1.Expert consensus on the application of nasal cavity filling substances in nasal surgery patients(2025, Shanghai).
Keqing ZHAO ; Shaoqing YU ; Hongquan WEI ; Chenjie YU ; Guangke WANG ; Shijie QIU ; Yanjun WANG ; Hongtao ZHEN ; Yucheng YANG ; Yurong GU ; Tao GUO ; Feng LIU ; Meiping LU ; Bin SUN ; Yanli YANG ; Yuzhu WAN ; Cuida MENG ; Yanan SUN ; Yi ZHAO ; Qun LI ; An LI ; Luo BA ; Linli TIAN ; Guodong YU ; Xin FENG ; Wen LIU ; Yongtuan LI ; Jian WU ; De HUAI ; Dongsheng GU ; Hanqiang LU ; Xinyi SHI ; Huiping YE ; Yan JIANG ; Weitian ZHANG ; Yu XU ; Zhenxiao HUANG ; Huabin LI
Journal of Clinical Otorhinolaryngology Head and Neck Surgery 2025;39(4):285-291
This consensus will introduce the characteristics of fillers used in the surgical cavities of domestic nasal surgery patients based on relevant literature and expert opinions. It will also provide recommendations for the selection of cavity fillers for different nasal diseases, with chronic sinusitis as a representative example.
Humans
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Nasal Cavity/surgery*
;
Nasal Surgical Procedures
;
China
;
Consensus
;
Sinusitis/surgery*
;
Dermal Fillers
2.Chinese expert consensus on the evaluation of allergen-specific immunotherapy outcomes(Wuhan, 2025).
Yuqin DENG ; Xi LUO ; Zhuofu LIU ; Shuguang SUN ; Jing YE ; Tiansheng WANG ; Jianjun CHEN ; Meiping LU ; Yin YAO ; Ying WANG ; Wei ZHOU ; Bei LIU ; Qingxiang ZENG ; Yuanteng XU ; Qintai YANG ; Yucheng YANG ; Feng LIU ; Chengli XU ; Yanan SUN ; Haiyu HONG ; Haibo YE ; Liqiang ZHANG ; Fenghong CHEN ; Huabin LI ; Hongtian WANG ; Yuncheng LI ; Wenlong LIU ; Yu XU ; Hongfei LOU
Journal of Clinical Otorhinolaryngology Head and Neck Surgery 2025;39(11):1075-1085
Allergen-specific immunotherapy(AIT) remains the only therapeutic approach with the potential to modify the natural course of allergic rhinitis(AR). Nevertheless, considerable inter-individual variability exists in patients'responses to AIT. To facilitate more reliable assessment of treatment efficacy, the China Rhinopathy Research Cooperation Group(CRRCG) convened young and middle-aged nasal experts in China to formulate the present consensus. The recommended subjective outcome measures for AIT comprise symptom scores, medication scores, combined symptom and medication scores, quality-of-life assessments, evaluation of disease control, and assessment of comorbidities. Objective indicators may supplement these measures. Currently available objective approaches include skin prick testing, nasal provocation testing, and allergen exposure chambers. However, these methods remain constrained by practical limitations and are not yet appropriate for routine implementation in clinical efficacy evaluation. In addition, several biomarkers, including sIgE and the sIgE/tIgE ratio, sIgG4, serum IgE-blocking activity, IgA, cytokines and chemokines, as well as immune cell surface molecules and their functional activity, have been shown to have associations with AIT outcomes. While these biomarkers may complement subjective assessments, they are subject to significant limitations. Consequently, large-scale multicenter trials and real-world evidence are required to strengthen the evidence base. The present consensus underscores the necessity of integrating patients'subjective experiences with objective testing throughout the treatment process, thereby providing a more comprehensive and accurate framework for efficacy evaluation. Looking forward, future investigations should prioritize the incorporation of multi-omics data and artificial intelligence methodologies, which hold promise for overcoming current limitations in assessment strategies and for advancing both the standardization and personalization of AIT.
