1.Long-term prognostic follow-up analysis of multiphasic myelin oligodendrocyte glycoprotein antibody-associated disease in children
Xuting CHANG ; Shangru LI ; Jie ZHANG ; Cuijie WEI ; Han XIE ; Yuan WU ; Yuehua ZHANG ; Xinhua BAO ; Yao ZHANG ; Xingzhi CHANG ; Taoyun JI ; Yuwu JIANG ; Ye WU
Chinese Journal of Pediatrics 2025;63(10):1079-1084
Objective:To investigate the long-term prognosis and related factors in children with multiphasic myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD).Methods:A bidirectional cohort study was conducted. This study included 41 children with MOGAD who were treated at the Children′s Medical Center of Peking University First Hospital between January 2013 and December 2024, with a disease duration of ≥5 years. Demographic characteristics, clinical episodes, therapy, and prognostic indicators (including the expanded disability status scale (EDSS) and modified Rankin scale (mRS)) were collected. Children were stratified into relapse and non-relapse groups based on the presence or absence of relapse within 5 years of the last follow-up. χ2 test or Mann-Whitney U test was used to analyze factors associated with relapse. The Log-rank test was used to compare relapse-free rates between children with disease onset 0-<5 years and those with onset at 5-10 years. Results:A total of 41 children were enrolled, including 20 boys and 21 girls. The age at onset was 5.3 (3.8, 8.5) years, the age at last follow-up was 16.1 (13.2, 17.5) years, and the disease duration was 9.4 (8.1, 10.9) years. The annualized relapse rate (ARR) during follow-up was 0.34 (0.19, 0.56) times/year. The duration to first relapse was 0.8 (0.4, 1.5) years. At the last follow-up, the EDSS score was 0.0 (0.0, 0.0) score, and the mRS score was 0 (0, 0) score. A total of 40 children (98%) experienced relapses within the first 5 years after onset, while only 1 child (2%) relapsed at 6.7 years. The relapse rate between 5-10 years was lower than that between 0-<5 years ( HR=0.27, 95% CI 0.16-0.47, P<0.001). A total of 25 children (61.0%) exhibited clustered relapses during the disease course. There were 20 children (49%) in non-relapse groups, who were aged 16.6 (14.8, 17.6) years, disease duration 9.8 (9.3, 10.8) years at the last follow-up. Among those 20 children, 15 children (75%) had discontinued corticosteroids and immunosuppressants. The relapse group had higher clinical event rates and ARR compared to the relapse-free group (both P<0.01), the age at last follow-up was yonger ( P<0.05), while no significant differences were observed in age at onset, disease duration, or timing of immunosuppressant use (all P>0.05). Conclusions:Pediatric multiphasic MOGAD generally has a favorable prognosis, about half of patients remain relapse-free for ≥5 years at last follow-up. Relapses predominantly occur early in the disease course (mostly within 5 years of onset) and often exhibit a clustered pattern.
2.Clinical characteristics analysis of two Chinese siblings with Susac syndrome and literature review
Hui DONG ; Yulan LI ; Xiaoli XU ; Shulei LIU ; Shuyi LIU ; Han XIE ; Yuan WU ; Xingzhi CHANG ; Jing ZHANG ; Chen XING ; Chunying GUO ; Jun WANG ; Ye WU ; Xinhua BAO
Chinese Journal of Applied Clinical Pediatrics 2025;40(11):856-860
Objective:To investigate the clinical manifestation, therapy, and prognosis of Susac syndrome and enhance the understanding of this disease.Methods:A case summary was made.The clinical data of two siblings with Susac syndrome treated at Children′s Medical Center, Peking University First Hospital in January 2024 were summarized.Reported cases of pediatric Susac syndrome were reviewed.Results:The onset of the disease in the two siblings was at the age of 3.00 and 6.75 years, with recurrent headaches, tinnitus, hearing loss and encephalopathy symptoms.Cranial magnetic resonance imaging showed multiple cerebral microbleeding and microinfarction lesions, " snowball like" in the corpus callosum and diffuse white matter edema in the brain.Audiometry revealed sensorineural hearing loss.In one case, ophthalmic fluorescein angiography revealed ischemic changes due to branch retinal artery occlusions.No pathogenic variants were detected in gene testing.This child was diagnosed with Susac syndrome, and the symptoms were improved after treatment with Corticosteroids and Rituximab.No relapse was observed during the 9-month follow-up.A total of 20 pediatric cases of Susac syndrome were retrieved, including 18 reported previously and 2 cases from this study.There were 2 boys and 18 girls, with the age of onset ranging from 2.5 to 17.0 years.The common initial symptoms included headache (19 cases), vertigo and tinnitus or hearing loss (9 cases), and vision impairment or visual field defect (4 cases). The symptoms were improved after immunotherapy.Conclusions:With a low incidence, Susac syndrome is rare in children and difficult to diagnose.There may be a genetic predisposition in such disease.Early diagnosis and immunotherapy can low the relapse and improve the prognosis.
