AIM: To assess whether the polymorphisms of NOD2/ CARD15 , autophagy-related 16-like 1 (ATG16L1 ), and interleukin-23 receptor (IL23R ) genes play a more critical role in the susceptibility of childhood-onset than in ...AIM: To assess whether the polymorphisms of NOD2/ CARD15 , autophagy-related 16-like 1 (ATG16L1 ), and interleukin-23 receptor (IL23R ) genes play a more critical role in the susceptibility of childhood-onset than in adult-onset Crohn’s disease (CD). METHODS: Polymorphisms R702W, G908R, and 3020insC of NOD2/CARD15 ; rs2241880 A/G of ATG16L1 , and rs11209026 (R381Q) of IL23R gene were assessed in 110 childhood-onset CD, 364 adult-onset CD, and 539 healthy individuals. Analysis of polymorphisms R702W, G908R, and 3020insC of NOD2/CARD15 genotyping was performed by allele specific polymerase chain reaction (PCR) or by PCR-restriction fragment length polymor-phism analysis. The polymorphisms rs2241880 A/G of the ATG16L1 , and rs11209026 (R381Q) of the IL23R gene in the children’s cohort were genotyped by PCR and melting curve analysis whereas adult group genotyping was performed using the Affymetrix Genome-Wide Human SNP Array 5.0 (500K). RESULTS: The 3020insC allele in NOD2/CARD15 was significantly higher in childhood than in adult-onset CD (P = 0.0067). Association with at least 1 NOD2/CARD15 variant was specific for ileal disease (with or without co- lonic involvement). Even if the frequency of G allele of the rs2241880 ATG16L1 polymorphism was increased in both paediatric and adult CD patients compared to con- trols (P = 0.017 and P = 0.001, respectively), no difference was observed between the childhood and the adult cohort. The rare Q allele of IL23R rs11209026 polymorphism was underrepresented in both paediatric and adult CD cases (P = 0.0018 and P = 0.04, respectively) and no difference was observed between the childhood and the adult cohort. The presence of the rs2241880 ATG16L1 and rs11209026 IL23R polymorphisms did not influence disease phenotype. CONCLUSION: Polymorphism 3020insC in NOD2/ CARD15 occurs statistically significantly more often in patients with childhood-onset CD than in patients with adult-onset CD. The ATG16L1 and IL23R variants are associated with susceptibility to CD, but not earlyonset disease.展开更多
We present a retrospective review of DNA immunoadsorption (DNA-IA) therapy on clinical symptoms as well as indicators in pediatric cases with systemic lupus erythematosus (SLE), and follow up the short-term curative e...We present a retrospective review of DNA immunoadsorption (DNA-IA) therapy on clinical symptoms as well as indicators in pediatric cases with systemic lupus erythematosus (SLE), and follow up the short-term curative effects. 16 SLE cases were treated by DNA-IA for 3 times every other day. We observed the changes on clinical manifestations and immunological indicators, in order to compare the alteration of these indicators including clinical manifestations, Systemic Lupus Erythematosus Disease Active Index (SLEDAI) scores, 24 hurinary protein excretion, autoantibodies, serum IgG and complement C3. 13 cases were followed up regularly, within 3 months after DNA-IA therapy, 12 cases of clinical manifestations improved (92.3%). SLEDAI scores in 10 cases decreased from (16.20 ± 12.54) to less than 5 (76.9%), 8 cases of ANA, anti-DNA antibodies were negative (61.5%), 13 cases with IgG level in serum recovered to normal (10.39 ± 4.38) g/L, C3 level rose to normal (1.06 ± 0.23) g/L. 3 to 6 months after IA, clinical manifestations and laboratory examinations in all cases got maximum improved. 9 months after IA, SLEDAI score in 2 cases (15.4%) rose to more than 5, anti-DNA antibody in 2 cases (15.4%) became positive, and 1case (7.7%) with serum C3 decreased again. 2 cases died from multiple organs dysfunction within 3 to 6 months after IA. No serious complications were found during DNA-IA. We recommend that DNA immunoadsorption is a safe and effective therapy for active childhood-onset SLE, which could improve clinical symptoms, eliminate ANA and anti-DNA antibodies. Combining with corticosteroids and immunosuppressive drugs, DNA-IA could significantly reduce the activity of disease and protect vital organs function in the short term.展开更多