Humans
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Allergens/immunology*
;
China
;
Consensus
;
Desensitization, Immunologic
;
Immunoglobulin E
;
Quality of Life
;
Rhinitis, Allergic/therapy*
;
Treatment Outcome
;
East Asian People
3.Clinical Evaluation of the Short-term Effects Assessment of the Condyle-stimulated Headband Bone Conduction Hearing Aids on Conductive,Mixed,and Sensorineural Hearing Loss
Yun LI ; Hongzheng ZHANG ; Jieqing CAI ; Meiping HUANG ; Lu YANG ; Bingyan YAN ; Yihang SONG ; Xin XI
Journal of Audiology and Speech Pathology 2025;33(1):50-54
Objective To study the hearing intervention effects of the Shokz condyle-stimulated headband bone-conduction hearing aid on patients with conductive,mixed,and sensorineural hearing loss and to explore its clinical application prospects.Methods A total of 55 patients with hearing loss(age 18~82)participated in the study.Among them,9 had conductive hearing loss,15 had sensorineural hearing loss,and 31 had mixed hearing loss.Their bilat-eral bone conduction pure tone thresholds at 0.5,1,2,and 4 kHz were all ≤60 dB HL.The patients were fitted with the condyle-stimulated headband bone-conduction hearing aid.Hearing thresholds in sound field,single-syllable speech recogni-tion scores in quiet,and sentence recognition thresholds in quiet were assessed before fitting and on the day of 14±2 after fitting to compare differences in results.The effectiveness of the hearing aids on the day of 14±2 after fitting was also eval-uated using the IOI-HA questionnaire.Results After wearing the bone-conduction hearing aid,the average hearing thresh-old and sentence recognition threshold of the patients decreased significantly compared with before fitting(the average hear-ing threshold:56.5±8.2 dB HL before fitting,39.3±4.9 dB HL on the day of 14±2 after fitting;sentence recognition threshold:48.6±9.7 dB HL before fitting,34.3±5.6 dB HL on the day of 14±2 after fitting),and the difference was statistically significant(P<0.001).The single-syllable speech recognition score before fitting was 29.8%±11.4%,and on the day of 14±2 after fitting,it was 72.4%±14.4%,the difference was statistically significant(P<0.001).The av-erage total score of the IOI-HA questionnaire was 29.0±3.8 points.Conclusion Condyle-stimulated headband bone-con-duction hearing aids can significantly improve the hearing and speech recognition ability of patients with conductive,mixed and sensorineural hearing loss whose bone conduction pure tone thresholds at 0.5~4 kHz were ≤60 dB HL.It may poten-tially improve the quality of life for patients with hearing loss and holds substantial clinical application value.
4.Surgical treatment of primary giant gastrointestinal stromal tumor
Zaizhong ZHANG ; Pan ZHAO ; Chunhong XIAO ; Meiping WANG ; Weixuan HONG ; Junwei FANG ; Lie WANG
Journal of Clinical Surgery 2025;33(6):581-584
Objective To explore the surgical treatment experience of primary giant gastrointestinal stromal tumors(GIST)(with isolated lesions with a maximum diameter>10 cm).Methods A retrospective analysis was conducted on the clinical and pathological data of 67 patients with primary giant GIST admitted from January 2018 to December 2024.Among them,35 cases underwent surgical operations after preoperative neoadjuvant therapy(25 effective cases and 10 ineffective cases)(neoadjuvant therapy group).Due to the initial diagnosis assessment expecting radical(R0)resection(13 cases),or preoperative complications(12 cases),or difficulty in obtaining a pathological diagnosis through puncture biopsy(7 cases),32 cases underwent direct surgery without neoadjuvant therapy(direct surgery group).Compare the general information,tumor condition,surgical condition,postoperative recovery,postoperative pathology,postoperative adjuvant therapy,and recurrence between two groups.Results Comparative analysis revealed that there was no statistically significant difference(P>0.05)between the neoadjuvant therapy group and the direct surgery group in terms of gender,age,primary tumor location,initial maximum diameter,growth type,localized or locally advanced stage,and postoperative follow-up time.The maximum diameters of the tumors before surgery in the neoadjuvant therapy group and the direct surgery group were(12.4±7.1)cm and(18.2±5.0)cm respectively,and the operation times were(125.4±30.6)minutes and(153.0±31.7)minutes respectively.The intraoperative blood loss was(228.3±76.4)ml and(300.3±67.2)ml,respectively.The postoperative hospital stay was(9.1±2.6)days and(11.1±3.2)days,respectively.There was a statistically significant difference between the two groups(P<0.05).The proportion of laparoscopic surgery in the neoadjuvant therapy group was 17.1%,which was higher than that in the direct surgery group(0),and the difference was statistically significant(P<0.05).There was no statistically significant difference between the two groups in terms of the proportion of tumor rupture,combined organ resection,postoperative complications and postoperative recurrence(P>0.05).Conclusion Primary giant gastrointestinal stromal tumors can mostly be reduced in size and progression through neoadjuvant therapy,improving the chances of minimally invasive surgery.However,there is also a risk of tumor progression during neoadjuvant therapy leading to increased surgical difficulty or even loss of curative surgical opportunities.
