exercise tests, treatment efficacy, blood uric acid levels, liver and kidney function indicators, and incidence of adverse events were compare and analyzed between two groups of patients.Results:The observation group had better exercise termination time, maximum ST descent time, and ST recovery time than the control group (all P<0.05). The total effective rate of the observation group after 6 weeks of treatment was significantly higher than that of the control group, and the difference was statistically significant (χ 2=5.455, P=0.02). There was no statistically significant difference in blood uric acid levels and liver and kidney function indicators between the two groups before treatment (all P>0.05). After treatment, both groups showed significant improvement in blood uric acid levels and liver and kidney function indicators compared to before treatment (all P<0.05). The levels of blood uric acid and liver and kidney function indicators in the observation group were significantly better than those in the control group after treatment (all P<0.05). The incidence of adverse events in the observation group during treatment was lower than that in the control group (χ 2=5.192, P=0.023). Conclusions:Allopurinol has a certain therapeutic effect on stable coronary heart disease with asymptomatic hyperuricemia, which helps patients enhance their physical activity and reduce the incidence of cardiovascular events.