目的评价贝利尤单抗治疗儿童系统性红斑狼疮(cSLE)的有效性和安全性,为临床用药提供循证参考。方法计算机检索中国知网、万方数据、维普网、SinoMed、PubMed、Embase、Web of Science、Cochrane图书馆,收集贝利尤单抗或贝利尤单抗联合...目的评价贝利尤单抗治疗儿童系统性红斑狼疮(cSLE)的有效性和安全性,为临床用药提供循证参考。方法计算机检索中国知网、万方数据、维普网、SinoMed、PubMed、Embase、Web of Science、Cochrane图书馆,收集贝利尤单抗或贝利尤单抗联合激素或贝利尤单抗联合激素和传统药物(试验组)对比安慰剂或激素或传统药物或传统药物联合激素等基础治疗(对照组)的随机对照试验(RCT),检索时限均为建库至2023年4月9日。筛选文献、提取资料后,采用Cochrane系统评价员手册5.1.0推荐的偏倚风险评估工具对纳入文献进行质量评价,采用RevMan 5.4软件进行Meta分析、敏感性分析。结果纳入7项RCT,共计510例患儿。Meta分析结果显示,试验组患儿的临床有效率显著优于对照组[OR=6.16,95%CI(2.23,17.00),P=0.0004],两组患儿的系统性红斑狼疮活动指数[MD=-1.73,95%CI(-3.50,0.05),P=0.06]、不良反应发生率[OR=0.72,95%CI(0.43,1.19),P=0.02]、补体C3水平[MD=0.12,95%CI(-0.06,0.30),P=0.18]、补体C4水平[MD=0.08,95%CI(-0.07,0.24),P=0.30]、系统性红斑狼疮反应指数4反应率[OR=1.52,95%CI(0.94,2.44),P=0.09]比较,差异均无统计学意义。敏感性分析结果显示,以系统性红斑狼疮活动指数、补体C3水平、补体C4水平为指标时,本研究所得结果稳健。结论贝利尤单抗治疗cSLE的疗效较好,且安全性与基础治疗相当。展开更多
Background： Approximately 15-20% cases of systemic lupus erythematosus （SLE） are diagnosed in children. There have been a Few studies reporting the epidemiological data of pediatric-onset SLE （cSLE） in China, nei...Background： Approximately 15-20% cases of systemic lupus erythematosus （SLE） are diagnosed in children. There have been a Few studies reporting the epidemiological data of pediatric-onset SLE （cSLE） in China, neither comparing the differences between cSLE and adult-onset SLE （aSLE）. The aim of this study was to describe the impact of age of onset on clinical features and survival in cSLE patients in China based on the Chinese SLE Treatment and Research group （CSTAR） database. Methods： We made a prospective study of 225 cSLE patients （aged 〈16 years） and 1759 patients aged 16-50 years based on CSTAR registry. We analyzed initial symptoms, clinical presentations, SLE disease activity, damages, and outcomes ofcSLE, as well as compared with aSLE patients. Results： The mean age ofcSLE patients was 12.16 ± 2.92 years, with 187 （83.1%） females. Fever （P 〈 0.001） as well as mucocutaneous （P 〈 0.001 ） and renal （P = 0.006） disorders were found to be significantly more frequent in cSLE patients as initial symptoms, while muscle and joint lesions were significantly less common compared to aSLE subjects （P 〈 0.001 ）. The eSLE patients were found to present more fi＇equently with malar rash （P = 0.001; odds ratio {OR], 0.624; 95% confidence interval [CI ], 0.470 0.829） but less tYequently with arthritis （P 〈 0.001 ; OR, 2.013; 95% CI, 1.512-2.679） and serositis （P = 0.030; OR, 1.629; 95% CI, 1.053 2.520）. There was no significant difl＇erence in SLE disease activity index scores between cSLE and aSLE groups （P = 0.478）. Cox regression indicated that childhood onset was the risk factor for organ damage in lupus patients （hazard ratio 0.335 [0.170 0.658], P = 0.001）. The survival curves between the cSLE and aSLE groups had no significant difference as determined by the log-rank test （0.557, P = 0.455）. Conclusions： cSLE in China has different clinical features and more inflammation than aSLE patients. Damage may be less in children and there is no difl＇erence in 5- year survival between cSLE and aSLE groups.展开更多
Objective: To investigate the characteristics of brain laterality in patients with childhood onset schizophrenia. Methods: The study employed a dichotic Chinese word listening test(DL) as a chief measure and WISC-R as...Objective: To investigate the characteristics of brain laterality in patients with childhood onset schizophrenia. Methods: The study employed a dichotic Chinese word listening test(DL) as a chief measure and WISC-R as an assistant measure to examine 16 patients and 20 normal children as controls. Results: DL indicated that the patients differed from the controls in ear advantages and their performances were significantly poorer than the controls in left ear. The performances of the patients on WISC-R showed that their PIQ was significantly lower than VIQ. Conclusion:The patients with childhood onset schizophrenia differed from the controls in brain laterality. They might be involved with right hemisphere impairment.展开更多