5.Clinical features and prognosis of Castleman disease in 29 children
Xiaofei ZHU ; Zhaoling WANG ; Lifeng ZHANG ; Xiaojun XU ; Meiping LU
Chinese Journal of Rheumatology 2025;29(7):583-589
Objective:To investigate the clinical features, treatment strategies, and prognosis of pediatric Castleman disease (CD), with the aim of enhancing disease recognition and improving diagnostic and therapeutic approaches.Methods:A retrospective analysis was conducted on the clinical data of 29 children diagnosed with CD at the Children′s Hospital, Zhejiang University School of Medicine, between January 2016 and January 2024. Age, sex, initial presentations, routine blood test, inflammatory indicators, coagulation function test, immunoglobulin level, histopathological examination, imaging examination, treatment and follow-ups were recorded. Patients were classified into two groups based on the presence or absence of symptoms: the symptomatic Castleman disease group (SC) and the asymptomatic Castleman disease group (AC). All patients were followed for a minimum of six months. Clinical characteristics, therapeutic regimens, and outcomes were compared between the two groups.For the measurement information, the independent samples t test was used for comparison between groups of data that conformed to normal distribution; the Mann-Whitney U test was used for comparison between groups of data that did not conform to normal distribution. The Chi-square test or Fisher′s exact probability method was used for comparison between groups of count data. Results:A total of 29 pediatric CD cases were included, comprising 15 males and 14 females. The number of patients diagnosed as unicentric Castleman disease (UCD) was 21, with the rest 8 as multicentric Castleman disease (MCD). There were 11 patients (37.9%) in the SC group and 18 patients (62.1%) in the AC group. The median age at onset was 10.0 years (interquartile range: 6.5-12.2 years). The most common initial presentation was mass ( n=20), followed by fever ( n=2), amaemia ( n=2), fever with fatigue ( n=2), rash ( n=1), abdominal pain ( n=1), and cough ( n=1). The frequently involved sites were neck ( n=8), abdominal cavity ( n=5), mediastinum ( n=3), retroperitoneum ( n=2), and one case each in the back, upper arm, and pelvis. The maximum lesion volume reached 1 040 cm 3. Compared to the AC group, the SC group had significantly lower hemoglobin levels [108.0(92.0, 123.0)g/L vs. 127.0(117.5, 139.0)g/L, Z=-2.35, P=0.019] and significantly higher levels of C-reactive protein (CRP) [38.0(3.0, 87.0)mg/L vs. 0.6(0.5, 3.8)mg/L, Z=-2.19, P=0.029], prothrombin time[12.6(11.4, 13.3)s vs. 11.3(10.5, 11.7)s, Z=-2.64, P=0.008], and fibrinogen [4.5(3.5, 5.4)g/L vs. 2.1(1.9, 2.6)g/L, Z=-3.04, P=0.002]. All patients underwent MRI/CT and ultrasonography; however, only 2 cases were diagnosed as CD by ultrasound. All patients underwent surgical excision of the mass, and diagnoses were confirmed by histopathological examination. Among the SC group, 4 patients (4/11, 36.4%) required postoperative treatment with glucocorticoids alone or in combination with immunosuppressants and biologics. The overall prognosis was favorable, with follow-up ranging from 6 months to 7 years and no reported mortality. One case, initially misdiagnosed as systemic lupus erythematosus, was later confirmed to be CD complicated with paraneoplastic pemphigus (PNP); this patient experienced recurrent fever, rash, and elevated CRP, and continued to require low-dose glucocorticoids and tacrolimus during the 4-year follow-up. Conclusion:In chileren, CD is most common in the neck and surgical resection yields favorable outcomes. Approximately 37.9% of patients present with systemic symptoms, of which about 36.4% require postoperative systemic therapy such as glucocorticosteroids. CD complicated with PNP may be misdiagnosed. Symptomatic cases are often associated with anemia, elevated CRP levels, and coagulation abnormalities. UCD pediatric patients without complications have a good prognosis and there′s no need for long-term medications after surgery. About 37.5% of MCD pediatric patients require glucocorticoids, immunosuppressants, biotherapy or chemotherapy to control symptoms in the postoperative period. Imaging modalities have limited diagnostic value, and histopathological examination remains the gold standard for diagnosis.