3.Clinical characteristics analysis of two Chinese siblings with Susac syndrome and literature review
Hui DONG ; Yulan LI ; Xiaoli XU ; Shulei LIU ; Shuyi LIU ; Han XIE ; Yuan WU ; Xingzhi CHANG ; Jing ZHANG ; Chen XING ; Chunying GUO ; Jun WANG ; Ye WU ; Xinhua BAO
Chinese Journal of Applied Clinical Pediatrics 2025;40(11):856-860
Objective:To investigate the clinical manifestation, therapy, and prognosis of Susac syndrome and enhance the understanding of this disease.Methods:A case summary was made.The clinical data of two siblings with Susac syndrome treated at Children′s Medical Center, Peking University First Hospital in January 2024 were summarized.Reported cases of pediatric Susac syndrome were reviewed.Results:The onset of the disease in the two siblings was at the age of 3.00 and 6.75 years, with recurrent headaches, tinnitus, hearing loss and encephalopathy symptoms.Cranial magnetic resonance imaging showed multiple cerebral microbleeding and microinfarction lesions, " snowball like" in the corpus callosum and diffuse white matter edema in the brain.Audiometry revealed sensorineural hearing loss.In one case, ophthalmic fluorescein angiography revealed ischemic changes due to branch retinal artery occlusions.No pathogenic variants were detected in gene testing.This child was diagnosed with Susac syndrome, and the symptoms were improved after treatment with Corticosteroids and Rituximab.No relapse was observed during the 9-month follow-up.A total of 20 pediatric cases of Susac syndrome were retrieved, including 18 reported previously and 2 cases from this study.There were 2 boys and 18 girls, with the age of onset ranging from 2.5 to 17.0 years.The common initial symptoms included headache (19 cases), vertigo and tinnitus or hearing loss (9 cases), and vision impairment or visual field defect (4 cases). The symptoms were improved after immunotherapy.Conclusions:With a low incidence, Susac syndrome is rare in children and difficult to diagnose.There may be a genetic predisposition in such disease.Early diagnosis and immunotherapy can low the relapse and improve the prognosis.