文摘AIM: To assess whether the polymorphisms of NOD2/ CARD15 , autophagy-related 16-like 1 (ATG16L1 ), and interleukin-23 receptor (IL23R ) genes play a more critical role in the susceptibility of childhood-onset than in adult-onset Crohn’s disease (CD). METHODS: Polymorphisms R702W, G908R, and 3020insC of NOD2/CARD15 ; rs2241880 A/G of ATG16L1 , and rs11209026 (R381Q) of IL23R gene were assessed in 110 childhood-onset CD, 364 adult-onset CD, and 539 healthy individuals. Analysis of polymorphisms R702W, G908R, and 3020insC of NOD2/CARD15 genotyping was performed by allele specific polymerase chain reaction (PCR) or by PCR-restriction fragment length polymor-phism analysis. The polymorphisms rs2241880 A/G of the ATG16L1 , and rs11209026 (R381Q) of the IL23R gene in the children’s cohort were genotyped by PCR and melting curve analysis whereas adult group genotyping was performed using the Affymetrix Genome-Wide Human SNP Array 5.0 (500K). RESULTS: The 3020insC allele in NOD2/CARD15 was significantly higher in childhood than in adult-onset CD (P = 0.0067). Association with at least 1 NOD2/CARD15 variant was specific for ileal disease (with or without co- lonic involvement). Even if the frequency of G allele of the rs2241880 ATG16L1 polymorphism was increased in both paediatric and adult CD patients compared to con- trols (P = 0.017 and P = 0.001, respectively), no difference was observed between the childhood and the adult cohort. The rare Q allele of IL23R rs11209026 polymorphism was underrepresented in both paediatric and adult CD cases (P = 0.0018 and P = 0.04, respectively) and no difference was observed between the childhood and the adult cohort. The presence of the rs2241880 ATG16L1 and rs11209026 IL23R polymorphisms did not influence disease phenotype. CONCLUSION: Polymorphism 3020insC in NOD2/ CARD15 occurs statistically significantly more often in patients with childhood-onset CD than in patients with adult-onset CD. The ATG16L1 and IL23R variants are associated with susceptibility to CD, but not earlyonset disease.
文摘We present a retrospective review of DNA immunoadsorption (DNA-IA) therapy on clinical symptoms as well as indicators in pediatric cases with systemic lupus erythematosus (SLE), and follow up the short-term curative effects. 16 SLE cases were treated by DNA-IA for 3 times every other day. We observed the changes on clinical manifestations and immunological indicators, in order to compare the alteration of these indicators including clinical manifestations, Systemic Lupus Erythematosus Disease Active Index (SLEDAI) scores, 24 hurinary protein excretion, autoantibodies, serum IgG and complement C3. 13 cases were followed up regularly, within 3 months after DNA-IA therapy, 12 cases of clinical manifestations improved (92.3%). SLEDAI scores in 10 cases decreased from (16.20 ± 12.54) to less than 5 (76.9%), 8 cases of ANA, anti-DNA antibodies were negative (61.5%), 13 cases with IgG level in serum recovered to normal (10.39 ± 4.38) g/L, C3 level rose to normal (1.06 ± 0.23) g/L. 3 to 6 months after IA, clinical manifestations and laboratory examinations in all cases got maximum improved. 9 months after IA, SLEDAI score in 2 cases (15.4%) rose to more than 5, anti-DNA antibody in 2 cases (15.4%) became positive, and 1case (7.7%) with serum C3 decreased again. 2 cases died from multiple organs dysfunction within 3 to 6 months after IA. No serious complications were found during DNA-IA. We recommend that DNA immunoadsorption is a safe and effective therapy for active childhood-onset SLE, which could improve clinical symptoms, eliminate ANA and anti-DNA antibodies. Combining with corticosteroids and immunosuppressive drugs, DNA-IA could significantly reduce the activity of disease and protect vital organs function in the short term.