6.Clinical characteristics of juvenile dermatomyositis in anti-nuclear matrix protein 2 antibody-positive patients and risk factors for severity: a national multicenter retrospective study
Huiyuan YANG ; Wanzhen GUAN ; Ling2 YANG ; Haimei LIU ; Xiaoqing3 LI ; Haiguo YU ; Meiping LU ; Jun YANG ; Xiaohui LIU ; Hongxia ZHANG ; Wei ZHANG ; Jihong XIAO ; Xiaozhong LI ; Guomin LI ; Hong CHANG ; Sheng HAO ; Yue DU ; Daliang XU ; Ling WU ; Wenjie ZHENG ; Li LIU ; Xinhui JIANG ; Shaohui ZHU ; Dongmei ZHAO ; Xuemei TANG ; Li SUN
Chinese Journal of Pediatrics 2025;63(12):1299-1305
Objective:To investigate the clinical characteristics and independent risk factors of severe disease in patients with anti-nuclear matrix protein (NXP) 2 antibody-positive juvenile dermatomyositis (JDM).Methods:A retrospective cohort study was conducted, including 219 anti-NXP2 antibody-positive JDM patients admitted to 23 children′s hospitals across China from July 2011 to July 2023. Patients were classified into severe and non-severe groups based on classification criteria for severe dermatomyositis. Demographic characteristics, clinical manifestations, and laboratory parameters were compared between the 2 groups using independent sample t-test, Mann-Whitney U test, or χ2 test. Univariate and multivariate Logistic regression analyses were performed to identify risk factors for severe disease. The receiver operating characteristic curve was employed to calculate optimal cut-off values. Results:Among the 219 patients, 108 were male and 111 were female, with an age at onset of 6.3 (3.5, 9.4) years. The severe group comprised 69 patients, and the non-severe group 150 patients. The severe group had significantly higher rates of fever, heliotrope rash, subcutaneous edema, periorbital edema, anti-Ro52 antibody positivity, as well as elevated levels of ferritin-to-albumin ratio (FAR), creatine kinase (CK), aspartate aminotransferase (AST), and lactate dehydrogenase (LDH) (all P<0.05). Multivariate analysis identified anti-Ro52 antibody positivity ( OR=13.26, 95% CI 1.37-128.29) and elevated FAR ( OR=1.90, 95% CI 1.09-2.31) as independent risk factors for severe anti-NXP2 antibody-positive JDM (both P<0.05). Receiver operating characteristic curve analysis revealed that a FAR cutoff value of 6.82 predicted severe disease with an area under the curve of 0.87 (95% CI 0.81-0.94, P<0.001), sensitivity of 0.85, and specificity of 0.70. All patients received glucocorticoid therapy, and the severe group received higher proportions of steroid pulse therapy, cyclophosphamide, mycophenolate mofetil, intravenous immunoglobulin, biologics, and adjuvant treatments compared to the non-severe group (all P<0.05). In terms of outcomes, 2 patients (2.9%) in the severe group died (due to neurological involvement and intestinal perforation, respectively), while the remaining patients achieved complete clinical response or remission. All patients in the non-severe group achieved remission. Conclusions:The primary clinical features of anti-NXP2 antibody-positive JDM included fever, heliotrope rash, subcutaneous edema, periorbital edema, anti-Ro52 antibody positivity, and elevated levels of CK, AST, LDH, and FAR. Furthermore, anti-Ro52 antibody positivity and a FAR>6.82 were identified as independent risk factors.