4.Long-term prognostic follow-up analysis of multiphasic myelin oligodendrocyte glycoprotein antibody-associated disease in children
Xuting CHANG ; Shangru LI ; Jie ZHANG ; Cuijie WEI ; Han XIE ; Yuan WU ; Yuehua ZHANG ; Xinhua BAO ; Yao ZHANG ; Xingzhi CHANG ; Taoyun JI ; Yuwu JIANG ; Ye WU
Chinese Journal of Pediatrics 2025;63(10):1079-1084
Objective:To investigate the long-term prognosis and related factors in children with multiphasic myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD).Methods:A bidirectional cohort study was conducted. This study included 41 children with MOGAD who were treated at the Children′s Medical Center of Peking University First Hospital between January 2013 and December 2024, with a disease duration of ≥5 years. Demographic characteristics, clinical episodes, therapy, and prognostic indicators (including the expanded disability status scale (EDSS) and modified Rankin scale (mRS)) were collected. Children were stratified into relapse and non-relapse groups based on the presence or absence of relapse within 5 years of the last follow-up. χ2 test or Mann-Whitney U test was used to analyze factors associated with relapse. The Log-rank test was used to compare relapse-free rates between children with disease onset 0-<5 years and those with onset at 5-10 years. Results:A total of 41 children were enrolled, including 20 boys and 21 girls. The age at onset was 5.3 (3.8, 8.5) years, the age at last follow-up was 16.1 (13.2, 17.5) years, and the disease duration was 9.4 (8.1, 10.9) years. The annualized relapse rate (ARR) during follow-up was 0.34 (0.19, 0.56) times/year. The duration to first relapse was 0.8 (0.4, 1.5) years. At the last follow-up, the EDSS score was 0.0 (0.0, 0.0) score, and the mRS score was 0 (0, 0) score. A total of 40 children (98%) experienced relapses within the first 5 years after onset, while only 1 child (2%) relapsed at 6.7 years. The relapse rate between 5-10 years was lower than that between 0-<5 years ( HR=0.27, 95% CI 0.16-0.47, P<0.001). A total of 25 children (61.0%) exhibited clustered relapses during the disease course. There were 20 children (49%) in non-relapse groups, who were aged 16.6 (14.8, 17.6) years, disease duration 9.8 (9.3, 10.8) years at the last follow-up. Among those 20 children, 15 children (75%) had discontinued corticosteroids and immunosuppressants. The relapse group had higher clinical event rates and ARR compared to the relapse-free group (both P<0.01), the age at last follow-up was yonger ( P<0.05), while no significant differences were observed in age at onset, disease duration, or timing of immunosuppressant use (all P>0.05). Conclusions:Pediatric multiphasic MOGAD generally has a favorable prognosis, about half of patients remain relapse-free for ≥5 years at last follow-up. Relapses predominantly occur early in the disease course (mostly within 5 years of onset) and often exhibit a clustered pattern.
5.Chest CT findings and clinical features of coronavirus disease 2019 in children
Zheng ZHONG ; Xingzhi XIE ; Wei HUANG ; Wei ZHAO ; Qizhi YU ; Jun LIU
Journal of Central South University(Medical Sciences) 2020;45(3):236-242
Objective: To describe the CT features and clinical characteristics of pediatric patients with coronavirus disease 2019 (COVID-19).Method: A total of 9 COVID-19 infected pediatric patients were included in this study.Clinical history,laboratory examination,and detailed CT imaging features were analyzed.All patients underwent the first CT scanning on the same day of being diagnosed by realtime reverse-transcription polymerase chain reaction (rRT-PCR).A low-dose CT scan was performed during follow-up.Results: All the child patients had positive results.Four patients had cough and one patient had fever.One patient presented both cough and fever.Two children presented other symptoms like sore throat and stuffy nose.One child showed no clinical symptom.Five patients had positive initial CT findings with subtle lesions like ground-glass opacity (GGO) or spot-like mixed consolidation.Three patients were reported with negative results in the initial and follow-up CT examination.One patient was reported with initial negative CT findings but turning positive during the first follow-up.All patients had absorbed lesions on follow-up CT images after treatment.Conclusion: Pediatric COVID-19 patients have certain imaging and clinical features as well as disease prognosis.Children with COVID-19 tend to have normal or subtle CT findings and relatively better outcome.
6.Changes of biochemical markers of bone turnover and bone mineral density in mid- and old-aged women
Hong LIU ; Eryuan LIAO ; Xianping WU ; Hong ZHANG ; Hui XIE ; Xingzhi CAO
Chinese Journal of Endocrinology and Metabolism 2009;25(2):164-167
Objective To study the changes of biochemical markers of bone turnover and bone mineral density (BMD) in mid- and old-aged women.Methods A total of 408 female volunteers aged 40 to 86 years were enrolled.Serum bone alkaline phosphatase (BAP),osteoealtin and urine type I collagen N-telopeptide(uN'TX) were measured using ELISA,and BM D at anteroposterior lumbar spine 1-4(L1-4)and femoral neck (FN) was detected by dual energy X-ray absorptiometry (QDR4500A).Results (1) Serum BAP,osteocaltin and uNTX levels were positively correlated with age,parity,number of births and duration of menopause (all P

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