文摘目的评价贝利尤单抗治疗儿童系统性红斑狼疮(cSLE)的有效性和安全性,为临床用药提供循证参考。方法计算机检索中国知网、万方数据、维普网、SinoMed、PubMed、Embase、Web of Science、Cochrane图书馆,收集贝利尤单抗或贝利尤单抗联合激素或贝利尤单抗联合激素和传统药物(试验组)对比安慰剂或激素或传统药物或传统药物联合激素等基础治疗(对照组)的随机对照试验(RCT),检索时限均为建库至2023年4月9日。筛选文献、提取资料后,采用Cochrane系统评价员手册5.1.0推荐的偏倚风险评估工具对纳入文献进行质量评价,采用RevMan 5.4软件进行Meta分析、敏感性分析。结果纳入7项RCT,共计510例患儿。Meta分析结果显示,试验组患儿的临床有效率显著优于对照组[OR=6.16,95%CI(2.23,17.00),P=0.0004],两组患儿的系统性红斑狼疮活动指数[MD=-1.73,95%CI(-3.50,0.05),P=0.06]、不良反应发生率[OR=0.72,95%CI(0.43,1.19),P=0.02]、补体C3水平[MD=0.12,95%CI(-0.06,0.30),P=0.18]、补体C4水平[MD=0.08,95%CI(-0.07,0.24),P=0.30]、系统性红斑狼疮反应指数4反应率[OR=1.52,95%CI(0.94,2.44),P=0.09]比较,差异均无统计学意义。敏感性分析结果显示,以系统性红斑狼疮活动指数、补体C3水平、补体C4水平为指标时,本研究所得结果稳健。结论贝利尤单抗治疗cSLE的疗效较好,且安全性与基础治疗相当。
文摘Background： Approximately 15-20% cases of systemic lupus erythematosus （SLE） are diagnosed in children. There have been a Few studies reporting the epidemiological data of pediatric-onset SLE （cSLE） in China, neither comparing the differences between cSLE and adult-onset SLE （aSLE）. The aim of this study was to describe the impact of age of onset on clinical features and survival in cSLE patients in China based on the Chinese SLE Treatment and Research group （CSTAR） database. Methods： We made a prospective study of 225 cSLE patients （aged 〈16 years） and 1759 patients aged 16-50 years based on CSTAR registry. We analyzed initial symptoms, clinical presentations, SLE disease activity, damages, and outcomes ofcSLE, as well as compared with aSLE patients. Results： The mean age ofcSLE patients was 12.16 ± 2.92 years, with 187 （83.1%） females. Fever （P 〈 0.001） as well as mucocutaneous （P 〈 0.001 ） and renal （P = 0.006） disorders were found to be significantly more frequent in cSLE patients as initial symptoms, while muscle and joint lesions were significantly less common compared to aSLE subjects （P 〈 0.001 ）. The eSLE patients were found to present more fi＇equently with malar rash （P = 0.001; odds ratio {OR], 0.624; 95% confidence interval [CI ], 0.470 0.829） but less tYequently with arthritis （P 〈 0.001 ; OR, 2.013; 95% CI, 1.512-2.679） and serositis （P = 0.030; OR, 1.629; 95% CI, 1.053 2.520）. There was no significant difl＇erence in SLE disease activity index scores between cSLE and aSLE groups （P = 0.478）. Cox regression indicated that childhood onset was the risk factor for organ damage in lupus patients （hazard ratio 0.335 [0.170 0.658], P = 0.001）. The survival curves between the cSLE and aSLE groups had no significant difference as determined by the log-rank test （0.557, P = 0.455）. Conclusions： cSLE in China has different clinical features and more inflammation than aSLE patients. Damage may be less in children and there is no difl＇erence in 5- year survival between cSLE and aSLE groups.
文摘Objective: To investigate the characteristics of brain laterality in patients with childhood onset schizophrenia. Methods: The study employed a dichotic Chinese word listening test(DL) as a chief measure and WISC-R as an assistant measure to examine 16 patients and 20 normal children as controls. Results: DL indicated that the patients differed from the controls in ear advantages and their performances were significantly poorer than the controls in left ear. The performances of the patients on WISC-R showed that their PIQ was significantly lower than VIQ. Conclusion:The patients with childhood onset schizophrenia differed from the controls in brain laterality. They might be involved with right hemisphere impairment.