7.Surgical treatment of primary giant gastrointestinal stromal tumor
Zaizhong ZHANG ; Pan ZHAO ; Chunhong XIAO ; Meiping WANG ; Weixuan HONG ; Junwei FANG ; Lie WANG
Journal of Clinical Surgery 2025;33(6):581-584
Objective To explore the surgical treatment experience of primary giant gastrointestinal stromal tumors(GIST)(with isolated lesions with a maximum diameter>10 cm).Methods A retrospective analysis was conducted on the clinical and pathological data of 67 patients with primary giant GIST admitted from January 2018 to December 2024.Among them,35 cases underwent surgical operations after preoperative neoadjuvant therapy(25 effective cases and 10 ineffective cases)(neoadjuvant therapy group).Due to the initial diagnosis assessment expecting radical(R0)resection(13 cases),or preoperative complications(12 cases),or difficulty in obtaining a pathological diagnosis through puncture biopsy(7 cases),32 cases underwent direct surgery without neoadjuvant therapy(direct surgery group).Compare the general information,tumor condition,surgical condition,postoperative recovery,postoperative pathology,postoperative adjuvant therapy,and recurrence between two groups.Results Comparative analysis revealed that there was no statistically significant difference(P>0.05)between the neoadjuvant therapy group and the direct surgery group in terms of gender,age,primary tumor location,initial maximum diameter,growth type,localized or locally advanced stage,and postoperative follow-up time.The maximum diameters of the tumors before surgery in the neoadjuvant therapy group and the direct surgery group were(12.4±7.1)cm and(18.2±5.0)cm respectively,and the operation times were(125.4±30.6)minutes and(153.0±31.7)minutes respectively.The intraoperative blood loss was(228.3±76.4)ml and(300.3±67.2)ml,respectively.The postoperative hospital stay was(9.1±2.6)days and(11.1±3.2)days,respectively.There was a statistically significant difference between the two groups(P<0.05).The proportion of laparoscopic surgery in the neoadjuvant therapy group was 17.1%,which was higher than that in the direct surgery group(0),and the difference was statistically significant(P<0.05).There was no statistically significant difference between the two groups in terms of the proportion of tumor rupture,combined organ resection,postoperative complications and postoperative recurrence(P>0.05).Conclusion Primary giant gastrointestinal stromal tumors can mostly be reduced in size and progression through neoadjuvant therapy,improving the chances of minimally invasive surgery.However,there is also a risk of tumor progression during neoadjuvant therapy leading to increased surgical difficulty or even loss of curative surgical opportunities.
8.Clinical Evaluation of the Short-term Effects Assessment of the Condyle-stimulated Headband Bone Conduction Hearing Aids on Conductive,Mixed,and Sensorineural Hearing Loss
Yun LI ; Hongzheng ZHANG ; Jieqing CAI ; Meiping HUANG ; Lu YANG ; Bingyan YAN ; Yihang SONG ; Xin XI
Journal of Audiology and Speech Pathology 2025;33(1):50-54
Objective To study the hearing intervention effects of the Shokz condyle-stimulated headband bone-conduction hearing aid on patients with conductive,mixed,and sensorineural hearing loss and to explore its clinical application prospects.Methods A total of 55 patients with hearing loss(age 18~82)participated in the study.Among them,9 had conductive hearing loss,15 had sensorineural hearing loss,and 31 had mixed hearing loss.Their bilat-eral bone conduction pure tone thresholds at 0.5,1,2,and 4 kHz were all ≤60 dB HL.The patients were fitted with the condyle-stimulated headband bone-conduction hearing aid.Hearing thresholds in sound field,single-syllable speech recogni-tion scores in quiet,and sentence recognition thresholds in quiet were assessed before fitting and on the day of 14±2 after fitting to compare differences in results.The effectiveness of the hearing aids on the day of 14±2 after fitting was also eval-uated using the IOI-HA questionnaire.Results After wearing the bone-conduction hearing aid,the average hearing thresh-old and sentence recognition threshold of the patients decreased significantly compared with before fitting(the average hear-ing threshold:56.5±8.2 dB HL before fitting,39.3±4.9 dB HL on the day of 14±2 after fitting;sentence recognition threshold:48.6±9.7 dB HL before fitting,34.3±5.6 dB HL on the day of 14±2 after fitting),and the difference was statistically significant(P<0.001).The single-syllable speech recognition score before fitting was 29.8%±11.4%,and on the day of 14±2 after fitting,it was 72.4%±14.4%,the difference was statistically significant(P<0.001).The av-erage total score of the IOI-HA questionnaire was 29.0±3.8 points.Conclusion Condyle-stimulated headband bone-con-duction hearing aids can significantly improve the hearing and speech recognition ability of patients with conductive,mixed and sensorineural hearing loss whose bone conduction pure tone thresholds at 0.5~4 kHz were ≤60 dB HL.It may poten-tially improve the quality of life for patients with hearing loss and holds substantial clinical application value.
9.Clinical features and prognosis of Castleman disease in 29 children
Xiaofei ZHU ; Zhaoling WANG ; Lifeng ZHANG ; Xiaojun XU ; Meiping LU
Chinese Journal of Rheumatology 2025;29(7):583-589
Objective:To investigate the clinical features, treatment strategies, and prognosis of pediatric Castleman disease (CD), with the aim of enhancing disease recognition and improving diagnostic and therapeutic approaches.Methods:A retrospective analysis was conducted on the clinical data of 29 children diagnosed with CD at the Children′s Hospital, Zhejiang University School of Medicine, between January 2016 and January 2024. Age, sex, initial presentations, routine blood test, inflammatory indicators, coagulation function test, immunoglobulin level, histopathological examination, imaging examination, treatment and follow-ups were recorded. Patients were classified into two groups based on the presence or absence of symptoms: the symptomatic Castleman disease group (SC) and the asymptomatic Castleman disease group (AC). All patients were followed for a minimum of six months. Clinical characteristics, therapeutic regimens, and outcomes were compared between the two groups.For the measurement information, the independent samples t test was used for comparison between groups of data that conformed to normal distribution; the Mann-Whitney U test was used for comparison between groups of data that did not conform to normal distribution. The Chi-square test or Fisher′s exact probability method was used for comparison between groups of count data. Results:A total of 29 pediatric CD cases were included, comprising 15 males and 14 females. The number of patients diagnosed as unicentric Castleman disease (UCD) was 21, with the rest 8 as multicentric Castleman disease (MCD). There were 11 patients (37.9%) in the SC group and 18 patients (62.1%) in the AC group. The median age at onset was 10.0 years (interquartile range: 6.5-12.2 years). The most common initial presentation was mass ( n=20), followed by fever ( n=2), amaemia ( n=2), fever with fatigue ( n=2), rash ( n=1), abdominal pain ( n=1), and cough ( n=1). The frequently involved sites were neck ( n=8), abdominal cavity ( n=5), mediastinum ( n=3), retroperitoneum ( n=2), and one case each in the back, upper arm, and pelvis. The maximum lesion volume reached 1 040 cm 3. Compared to the AC group, the SC group had significantly lower hemoglobin levels [108.0(92.0, 123.0)g/L vs. 127.0(117.5, 139.0)g/L, Z=-2.35, P=0.019] and significantly higher levels of C-reactive protein (CRP) [38.0(3.0, 87.0)mg/L vs. 0.6(0.5, 3.8)mg/L, Z=-2.19, P=0.029], prothrombin time[12.6(11.4, 13.3)s vs. 11.3(10.5, 11.7)s, Z=-2.64, P=0.008], and fibrinogen [4.5(3.5, 5.4)g/L vs. 2.1(1.9, 2.6)g/L, Z=-3.04, P=0.002]. All patients underwent MRI/CT and ultrasonography; however, only 2 cases were diagnosed as CD by ultrasound. All patients underwent surgical excision of the mass, and diagnoses were confirmed by histopathological examination. Among the SC group, 4 patients (4/11, 36.4%) required postoperative treatment with glucocorticoids alone or in combination with immunosuppressants and biologics. The overall prognosis was favorable, with follow-up ranging from 6 months to 7 years and no reported mortality. One case, initially misdiagnosed as systemic lupus erythematosus, was later confirmed to be CD complicated with paraneoplastic pemphigus (PNP); this patient experienced recurrent fever, rash, and elevated CRP, and continued to require low-dose glucocorticoids and tacrolimus during the 4-year follow-up. Conclusion:In chileren, CD is most common in the neck and surgical resection yields favorable outcomes. Approximately 37.9% of patients present with systemic symptoms, of which about 36.4% require postoperative systemic therapy such as glucocorticosteroids. CD complicated with PNP may be misdiagnosed. Symptomatic cases are often associated with anemia, elevated CRP levels, and coagulation abnormalities. UCD pediatric patients without complications have a good prognosis and there′s no need for long-term medications after surgery. About 37.5% of MCD pediatric patients require glucocorticoids, immunosuppressants, biotherapy or chemotherapy to control symptoms in the postoperative period. Imaging modalities have limited diagnostic value, and histopathological examination remains the gold standard for diagnosis.
10.Clinical characteristics of juvenile dermatomyositis in anti-nuclear matrix protein 2 antibody-positive patients and risk factors for severity: a national multicenter retrospective study
Huiyuan YANG ; Wanzhen GUAN ; Ling2 YANG ; Haimei LIU ; Xiaoqing3 LI ; Haiguo YU ; Meiping LU ; Jun YANG ; Xiaohui LIU ; Hongxia ZHANG ; Wei ZHANG ; Jihong XIAO ; Xiaozhong LI ; Guomin LI ; Hong CHANG ; Sheng HAO ; Yue DU ; Daliang XU ; Ling WU ; Wenjie ZHENG ; Li LIU ; Xinhui JIANG ; Shaohui ZHU ; Dongmei ZHAO ; Xuemei TANG ; Li SUN
Chinese Journal of Pediatrics 2025;63(12):1299-1305
Objective:To investigate the clinical characteristics and independent risk factors of severe disease in patients with anti-nuclear matrix protein (NXP) 2 antibody-positive juvenile dermatomyositis (JDM).Methods:A retrospective cohort study was conducted, including 219 anti-NXP2 antibody-positive JDM patients admitted to 23 children′s hospitals across China from July 2011 to July 2023. Patients were classified into severe and non-severe groups based on classification criteria for severe dermatomyositis. Demographic characteristics, clinical manifestations, and laboratory parameters were compared between the 2 groups using independent sample t-test, Mann-Whitney U test, or χ2 test. Univariate and multivariate Logistic regression analyses were performed to identify risk factors for severe disease. The receiver operating characteristic curve was employed to calculate optimal cut-off values. Results:Among the 219 patients, 108 were male and 111 were female, with an age at onset of 6.3 (3.5, 9.4) years. The severe group comprised 69 patients, and the non-severe group 150 patients. The severe group had significantly higher rates of fever, heliotrope rash, subcutaneous edema, periorbital edema, anti-Ro52 antibody positivity, as well as elevated levels of ferritin-to-albumin ratio (FAR), creatine kinase (CK), aspartate aminotransferase (AST), and lactate dehydrogenase (LDH) (all P<0.05). Multivariate analysis identified anti-Ro52 antibody positivity ( OR=13.26, 95% CI 1.37-128.29) and elevated FAR ( OR=1.90, 95% CI 1.09-2.31) as independent risk factors for severe anti-NXP2 antibody-positive JDM (both P<0.05). Receiver operating characteristic curve analysis revealed that a FAR cutoff value of 6.82 predicted severe disease with an area under the curve of 0.87 (95% CI 0.81-0.94, P<0.001), sensitivity of 0.85, and specificity of 0.70. All patients received glucocorticoid therapy, and the severe group received higher proportions of steroid pulse therapy, cyclophosphamide, mycophenolate mofetil, intravenous immunoglobulin, biologics, and adjuvant treatments compared to the non-severe group (all P<0.05). In terms of outcomes, 2 patients (2.9%) in the severe group died (due to neurological involvement and intestinal perforation, respectively), while the remaining patients achieved complete clinical response or remission. All patients in the non-severe group achieved remission. Conclusions:The primary clinical features of anti-NXP2 antibody-positive JDM included fever, heliotrope rash, subcutaneous edema, periorbital edema, anti-Ro52 antibody positivity, and elevated levels of CK, AST, LDH, and FAR. Furthermore, anti-Ro52 antibody positivity and a FAR>6.82 were identified as independent risk factors.

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